Title: A system of practical medicine. By American authors. Vol. 2
General diseases (continued) and diseases of the digestive system
Editor: William Pepper
Louis Starr
Release date: April 3, 2014 [eBook #45313]
Most recently updated: October 24, 2024
Language: English
Credits: Produced by Ron Swanson
RHEUMATISM. By R. PALMER HOWARD, M.D.
GOUT. By W. H. DRAPER, M.D.
RACHITIS. By ABRAHAM JACOBI, M.D.
SCURVY. By PHILIP S. WALES, M.D.
PURPURA. By I. EDMONDSON ATKINSON, M.D.
DIABETES MELLITUS. By JAMES TYSON, A.M., M.D.
SCROFULA. By JOHN S. LYNCH, M.D.
HEREDITARY SYPHILIS. By J. WILLIAM WHITE, M.D.
DISEASES OF THE MOUTH AND TONGUE. By J. SOLIS COHEN, M.D.
DISEASES OF THE TONSILS. By J. SOLIS COHEN, M.D.
DISEASES OF THE PHARYNX. By J. SOLIS COHEN, M.D.
DISEASES OF THE OESOPHAGUS. By J. SOLIS COHEN, M.D.
FUNCTIONAL AND INFLAMMATORY DISEASES OF THE STOMACH. By SAMUEL G. ARMOR, M.D., LL.D.
SIMPLE ULCER OF THE STOMACH. By W. H. WELCH, M.D.
CANCER OF THE STOMACH. By W. H. WELCH, M.D.
HEMORRHAGE FROM THE STOMACH. By W. H. WELCH, M.D.
DILATATION OF THE STOMACH. By W. H. WELCH, M.D.
MINOR ORGANIC AFFECTIONS OF THE STOMACH (Cirrhosis; Hypertrophic Stenosis of Pylorus; Atrophy; Anomalies in the Form and the Position of the Stomach; Rupture; Gastromalacia). By W. H. WELCH, M.D.
INTESTINAL INDIGESTION. By W. W. JOHNSTON, M.D.
CONSTIPATION. By W. W. JOHNSTON, M.D.
ENTERALGIA (INTESTINAL COLIC). By W. W. JOHNSTON, M.D.
ACUTE INTESTINAL CATARRH (DUODENITIS, JEJUNITIS, ILEITIS, COLITIS, PROCTITIS). By W. W. JOHNSTON, M.D.
CHRONIC INTESTINAL CATARRH. By W. W. JOHNSTON, M.D.
CHOLERA MORBUS. By W. W. JOHNSTON, M.D.
INTESTINAL AFFECTIONS OF CHILDREN IN HOT WEATHER. By J. LEWIS SMITH, M.D.
PSEUDO-MEMBRANOUS ENTERITIS. By PHILIP S. WALES, M.D.
DYSENTERY. By JAMES T. WHITTAKER, A.M., M.D.
TYPHLITIS, PERITYPHLITIS, AND PARATYPHLITIS. By JAMES T. WHITTAKER, A.M., M.D.
INTESTINAL ULCER. By JAMES T. WHITTAKER, A.M., M.D.
HEMORRHAGE OF THE BOWELS. By JAMES T. WHITTAKER, A.M., M.D.
INTESTINAL OBSTRUCTION. By HUNTER MCGUIRE, M.D.
CANCER AND LARDACEOUS DEGENERATION OF THE INTESTINES. By I. EDMONSON ATKINSON, M.D.
DISEASES OF THE RECTUM AND ANUS. By THOMAS G. MORTON, M.D., and HENRY M. WETHERILL, JR., M.D., PH.G.
INTESTINAL WORMS. By JOSEPH LEIDY, M.D., LL.D.
DISEASES OF THE LIVER. By ROBERTS BARTHOLOW, A.M., M.D., LL.D.
DISEASES OF THE PANCREAS. By LOUIS STARR, M.D.
PERITONITIS. By ALONZO CLARK, M.D., LL.D.
DISEASES OF THE ABDOMINAL GLANDS (TABES MESENTERICA). By SAMUEL C. BUSEY, M.D.
ARMOR, SAMUEL G., M.D., LL.D.,
Brooklyn.
ATKINSON, I. EDMONDSON, M.D.,
Professor of Pathology and Clinical Medicine and Clinical Professor of Dermatology in the University of Maryland, Baltimore.
BARTHOLOW, ROBERTS, A.M., M.D., LL.D.,
Professor of Materia Medica, General Therapeutics, and Hygiene in the Jefferson Medical College, Philadelphia.
BUSEY, SAMUEL C., M.D.,
An Attending Physician and Chairman of the Board of Hospital Administration of the Children's Hospital, Washington, D.C.
CLARK, ALONZO, M.D., LL.D.,
Late Professor of Pathology and Practical Medicine in the College of Physicians and Surgeons, New York.
COHEN, J. SOLIS, M.D.,
Professor in Diseases of the Throat and Chest in the Philadelphia Polyclinic; Physician to the German Hospital, Philadelphia.
DRAPER, W. H., M.D.,
Attending Physician to the New York and Roosevelt Hospitals, New York.
HOWARD, R. PALMER, M.D.,
Professor of Theory and Practice of Medicine in McGill University, Montreal; Consulting Physician to Montreal General Hospital, Canada.
JACOBI, ABRAHAM, M.D.,
Clinical Professor of Diseases of Children in the College of Physicians and Surgeons, New York, etc.
JOHNSTON, W. W., M.D.,
Professor of Theory and Practice of Medicine in the Columbian University, Washington.
LEIDY, JOSEPH, M.D., LL.D.,
Professor of Anatomy in the University of Pennsylvania, Philadelphia.
LYNCH, JOHN S., M.D.,
Professor of Principles and Practice of Medicine in the College of Physicians and Surgeons, Baltimore.
MORTON, THOMAS G., M.D.,
Surgeon to the Pennsylvania Hospital, Philadelphia.
MCGUIRE, HUNTER, M.D.,
Richmond, Va.
SMITH, J. LEWIS, M.D.,
Clinical Professor of Diseases of Children in the Bellevue Hospital Medical College, New York.
STARR, LOUIS, M.D.,
Clinical Professor of Diseases of Children in the Hospital of the University of Pennsylvania, Philadelphia.
TYSON, JAMES, A.M., M.D.,
Professor of General Pathology and Morbid Anatomy in the University of Pennsylvania, Philadelphia.
WALES, PHILIP S., M.D.,
Washington.
WELCH, WILLIAM H., M.D.,
Professor of Pathology in Johns Hopkins University, Baltimore.
WETHERILL, HENRY M., JR., M.D.,
Assistant Physician to the Pennsylvania Hospital for the Insane, Philadelphia.
WHITE, J. WILLIAM, M.D.,
Surgeon to the Philadelphia Hospital; Assistant Surgeon to the University Hospital; Demonstrator of Surgery and Lecturer on Venereal Diseases and Operative Surgery in the University of Pennsylvania, Philadelphia.
WHITTAKER, JAMES T., M.D.,
Professor of Theory and Practice of Medicine in the Medical College of Ohio, Cincinnati.
FIGURE | |
1. | POSITION OF PUNCTURES IN DIABETIC AREA OF MEDULLA OBLONGATA NECESSARY TO PRODUCE GLYCOSURIA |
2. | THE LAST CERVICAL AND FIRST THORACIC GANGLIA, WITH CIRCLE OF VIEUSSENS, IN THE RABBIT, LEFT SIDE |
3. | DIAGRAM SHOWING COURSE OF THE VASO-MOTOR NERVES OF THE LIVER, ACCORDING TO CYON AND ALADOFF |
4. | DIAGRAM SHOWING ANOTHER COURSE WHICH THE VASO-MOTOR NERVES OF THE LIVER MAY TAKE |
5. | JOHNSON'S PICRO-SACCHARIMETER |
6. | PEMPHIGUS BULLA FROM A NEW-BORN SYPHILITIC CHILD |
7. | SECTION OF RETE MUCOSUM AND PAPILLÆ FROM SAME CASE OF PEMPHIGUS AS FIG. 6 |
8. | SECTION OF AN OLD GUMMA OF THE LIVER |
9. | SYPHILITIC DACTYLITIS, FROM BUMSTEAD |
10. | THE SAME AS FIG. 9 |
11. | SERRATIONS OF NORMAL INCISOR TEETH |
12. | NOTCHING OF SYPHILITIC INCISOR TEETH |
13. | OÏDIUM ALBICANS FROM THE MOUTH IN A CASE OF THRUSH |
14. | CHRONIC INTUMESCENCE OF THE TONGUE (HARRIS) |
15. | HYPERTROPHY OF TONGUE (HARRIS), BEFORE OPERATION AND AFTER |
16. | GLOSSITIS (LISTON) |
17. | INCISION FOR A CUSPID TOOTH (WHITE) |
18. | INCISION FOR A MOLAR TOOTH (WHITE) |
19. | FUSIFORM DILATATION OF OESOPHAGUS (LUSCHKA) |
20. | FAUCHER'S TUBE FOR WASHING OUT THE STOMACH |
21. | FAUCHER'S TUBE FOR WASHING OUT THE STOMACH |
22. | ROSENTHAL'S METHOD OF WASHING OUT THE STOMACH |
23. | ANTERIOR VIEW OF A STRANGLUATED INTESTINE AND STRICTURE |
24. | POSTERIOR VIEW OF A STRANGULATED INTESTINE AND STRICTURE |
25. | APPEARANCE OF THE NATURAL RELATIONS OF THE DIVERTICULUM TO THE INTESTINE |
26. | SIMPLE INVAGINATION OF THE ILEUM |
27. | SIMPLE INVAGINATION, WITH OCCLUSION OF BOWEL, FROM INFLAMMATORY CHANGES |
RHEUMATISM. | PURPURA. |
GOUT. | DIABETES MELLITUS. |
RACHITIS. | SCROFULA. |
SCURVY. | HEREDITARY SYPHILIS. |
SYNONYMS AND DEFINITION.—Acute Rheumatism, Acute Rheumatic Polyarthritis, Rheumarthritis, Rheumatic Fever, Polyarthritis Synovialis Acuta (Heuter).
Acute articular rheumatism is a general non-contagious, febrile affection, attended with multiple inflammations, pre-eminently of the large joints and very frequently of the heart, but also of many other organs; these inflammations observing no order in their invasion, succession, or localization, but when affecting the articulations tending to be temporary, erratic, and non-suppurating; when involving the internal organs proving more abiding, and often producing suppuration in serous membranes. It is probably connected with a diathesis—the arthritic—which may be inherited or acquired. It may present such modifications of its ordinary characters as to justify being called (2d) subacute articular rheumatism, and it may sometimes pass into the (3d) chronic form.
ETIOLOGY.—There is a general consensus of opinion that acute articular rheumatism belongs especially to temperate climates, and that it is exceedingly rare in polar regions; but respecting its prevalence in the tropics contradictory statements are made. Saint-Vel declares that it is not a disease of hot climates; Rufz de Levison saw only four cases of acute articular rheumatism, and not one of chorea, in Martinique during twenty years' practice; while Pruner Bey says it is common in Egypt, and Webb remarks the same for the East Indies. Even in temperate climates, like those of the Isle of Wight, Guernsey, Cornwall, some parts of Belgium (Hirsch), the disease is very rare—a circumstance not to be satisfactorily explained at present.
Acute articular rheumatism is never absent; it occurs at all seasons of the year, although subject to moderate variations depending mainly upon atmospheric conditions. It is the general opinion that it prevails most during the cold and variable months of spring, but this is not true of every place, nor invariably of the same place. Indeed, Besnier,1 after a long and special observation of the disease in Paris, concludes that there it is most frequent in summer and in spring. In Montreal, during ten years, the largest number of cases of acute rheumatism admitted to the General Hospital obtained in the spring months (March to June [p. 20]inclusive), when they averaged 51 a month; 33 was the average for all the other months, except October and November, when 26½ was the average. The statistics of Copenhagen, Berlin, and Zurich show a minimum prevalence in summer or in summer and autumn.
1 Dictionnaire Encyclopédique des Sciences Méd., Troisième Serie, t. iv.
Occupations involving muscular fatigue or exposure to sudden and extreme changes of temperature, especially during active bodily exertion, predispose to acute articular rheumatism; hence its frequency amongst cooks, maid-servants, washerwomen, smiths, coachmen, bakers, soldiers, sailors, and laborers generally.
While no age is exempt from acute articular rheumatism, it is, par excellence, an affection of early adult life, the largest number of cases occurring between fifteen and twenty-five years of age, and the next probably between twenty-five and thirty-five. A marked decline in its frequency takes place after the age of thirty-five, and a still greater after forty-five. It is not uncommon in children between five and ten, and especially between ten and fifteen, but is very rare under five, although now and then one meets with an example of the disease in children three or four years of age. While the acute articular affections observed in sucklings are, as a general rule, either syphilitic or pyæmic, some authentic instances of rheumatic polyarthritis are recorded. Kauchfuss's two cases among 15,000 infants at the breast, Widerhofer's case, only twenty-three days old, Stager's, four weeks old, and others, are cited by Senator.2
2 Ziemssen's Cyclop. of Pract. Med., xvi. 17.
An analysis of 4908 cases of acute rheumatism admitted to St. Bartholomew's Hospital, London,3 during fifteen years, and of 456 treated in the Montreal General Hospital during ten years,4 gives the following percentages at given periods of life:
London. | Montreal. | ||
Under 10 years | 1.79 per cent. | ||
From 10 to 15 years | 8.1 per cent. | Under 15 years | 4.38 per cent. |
From 15 to 25 years | 41.8 per cent. | From 15 to 25 years | 48.68 per cent. |
From 25 to 35 years | 24.5 per cent. | From 25 to 35 years | 25.87 per cent. |
From 35 to 45 years | 14.2 per cent. | From 35 to 45 years | 13.6 per cent. |
Above 45 years | 9.5 per cent. | Above 45 years | 7.4 per cent. |
The close correspondence existing in the two tables for all the periods of life above fifteen is very striking: the disparity between them below the age of fifteen may, I believe, be explained by the circumstance that the pauper population of Montreal is, when compared with that of London, relatively very small, and by the further fact that the practice of sending children into hospitals hardly obtains here.
3 St. Bartholomew's Hospital Reports, xiv. 4.
4 Dr. James Bell, in Montreal General Hospital Reports, i. 350.
No doubt the above tables do not correctly represent the liability of children to acute articular rheumatism, but they are probably a fair statement of the relative frequency of the disease in the adult hospital populations of London and Montreal. If primary attacks of the disease only were tabulated, the influence of youth would be more evident, for it is scarcely possible to find on record an authentic instance of the disease showing itself for the first time after sixty. Dr. Pye-Smith5 has done [p. 21]this in 365 cases, and the results prove the great proclivity of very young persons to acute rheumatism: Between five and ten years, 6 per cent. occurred; between eleven and twenty, 49 per cent.; from twenty-one to thirty, 32.3 per cent.; from thirty-one to forty, 9.5 per cent.; from forty-one to fifty, 2.2 per cent.; and from fifty-one to sixty-one, 1.1 per cent. The same author has also shown that secondary attacks are most common in the young; so that advancing age not only renders a first attack of the disease improbable, but lessens the risk of a recurrence of it. The influence of age upon acute rheumatism is further shown in the fact that the disease is less severe, and less apt to invade the heart, in elderly than in young persons.
5 Guy's Hospital Reports, 3d Series, xix. 317.
The general opinion that sex exercises no direct influence beyond exposing males more than females to some of the predisposing and exciting causes of acute rheumatism is perhaps true if the statement be confined to adults, to whom, indeed, most of the available statistics apply; but it should be borne in mind that a larger proportion of men than of women resort to hospitals, and there is some reason to believe that in childhood the greater liability to the disease is on the part of the female sex. Thus, the number of cases of rheumatism treated at the Children's Hospital in London from 1852 to 1868 was 478, of whom 226 were males and 252 females.6 Of Goodhardt's 44 cases of acute rheumatism in children, 26 were girls and 18 were boys.7 Of 57 examples of rheumatism in connection with chorea observed by Roger in children under fourteen, 33 were girls and 24 were boys.8
6 Vide Dr. Tuckwell's "Contributions to the Pathology of Chorea," in St. Bartholomew's Hospital Reports, v. 102.
7 Guy's Hospital Reports, 3d Series, xxv. 106.
8 Arch. Gén., vol. ii. 641, 1866, and vol. i. 54, 1867, quoted by Tuckwell.
That heredity predisposes to acute articular rheumatism is admitted by nearly all modern authorities, even Senator, while speaking of it as "a traditional belief," not venturing to deny it. The frequency of the inherited predisposition Fuller placed at 34 per cent.; Beneke, quoted by Homolle,9 at 34.6 per cent; Pye-Smith at 23 per cent.10 Such predisposition favors the occurrence of the disease in early life, but does not necessarily determine an attack of acute rheumatism in the absence of the other predisposing or exciting causes. That the inherited bias or mode of vital action or condition of tissue-health may be so great as, per se, to induce an attack of the disease, is held by some authorities. It is probable that not only acute articular rheumatism in the parents, but simple chronic articular rheumatism and those forms grouped under the epithet rheumatoid arthritis, may impart a predisposition to the acute as well as to the chronic varieties of articular disease just mentioned. But owing to the obscurity which still surrounds the relations existing between acute articular rheumatism and rheumatoid arthritis this point needs further investigation. In what the inherited predisposition to acute articular rheumatism consists we are ignorant; to say that it imparts to the tissues or organs a disposition to react or act according to a fixed morbid type, or that some of the nutritive processes are perverted by it, is merely to state a theory, not to explain the nature of the predisposition.
9 Nouv. Dict. de Méd. et de Chir., t. 31, 557.
10 Guy's Hospital Reports, 3d Series, xix. 320.
No type of bodily conformation or temperament can be described that [p. 22]certainly indicates a proclivity to acute articular rheumatism; nor is there any change in the constitution of the tissues or fluids of the body by which the proclivity may be recognized. We infer the existence of the inherited predisposition—the innate bias—when rheumatism is found in the family history; when acute rheumatism or cardiac disease, or chorea not produced by mental causes, occurs in childhood; when the first attack of acute articular rheumatism is succeeded by subsequent attacks; and especially when the intervals between the attacks are short. Goodhardt has recently furnished valuable, but not conclusive, evidence to prove that in children obstinate headaches, night-terrors, severe anæmia, various neuro-muscular derangements, such as torticollis, tetany, muscular tremors, stammering, incontinence of urine, recurring attacks of abdominal pain, with looseness of the bowels quickly succeeding a meal, the cutaneous affection erythema nodosum, are indications of a rheumatic bias or predisposition.11
11 Guy's Hospital Reports, 3d Series, xxv.
There is some basis for the opinion that residence in damp, cold dwellings predisposes somewhat to acute articular rheumatism, although not at all to the same degree that it does to the chronic articular and muscular forms. Chomel and Jaccoud especially have insisted that it will gradually create a predisposition to the disease, even if it has not been inherited. All pathologists agree that cold is the most frequent exciting cause of acute articular rheumatism, and that it is especially effective when applied while the body is perspiring freely or is overheated or fatigued by exercise. There is no necessary ratio between the degree of cold or its duration and the severity of the resulting rheumatism. A slight chilling or a momentary exposure to a current of cold air will in some act as powerfully and as certainly as a prolonged immersion in cold water or a night spent sleeping on the damp grass. This circumstance, together with the fact that cold applied in the same way may also produce a pharyngitis or a bronchitis, a pneumonia or a nephritis, etc., is held to indicate that the cold acts according to individual predisposition; and Jaccoud, Flint, and others maintain that unless a rheumatic proclivity exists cold will not produce an attack of the disease under consideration. I doubt that we are yet in a position to assert that absolutely, although the weight of argument is in its favor. Let it suffice to say, that while a prolonged residence in a cold, damp dwelling may gradually develop a predisposition to rheumatism, a short exposure to cold will be likely to induce an attack of rheumatism if the predisposition exist.
There are other influences which may be regarded as auxiliaries to cold in exciting an attack, as they seem to increase the susceptibility of the patient to its operation: they establish what has been felicitously called a state of morbid opportunity. Such are all influences that reduce the resisting powers of the organs and organism, as bodily fatigue, mental exhaustion, the depressing passions, excessive venery, prolonged lactation, losses of blood, etc. It is probably in such a manner that local injuries (traumatism) sometimes appear to induce an attack of rheumatism. A blow on a finger (Cotain), the extraction of a tooth (Homolle), a hypodermic injection (ibid.), etc., may act powerfully in some persons upon and through the nervous system, and by lessening their resisting power [p. 23]may favor the overt manifestation of the rheumatic predisposition. But doubtless some such cases have been examples of mere coincidence.
There are certain pathological and even physiological conditions during or after which an inflammatory affection of one or several joints closely resembling acute articular rheumatism more or less frequently arises. Thus, during the early desquamating stage of scarlatina a mild inflammation of the joints of the hands and feet, and frequently of the large articulations as well, is very often seen, and it is attended with profuse perspiration, with a condition of urine like that of ordinary acute rheumatism, and occasionally with inflammation of the heart or pleura. During convalescence from dysentery an affection of a single or of several articulations resembling rheumatism has been noticed, and the two affections have even alternated in the same patient. That singular epidemic disease dengue is attended with a polyarticular affection closely resembling acute articular rheumatism, occasionally pursuing a protracted course, and not seldom leaving after it a cardiac lesion. In hæmophilia polyarticular and muscular disorders frequently arise which closely resemble, and appear to be sometimes identical with, ordinary acute articular and muscular rheumatism. Gonorrhoea too is often associated with a febrile polyarthritis, and rarely with an endocarditis at the same time. In the puerperal state an inflammation of one or several articulations is not unfrequently observed (puerperal rheumatism).
Respecting the real nature of these polyarticular inflammations very much has to be made out; and it must suffice at present to say that while many of them are of a pyæmic nature, as some examples of puerperal and scarlatinal arthritis, in which pus forms in or about the joints and in the serous cavities and viscera, some of them are no doubt examples of genuine rheumatism occurring in persons of rheumatic predisposition, which have either been induced by the lowering influence of the disease upon which they have supervened, or by the accidental coincidence of some of the other causes of acute rheumatism. There remains, however, the ordinary form of scarlatinal arthritis, which so closely resembles true acute articular rheumatism in its symptoms, course, visceral complications, and morbid anatomy that it cannot be said that the two affections are distinct and different. And much the same appears to be true of the articular affection of dengue. Yet so frequently does the articular affection accompany scarlatina and dengue respectively that it cannot logically be referred to a coexisting rheumatic predisposition, and must be a consequence of the disturbing influences of the specific poison of those zymotic affections per se.
PATHOLOGY.—The pathology of acute articular rheumatism is a very much debated question, and is not at all satisfactorily known. Hence a mere statement of the most prominent theories now held by different pathologists will be given.12
12 The reader may consult with advantage Dr. Morris Longstreth's fourth chapter in his recent excellent monograph upon Rheumatism, Gout, and some Allied Disorders, New York, 1882.
The latest modification of the lactic-acid theory of Prout is founded upon the modern physiological teaching that during muscular exercise sarcolactic acid and acid phosphate of potassium are formed, and carbon dioxide set free, in the muscular tissue, and that cold, acting on [p. 24]the surface under such circumstances, may check the elimination of these substances and cause their accumulation in the system. This view, it is held, explains why the muscles and their associated organs, the joints and tendons, suffer first and chiefly, because the morbific influence is exerted upon them when exhausted by functional activity; and it further accounts for the visceral manifestations and the apparent excess of acid eliminated during the course of the disease. The circumstance that in three cases of diabetes (Foster,13 Kuelz14) the administration of lactic acid appeared to induce polyarticular rheumatism favors the idea that acid is the materies morbi in rheumatism.
13 Brit. Med, Jour., ii. 1871.
14 Beiträge zur Path. und Therapie des Diabetes, u. s. w., ii. 1875.
Now it must be admitted that, as yet, no sufficient proof is forthcoming that a considerable excess of lactic acid exists in the fluids or solids of the body or in the excretions in rheumatism (it is true the point has not been sufficiently investigated). On the other hand, that acid has been found in the urine of rickets, and its excess in the system is regarded by Heitzmann and Senator15 as the cause of the peculiar osteoplastic disturbances of that disease—an affection altogether different from rheumatism. It is quite improbable that the amount of sarcolactic acid produced by over-prolonged muscular exertion, and whose elimination has been prevented by a chill or a mental emotion, is sufficient to maintain the excessive acidity of the urine and other fluids during a long rheumatic fever; and arguments can be adduced favorable to the view that excessive formation of acid is an effect rather than the cause of rheumatism: cases of that disease occur in which neither excessive muscular exertion nor exposure to chill have preceded the rheumatic outbreak. Lastly, lactic acid is not the only principle retained when the functions of the skin are arrested by cold, the usual exciting cause of rheumatism; why should not the retained acetic, formic, butyric, and other acids, for example, play their rôle in the production of the symptoms observed under such conditions?
15 Ziemssen's Cyclop., xvi. p. 177.
The same objections apply to Latham's16 hypothesis that hyperoxidation of the muscular tissue is the starting-point of acute rheumatism. He assumes, with other physiologists, the existence of a nervous centre which inhibits the chemical changes that would take place if the tissues were out of the body. If this centre be changed or weakened, the muscle, instead of absorbing and fixing the oxygen and giving out carbonic acid, disintegrates; lactic acid is formed, and, passing into the blood, may be there oxidized and produce the pyrexia of acute rheumatism. It need hardly be remarked that the existence of a chemical inhibitory centre has yet to be proved, although much may be advanced in its favor; and, secondly, the recent investigations of Zuntz render it highly probable that in all febrile affections it is the muscles chiefly, if not solely, which suffer increased oxidation, and that this is due to increased innervation—views not easily reconciled with Latham's theory.
16 Brit. Med. Jour., ii. 1880, p. 977.
The nervous theory of rheumatism and of articular diseases originated with Dr. J. K. Mitchell of Philadelphia17 in 1831, and was afterward elaborated by Froriep in 1843,18 Scott Alison19 in 1846, Constatt in 1847,20 [p. 25]Gull in 1858, Weir Mitchell in 1864,21 Charcot in 1872, and by very many others since. According to present physiological doctrine, the exciting cause of rheumatism, cold, either acts directly upon the vaso-motor or the trophic (?) nerves of the articulations, and excites inflammation of them, or else it irritates the peripheral ends of the centripetal nerves, and through these excites actively the vaso-motor and trophic nerve-centres. The local lesions, on this hypothesis, are of trophic origin; the fever is due to hyperactivity of the centres supposed to control the chemical changes going on in the tissues; the excessive perspiration to stimulation of the sweat-centres; and so on. It is not held that a definite centric lesion of the nervous system exists in rheumatism, analogous to the lesions which in myelitis or locomotor ataxia develop the arthropathies of those affections, but rather a functional disturbance. One of the latest and ablest advocates of the neurosal theory of rheumatism in all its forms (simple, rheumatoid, gonorrhoeal, urethral, etc.), Jonathan Hutchinson, calls it "a catarrhal neurosis, the exposure of some tract of skin or mucous membrane to cold or irritation acting as the incident excitor influence."22
17 Am. Jour. Med. Sci., 1831; ib., 1833.
18 Die Rheumatische Schwiele, Weimar, 1843.
19 Lancet, 1846, i. 227.
20 Spec. Pathologie und Therapie, 1847, ii. p. 609.
21 Vide Am. Jour. Med. Sciences, April, 1875, vol. lxix. 339-348.
22 Trans. International Med. Congress, 1881, ii. 93.
In order that peripheral irritation shall thus induce inflammation of the joints and the other affections of muscles, tendons, fasciæ, etc. which are called rheumatic, he holds with the French School that the arthritic diathesis must exist, or that state of tissue-health which involves a tendency to temporary inflammation of many joints or fibrous structures at once, or to repeatedly recurrent attacks of inflammation of one joint or fibrous structure. If I understand Mr. Hutchinson correctly, he also holds that a nerve-tissue peculiarity exists which renders persons liable to rheumatism. He does not indicate either the cause or the nature of the nerve-tissue peculiarity. But modern pathology teaches that the functional conditions of the nervous centres known as neuroses, whether inherited or acquired, reveal themselves as morbid manifestations of nerve-function on the part of special portions of or the entire nervous system, and, as Dr. Dyce Duckworth has well pointed out, these neuroses may be originated, when not inherited, in various ways, as by excessive activity of the nervous system, by prolonged or habitual excesses, etc. "Thus, undue mental labor, gluttony, alcoholic intemperance, debauchery, and other indulged evil propensities in the parent come to be developed into definite neurotic taint and tendency in the offspring."
But is there nothing more in acute articular rheumatism than an inflammation of certain structures, articular and visceral, lighted up in an individual of a neuro-arthritic diathesis? What do we learn from that closely-allied affection, gout, which involves especially the same organs as rheumatism, and is held by many of the ablest pathologists to belong to the same basic diathesis as it? Duckworth23 has very ably advocated a neurotic theory of gout, but it is admitted on all hands—and by Duckworth himself—that in gout a large part of the phenomena is due to perverted relations of uric acid and sodium and to the presence of urate of soda in the blood. May we not from analogy, as well as from other evidence, infer that in that so-called other neurosis, rheumatism, a considerable part of the phenomena is due to perversions of [p. 26]the processes of assimilation and excretion, and to the presence of some unknown intermediate product of destructive metamorphosis—lactic or other acid? This is admitted by Maclagan and strongly advocated by Senator; and in this way the pathology of the disease may be said to embrace the humoral as well as the solidist doctrines—the resulting theory being a neuro-humoral one. No doubt pathological chemistry and clinical investigation will ere long make important discoveries respecting the pathology of acute rheumatism which shall maintain the close alliance believed to exist between that affection and gout.
23 Brain, April, 1880.
The miasmatic theory, so ably advocated by Maclagan,24 assumes that rheumatism is due to the entrance into the system from without of a miasm closely allied to, but quite distinct from, malaria. His argument on this topic is ingenious and elaborate, yet has not been favorably received by pathologists. Opposed to it are the following amongst other considerations: Heredity exercises a marked influence upon the occurrence of rheumatism; unlike malarial disease, no climate or locality is immune from rheumatism; the many indications that a diathesis plays a chief rôle in rheumatism; the remarkable influence exerted by cold and dampness in the etiology of the disease.
24 Rheumatism: its Nature, Path., etc., London, 1881, pp. 60-95.
Heuter's25 infective-germ theory, like the miasmatic, refers rheumatism to a principle not generated in the system, but introduced from without. A micrococcus enters the dilated orifices of the sweat-glands, and, reaching the blood, first sets up an endocarditis, and then capillary emboli produce the articular inflammations. This is a reversal of what really happens, so far as the time of invasion of the endocardium and the synovial membranes is concerned; and Fleischauer's case, in which miliary abscesses were found in the heart, lungs, and kidneys, was probably one of ulcerative endocarditis, which, after all, is a rare complication of acute articular rheumatism. Moreover, it is a gratuitous assertion to say that endocarditis exists in all cases of the disease. If, however, Heuter were content to say that acute articular rheumatism was produced by a specific germ, as held by Recklinghausen and Klebs, which on entering the system acted specially upon the joints and the fibro-serous tissues, as the poison of small-pox does upon the skin, while at the same time it sets up general disturbances of the entire economy as other zymotic poisons do, there would be nothing opposed to general pathological laws. Even the existence of a diathesis capable of favoring the action of the specific germ would be analogous to the tuberculous diathesis, which favors the action of the bacillus of tubercle; and cold, its ordinary exciting cause, might be regarded simply as a condition which renders the system more susceptible to the action of the germ, and the modus operandi of cold in doing this might be variously explained.
25 Klinik der Gelenkkrankheiten, Leipzig, 1871.
SYMPTOMS.—The disease has no uniform mode of invasion. (a) Very frequently slight disorder of health, such as debility, pallor, failure of appetite, unusual sensibility to atmospheric changes, grumbling pains in the joints or limbs, or even in some muscle or fascia, precedes by one or more days the fever and general disturbance. (b) Not infrequently a mild rigor or repeated chilliness, accompanied or soon followed by moderate or high fever, ushers in the illness, and in from a few hours to one [p. 27]or at most two days the characteristic articular symptoms ensue. (c) In very rare cases febrile disturbance, ushered in by chills, may be followed by inflammation of the endo- or pericardium or pleura before the joints become affected.
Whatever the mode of invasion, the symptoms of the established disease are well defined, and marked febrile disturbance, transient inflammation of several of the larger articulations, excessive activity of the cutaneous functions, and a great proclivity to inflammation of the endo- and pericardium constitute the stereotyped features of the disease.
As a very general rule, the temperature early in the disease promptly attains its maximum of 102° F. to 104° F., yet the surface does not feel very hot; the pulse ranges from 90 to 100 or 110, and is regular, large, and often bounding; the tongue is moist, but thickly coated with a white fur; there are marked thirst, impaired appetite, and constipation; the stools are usually dark; the urine scanty, high colored, very acid, of great density, and holding in solution an excess of uric acid and urates, which are frequently deposited when the urine cools. The general surface is covered with a profuse sour-smelling perspiration, whose natural acid reaction, as a general rule, is markedly increased; indeed, the naturally alkaline saliva is also acid. Beyond a little wandering during sleep, occasionally observed in irritable, nervous patients, there is very rarely any delirium, and this notwithstanding that sleep is frequently much disturbed by the pain in the joints and the excessive sweating.
If the local articular symptoms have not set in almost simultaneously with the pyrexia, or even preceded it, they will follow it in from a few to twenty-four or forty-eight hours. At first one or more joints, usually the knees or ankles, become painful, sensitive to pressure, hot, more or less swollen, and exhibiting a slight blush of redness or none at all. The swelling may consist of a mere puffiness, due to slight infiltration of the soft parts external to the joint, or of a more or less considerable tumefaction, caused by effusion into the synovial capsule. In the knees, elbows, shoulders, and hips the swelling is usually confined to the articulations, and there is but little redness of the integument, but in the wrists and ankles the inflammatory process is often more severe, and may invade the whole dorsum of the hand or foot, rendering the integument tense, tumid red, and shining. Pitting of the swollen parts, although quite exceptional in acute articular rheumatism, will exist under the conditions just mentioned. The metacarpo-phalangeal articulations are likewise often a good deal swollen and of a bright-red color.
The pain in the affected articulations varies from a trifling uneasiness or dull ache to excruciating anguish; sometimes the pain is felt only on moving or pressing the joint; pressure always aggravates it; even the weight of the bed-clothes may be intolerable; and in severe cases the slightest movement of the joint or a jar of the bed produces great suffering. The pain, like the swelling, sometimes extends beyond the affected joints to the tendinous sheaths, the tendons, and muscles, and even to the nerves of the neighborhood.
It is a striking peculiarity of acute rheumatism that the inflammation tends to invade fresh joints from day to day, the inflammation usually, but not invariably, declining in those first affected; and sometimes this retrocession of the inflammation in a joint is so sudden, and so coincident [p. 28]with the invasion of a different one, that it is often regarded as a true metastasis. Exceptionally, however, one or several joints remain painful and swollen, although this occurs chiefly in subacute attacks. In this way most of the large joints may successively suffer once, twice, or oftener during an attack of acute rheumatism. And as the inflammation commonly lasts in each articulation from two to four or more days, it is usual to have six or eight of the joints affected by the end of the first week. While the ankles and knees, wrists, elbows, and shoulders, are especially liable to be affected, and with a frequency pretty closely corresponding to the above order, the joints of the hands occasionally, and the hips even more frequently, escape. The intervertebral and tempero-maxillary articulations have very rarely suffered in the writer's experience.
If the ear be applied to the cardiac region in acute rheumarthritis, another local inflammation than the articular will very frequently be detected, which otherwise would probably be unrecognized, and yet it is the most important feature of the disease. In the first or second, or even as late as the fourth, week of the fever the signs of endocarditis of the mitral valve, occasionally of the aortic, and sometimes of both, will exist in an uncertain but large proportion of cases, or those of pericarditis, but in a less proportion, will obtain. Indeed, the cardiac inflammation may even precede the articular, and some believe it may be the only local evidence of rheumatic fever. As a general rule, the implication of the endo- or pericardium in acute rheumarthritis gives rise to no marked symptoms or abrupt modification of the clinical features of the case, and a careful physical examination must be instituted to discover its existence. But the recurrence of pain or tightness either in the precordial or sternal region, of marked anxiety or pallor of the face, of sudden increase in the weakness or frequency of the pulse, or of irregularity in its rhythm, of restlessness or delirium, of oppression of breathing, or of short, dry cough,—may indicate the invasion of the endo- or peri- or myocardium, and a physical examination will be needed to detect the cardiac disease and to exclude the presence of pleuritis, pneumonia, or bronchitis. Sometimes, however, especially in severe cases, an extensive pericarditis, with or without myocarditis, will produce grave constitutional disturbance, in which sleeplessness, delirium, stupor, generally associated with a very high temperature and marked prostration, will, as it were, mask both the articular and the cardiac affection.26
26 See Stanley's case, Med.-Chir. Trans., 1816, vol. vii. 323, and Andral's Clinique Médicale, t. i. 34.
As regards the murmurs which arise in acute rheumatic endo- or pericarditis, while they are usually present and quite typical, this is not always so. The only alteration of the cardiac sounds may be at first and for some time a loss of clearness and sharpness, passing into a prolongation of the sound, which usually develops into a distinct murmur, or the sounds may be simply muffled. In pericarditis limited to that portion of the membrane which covers the great vessels no friction murmur may be audible, or it may be heard and be with difficulty distinguished from an endocardial murmur. On the other hand, a systolic basic murmur not due to endo- or pericarditis frequently exists, sometimes in the early, but usually in the later, stages of rheumatic fever.
[p. 29]Other local inflammations occasionally arise in the course of acute rheumatism: pneumonia is one of the most frequent; left pleuritis is not infrequent, and is doubtless often caused by the extension of a pericarditis; but both pneumonia and pleurisy are occasionally double in rheumatic fever. Severe bronchitis is observed now and then, and very rarely peritonitis, and even meningitis. These several affections, together with delirium, coma, convulsions, chorea, and hyperpyrexia, which are likewise occasional incidents of the disease, will be considered under the head of non-articular manifestations and complications of acute articular rheumatism.27
27 See observations of W. S. Cheesman, M.D., New York Medical Record, Feb. 25, 1882, 202.
Some of the symptoms of acute articular rheumatism need individual notice.
The temperature in acute articular rheumatism maintains no typical course, and usually exhibits a series of exacerbations and remissions, which correspond closely in time and degree with the period, duration, and severity of the local inflammatory attacks. As a very general rule in average cases, the temperature attains by the end of the first or second day to 102° F., and while the subsequent evening exacerbations may reach 104°, 104.4°, or very rarely 105°, yet in the great majority of cases the maximum temperature does not exceed 103° F., and in a very considerable number falls short of 102°. An analysis of one of Dr. Southey's tables28 shows that in 84 cases of acute rheumatism 1 attained the temperature of 105.8°; 8, that of 104° to 105°; 15, that of 103° to 104°; 32, that of 102° to 103°; 17, that of 101° to 102°; 10, that of 100° to 101°; and 1, that of 99.8°; that is, the temperature was below 103° in five-sevenths, and below 104° in about ten-twelfths, of the whole. In very mild cases, in which but a few joints are inflamed, and only to a slight degree, the temperature may not reach 100° at any time, and there may be intervals of complete apyrexia. On the other hand, in a few rare severe cases of rheumatic fever, especially when complicated with pericarditis, pneumonia, or delirium, or other disturbance of the cerebral functions, the temperature attains to 106°, 108°,29 109.4°,30 110.2°,31 or even 111°,32 or 112°. Such cases are now spoken of as examples of rheumatic hyperpyrexia.
28 St. Bartholomew's Hospital Reports, xiv. p. 12.
29 Weber, Clinical Society's Trans., vol. v. p. 136.
30 Th. Simon, quoted by Senator, Ziemssen's Cyclop. of Prac. Med., xvi. p. 46.
31 Murchison and Burdon-Sanderson, two cases, Clinical Society's Trans., vol. i. pp. 32-34.
32 Ringer, Med. Times and Gaz., vol. ii., 1867, p. 378.
There is no rule about the mode of invasion of this high temperature. It may ensue gradually or suddenly, the previous range having been low, moderate, or high, steady or oscillating.
Defervescence in rheumatic fever takes place, as a very general rule, gradually—i.e. by lysis—but exceptionally it is completed in forty-eight or even twenty-four hours. An interesting observation, which will be of much prognostic value if it be confirmed hereafter, has been made by Reginald Southey,33 to the effect "that a short period of defervescence, or a sudden remission and an early remission, betokens the relapsing form of the disease, and the likelihood of frequent relapses, as well as of slow ultimate recovery, in the direct ratio as this defervescence has been early and abrupt."
33 St. Bartholomew's Hospital Reports, xiv. p. 16.
[p. 30]The characters of the urine in acute rheumatism are tolerably uniform, but far from constantly so. Its quantity in the majority of cases is reduced, frequently not exceeding twenty-four ounces per diem, and occasionally not exceeding fourteen. This is owing in some degree to profuse sweating, but also, as in other febrile affections, to retention of water. Its density is usually high—1020 to 1030, or even 1035—which is due chiefly to its concentration, and not, as has been generally supposed, mainly to an increase in the total solids excreted.34 Its color is a very dark red or deep reddish-yellow, partly from concentration; but it is yet not known whether the deep hue is partly from increase of the normal pigments or of one of them (urobilin),35 or from the presence of some abnormal coloring matter. Its reaction is generally highly acid, and continues so for many hours after its discharge, unless in subacute cases, when it is occasionally neutral or sometimes alkaline at the time of its escape, or becomes so in a very short time afterward. It is commonly toward the decline of the attack that the urine becomes neutral or alkaline. As a very general rule, the amount of urea and of uric acid excreted during the febrile stage exceeds what is physiological, and begins to decline when convalescence commences; but this may be reversed (Parkes,36 Lede,37 Marrot38). The sulphuric acid is notably increased (Parkes), the chlorides often diminished and sometimes absent, and the phosphoric acid very variable (Beneke, Brattler39), but usually lessened (Marrot).
34 See Guy's Hospital Reports, 3d Series, vol. xii. 441.
35 Jaffe, Virchow's Archiv, xlvii. 405, quoted in Ziemssen's Cyclopæd. Prac. Med., xvi. 41.
36 On Urine, p. 286.
37 Recherches sur l'Urine dans le Rheumatisme Artic. Aigue, Paris, 1879.
38 Contribution à l'Étude du Rheumatisme Artic., etc., Paris, 1879, 41.
39 Quoted by Parkes, op. cit., 290.
During convalescence the urine increases in quantity, while, as a general rule, the urea and uric acid lessen relatively and absolutely, and the chlorides resume their normal proportions to the other ingredients. The reaction frequently becomes alkaline, and the specific gravity falls considerably, although not always as soon as the articular inflammation subsides. Temporary albuminuria occurs very frequently in the febrile and occasionally in the declining stage, but generally disappears when convalescence is completed. It obtained on admission in 8 out of 43 cases lately reported by Dr. Greenhow.40 A more abiding albuminuria, due very rarely to acute parenchymatous nephritis, may be met with (Johnson, Bartels, Hartmann, Corm). Blood, even in considerable amounts, has also rarely appeared in the urine,41 sometimes in connection with embolic nephritis and endocarditis, for such appear to have been the nature of Rayer's nephrite rheumatismale.42
40 Lancet, 1882, i. 913.
41 Clinical Lectures, R. B. Todd, edited by Beale, 1861, p. 346.
42 Traité des Maladies Reins. See also Dr. Weber, Path. Trans. of London, xvi. p. 166.
The saliva, which is normally alkaline, has usually a decidedly acid reaction in acute articular rheumatism, and Dr. Bedford Fenwick states that it always in this disease contains a great excess of the sulpho-cyanides, and that these slowly and steadily diminish, till at the end of the third week or so they become normal in amount.
A profuse, very acid, sour-smelling perspiration is one of the striking symptoms occurring in the course of acute articular rheumatism, and [p. 31]until very lately it has been generally held to indicate an excessive formation in, and elimination of acid from, the system, either lactic acid or some of the acids normal to the perspiration, as acetic, butyric, and formic. However, not only have chemists failed to detect lactic acid in the perspiration of acute rheumatism, but late research tends to show that the excessive acidity of the perspiration in this disease is but very partially due to the perspiration itself, and is chiefly owing to chemical changes taking place in the overheated and macerated surface of the skin and its epidermis, and to the retention of solid products accumulated on that surface. Besnier says that if in acute articular rheumatism or other disease attended with much perspiration the surface be kept well washed, the sweat will be found in the greater number of cases at the moment of its secretion to be nearly neutral as soon as actual diaphoresis occurs, more decidedly acid when the perspiration is less abundant or begins to flow, and exceptionally alkaline. Most physicians are aware that the profuse perspiration of acute rheumatism is non-alleviating; it is not a real critical discharge of noxious materials from the system, nor is it followed by prompt reduction of the temperature and other symptoms. It is but a symptom of the disease, and occurs especially in severe cases, and when it continues long after the reduction of the temperature it is a source of exhaustion, and may be checked with advantage.
The blood is deficient in red globules, Malassez finding in men from 2,850,000 to 3,700,000 per cubic millimeter instead of 4,500,000 to 5,000,000, and in women 2,300,000 to 2,570,000 instead of 3,500,000 to 4,000,000. The hæmoglobin and the oxidizing power of the blood are also considerably reduced; the fibrin is largely increased (6 to 10 parts in 1000 instead of 3); the albumen and albuminates are lessened, the extractives increased; the proportion of urea is normal, and no excess of uric acid is found in the blood. Instead of that fluid being less alkaline than normal, Lepine and Conard have recently stated that its alkalinity is increased in acute rheumatism, but constantly diminished in chronic rheumatism,43 and no excess of lactic acid has been proved to exist in the blood in either acute or chronic rheumatism. A condition of excessive coagulability of the fibrin, independently of its excessive amount (inopexia), is an habitual character of acute rheumatism; however, in very bad cases, especially those attended with hyperpyrexia and grave cerebral symptoms, the blood after death has been black and coagulated and the fluid in the serous cavities has given an acid reaction. The above alterations in the blood usually are proportionate to the intensity of the fever and the number of the joints and viscera involved.
43 Lepine, "Note sur la determination de l'Alcalinité du Sang," Gaz. Méd. de Paris, 1878, 149; Conard, Essai sur l'Alcalinité du Sang dans l'État de Sante, etc., Thèse, Paris, 1878.
The manifestations of acute articular rheumatism other than the articular are various, and some of them, more especially those observed in the heart, may be regarded as integral elements of the disease, for they occur in a large proportion of the cases, often coincidentally with the articular affection, and may even precede it, and probably may be the sole local manifestation of acute rheumatism, although under the last-mentioned circumstances it is difficult to prove the rheumatic nature of the ailment.
The cardiac affections may be divided into inflammatory and [p. 32] non-inflammatory. The former consist of pericarditis, endocarditis, and myocarditis; the latter embrace deposition of fibrin on the valves, temporary incompetence of the mitral or tricuspid valves, and the formation of thrombi in the cavities of the heart. For practical purposes hæmic murmurs may be included in the latter group.
No reliable conclusions can be drawn respecting the gross frequency of recent cardiac affections in rheumatic fever, for not only do authors differ widely on this point, but they do not all distinguish recent from old disease, nor inflammatory from non-inflammatory affections, nor hæmic from organic murmurs. Nor does it appear probable, from the published statistics, that these differences are owing to peculiarities of country or race. The gross proportion of heart disease of recent origin in acute and subacute articular rheumatism was in Fuller's44 cases 34.3 per cent.; in Peacock's,45 32.7 per cent.; in Sibson's46 (omitting his threatened or probable cases), 52.3 per cent.;47 in 3552 St. Bartholomew's Hospital cases analyzed by Southey,48 29.8 per cent.; in Bouilland's cases, quoted by Fuller,49 5.7 per cent.; in Lebert's,50 23.6 per cent.; in Vogel's,50 50 per cent.; in Wunderlich's,50 26.3 per cent. I am not aware of any analysis, published in this country, of a large number of cases of rheumatism with reference to cardiac complications, but Dr. Austin Flint,51 after quoting Sibson's percentage of cases of pericarditis, which was (63 in 326 or) 19 to the 100, remarks, "I am sure that this proportion is considerably higher than in my experience."
44 On Rheumatism, Rheumatic Gout, etc., 3d ed., p. 280.
45 St. Thomas's Hospital Reports, vol. x. p. 19.
46 Reynolds's Syst. of Med., Eng. ed., vol. iv. 186.
47 Those familiar with the accuracy and diagnostic skill of the lamented Sibson will not hesitate to add his 13 cases of very probable endocarditis to his 170 positive cases of cardiac inflammation in 325 examples of acute rheumatism, which will raise his percentage to 56.3.
48 Lib. cit., vol. xiv. 6.
49 Lib. cit., 264.
50 See Senator in Ziemssen's Cyclopæd. Pract. of Med., xvi. 49.
51 Pract. Med., 5th ed., 314.
The frequency of cardiac complications in rheumatism is influenced by several circumstances. Some unexplained influence, such as is implied in the terms epidemic and endemic constitution, appears to obtain. Peacock found the proportion of cardiac complications in rheumatism to range from 16 to 40 per cent. during the five years from 1872 to 1876, and a similar variability is shown in Southey's statistical table52 covering the eleven years from 1867 to 1877. Be it observed that these variations occurred in the same hospitals and under, it may be presumed, very similar conditions of hygiene and therapeusis. Youth predisposes to rheumatic inflammation of the heart, so that it may still be said that the younger the patient the greater the proclivity. Of Fuller's cases, 58 per cent. were under twenty-one, and the liability diminished very markedly after thirty. Of Sibson's cases, 62 per cent. were under twenty-one. In infancy and early childhood the liability is very great, and at those periods of life the heart, and more especially the endocardium, rarely escapes; and the cardiac inflammation often precedes by one or two days the articular. The careful observations of Sibson confirm the spirit, but not the letter, of Bouilland's original statement, and proves that the danger of heart disease is greater in severe than in mild cases of acute rheumatism, and that this is especially true of pericarditis. (It may be remarked here, en parenthese, that the number of joints affected is [p. 33]very generally in proportion to the severity of the attacks.) However, the mildest case of subacute rheumatism is not immune from cardiac inflammation, and it has occasionally been observed even in primary chronic rheumatism.53 Occupations involving hard bodily labor or fatigue, whether in indoor or outdoor service, render the heart very obnoxious to rheumatic inflammation. Existing valvular disease, the result of a previous attack of rheumatism, favors the occurrence of endocarditis in that disease. Some authorities maintain that treatment modifies the liability to rheumatic affection of the heart, and this will be spoken of hereafter. The period of the rheumatic fever at which cardiac inflammation sets in varies very much, but it may be confidently stated that it occurs most frequently in the first and second weeks, not infrequently in the third week, seldom in the fourth, and very exceptionally after that, although it has happened in the seventh. An analysis of Fuller's experience54 in 22 cases of rheumatic fever and 56 of endocarditis—a total of 78—shows that the disease declared itself under the sixth day in 8; from the sixth to the tenth in 29; from the tenth to the fifteenth in 17; from the fifteenth to the twenty-fifth in 18; and after the twenty-fifth in 6. The friction sound was audible in Sibson's 63 cases of rheumatic pericarditis—from the third to the sixth day in 10, and before the eleventh day in 30, or nearly one-half of the whole. That observer concludes "that in a certain small proportion of the cases, amounting to one-eighth of the whole," the cardiac inflammation took place at the very commencement of the disease, and simultaneously with the invasion of the joints.55
52 Lib. cit.
53 Raynaud, Nouveau Dict. de Méd. et de Chir., t. viii. 367.
54 Lib. cit., pp. 77-278.
55 Lib. cit., p. 209. See also Dickinson in Lancet, i., 1869, 254; Bauer in Ziemssen's Cyclopæd., vi. 557.
Of the several forms of rheumatic cardiac inflammation, endocarditis is the most frequent, and in a large proportion of cases it may exist alone; pericarditis is also very often observed, but it seldom is found per se, being in the vast majority of cases combined with endo- and occasionally with myocarditis. It is generally the ordinary verrucose endocarditis that obtains. The ulcerative form occurs sometimes, and should be suspected if in a mild or protracted case of acute rheumatism endocarditis sets in with, or is accompanied by, rigors, and the general symptoms are of pyæmic or typhoid character or both, even although an endocardial murmur is not present, for extensive vegetating ulcerative endocarditis frequently exists without audible murmur. It is remarkable, as Osler has shown,56 how few instances of ulcerative endocarditis developing during the course of acute rheumatism are reported; and I would add that by no means all of these were examples of first attacks, chronic valvular lesions, the consequence of former illness, existing in many of them at the time of the final acute attack. Southey's57 patient, and both of Bristowe's,58 had had previous rheumatic seizures. However, Peabody's case,59 one of Ross's three cases,60 and Pollock's61 case appear to have been examples of ulcerative [p. 34]endocarditis occurring during a first attack of acute articular rheumatism. The united and thickened condition of two segments of the aortic valve in one of Ross's cases indicates old-standing disease, although no history of former rheumatism is given. Goodhardt62 has lately insisted upon the tendency of ulcerative endocarditis to appear in groups or epidemics, but the evidence is not conclusive.
56 Archives Médecine, vol. v., 1881; Trans. International Med. Cong., vol. i. 341.
57 Clin. Soc. Trans., xiii. 227.
58 Brit. Med. Jour., i., 1880, 798.
59 Medical Record N.Y., 24th Sept., 1881, 361.
60 Canada Med. and Surg. Journ., vol. xi., 1882, 1, and ib., vol. ix., 1881, 673.
61 Lancet, ii., 1882, 976.
62 Trans. Path. Soc. London, xxxiii. 52.
Space will not permit any detailed description of the symptoms and signs of endo- or pericarditis: these will be found in their proper places in this work, but a few observations are needed upon myocarditis, which occasionally occurs in combination with rheumatic pericarditis, and is a source of much more danger than the latter is, per se. Dr. Maclagan63 is almost the only authority who recognizes the occurrence of rheumatic myocarditis independently of inflammation of the membranes of the heart. He maintains that the rheumatic poison probably and not infrequently acts directly on the cardiac muscle; in which case the resulting inflammation is apt to be diffused over the left ventricle and to produce grave symptoms, while in other instances the inflammatory process begins in the fibrous rings which surround the orifices of the heart (especially the mitral), extends to the substance at the base of the heart, and is there localized. As in this latter form the inflammation usually extends also to the valves, "any symptoms to which the myocarditis gives rise are lost in the more obvious indications of the valvulitis." However, this limited inflammation of the myocardium is not dangerous. Dr. Maclagan asserts that the more diffused and dangerous inflammation of the walls of the left ventricle, while always difficult, and sometimes impossible, of diagnosis, can be determined with tolerable certainty in some cases. In this view, however, he has been preceded by Dr. Hayden,64 who states that the diagnosis of myocarditis is quite practicable irrespective of the accompanying inflammation of the membranes of the heart.
63 Rheumatism: its Nature, Pathology, and Successful Treatment, 1881.
64 Diseases of the Heart and Aorta, 1875, 746.
From the observations of the author just named, as well as of many others, it may be inferred that acute diffused myocarditis of the left ventricle exists in rheumatic fever when either with or without coexisting pericarditis there are marked smallness, weakness, and frequency of pulse, anguish or pain or great oppression at the præcordia, severe dyspnoea, the respiration being gasping and suspirious, feeble, rapid, and irregular action of the heart, great weakness of the cardiac sounds, and almost extinction of the impulse, evidence of deficient aëration of the blood combined with coldness of surface, tendency to deliquium, and when these symptoms and signs cannot be fairly attributed to extensive pericardial effusion or to pulmonary disease, or to obstructed circulation in the heart consequent upon endocarditis with intra-cardiac thrombosis or upon rupture of a valve. It might, however, be impossible to exclude endocarditis complicated with thrombosis, conditions which do occur in rheumatic endocarditis, or a ruptured valve, which, although rarely, has been occasionally observed. Grave cerebral symptoms, delirium, convulsions, coma, though frequently present, are not peculiar to acute myocarditis.65 [p. 35]Hence, even with the above group of clinical facts, the diagnosis at best can be but probable. The disease, too, may be latent, or, like Stanley's66 celebrated case, produce disturbances of the cerebral system rather than of the circulatory.
65 In illustration see case by Southey in which the symptoms and signs agree very well with the above description, and yet, although the heart's substance was of dirty-brown color and the striation of its fibre lost, Southey did not believe these appearances due to carditis. (Clin. Trans., xiii. p. 29.)
66 Med.-Chir. Trans., vol. vii.
Dr. Maclagan has advanced the opinion that a subacute myocarditis is not of uncommon occurrence in acute articular rheumatism, and may be unattended by endo- or pericarditis. Such a condition, he says, may be diagnosed when early in the course of the case the heart's sounds quickly become muffled rather than feeble. As he quotes but one case67 in which an autopsy revealed alterations in the walls of the heart, and as endocarditis and a little effusion in the pericardium coexisted, it is premature to accept the evidence as final, and the great importance of the subject demands further investigation.
67 Lib. cit., p. 175.
Admitting with Fuller the occasional deposition of fibrin upon the valves and endocardium in rheumatic fever independently of endocarditis, the murmur resulting therefrom could not be reliably distinguished from that of inflammatory origin. It remains to speak briefly of temporary incompetence of the mitral and tricuspid valves and their dynamic murmurs, and of hæmic murmurs. Occasionally, in severe cases of rheumatic fever, more especially in the advanced stage, there may be heard a systolic murmur of maximum intensity either in the mitral area or over the body of the left ventricle, unaccompanied by accentuation of the second sound, or, as a general rule, by evidence of pulmonary obstruction. Such murmurs are apt to be intermittent, and as they disappear on the return of health, they have been satisfactorily referred to temporary weakness of the walls of the heart, so that the auriculo-ventricular orifices are not sufficiently contracted during the ventricular systole for their valves to close them, and regurgitation follows. Yet, inasmuch as Stokes distinctly mentions the absence of murmur in many cases of softening of the heart in typhus, it is probable that an excessive weakness of the ventricular wall is incompatible with the production of murmur, and that the presence of murmur in such circumstances is evidence of some remaining power in the heart.
Dr. D. West68 has published some cases of acute dilatation of the heart in rheumatic fever which strongly corroborate these views. The murmur in one of them became appreciable only as the heart's sounds increased in loudness and the dilatation lessened. One ended fatally, and acute fatty degeneration of the heart's fibres was found in patches.69 I believe that some of these temporary mitral murmurs in acute rheumatism depend upon a moderate degree of valvulitis quite capable of complete resolution. Sibson70 has lately stated that he has met with the murmur of tricuspid regurgitation without a mitral murmur in 13 out of 107 cases of rheumatic endocarditis, and with a recent mitral murmur in 27 out of 50 [p. 36]cases. "The tricuspid murmur generally comes into play about the tenth or twelfth day of the primary attack, along with symptoms of great general illness;" it appears earlier, as a rule, in those cases in which it is associated with mitral regurgitation than when it exists alone; it is of variable duration, but usually short—from one to nineteen days or more. He regards it as of non-inflammatory origin, and dependent upon regurgitation due to the so-called safety-valve function of the tricuspid valve; and when limited to the region of the right ventricle he infers that it is usually the effect and the evidence of endocarditis affecting the left side of the heart. These novel statements are confirmed by the observations of Parrot, Balfour, and William Russell,71 which go to prove that tricuspid regurgitation occurs frequently in the more advanced stages of debility. No other authority than Sibson, however, insists upon its frequent occurrence in acute rheumatism.
68 Barth. Hosp. Repts., xiv. 228.
69 On this subject see Stokes, Dis. Heart and Aorta, pp. 423, 435, 502; Stark, Archives générales de Méd., 1866; DaCosta, American Journal Med. Sci., July, 1869; Hayden, Dis. Heart and Aorta, 1875; Balfour, Clin. Lects. on Heart and Aorta, 1876; Cuming, Dublin Quart. Jour. Med. Sci., May, 1869; Nixon, ib., June, 1873. I. A. Fothergill has seen several cases in which such mitral murmurs have followed sustained effort in boys, and have disappeared after a time: The Heart and its Diseases, 2d ed., 1879, p. 177.
70 Reynolds's System. Med., Eng. ed., vol. iv. 463.
71 See Brit. Med. Jour., i. 1883, 1053.
The anæmia which is so striking a symptom of rheumatic fever, especially when several joints are severely inflamed, coexists very frequently with a systolic basic murmur, which is most often louder over the pulmonary artery (in second left intercostal space and more or less to left of sternum) than over the aorta. The murmur may appear early in the disease, but sets in most frequently when the disease is subsiding. When thus appearing late in a case accompanied by endocarditis and pulmonary congestion, it is of favorable omen and indicates improvement in the thoracic affection. The growing opinion, however, respecting so-called anæmic murmurs is, that they depend chiefly upon regurgitation through the tricuspid orifice, although Dr. W. Russell refers them to pressure of a distended left auricle upon the pulmonary artery.72
72 Ib., 1065.
Pulmonary affections in form of pleuritis, pneumonia, or bronchitis are common complications of rheumatic fever. Adding Latham's,73 Fuller's,74 Southey's,75 Gull and Sutton's,76 Pye-Smith's,77 and Peacock's78 cases together, we have a total of 920 in which some one or more of the above pulmonary affections obtained in 109 instances, or 11.8 per centum. A further analysis of Latham's and Fuller's cases shows that it is especially when rheumatic fever is complicated with cardiac disease that the lungs suffer; thus, pulmonary affections obtained in 26.5 per cent. of cases complicated with heart disease, and in only 7 per cent. of cases free from that disease. It is more especially when pericarditis complicates rheumatic polyarthritis that pulmonary affections occur. Thus, these were found in only 10.5 per cent. of cases of recent rheumatic endocarditis, in 58 per cent. of cases of pericarditis, and in 71 per cent. of cases of endo-pericarditis. The tendency which inflammation of the pericardium has to extend to the pleura probably partially accounts for the more frequent association of the pulmonary affections with rheumatic peri- than with rheumatic endocarditis. (Sibson found pleuritic pain in the side twice as frequent in pericarditis, usually accompanied with endocarditis (31 in 63), as in simple endocarditis, 26 in 108.79) But the greater severity of those cases of rheumatic fever complicated with peri- or endo-pericarditis must also have a decided influence in developing the pulmonary affections. [p. 37]Pneumonia and pleuritis are very frequently double in rheumatic fever, and are often latent, requiring a careful physical examination for their detection. So suddenly does the exudation take place in some cases of rheumatic pneumonia that the first stage is not to be detected either by symptoms or signs. On the other hand, in some cases the absence of the typical signs of hepatization, the want of persistence in the physical signs, and their rapid removal, and even in rare instances an obvious alternation between the pulmonary and the articular symptoms, suggest that the process often stops short of true hepatization, and partakes rather of congestion and splenization, with or without pulmonary apoplexy—a view which has been occasionally confirmed by the autopsy.80
73 Latham's Works, Syd. Soc., i. 98 et seq.
74 Lib. cit., 317.
75 Bartholomew Hospital Reports, xv. 14.
76 Guy's Hosp. Reports, 3d Series, xi. 434.
77 Ib. xix. 324.
78 St. Thomas's Hospital Reports, x. 12-17.
79 Reynolds's System Med., iv. 233.
80 Vide Sturges, Natural History and Relations of Pneumonia, 1876, pp. 70-78; T. Vasquez, Thèse, Des complications Pleuro-pulmonaires du Rheumatisme Artic. Aigue, Paris, 1878, pp. 25-31; M. Duveau, Dictionnaire de Méd. et de Chir., t. xxviii. p. 443.
Active general congestion of the lungs has occasionally been observed in this disease, and has proved fatal in five minutes81 and in an hour and a half82 from the invasion of the symptoms. The rheumatic poison frequently excites pleuritis, some of the characters of which are—the suddenness with which free effusion occurs; the promptness with which it is removed, only perhaps to invade the other pleura, and then to reappear in the cavity first affected; the diffusion of the pain over the side and its persistence during the effusion; and its frequent concurrence with pericarditis, and in children with endocarditis; its little tendency to become chronic, and its marked proclivity to become double. It is often latent and unattended with pain. Sibson asserts that if in rheumatic pericarditis "pain over the heart is increased or excited by pressure over the region of the organ, it may with an approach to certainty be attributed to inflammation of the pleura," etc. The product of the inflammation is commonly serous, but occasionally purulent.
81 Thèse d'Aigue pleur., 1866, par B. Ball.
82 M. Aran, quoted by Vasquez, lib. cit., p. 14.
The disturbances of the nervous system are amongst the most important complications of acute rheumatism, and are due either to functional disorder or very rarely to obvious organic lesions of the nerve-centres or their membranes. The dominant functional disturbance may be delirium, which is greatly the most frequent; or coma, which is rare; or chorea, very frequently observed in children; or tetaniform convulsions, which occur very seldom per se. As a rule, two or more of these forms coexist or alternate with or succeed one another, and the grouping, as well as the variety, of the symptoms may be greatly diversified. In 127 observations there were 37 of delirium only, 7 of convulsions, 17 of coma and convulsions, 54 of delirium, convulsions, and coma, 3 of other varieties (Ollivier et R., cited by Besnier).
Rheumatic Delirium.—Either with or without subsidence of the articular inflammation, about from the eighth to the fourteenth day of the illness, but occasionally at its beginning, or sometimes on the eve of apparent convalescence, the patient becomes restless, irritable, excited, and talkative; sleep is wanting or disturbed; some excessive discharge from the bowels or kidneys occasionally occurs; profuse perspiration is usually present, and may continue, but frequently lessens or altogether ceases; the skin becomes pungently hot, the temperature generally—not always, however—rising rapidly toward a hyperpyrexial point, and ranging from [p. 38]104° to 111°; and transient severe headache and disturbances of special sense sometimes obtain. At a later period, or from the outset in hyperacute cases, flightiness of manner or incoherence in ideas is quickly succeeded either by a low muttering delirium, twitchings of the muscles, violent tetaniform movements and general tremors, and a condition perhaps of coma-vigil, or by an active, noisy, even furious, delirium. The articular pains are no longer complained of, and sometimes the local signs of arthritis also quickly disappear; but neither statement is uniformly true. The pulse becomes rapid; prostration extreme; semi-consciousness or marked stupor gradually or rapidly supervenes; the temperature continues to rise; the face, previously pale or flushed, becomes cyanotic; and very frequently death ensues, either by gradual asthenia or rapid collapse, often preceded by profound coma or rarely by convulsions. Deep sleep often precedes prompt recovery.
The duration of the nervous symptoms varies from one or two, or more usually six or seven, hours in very severe cases, to three or four days in moderate ones, or occasionally seven, eight, or sixteen83 or twenty-nine days84 in unusually protracted cases. In the last-mentioned, however, the delirium is not usually constant, and frequently disappears as the temperature falls, and recurs when its rises. Moreover, a rapid and extreme elevation of temperature is frequently altogether wanting.
83 Southey's case, Clin. Soc. Trans., xiii. p. 25. Sleeplessness preceded it for four days, and there was no hyperpyrexia.
84 Graham's case, ib., vi. p. 7. Delirium set in on the seventh day of illness, and three days after invasion of joints. Temperature 104.8° early in disease; never exceeded 106°, probably owing to repeated use of cold baths. Temperature at death, 104.2°.
No real distinction can be established between these protracted cases of rheumatic delirium and so-called rheumatic insanity, in which occur prolonged melancholia, with stupor, mania, hallucinations, illusions, etc., often associated with choreiform attacks. This variety may be of short duration or continue until convalescence is established, or may rarely persist after complete recovery from the articular affection.
Coma may occur in acute rheumatism without having been preceded or followed by delirium or convulsions, although it is very rare; and, like delirium, it may obtain without as well as with peri- or endocarditis or hyperpyrexia. It usually proves very rapidly fatal. In Priestly's case, an anæmic woman of twenty-seven, during a mild attack of acute rheumatism, one night became restless; at 3 A.M. the pain suddenly left the joints; apparent sleep proved to be profound coma, and at 6 A.M. she was in articulo mortis.85 Southey relates the history of a girl of twenty who, without previous delirium or high temperature, suddenly became unconscious, and died in half an hour.86 One of Wilson Fox's cases had become completely comatose, and was apparently dying nine hours after the temperature had rapidly risen to 109.1°, when she was restored to consciousness by a cold bath and ice to her chest and spine.87
85 Lancet, ii., 1870, 467.
86 Clin. Soc. Trans., xiii. p. 29.
87 The Treatment of Hyperpyrexia, 1871, 4.
Convulsions of epileptiform, choreiform, or tetaniform character frequently succeed the delirium, but in exceptional cases they occur independently of it, and may even prove fatal.
Besides the choreiform disturbances which occur in connection with delirium, stupor, tremor, etc. in cerebral rheumatism, simple chorea is [p. 39]frequently observed as a complication or a sequence, or even as an antecedent, of acute articular rheumatism, and they occasionally alternate in the same patient and in the same family. Chorea is perhaps most frequently seen in mild cases and in the declining and convalescent stages of rheumatic fever, and, while very common in childhood and adolescence (five to twenty), it is very rare later in life.
Such are the chief functional disturbances of the brain met with in rheumatic fever, and the post-mortem examination reveals in them either quite normal naked-eye appearances, or more frequently, especially in rapidly fatal cases, general congestion of the pia mater, and to a less degree of the cerebral substance, or in more protracted cases a greater or less increase of transparent or opalescent serum in the subarachnoid space and ventricles. The serum may be slightly or deeply tinged with blood. If the serous or sero-sanguinolent effusion be considerable, the encephalic mass or portions of it may be anæmic. But besides these conditions, which are also commonly observed in many other febrile diseases, and which are probably only concomitants of the functional disturbance arising in the advanced stage of acute articular rheumatism, certain organic affections of the nervous centres or their membranes occasionally occur in this disease, and are plainly the cause of the cerebral disturbance observed during life. Cerebral meningitis, although very rare as a complication of acute articular rheumatism, except in certain hot climates, like that of Turkey,88 does occur, and lymph or pus is found, usually over the convexity of the brain, but sometimes at the base and down the cord.89 The symptoms of rheumatic cerebral meningitis are very like those of rheumatic delirium; vomiting, and even, but less frequently, pain in the head, may be absent, while hyperpyrexia may coexist (Foster's case), although not necessarily present. Should the pulse from being frequent become slow and irregular, and any paralytic symptoms ensue, meningitis may be suspected. In some of these cases the meningitis is a consequence of ulcerative endocarditis and embolism of the cerebral vessels,90 but in others it obtains without endocarditis or any purulent formation elsewhere than in the meninges, as there is probably a true rheumatic localization like pericarditis. The articular inflammation may continue after the invasion of the meningitis, or the latter may promptly follow the disappearance of the former, as though a metastasis of morbid action had taken place.91 In many instances, according to Ollivier, Ranvier, Behier, and others, although the macroscopic signs of meningitis are absent, the microscope detects proof of its presence in the existence of an increased number of vessels, fatty granulations on their walls, proliferation of nuclei and capillary extravasations—histological conditions identical with those found in the mild degrees of rheumatic inflammation of the joints.
88 Senator, in Ziemssen, xvi. 50.
89 Watson's Prac. Physic, 1872, Am. ed. vii. 335; Fyfe, Med. Gazette, vol. xxix. 703; Fuller, lib. cit., 302; Leudet, Clin. Médicale, 139; Dowse, London Lancet, ii. 1872, 9; Foster, ib., ii. 1868, 115; Hicks, New York Medical Record, Nov., 1878, 404.
90 That ulcerative endocarditis frequently produces meningitis is illustrated by Osler's cases, 4 out of 7 of which were complicated with purulent meningitis: Transactions of International Med. Congress, 1881, i. 344.
91 See a case reported by W. L. Ramsey in New York Medical Record, i., 1881, p. 9.
Embolism of the cerebral arteries, producing meningitis, or more frequently softening of the cerebral substance or hemorrhage, or proving [p. 40]fatal before necrobiosis has time to set in, is an occasional complication of acute articular rheumatism. A young lady, while under my care suffering from her first attack of articular rheumatism complicated with endocarditis, became suddenly hemiplegic and aphasic, and died twelve hours later. In a girl of thirteen, the subject of acute articular rheumatism complicated with ulcerative endocarditis, right hemiplegia suddenly occurred, and at the autopsy Bristowe found an embolon in the left middle cerebral artery and a softened area in the left corpus striatum. Bradbury reports a primary acute rheumatism with endocarditis, delirium, and coma, but without paralysis, in which a plug was found in the right middle cerebral artery, but the brain was quite healthy.92
92 Lancet, ii., 1870, 148; also a case in Lancet, i., 1882, p. 605: in eighth week of subacute articular rheumatism; embolism; right hemiplegia. Autopsy: large vegetations on valves; obstruction in middle cerebral artery.
Very much the same observations are applicable to the disturbances of the spinal cord and its envelopes in rheumatic fever as have been made in reference to those of the cerebrum and its coverings. They may exist with or without any alteration of the cord or membranes to which they can be reliably referred; that is to say, they may be simply functional in the peculiar sense in which that word is now understood, or they may be connected with obvious structural changes, and chiefly with those indicating inflammation of the membranes or substance of the cord. The spinal symptoms may precede the articular affection, but generally appear after it. They sometimes closely resemble those of idiopathic tetanus,93 or of spinal meningitis, or of myelitis, or of meningo-myelitis; and in the last case, along with severe rachialgia, muscular rigidity, cutaneous and muscular hyperæsthesia, and neuralgic pains, there will occur numbness and more or less paralysis of the lower extremities,94 bladder, and rectum (paraplegia). These spinal disturbances may or may not be accompanied by hyperpyrexia, and when simply functional they are usually less severe and persistent, have a greater tendency to alternate with one another and with the articular affection, and are more amenable to treatment, than when due to those very rare complications of rheumatic fever, spinal meningitis or meningo-myelitis. The inflammation may involve both the cerebral and spinal membranes at the same time.
93 Bright's case, 2, Med.-Chirurgical Transactions, xxii. 4; Dr. E. C. Mann, N.Y. Medical Record, 1875, 38; Bouilland, Traité sur les Maladies du Coeur, t. i. p. 33.
94 Leudet, lib. cit., p. 139; Dowse, Lancet, i., 1872, 9.
The causes of these disturbances of the nervous system, when not attributable to appreciable lesions, such as congestion, inflammation, hemorrhage, embolism, thrombosis, and softening, are not established. The following appear to be reasonable conclusions from the facts at present known:
The most constant condition, and without which these cerebral symptoms very rarely arise, appears to be some susceptibility or vulnerability of the nervous system, inherited or acquired, rendering it apt to be disturbed by influences which less susceptible centres would successfully resist. Trousseau, who has especially advocated this view,95 considered intemperance in the use of spirits to be a frequent source of this nervous predisposition. Accepting this neurotic predisposition as the factor generally present when acute articular rheumatism is complicated [p. 41]with disturbances of the nerve-centres, we may inquire what are the circumstances in the disease capable of developing into activity the predisposition.
95 Clin.-Med., Syd. ed., i. 513 et seq.
Unquestionably, the existence of acute pericarditis, or of endocarditis, or of inflammation of the lungs or pleura, is one of those conditions.
Probably hyperpyrexia acts in some cases as an exciting cause of the nervous phenomena, for while the delirium preceded the hyperpyrexia in 6 cases, it accompanied it in 19 and followed it in 10;96 and the nervous symptoms disappear when the hyperthermia is removed by the employment of cold, and recur with the return of high temperature. The phenomena of sunstroke and heat-apoplexy prove that a high temperature is capable of producing convulsions and coma. That these grave cerebral disturbances are so infrequent in acute rheumatism (obtaining in about 3 or 4 per cent. only) is probably owing to the usual moderate range of temperature and the rarity of hyperpyrexia in the disease. Still, while hyperpyrexia is a disturber of cerebro-spinal function, too much importance must not be attached to it, for not only does such disturbance very frequently precede the hyperpyrexia, but there are many facts indicating that the hyperpyrexia is itself very frequently, like the delirium, tremor, and coma which precede or accompany it, but a consequence of disorder, usually of a paralyzing kind, of the nerve-centres. It has been met with in lesions of the pons, in tetanus, in injuries of the cord, in some cases of non-inflammatory softening of the brain and of cerebral hemorrhage; that is, in a class of affections not belonging to the specific fevers, but to those directly disturbing or destroying the functions of the nerve-centres. And cases of acute rheumatism do rarely occur in which a very high temperature is not accompanied by cerebral disturbances. Sibson quotes two such,97 one of which, with a temperature of 110.8°, was only restless and talked when asleep, and the other, with a temperature of 106.3°, presented only vomiting and dyspnoea. Cardiac inflammation was absent in both. DaCosta relates one in his valuable paper upon cerebral rheumatism in which, although the temperature was 110°, no cerebral symptoms nor cardiac affection existed.98
96 "Abstract Report upon Hyperpyrexia in Ac. Rheum.," Brit. Med. Jour., 1882, p. 807.
97 Lib. cit., p. 264.
98 This essay contains a record of 11 cases of cerebral rheumatism and several autopsies: Am. Jour. Med. Sci., 69, 1845, p. 36, case xi.
The goodly number of instances lately published in which grave cerebral symptoms have obtained in acute articular rheumatism at ordinary febrile temperatures, while they prove that hyperthermia is not an essential condition productive of such symptoms, require to be explained. Some such, no doubt, have been instances of marked predisposition, so that a moderate febrile temperature or some complication sufficed to disturb the brain, as we see in typhoid and other fevers, in pneumonia, etc. If there be a rheumatic poison—which has not yet been proved—it may, in predisposed persons, produce the cerebral symptoms. The argument99 that such poison should produce inflammation of the nervous centres if it acted directly on them is not convincing. It need not necessarily produce similar alterations in serous or synovial membranes and in nervous tissues. Many toxic agents disturb, and even suspend, the [p. 42]cerebro-spinal functions, and leave no appreciable changes in them. Do these cases prove that there is something peculiar to rheumatic fever which tends to disturb the nervous centres? Hardly; for while such disturbance is comparatively rare in that disease, it is observed frequently in many other febrile affections, notably in typhus, scarlatina, and small-pox; and as in these, so in rheumatic fever, it is more often observed in the severe than in the mild cases, as though it were a part of the systemic disturbance incident to the febrile affection and largely proportionate to its severity.
99 Maclagan, Rheumatism: Its Nature, Pathology, etc., 1881, 287.
Yet there is something special in acute rheumatism which perhaps has to do with the occurrence as well as the severity of the cerebro-spinal symptoms and of the hyperpyrexia; viz. the long duration and severity of the pain, and the number and importance of the parts, in addition to the articulations, which are one after the other or simultaneously involved in severe inflammation—peri-, endo-, myocardium, lungs, pleura, etc. Perhaps in no other acute febrile disease are so many distinct and important organs involved in inflammation at the same time or in rapid succession; and it is no wonder that the functions of the nervous system should in consequence become greatly depressed, exhausted, or disturbed.
The kidneys appear very rarely to suffer serious disease in acute rheumatism, if we except embolism of their arteries due to endocarditis; and it is very doubtful whether the rare instances100 in which an acute parenchymatous nephritis has been observed in acute rheumatism can be referred to direct rheumatic inflammation, or not, rather, to the operation of the exposure which induced the rheumatism. Further investigation is needed to determine whether interstitial nephritis is even very exceptionally an indirect consequence of rheumatism, as Lancereaux admits.
100 See DaCosta's cases 1 and 2, Cerebral Rheumatism, lib. cit.; case 1 certainly favors the view that either the rheumatic poison, if there be such, or the constitutional disturbance incident to acute polyarticular rheumatism, may sometimes produce nephritis. See also a case by A. Deroye, Thèse, Doctorat, Paris, 1874, quoted by P. Coubere in Contribution à l'Étude des Complications Renales du Rheumatisme Artic. Aigue, Paris, 1877.
The other complications, being of less importance, must be but barely alluded to. A pharyngitis attended with severe dysphagia and high fever occasionally precedes the other symptoms or occurs in the early stage of the disease. Gastralgia, enteralgia, simple serous diarrhoea, and dysentery also rarely occur in acute rheumatism. That they are sometimes, at least, truly rheumatic appears probable from the circumstance that they may precede, follow, or alternate with the articular affection, and are all intensely painful. I have but once met with acute peritonitis as a complication of acute rheumatism; the immunity of this serous membrane from rheumatic inflammation is an inexplicable anomaly in view of the proclivity of the pericardium and pleura to that process. Cystitis and orchitis are rare.
Several cutaneous affections are not unfrequently observed in relation with acute rheumatism. Besides sudamina and miliaria rubra, which are very common as consequences of the excessive perspiration,101 there [p. 43]are others which may be themselves rheumatic manifestations. Such are especially erythema marginatum,102 e. papulatum, and e. nodosum. A well-marked urticaria frequently precedes acute rheumatism in a friend of the writer's; it may occur during its course or soon after the cessation of the pains. Scarlatiniform eruptions are occasionally observed, and very rarely punctiform hemorrhages—peliosis rheumatica or rheumatic purpura. The purpuric symptom may be accompanied by erythema or urticaria, and may precede, accompany, or alternate with other rheumatic manifestations. Unlike purpura variolosa and idiopathic purpura hæmorrhagica, this variety appears to be free from danger.
101 Dr. J. T. Metcalfe of New York many years ago showed me a case of rheumatic fever in which the sweat-vesicles had run together, forming, instead of the usual pearly globular vesicles, irregular flat blebs, some of them equal in area to seven or nine primary vesicles, filled with transparent fluid, and this fluid could be displaced by pressure to adjacent parts, as though it lay simply under the superficial epidermic layer. I have seen several similar cases since.
102 Dr. Palmer relates a case complicated with erysipelas and peritonitis in Boston Med. and Surg. Journal, 1868.
Besides a slight local oedema affecting the malleoli, scrotum, eyelids, etc., or accompanying the cutaneous eruptions just mentioned, a more decided infiltration of the subcutaneous cellular tissue occasionally exists in the vicinity of the inflamed joints and tendinous sheaths, and more rarely extends to an entire limb, which may not only be considerably enlarged and painful and resemble a milk leg, but may be red, hot, and tender, and excite suspicion of phlegmonous erysipelas. Phlebitis, although infinitely less frequent than in gout, has been observed in acute articular rheumatism.103 Jaccoud in 1871104 mentioned the exceptional occurrence of subcutaneous nodosities in rheumatism, which he says Froriep first pointed out;105 but Homolle states that they had been previously mentioned by Sauvage and Chomel.106 Since then several independent observers have met with this affection, and Drs. Thomas Barlow and Francis Warner of London have lately written a short valuable paper upon the subject based upon 27 cases which they had separately or conjointly investigated. From their paper the following account is chiefly derived:107 These nodules may vary in number from one to fifty, and in size from that of a pin's head to the volume of an almond, and are quite subcutaneous, firm and elastic, painless, and freely movable. They are not usually attached to the skin, but to the tendons, deep fasciæ, pericranium, periosteum, etc.; the integument over them is free from heat, redness, and infiltration, although exceptionally tenderness on pressure and slight redness may exist over them. They are found most frequently on the back of the elbow, the malleoli, and margins of the patella, but occur occasionally on the extensor tendons of the hand and foot, the scapular spine and iliac crest, the temporal ridge and superior occipital curved line, the ear, etc. These nodules occur singly or in clusters, and are often symmetrical; they are very rapidly developed in crops or in succession, and last sometimes for a few hours, more frequently from three or four days to four or five months, or even eighteen to thirty months. The original formations may disappear, and be succeeded by fresh ones; and sometimes, when no longer perceptible by touch, they may be found post-mortem. Their development is unattended by pyrexia, unless pleuritis, pericarditis, or other condition coexist to which the pyrexia might [p. 44]be referred. These nodosities do not appear to suppurate or ossify or become infiltrated with urate of soda, and histologically they resemble organizing granulative tissue. As regards their pathological associations, Drs. Barlow and Warner found evidences of rheumatism in 25 out of 27 cases; a morbid condition of the heart existed in all of them, and chorea in 10 of them. Two of the conclusions formulated by the authors just mentioned are of great importance: that these subcutaneous nodosities "may be considered as in themselves indicative of rheumatism, even in the absence of pain;" that, while unimportant in themselves, they are "of serious import, because in several cases the associated heart disease has been found actively progressive." Dr. Dyce Duckworth has reported two cases in which these nodules occurred in adults, lasted eighteen months in one, and were still present in the other case after thirty months, and were attached to the skin and periosteum. In one of them the nodules were very painful and ached more in cold weather, and the patient had no history of rheumatism or of chorea, although her mother and one sister had.108 In Dr. Stephen Mackenzie's case the woman was the subject of tertiary syphilis, and had no personal history of rheumatism or chorea, and she was free from heart disease; but her family history was not given.109
103 Phlebite Rheumatismale Aigue, Paris, 1869, par M. Lelong. In Revue de Méd., t. i. 492-499, 1881, a case by Dr. Launois.
104 Pathologie Interne, ii. 546, 1871.
105 Die Rheumatische Schwiele, Weimar, 1843.
106 Lib. cit., p. 628.
107 Trans. International Medical Congress, London, vol. iv. pp. 116-128, 1881. In this paper, and in an article by MM. E. Troisier and L. Brock, to be found in Revue de Médecine, t. i. 297-308, 1881, are references to the authors who had written upon it.
108 Brit. Med. Journ., i., 1883, 868.
109 Ibid., i., 1883, 867.
The course and duration of acute polyarticular rheumatism vary very much, and are apparently influenced by several circumstances, such as the severity or the mildness of the articular affection, as well as of the constitutional disturbance; the presence or not of complications; the state of health of the patient about the time of the attack, and, probably, the existence or not of a proclivity to the disease; and whether the disease present the continued or the relapsing type. As a tolerably general rule, when the constitutional symptoms are acute, the skin hot, the perspiration free and very acid, the urine of high density, color, and acidity, and several of the articulations are swollen and very painful—when no serious complication, and especially no severe cardiac affection, exists, and when the patient is endowed with a fair constitution and with organs not damaged by previous disease, the course of the fever is tolerably short and continuous, and the recovery more or less prompt. Amongst the most reliable evidences of approaching recovery in such cases is the tongue becoming clean and losing its red color and the urine increasing considerably in quantity, but containing a large proportion of solid matter, as indicated by a high density.
On the other hand, a large proportion of cases run a more irregular and protracted course, and more or less marked relapses succeed real but temporary improvements, the local disturbance affecting fresh joints or reappearing in those previously attacked, and the general symptoms resuming renewed activity. The duration of the active symptoms in these cases is considerable, seldom under six weeks, and frequently occupying seven, eight, or more. In these protracted cases the symptoms, as a rule, are usually rather milder, the perspiration not as profuse or sour, the urine of less density and acidity, the articulations less hot and painful, than in the previously described group. Sometimes, indeed, the perspiration and the urine are of neutral or even faintly alkaline reaction. It is not only the unexplained tendency to relapse which protracts these [p. 45]cases, but sometimes in addition an established proclivity to the disease—the rheumatic habit—or a condition of previous unsound or frail health.
Such cases occasionally pass into the subacute form, or the mild febrile symptoms gradually and finally decline, and the joints may either remain tender, swollen, and stiff some time longer, or these signs of recent inflammation may soon disappear and leave the articulations merely weak.
Many cases of acute rheumatism embody several of the features of the two groups just described, and no definite course or duration of acute articular rheumatism can be accurately laid down.
The course and duration of acute polyarticular rheumatism have received a good deal of attention of late years. But Dr. Austin Flint110 was one of the first to study the natural history of the disease uninfluenced by active treatment, and he was followed in 1865,111 1866,112 and 1869113 by Sir William Gull and Dr. Sutton, who treated a series of cases without medicine, unless mint-water be so regarded. The mean duration of Flint's 13 cases from the date of attack to convalescence, excluding one in which pericarditis and pneumonia occurred, was a fraction under twenty-six days. It is unfortunate that the number of cases was so small, and that 11 of the patients were females, who appear to be especially subject to the milder and more protracted attacks of the disease. A larger number, with an equal proportion of the sexes, would probably have given a different result.
110 American Journal of Med. Sciences, July, 1863.
111 Ib., vol. xii.
112 Medico-Chirurgical Transactions, vol. lii.
113 Guy's Hospital Reports, 2d Series, vol. xi.
Gull and Sutton have published the natural histories of 62 cases—viz. of 41 in their first series, of 8 more in their second, and of 13 more in their third. The average duration of the acute symptoms was, in the first series, 8.5 days, in the second, 9 days, and in the third, 10 days, giving an average of 9.1 days for the duration, after admission to hospital, of the acute symptoms of acute polyarticular rheumatism when there is no very severe cardiac disease. In their third paper, based upon 13 new cases and 12 of those published in their two previous communications, they conclude "that rheumatic fever uncomplicated with any very severe heart affection tends to run its course in nineteen days, calculating from the time the rheumatic symptoms first set in to their termination."114 Yet an analysis of the 23 of the 41 cases contained in their first series115 respecting which the duration of the rheumatic symptoms before admission and from admission to complete convalescence is given, shows that the period occupied from the setting in of the rheumatic symptoms to convalescence was in the 13 male subjects 25.8 days, and in the 10 female 42 days, or, including both sexes, the average duration was 32.8 days—i.e. 6.8 days longer than Flint's result.
114 Med.-Chir. Trans., lii. 82.
115 Guy's Hospital Reports, xi. 435.
As Gull and Sutton had especially pointed out the class that tends to assume acute characters and recover more quickly than any other, and the class that runs a protracted course and tends to relapse, it is somewhat remarkable that they did not tabulate the cases belonging to those classes separately, and show distinctly their differences in duration and [p. 46]modes of convalescence. This has been attempted by Southey,116 but, unfortunately, his conclusions, as will hereafter appear, have not been confirmed by other observers.
116 St. Bartholomew's Hospital Reports, xiv., and ib., xv.
Finally, in this connection, after carefully weighing ten subjects of acute articular rheumatism during their illness and until they had regained their usual weight, A. Roussel117 found that the time during convalescence occupied in regaining the weight previously lost was inversely proportional to the duration of the attack.
117 Essai sur la Convalescence du Rheumatisme Artic. Aigue, Paris, 1881, 66.
Under this head Charcot, Besnier, and Homolle describe an affection which corresponds closely with one variety of the disease commonly called rheumatoid arthritis, but the writer employs the term with the same significance as most modern English authors (Garrod, Sutton, Flint, Maclagan). It is milder yet more enduring than the acute form, but their symptoms are identical in kind. It is usually subacute from the outset, although occasionally succeeding the acute type. The febrile disturbance is but slight, rarely reaching 101°, and the perspiration is less abundant; there is less pain, heat, and tenderness in the joints, and only a few of them are involved together; but although the articular affection moves from joint to joint, it persists for weeks or months in several of them or in one only, improving and relapsing generally without apparent reason. However, it does not seriously damage the articulations, and they ultimately quite recover. Mild cardiac affections also occur, but less frequently, and the serious disturbances of the cerebral and respiratory systems are very seldom met with. The gradations between subacute articular rheumatism and the acute form on the one hand, and the simple chronic form on the other, are almost innumerable. Marked anæmia is as much a feature of subacute as of acute articular rheumatism, and its victims are often of unhealthy or asthenic constitution, and subject to recurring attacks of the disease on but slight provocation. The return of warm weather often relieves such cases.
THE MORBID ANATOMY OF ACUTE AND SUBACUTE ARTICULAR RHEUMATISM.—Although opportunities of ascertaining the conditions of the articulations in acute articular rheumatism are rare, yet it is now established that the process is an inflammation involving chiefly the synovial membrane, and to a less degree the cartilages, ligaments, tendinous sheaths, and in some cases even the bones and periarticular soft parts. The synovial membrane is more or less injected and reddened diffusely or in patches, especially where it forms fringe-like folds and at its line of union with the cartilage. It is somewhat thickened, opaque, and devoid of its satin-like lustre, and in somewhat protracted cases covered here and there with a thin, easily detached neo-membranous formation. Within the articulations will be found from a few drops to one or two ounces of a viscid, pale, citron- or reddish-colored fluid, like synovia, but more fluid, and generally turbid and containing transparent or semi-opaque gelatinous masses or albumino-fibrinous flocculi. The [p. 47]microscope reveals in the effusion large detached spherical epithelial cells in various stages of germination or of fatty degeneration, and a variable number of red blood-corpuscles and pus-cells. Very exceptionally, the effusion is mixed with more or less true pus. In two out of the eight fatal cases reported by Fuller, in which the joints were examined, pus in moderate quantity was found along with other products in some, but not in all, of the inflamed articulations, and one of them was complicated with erysipelas, the other with sloughs over both trochanters. In very severe forms complicated with hemorrhagic tendencies the inflammatory products have contained a large proportion of blood. Cornil et Ranvier118 insist that even in slight cases of rheumatic arthritis the diarthrodial cartilage constantly suffers changes arising from nutritive irritation and proliferation of the cartilage-cells. At first the cartilage loses here and there some of its polished hyaline appearance, and the microscope reveals a finely-striated condition of its structure which gives it a velvety aspect. When the inflammation has been more severe and of longer duration, so that the deeper layers have been involved, the unaided eye will perceive local swellings in which the natural elasticity and resistance of the cartilage are impaired, and its surface is fissured or villous-like in appearance. "In certain rare cases of mono-articular acute arthritis true ulcerations of the cartilage are observed."
118 Manual d'Histologie pathologique, Paris, 1869, 406.
The soft parts in the immediate vicinity of the inflamed joints may be in some cases more or less congested and oedematous, and the tendinous sheaths, and even the bursæ mucosæ, inflamed and distended with inflammatory products like those in the articulations. Charcot,119 holding the opinion that arthritis deformans is but a chronic variety of articular rheumatism, quotes Gurlt's statement that in acute articular rheumatism "the medullary tissue of the ends of the bones undergoes a great increase of vascularity, with proliferation of its corpuscles," and remarks that Hasse and Kussmaul have also referred to lesions of the bone and periosteum in that disease. But the condition of the osseous parts of the joints in acute articular rheumatism can hardly be said to be known, and it is premature to speak positively respecting it.
119 Clinical Lectures on Acute and Chronic Diseases, Sydenham Soc., 1881, p. 148.
Finally, in subacute rheumatism the alterations in the synovial membrane, and especially in the cartilages just described, are likely to be more marked than in the acute form.
The DIAGNOSIS of acute polyarticular rheumatism is seldom difficult in adults, but when acute rheumatism localizes itself in one joint or occurs in infancy or early childhood, a diagnosis, especially an early one, sometimes cannot be easily established. The considerations by which acute polyarticular rheumatism may be distinguished from acute gout, subacute rheumatoid arthritis, and gonorrhoeal rheumatism will be given in connection with those topics.
Pyæmia has perhaps been confounded with acute articular rheumatism more than any other disease, but the rheumatic affection, unlike the pyæmic, is not necessarily connected with any pre-existing condition capable of causing purulent infection of the blood or system, such as a wound, fracture, abscess, or a local inflammation of bone, periosteum, vein, pelvic organ, or a specific fever (variola, relapsing, typhoid, [p. 48]glanders, etc.); it does not present severe rigors, which recur at irregular intervals and are attended with teeth-chattering and a high temperature, 104° to 105°, rapidly attained; its type of fever is not so intermittent or markedly remittent as that of pyæmia; its profuse sweating continues although the temperature remains febrile, but that of pyæmia coincides with the decline of the temperature; unlike pyæmia, it only very rarely produces profound constitutional disturbance of a typhoid character, and has no tendency to run a rapidly fatal course in eight to ten days or in two or three weeks; its visceral inflammations are chiefly cardiac, pleural, and pulmonary, and tend to resolve; those of pyæmia are especially pulmonary, pleural, and hepatic, although frequently cardiac also, and generally produce suppuration and destruction of tissue. Multiple subcutaneous abscesses and cutaneous blebs and pustules do not occur in acute articular rheumatism, and its articular affection differs in many respects from that of pyæmia; many more joints are involved; the inflammation is erratic, very rarely fixed, and generally resolves without damage to the articulation; the affected joint is usually hotter, redder, more painful, and more sensitive, and the swelling is less diffused, and its outline corresponds more accurately with that of the synovial capsule. Sometimes acute articular rheumatism is complicated with the phenomena of pyæmia, as when so-called ulcerative endocarditis obtains.
The acute inflammations which are occasionally observed in one or several articulations of newly-born infants are generally pyæmic. It is only in the early stage of acute glanders that the severe muscular and articular pains sometimes present in that very rare disease in man might lead to its being confounded with acute articular rheumatism; but the patient's occupation and history, the early and severe prostration, the absence, as a rule, of redness and swelling around the painful articulations, and, in some instances, the early appearance of pustules and blebs on the skin and of abscesses in the deeper tissues, will suggest the real nature of the case.
Acute periostitis frequently occurs in children in close proximity either to one joint, or less frequently to more than one, and may readily be confounded with acute articular rheumatism. But the constitutional disturbance in acute periostitis is prompt and severe at the outset; the swelling increases rapidly, is firmer than that of arthritis, does not involve the joint proper and its capsule, but, like the tenderness on pressure, exists above or below the articulations, especially around the head of the bone; there are no visceral complications, provided pyæmia has not supervened; the constitutional symptoms early assume a typhoid character, and unless an early incision be made a fatal issue soon ensues.
The enlarged ends of the long bones and the pains in the limbs of rickets might lead to a suspicion of acute articular rheumatism, but the early age of such children, the absence of pain and swelling in the joints, the beaded condition of the sternal ends of the ribs, the late dentition and locomotion, the peculiarly shaped head, and other evidences of that affection, would prevent a careful observer from making a mistake. Inherited syphilis in infants, like rickets, may produce fusiform swelling and thickening at the ends of the long bones, especially the humerus and femur, and sometimes pain in the joints on movement; but at first the swelling [p. 49]is confined to the epiphyseal line, and only later extends to the joint; there is a pseudo-paralysis of the limb, and but little pain or fever; bony osteophytes may often be felt under the skin at the line of union of the epiphysis with the shaft; the epiphysis often becomes separated from the shaft, and suppuration may ensue around the bone and in the articulation; sometimes adhesions and perforation of the integument take place, allowing of the escape of disintegrating osseous and cartilaginous tissue; and there will coexist either on the skin or mucous membrane some of the ordinary evidences of inherited syphilis.120 The acute and subacute articular inflammations occasionally observed in cerebral softening and hemorrhage, in injuries and inflammation of the spinal cord and caries of the vertebræ, may be distinguished from acute and subacute articular rheumatism by the following circumstances: the existence of some one of these diseases of the brain or cord, the articular affection being usually confined to the paralyzed limbs; its invasion about the time of the setting in of the late rigidity, or even still later; the absence of cardiac complications and the presence of other trophic or neuro-paralytic lesions, such as acute sloughings, rapid atrophy of the palsied muscles, cystitis, ammoniacal urine, etc.121
120 Vide Parrot, Archives de Physiol. Norm. et Path., 1872 and 1876; R. W. Taylor, Bone Syphilis in Children, New York, 1875.
121 See J. K. Mitchell, Am. Jour. Med. Science, vol. viii., 1831, and ib., 1833; Scott Alison, Lancet, i., 1846, 276; Brown-Séquard, Lancet, i., 1861; Gull, Guy's Hosp. Repts., 1858; Charcot, Archives de Physiologie, t. i. p. 396, 1868, and many others.
Acute articular rheumatism in children presents peculiarities. It often affects but one joint, and has little tendency to become general; the joints of the lower extremity, ankle, and knee are most obnoxious; the local signs of inflammation, redness, swelling, and pain, are feebly developed, and the child may walk as if nothing were wrong; the disease is usually subacute; the temperature rarely very high; the perspiration not profuse; the urine not scanty, and not often loaded with lithic acid. Cardiac and the other internal complications, except the cerebral, are more frequent than in adults; endocarditis is especially frequent, pericarditis and pleuritis not rare. It is almost exclusively in childhood that acute articular rheumatism becomes associated with or followed by chorea, and yet the delirium, coma, and convulsions frequently observed during rheumatic fever in the adult are very rarely seen in the child. Muscular rheumatism, however, in the form of torticollis, frequently coexists, and so do erythema nodosum and the subcutaneous fibrous nodules previously described.
It is very rarely indeed that acute rheumatism invades a single joint to the exclusion of the rest; and it is perhaps impossible to be certain that such an arthritis is rheumatic unless some of the other symptoms or complications of articular rheumatism supervene, or unless it have succeeded a polyarticular rheumatism, which it very rarely does. Mono-articular rheumatism is very generally of the subacute type, and unattended with fever from the outset, or only a moderate pyrexia obtains for a few days; there is generally considerable effusion into the joint, with [p. 50]swelling, pain, and moderate local heat; visceral complications very rarely arise, but the local inflammation persists most obstinately for six or eight weeks or three or four months, and often leaves the joint tender, stiffs, and weak for a long time or even permanently. In both the acute and subacute forms, before concluding that the uni-arthritis is rheumatic, we must exclude the probability of its being traumatic, strumous, syphilitic, gonorrhoeal, neurotic, or, above all, of the nature of rheumatoid arthritis, which many such cases really are.
PROGNOSIS.—The disease is rarely directly fatal during the attack, yet as the frequency of the complications varies unaccountably from time to time, so the mortality may be exceptionally large or small for even prolonged periods. It may be said that the average mortality ranges between 1.16 and 4 per cent. in the experience of modern authors. The average mortality in the Paris hospitals for four years (1868-69, 1872-73) Besnier fixes at 1.65 per cent.;122 in St. Bartholomew's, London, Southey found it for fifteen years (1861-75) to be 1.16 per cent.;123 Pye-Smith fixes the rate at 4 per cent. in 400 cases treated in Guy's;124 W. Carter gives 2.5 per cent. as the rate during ten years at the Southern and Royal Southern Hospitals of Liverpool.125 The death-rate appears to vary remarkably with age, as Southey's figures show:126 under ten years, 3.40 per cent.; between ten and fifteen, 1.5 per cent.; between fifteen and twenty-five, 1.4 per cent.; between twenty-five and thirty-five, 0.9 per cent.; between thirty-five and forty-five, 0.8 per cent., the mortality declining very greatly after the tenth, after the twenty-fifth, and after the forty-fifth year of life.
122 Dictionnaire Encyclopédique, Troisième serie, t. iv., p. 463.
123 Barth. Hospital Reports, vol. xiv., p. 4.
124 Guy's Hospital Reports, xix. p. 327.
125 The Liverpool Medico-Chirurgical Journal, July, 1881, p. 88.
126 Lib. cit., p. 4.
The danger of the case is usually proportionate to the youth of the patient, the degree of the pyrexia, the number of the joints involved, and the number and the character of the complications, the habits, and previous health of the patient. A fatal issue is most frequently observed in connection with hyperpyrexia alone, or in combination with delirium or coma. A rapid rise of temperature and a temperature over 105°, especially if cerebral disturbance coexist, indicate danger; and so does arrested perspiration while the temperature is high. In a much smaller number of cases death is due to some other complication, especially to purulent pericarditis or to that combined with pleuritis or pneumonia; in not a few cases the prior existence of chronic valvular disease, with fibroid induration of liver and kidneys, renders a fresh rheumatic endo- or pericarditis, occurring as part of acute articular rheumatism, fatal. There is good if not conclusive evidence that rather sudden death in acute articular rheumatism is occasionally due either to diffuse myocarditis or to fatty degeneration of the muscle of the heart. In Greenhow's 2 deaths out of 50 cases treated by sodium salicylate the pericardium was universally adherent and the heart's fibre fatty in one and pale and flabby in the other. Sudden death in this disease is very rarely due to embolism of the pulmonary artery or of the cerebral vessels, while ulcerative endocarditis is very exceptionally one of the sources of a fatal issue.127 But although acute articular rheumatism rarely kills [p. 51] directly, it frequently lays the foundation of subsequent ill-health, and ultimately proves fatal through organic disease of the heart and its many consequences. However, it is an interesting circumstance that while acute rheumatic inflammation is prone to damage the heart permanently, it very rarely, quite exceptionally, impairs the structure or functions of the articulations. It is almost solely the subacute form that now and then becomes chronic or renders a joint for a long time painful, swollen, and crippled in its movements. Whether acute rheumatism, however intense per se, ever ends in destructive suppuration and ulceration of a joint is doubted by some authorities, notwithstanding the cases published by Fuller and others. No doubt some of the cases were really pyæmic, or perhaps gonorrhoeal; and it must be borne in mind that acute articular rheumatism occasionally develops pyæmia, and then an arthritis might be considered rheumatic when truly pyæmic. The question of acute rheumatic arthritis exciting a chronic rheumatoid affection will arise hereafter.
127 See an article on the mortality among rheumatic risks by A. Huntingdon, M.D., in N.Y. Medical Record, 1875, p. 195.
TREATMENT.—Owing to our imperfect knowledge of the real nature of acute articular rheumatism, its treatment is still largely either empirical or intended to combat certain prominent symptoms or complications of the disease. Of the various methods of treatment which have been employed space will not permit a description; even of those advocated by authorities of the present hour only very few will be considered.
The method which is now unquestionably the favorite one in both Europe and America, and which in its power of promptly relieving the articular and muscular pains and reducing the fever of acute rheumatic polyarthritis may without exaggeration be compared to that exercised by quinia over the paroxysms of ague, is that in which salicylic acid or salicylate of sodium is given in repeated and full doses. It was in July, 1875,128 that Buss first asserted that salicylic acid was a specific for rheumatism, and in March, 1876,129 Maclagan, after having employed salicine from 1874, published his experience of it as a valuable remedy in the treatment of acute rheumatism, its beneficial action being "generally apparent within twenty-four, always within forty-eight, hours of its administration in sufficient dose." Perhaps a sufficient time has now elapsed to permit of a just opinion of the power of these new remedies, the salicyl compounds, over acute articular rheumatism. The facts presented at the discussion recently held at the Medical Society of London130 are sufficiently numerous and authoritative to justify, at least provisionally, some definite conclusions as to the remedial relations of the salicylates to acute articular rheumatism.
128 "Die Antepyr. Wirkung der Salycylsäure," Centralbl. f. d. Medic. Wissenschr., 1875, 276.
129 The Lancet, March 4 and 11, 1876.
130 The Lancet, Dec. 17, 24, 31, 1881; Jan. 7, 14, 28, 1882.
1. The articular pain and the fever of acute rheumatic polyarthritis are more or less speedily removed by the salicyl remedies (salicylic acid, sodium salicylate, and salicine); the pains very frequently persist after the temperature has become normal. Both symptoms were removed by five days' use of such agents in 50 per cent., and by eleven days' use in 80 per cent., of 355 cases treated at Guy's Hospital, and tabulated by Fagge,131 and by five days' use in 60 per cent., and by eleven days' use [p. 52]in 66 per cent., of the 60 severe cases treated and severely criticised by Greenhow.132
131 Ibid., ii., 1881, 1031.
132 Clinical Society's Transactions, vol. xiii., 1880. See Dr. Fagge's table iv., Lancet, ii., 1881, 1032.
Again, in 190 cases of acute and subacute rheumatism the average duration, under salicyl remedies, of pyrexia was 5.5 days and of joint disease, 5.3 days (Warner133); in 156 cases at St. George's Hospital the average duration of pyrexia was 3.66 days, of pain 4 days (Owen134); in 82 at the Middlesex the average duration of pyrexia was 5 days, of pain 5.6 days (Coupland135); and in 55 at the Westminster the average duration of pyrexia was 7 days, of pain 7.25 days136—that is, a general average duration in the whole series for the pain and pyrexia of 5.4 days.
133 Ibid., p. 1080.
134 Ibid., p. 1081.
135 Ibid., i., 1882, 10.
136 Ibid., ii., 1881, p. 1080.
Further, 36 per cent. of Fagge's cases and 58 per cent. of Greenhow's were relieved of both the above symptoms on the fourth day; 24.8 per cent. of Fagge's and 50 per cent. of Greenhow's on the third day; and 13.5 per cent. of Fagge's and 26.6 per cent. of Greenhow's on the second day. In Clouston's 27 cases, treated in private, 66.6 per cent. were free from pain and 59 per cent. from fever within three days, and 85.2 per cent. were devoid of pain and 72.7 per cent. of fever within four days.137 Finally, all who have had much experience of this method of treating acute rheumatism will agree that the first or second dose frequently relieves the articular pains like a charm, and the local swelling then frequently subsides in from sixteen to forty-eight hours.
137 The Practitioner, i., 1882.
2. Relapses are more frequent—probably considerably more frequent—under treatment by salicylates than under other methods. Thus, the average of relapses in eight different tables of cases treated by the salicyl remedies ranged from 16.6 per cent. to 35 per cent., giving a general average of 26 per cent.;138 while under other methods in three different tables the average ranged from 5.4 per cent. to 27.6 (this last under the full alkaline), giving a general average of 16 per cent.139 Relapses appeared to recur less frequently in those cases which yielded to the salicylates within five days than in those which took from six to eleven days to yield, in the ratio, according to Fagge's figures, of 26.6 per cent. for the first, and 29.4 per cent. for the second day; and, according to Hood's, as 18.4 per cent. to 24.4 per cent. There does not appear to be any regularity in the order of occurrence or recurrence of relapses, nor is Southey's definite statement that in "relapsing cases the temperature is nearly or quite normal on the eighth evening, and a slight relapse occurs on the thirteenth morning," borne out by the statistics produced at the London Medical Society. Moreover, W. Carter's cases140 have not confirmed Southey's precise statement respecting the gradual remission of the temperature on the eighth and ninth days of illness in the continued or non-relapsing, uncomplicated forms. Irregularity and inconstancy are the typical features of articular rheumatism. The relapses under the treatment by the salicylates have been referred to the premature disuse of those remedies, but they do occur notwithstanding [p. 53]the continued employment of them. It is a general opinion that exposure to cold, errors in diet, and an early return to work are frequent causes of relapse; and Broadbent refers the increased liability to relapse under the salicyl compounds to the rapidity with which those remedies relieve the acute symptoms of articular rheumatism, in consequence of which sufficient care is not observed either by the patients or their nurses, and they are exposed to some of the above exciting causes of relapse. All the above causes do probably play their part so long as the materies morbi (if that really exist either as a chemical principle or as a germ) has not been wholly eliminated or destroyed. Indeed, the short intervals which frequently obtain between the primary invasion of the so-called relapses, and the failure of the salicyl compounds to prevent peri- and endocarditis, render it probable that what are commonly spoken of as relapses are not due to a new infection, as in the case of the relapse of typhoid fever, but to the recrudescences of a disease not yet terminated, but over some of the manifestations of which—the articular inflammation and the pyrexia—the salicylates exercise some control.
138 Fagge's, 26.2 per cent.; Greenhow's, 35; Warner's, 33.6; Owen's, 30.2; Hood's, 18.8; Coupland's, 35.3; Broadbent's, 16.6; Powell's, 18.7; total, 214 ÷ 8 = 26 per cent.
139 Hood's, 5.4; Warner's, 14.9; Owen's, 27.6; total, 47.9 ÷ 3 = 16 per cent.
140 The Liverpool Med.-Chirurgical Journal, July, 1881, p. 101.
3. Authorities are generally agreed that the salicyl compounds do not arrest or control rheumatic inflammation of the endo- or pericardium or pleura, or subdue the pyrexia, if these complications in well-marked degree exist; and there is strong evidence to show that they do not at all constantly prevent the disease from involving those organs, even after the articular affection has subsided under their use. Inestimable as is the benefit conferred by these remedies in promptly relieving the articular pain and fever, they do not secure the great desideratum in the treatment of acute articular rheumatism—protection of the heart.
In 352 cases treated with salicylate of soda at the Westminster Hospital, heart disease developed in 13.6 per cent.; in 267 treated without the salicylate, heart disease developed in 14.2 per cent. (Warner's cases).141 In 350 cases treated with salicylates at Guy's, heart complications obtained in 68 per cent., while in 850 treated without them, the cardiac complications occurred in 58.8 per cent. (Hood).142 Gilbart-Smith collected a large number of cases from several of the London hospitals, and analyzed them with the following results: Of 1727 cases of acute rheumatism treated before the introduction of the salicyl compounds, the proportion of cardiac complications was 54.4 per cent.; in 1748 cases treated subsequently to their introduction, the cardiac affections obtained in 63.4 per cent.; and in 533 cases treated by the salicyl compounds, those affections obtained in 68.4 per cent.143
141 The Lancet, ii., 1881, 1080.
142 Ibid., ii., 1881, 1120.
143 Ibid., i., 1882, 136.
These facts certainly seem to prove that the salicyl compounds do not prevent the occurrence of the visceral complications or manifestations of acute articular rheumatism; and if space permitted instances might be quoted from many authors in which either endo- or pericarditis or pleuritis or pneumonia or other visceral manifestation had set in after the patient had been taking the salicylates long enough to have produced their usual physiological effects; some of these will be mentioned under the next section.
It may be objected that in the above estimates sufficient attention has not been paid to the period of the disease at which the treatment by the [p. 54]salicylates was begun, the time it was continued, the doses given, the age of the patient, the severity and other characters of the illness, such as whether acute or subacute, first or second attack, complicated or not.
4. It must be admitted that there are a few facts which render it very probable that the salicyl compounds do really reduce the frequency of these complications, and thus give some protection to the heart in rheumatism. Of Powell's 32 cases, 19 = 60 per cent. had heart disease when admitted; and of the remaining 13, 6 = 46 per cent. developed cardiac disease after admission and while under the salicylates.144 Of Dr. Jacobi's145 150 cases, 78 = 52 per cent. were admitted with unsound hearts, and of the other 72, only 5 = 6.9 per cent. developed cardiac disease after beginning salicylate treatment. Of Southey's 51 cases, 24 = 47 per cent. were admitted with diseased hearts; and of the remaining 27, only 4 = 14.8 per cent. developed a cardiac affection subsequent to beginning treatment by the salicylates.146 Of the Boston Hospital cases, 38 per cent. were affected with heart disease at entrance, and only 4.76 per cent. afterward. No heart affection was developed in any of Clouston's 27 private cases—a result he attributes to the early period at which the remedies are given in private practice. But the number is too small to permit of any conclusion being drawn, and 4 of the cases were examples of recurrence of the disease at short intervals (three and four weeks) in the same patient, in whom there appears to have existed no proclivity to cardiac complication, for he had had four attacks before he came under Clouston's care. Moreover, his cases were mild, but 16 of them being acute, and of these only 3 attaining a temperature of 103° and upward. Finally, Herman147 estimates the percentage of heart affections that developed after beginning the salicylates in the London Hospital at 18.7 per cent., and after other treatment at 30 per cent. Omitting Clouston's, the general average of the above results is, that in 49.2 per cent. cardiac disease existed before the patients began the salicyl treatment, and that in 18.2 per cent. it developed after that, while 30 per cent. of cardiac disease developed after other methods of treatment were begun.
144 Lancet, i., 1882, 134.
145 St. Thomas's Hospital Reports, New Series, viii. 252.
146 St. Bartholomew's Hospital Reports, xvi. 10.
147 Quoted by T. G. Smith, Lancet, i., 1882, 137.
The subject is one beset with difficulties, and still needs investigation. It is reasonable to infer that as the salicylates promptly arrest the articular inflammation and allay the fever of uncomplicated acute rheumarthritis, they will prevent the visceral inflammations so apt to develop when the disease runs its course uninfluenced by treatment; but experience has shown that they do not control or arrest rheumatic inflammation of the heart or pleura or the attending pyrexia, although capable of subduing the articular inflammation and the pyrexia that accompanies it. The most eminent therapeutists are divided on the subject. Maclagan, while admitting that the salicyl compounds do not ward off cardiac complications, or cure them when they exist, maintains that their existence is an additional reason for giving those remedies freely and in large doses.148 Broadbent,149 while believing in the protective influence of the salicylates "when brought to bear upon the fever in the first days of its existence," finds in the presence of any cardiac inflammation a reason for at once discontinuing those remedies. Flint150 believes that rheumatic endo- and [p. 55]pericarditis are more common since the introduction of the salicyl treatment than when the alkaline method was relied upon almost entirely, and advises151 the administration of alkalies with the salicylates to protect the heart. Vulpian152 thinks the protective power in question probable, but not established; while the latest French authority, Homolle, is of opinion that "cardiac affections are really less frequent in patients treated by salicylate of sodium than in others."153
148 Lib. cit., pp. 266, 275.
149 Lancet, i., 1882, 138.
150 New York Med. Record, 1882, 66.
151 Pract. Med., 5th ed., 1098.
152 Du Mode d'Action du Salicylate du Soude dans le Traitement du Rheum. Artic. Aigue, Paris, 1881, 11.
153 Nouveau Dict. de Méd. et de Chir., xxxi., 1882, 648.
5. The occurrence of hyperpyrexia is not always prevented by the salicyl remedies, even when they have produced their full physiological effects. Fagge endeavors to explain away the two cases of hyperpyrexia which occurred under Greenhow and the other two which happened amongst the cases tabulated by himself, and remarks that if the temperature should begin to fall under the use of salicylic acid, and then should change its course and rapidly attain a dangerous height, that would really show that the drug is sometimes incapable of preventing the occurrence of hyperpyrexia. This actually happened in one of Powell's two cases,154 and the patient died suddenly at a temperature of 107°. In Greenhow's first case the patient had been taking the salicylate for four days, and was deaf and delirious when the temperature became 105.8°.155 Finney reports a case in which drachm iss of salicine were given daily for two days, and drachm ij on the third day, when pericarditis set in, and on the fourth day hyperpyrexia supervened.156 Haviland Hall records an instance in which the temperature fell from 103.5° to 100.6° after twenty-grain doses of salicylate soda, every three hours, taken for two days; on the third day the medicine was given every four hours; the temperature rose in the evening to 103.4°, and on the next day it rose rapidly to 108.7°, and the patient became delirious. Patient recovered rapidly after two baths.157
154 Lancet, i., 1882, 135.
155 Clin. Soc. Trans., xiii. 264.
156 Brit. Med. Journ., ii., 1881, 932.
157 Lancet, ii., 1881, 1082. See also two cases in Med. Times and Gaz., ii., 1876, 383.
Pericarditis is not always present when hyperpyrexia arises during the administration of salicylic acid; it was absent in Powell's cases, is not mentioned in Hall's, and did not ensue in one of Greenhow's until two days after the temperature had reached 105.4° F. However, either pericarditis or pneumonia is very frequently present when the temperature is excessive. It is generally admitted that the salicylates do not control rheumatic hyperpyrexia once it exists.
6. Notwithstanding the prompt removal of the pain and reduction of the fever by the salicyl compounds, the average duration of acute articular rheumatism is not very considerably lessened by those remedies. Thus, of Hood's158 350 cases treated by salicylates the average duration of the illness was 35.95 days as against 38.75 under other methods. The average time spent in bed by Warner's 342 cases was 19.5 days under the salicylates, and by 352 patients under other remedies 23.5 days. Both estimates show a curtailment of the duration of the disease by the new treatment of three to four days only; which is not a very material improvement.
158 Calculation from Dr. Hood's Tables 1 and 1a, Lancet, ii., 1881, 1119.
[p. 56]7. Nor do the salicylates materially alter the time spent in hospital by rheumatic patients; some evidence indicates that they actually prolong that period. The following are the average residences in hospital under the salicylates, according to several recent authors, and they are remarkably uniform with two exceptions: Coupland, 36 days; Warner, 34.9; Hall, 34; Southey, 32.5; Broadbent, 31.2; Powell, 31; Finlay and Lucas, 29.7;159 Owen, 23; Brown, 21.9;160 or a general average of 30.4 days for the salicyl remedies. Under full alkaline treatment: Owen, 26 days; Dickinson, 25;161 Fuller, 22.2;162 Blakes, 24;163 or a general average of 24.3 days for full alkaline treatment. And if to these we add Finlay and Lucas's results, 27.7 days, under but two to three drachms of alkaline salts in the twenty-four hours—a quantity only the fourth of that given under the full alkaline method—the general average residence in hospital under alkaline treatment was but 25.4 days; that is, five less than under the salicylate.
159 Lancet, ii., 1879, 420.
160 Boston Med. and Surg. Journ., Feb., 1877. The four cases excluded by the reports are included in this calculation, that it may more fairly be compared with other reports.
161 Lancet, i., 1869.
162 The Practitioner, i., 1869, p. 137.
163 Boston City Hospital Reports, 1st Series.
These several estimates of the time spent in hospital under the salicylates, with the exception of Owen's and Brown's, correspond closely with that of the time spent by Gull's and Sutton's patients under mint-water—32.8 days—although the general average of them falls short of the latter by 2.4 days.
The following table (iii.) of Hood's164 shows that under the salicylate method 45.7 per cent. remained in hospital beyond forty days, and 39 per cent. under other methods, and that about 50 per cent. more were discharged within twenty days under the other methods than under the salicylate:
350 cases treated with salicylates: | ||||
Days. | ||||
Under 10. | Under 20. | Under 30. | Under 40. | Ill longer. |
3 = 0.84%. | 31 = 8.88%. | 76 = 21.7%. | 84 = 24%. | 160 = 45.7%. |
850 without salicylates: | ||||
Days. | ||||
Under 10. | Under 20. | Under 30. | Under 40. | Ill longer. |
12 = 1.4%. | 105 = 12.35%. | 175 = 20.1%. | 182 = 21.4%. | 331 = 39%. |
164 The Lancet, ii., 1881, 1120.
These statistics favor Greenhow's opinion that patients treated with salicylate of sodium regain their strength slowly, and are long in becoming able to resume their ordinary occupations. Some allowance, however, must be made for the precautions against relapse under salicylates observed in hospitals since the great tendency thereto has been recognized.
8. Certain unpleasant or toxic effects are produced by salicylic acid and salicylate of sodium; such are nausea, vomiting, abdominal pain, frontal headache, tinnitus, incomplete deafness, vertigo, tremor, quickened respiration, very rarely amblyopia and even temporary amaurosis, and not unfrequently delirium. A feeling of prostration and general misery is not uncommon. These phenomena of salicylism are in great measure proportionate to the dose employed, but they have followed moderate [p. 57]doses, owing sometimes to idiosyncrasy, and perhaps frequently to retarded elimination consequent upon previous disease of the kidneys or disturbance of their function by the salicylic acid or its salt. Those agents are usually completely excreted in forty-eight hours, but in one of Powell's165 cases elimination was not completed before the fifth day, and not before the eighth in Byanow's case.166 Possibly uræmia may in some cases cause the delirium.167 The delirium, which may be violent or not, is often preceded by dryness of the tongue, restlessness, and rapid breathing. Impurities in the acid may account for the inconstancy with which delirium has been noticed by different observers. While but 2 instances in 82 cases were met with by Coupland, 3 out of 90 cases by Broadbent, and 3 out of 109 by Brown,168 Charles Barrows169 encountered 8 instances in 28 cases. In one of these a boy of eleven became delirious in eighteen hours, having taken 10 grs. of salicylate of sodium every three hours. In another instance the drug had been in full use for five days before the delirium manifested itself. These phenomena of salicylism rapidly disappear when the medicine is stopped, and delirium has not always recurred on its resumption. They are less frequent in children, in whom elimination by the kidneys takes place very rapidly and a marked tolerance of salicyl compounds exists. Occasionally more serious effects appear to be produced by the salicylates, owing to their direct action on the heart, impairing its power, as evidenced by feeble impulse and sounds, increased frequency of the pulse, and diminution of the arterial pressure.170 But, notwithstanding the very large number of cases of acute rheumatism that have been treated by the salicyl compounds, very few clear instances of their toxic action on the heart have been recorded, and even in some of these there were other conditions present that may have played some part, perhaps a chief part, in the production of cardiac failure. In Greenhow's case171 the autopsy revealed a dilated fatty heart and slightly granular kidneys, and the cardiac failure coincided with a fall of temperature to 97° F. Goodhardt's172 patient died in nine hours after beginning the salicylic acid, of which she took but one drachm, in divided doses, every three hours. The pulse rose rapidly to 160; she was restless and moaning, but died quietly and suddenly. Recent pericarditis, with one or two points of fatty degeneration of the heart's substance, and sound kidneys were found. The reporter of the case inclines to the opinion that the acid produced sudden collapse and cardiac failure, while Bristowe referred them to the rheumatic poison itself. I have not been able to refer to Hoppe Seyler's paper,173 in which he relates that having given 5 grammes of salicylic acid to a child of seven and a half years affected with articular rheumatism, shortly afterward there occurred deafness, agitation, profuse sweating, dyspnoea, and finally fatal collapse. The condition of the heart and kidneys before and after death is not given. Weber [p. 58]published174 an instance in which 15-gr. doses of salicin given to a woman of twenty-seven produced in thirty-four hours a rapid fall of temperature from 103° to 96° F., accompanied by delirium and serious but not fatal collapse. It is well to remember that a similar failure of cardiac power is occasionally observed in other fevers when rapid defervescence occurs, although the salicyl compounds have not been taken; and it is certainly necessary to give these remedies cautiously, and often to administer alcohol with them, when the heart's action is at all enfeebled by protracted pyrexia and pain, or by disease (inflammatory or degenerative) of its substance or envelope. Indeed, if severe cardiac inflammation obtain in rheumatism, the remedies are powerless and perhaps unsafe. The sudden reduction of the temperature when much exhaustion obtains, even in the hyperpyrexia of rheumatic and other fevers, whether by salicylic acid or quinia or the cold bath, may be attended with fatal collapse of the heart.
165 Lancet, i., 1882, 135.
166 Quoted by Wood in his Therapeutics and Mat. Med., 1880, from Centralb. für Chir., 1877, 809.
167 See DaCosta's observations in Am. Med. Journal, vol. lxix., and Ackland's in B. Med. Journal, i., 1881, 337.
168 Boston Med. and Surg. Journal.
169 N.Y. Med. Record, April 29, 1882, 456.
170 Köhler, Centralb. f. Med. Wissensch., 1876, and Dunowsky, Arbeiter Pharm. Labor., Moskau, i. p. 190, quoted by H. C. Wood, Therapeutics, Mat. Med., etc., 3d ed., p. 639.
171 Clin. Soc. Trans., xiii. p. 266, c. iii.
172 Ibid., p. 123.
173 Quoted by D. Seille, Thèse, De la Méd. Salicylée dans le Rheumatism, Paris, 1879, p. 54.
174 Clin. Soc. Trans., x. p. 70, 1877.
Instead of the frequent weak pulse above mentioned, I have many times found salicylate of sodium render the pulse very slow, labored, and compressible in typhoid fever, and generally at the same time the temperature has been considerably reduced below what it had been.
A temporary albuminuria is not infrequent; excluding mere traces, it obtained in 52 per cent. of cases treated by the salicylates alone or in conjunction with full doses of alkali, and in but 25 per cent. of those in which full doses of alkali, with or without quinia, were employed.175
175 Isambard Owen, Lancet, ii., 1881, p. 1081.
Very rarely hæmaturia and even nephritis have occurred. The active principle is chiefly eliminated by the kidneys, which may account for a local irritating influence upon those organs.
Salicine is much preferred by Maclagan to salicylic acid and to salicylate of sodium, on the grounds that it is a bitter tonic and produces less debility and more rapid convalescence than those agents, and that it never produces delirium nor depresses the heart's action. Ringer176 and Charteris177 state that they have never seen salicine, even in large doses, cause delirium; and Prof. Gairdner has not found it produce any unfavorable symptoms.178 On the other hand, Greenhow179 found that marked depression of the heart's power ensued in 4 out of 10 cases whilst the patients were taking salicine, and entirely subsided after it was discontinued. Further careful and extended observation is needed before the relative value of salicine and salicylate of sodium can be reliably stated. It is probable that the salt is more active and prompt than the bitter principle; and this, with the greater cheapness of the former, may perhaps account for the more general employment in hospitals of the salicylate than of salicine. The latter, moreover, is often tolerated when the former is not.
176 Handbook Therapeutics, 8th ed., 1880, 587.
177 Brit. Med. Jour., i., 1881, 229.
178 Lancet, i., 1882, in table giving experience of British hospitals, prepared by Maclagan.
179 Trans. Path. Soc., xiii. 262.
As regards the doses of these agents required in acute rheumatic arthritis, practitioners are not agreed; Maclagan, Stricker, Fagge, Broadbent, Ringer, Flint, Sée, recommend large doses at short intervals at the outset, with the view of getting the patient rapidly under the influence of the drug. Maclagan gives salicine scruple i-ij at first hourly, then every two hours [p. 59]as the acute symptoms begin to decline; after the second day he allows 20 to 30 grs. every four hours for two or three days; "and for a week or ten days more that quantity should be taken three times a day." Stricker, Fagge, Broadbent, and Sée recommend about 20 to 30 grs. of salicylate of sodium every hour or two for six doses (= drachm ij-iij in the day), and Ringer would employ 10 grs. hourly, and if in twenty-four hours this dose has not either modified the disease or produced its characteristic symptoms, he would increase it to 15 and then to 20 grains hourly. On the other hand, Owen's180 results show practically no difference in the duration of pain and pyrexia and in the average duration of illness from the commencement, whether drachm iij or drachm ij or drachm iss were given every twenty-four hours; and C. G. Young181 found that 10 to 15 grs. every one, two, or three hours are sufficient.
180 Lancet, ii., 1881.
181 Dub. Journ. Med. Sci., Sept., 1880, 193.
Indeed, exceptionally good and exceptionally indifferent results are reported under similar doses. No such good results are reported as those of the Boston City Hospital under doses of drachm ij to drachm iv per diem, the average residence in hospital being only eighteen days if four cases which became chronic are excluded, or 21.9 days if they are included.
The plan in vogue at our hospital here and in my own private practice is to give about 15 grains every two or three hours, according to the severity of the case and until the articular pain and pyrexia are relieved. After the pain and pyrexia have yielded, the remedy should be continued in smaller doses, say 10 to 15 grs., three or four times a day, according to the severity of the case, for eight to ten days longer, to prevent relapse, and during this period exposure, exercise, and dietetic excesses must be carefully guarded against.
The salicine may be given dissolved in milk or enclosed in wafers; the salicylate of soda, in a solution of any aromatic water, to which extract of liquorice or syrup of lemon and a few drops of spirits of chloroform may be added. The French add a little rum to flavor the mixture. Should severe cardiac inflammation exist, and, even although not severe, should there exist signs of failure of cardiac power, salicylates and salicine had better be avoided. If the secretion of urine diminish considerably under their use, or hæmaturia supervene, or organic disease of the kidneys exist, they must be employed cautiously, and may require prompt suspension. If marked debility exist, stimulants, especially the alcoholic, should be combined with them.
The oil of wintergreen has recently been well spoken of by F. P. Kinnicutt of St. Luke's Hospital, New York,182 as a substitute for salicylate sodium. It is itself a methyl salicylate 90 per cent., plus terebene 10 per cent. Its officinal name is oleum gaultheria, and it is given in doses of minim x-xv every two hours except during sleep, and in severe cases of articular rheumatism during the twenty-four hours, either by floating the oil upon a wineglass of water or milk or in capsules or upon lumps of white sugar. It resembles in its influence upon acute rheumatism very closely the sodium salicylate, for which it may perhaps be substituted, and Kinnicutt maintains that it is quite as effectual, pleasanter to take, and free from the intoxicating properties of the salt and the salicylic acid. It requires to be continued during convalescence just like the salicylate.
182 Med. Record of New York, Nov., 1882, 505.
[p. 60]The alkalies—in this country at least—were the favorite remedies in the treatment of acute articular rheumatism before the powers of salicine and salicylic acid became generally known, and there are still authorities who maintain their excellence, if not their superiority over the salicylates, in protecting the heart against the recurrence of rheumatic inflammation (Flint, Dickinson, Sinclair, Stillé).
Under the term the alkaline treatment unfortunately are included two distinct methods of administering the salts composed of potash and soda and the vegetable acids, carbonic, tartaric, citric, etc.—viz.: that in which about half a drachm of one of these salts is given three or four times a day; and the other known as Fuller's method, in which large doses are prescribed, so that from an ounce to an ounce and a half is given in the first twenty-four hours, with the view of rapidly rendering the urine alkaline, and if possible the perspiration also; for I have frequently produced the former effect in less than twelve hours, yet have found the perspiration still redden litmus on the second, and even the third, day and later. A disregard of the essential differences existing between these two methods of employing alkalies in acute rheumatism may partially account for the differences of opinion existing as to the value of the alkaline treatment, and for the differences in the statistical results thereof published by various observers—a remark applicable to other methods and statistics also. Fuller commonly ordered every three or four hours bicarb. sodium drachm iss and acetate of potassium drachm ss dissolved in ounce iij of water and rendered effervescing at the moment of administration by the addition of an ounce of lemon-juice or drachm ss of citric acid. As soon as the urine presents an alkaline reaction—which is usually the case in twelve to twenty-four hours—the quantity of the alkali is reduced by one-half, or to about 8 drachms, during the succeeding twenty-four hours, and provided the urine continues alkaline to 3 drachms on the third day. On the fourth day and subsequently only a scruple to half a drachm of alkali is given three times a day, sufficient to keep the urine alkaline, and to each dose are added 3 grains of quinia dissolved in lemon-juice; and this combination is continued till convalescence sets in. An aperient pill is given whenever needed, but is administered "only under conditions of extreme nervous irritation." The method is not an exclusively alkaline one.
Space will not allow of a lengthened analysis of the statistics that have been published on this subject, and I will give only some of the more important statistical results. While, as we have seen, the average duration of pyrexia and articular pain under salicylate treatment is about 5.4 days, under moderate alkaline treatment, according to the recent statistics of Finlay and Lucas,183 the average duration of pyrexia was 10.3 days and of articular pain 12.2 days, and of Owen184 6.5 days for the first and 8 days for the second, or a general average for the pain and pyrexia together of 9.25 days, or about 3.85 days longer than under the salicylate treatment. Nor can it be said even of the full alkaline plan that the first or second dose frequently relieves the articular pains like a charm. On the other hand, it has been already shown that the average time spent in hospital was five days less under the full alkaline than under the salicylate treatment.
183 Lancet, ii. 1879, 420.
184 Ibid., ii., 1881, 1081.
As regards the relative power of the salicylates and of full alkaline [p. 61]treatment in protecting the heart, the following analysis and calculation deserve attention. The percentage of cases in which cardiac disease set in after the salicylate treatment began was, according to Powell, 18.75; according to Haviland Hall, 37.1; according to Finlay and Lucas, 11.60; Southey, 8; Brown, 4.76; Jacobi, 3.35, or a general average of 14 per cent.; whereas cardiac disease developed after the alkaline treatment had commenced in 13.6 per centum according to Blake;185 in 10.7 per cent. according to Dickinson;186 in 7 per cent. according to Owen; in 6.6 per cent. according to Finlay and Lucas; and in 2 per cent. according to Fuller; making a general average of only 7.8 per cent.
185 Med. and Surg. Reports of Boston City Hospital, 1st Series, 1870.
186 This percentage is obtained by adding together all the cases treated by alkalies given by Dickinson in his IX., X., XI., and XII. tables. Their total was 65 cases in which the heart was affected seven times. In table IX. from drachm ii-iv of alkaline salts were given daily, and in table X. about drachm iij daily.—Lancet, i., 1869.
Judging from these statistics, it is not improbable that a combination of sodium salicylate, with full doses of bicarbonate of sodium or chlorate of potassium, will give better results in the treatment of acute rheumatism than either of those classes of remedies singly. Indeed, Flint and others have advised such combinations, and Bedford Fenwick has recently stated, as a result of his experience in 30 cases, that if, after giving a free purge, followed by scruple doses of sodium salicylate hourly for six hours, that salt be stopped, and in twelve hours afterward half-drachm doses of citrate of potassium be administered every four or six hours until the saliva becomes alkaline, relapses will be extremely rare, and that this is the safest and most successful method of treating acute and subacute articular rheumatism.187
187 Lancet, i., 1882.
Having spoken somewhat fully upon the remedies of which I have most personal experience, and which have the largest number of advocates at the present time, and having advised the combination of these remedies, I shall only glance at some of the other remedies or methods of treating the disease still more or less employed.
Quinia, given in divided doses to the extent of 15 to 30 grains in the day, is still highly thought of in France in the early stages, during the course of and on the occurrence of relapses, in acute (especially febrile poly-) articular rheumatism. It is claimed by Briquet, Monneret,188 Legroux, and others that although not a specific for the disease it moderates the general disturbance, diminishes the local affections, and even retards the development or lessens the gravity of the cerebral symptoms—that, although it does not control the cardiac inflammations, it is not contraindicated by them. The only recent English authority who has strongly advocated full doses of quinia in this disease is Garrod,189 but he mixed the drug, in five-grain doses, with half a drachm of bicarbonate of potassium, a little mucilage, and spirits of chloroform, and gave it every four hours until the fever and articular affection had completely abated. Sufficient facts have not been published to permit of the formation of a reliable judgment as to the actual or the comparative value of either the simple quinia or the quino-alkaline treatment of acute and subacute articular rheumatism. There can be no doubt as to the value of quinia to meet certain conditions incident to the disease, such as debility, lingering [p. 62]convalescence, periodical relapse, excessive perspiration, failure of appetite, and perhaps, in some instances, high temperature. Barclay has found quinia of much service when depression has followed the long continuance of the alkaline treatment and is attended with alkaline urine and a deposit of the earthy phosphates.190 It may be given by the rectum if not tolerated by the stomach or if the alkalines are being taken.
188 La Goutte et le Rheumatisme, Paris, 1857.
189 Reynolds's Syst. Med., 1870, p. 951.
190 St. George's Hospital Reports, vol. vi. p. 111 et seq.
Greenhow191 has treated 43 cases with iodide of potassium and quinine, and says that his experience of this method contrasts favorably with that of salicine and salicylate of soda. However, pneumonia supervened in 3 cases while under treatment; cardiac inflammation arose in 6 cases (= 14 per cent.) after admission; single relapses of short duration occurred in 21 per cent.; and, excluding two cases in which the treatment was soon discontinued and 7 very mild cases, the remaining 34 cases were on the average each thirty-six days in hospital. Under this method relapses were less frequent (21 per cent. instead of 26 per cent.), and stay in hospital longer (36 instead of 30.4 days), than under that by the salicylates; but the number of cases treated is too small to base a final opinion upon. He prescribed 5 grains each of iodide of potassium and carbonate of ammonia three or four times a day, and 2 grains of quinia with three of extract of hyoscyamus in pill as often. This method, in principle at least, resembles that recommended by DaCosta, who administers in uncomplicated cases bromide of ammonium in 15- to 20-grain doses every three hours, and as soon as the acute symptoms have disappeared follows it by quinia in fair doses. It has not come into general use in this country, although its eminent proposer published his cases in 1869.192
191 The Lancet, i., 1882, 913.
192 Pennsylvania Hospital Reports, vol. ii., 1869; New York Medical Record, September, 1874, p. 481.
Notwithstanding the encomiums passed upon propylamine—or, more correctly, trimethylamine—as a remedy for acute and chronic rheumatism by Awenarius of St. Petersburg in 1856, by Gaston of Indiana in 1872, by Dujardin-Beaumetz in 1873, and Peltier in 1874 (both of France), and Spencer of England in 1875, it has not been much employed, especially since the salicylates have attracted attention. It appears that in a considerable proportion of cases the articular pains have subsided in two or three days under its employment, and then the temperature has declined, but the visceral complications have not been prevented. From 4 to 8 minims of trimethylamine in an ounce of peppermint-water, with a drachm of syrup of ginger, may be given every hour or two, the intervals to be increased as the pains diminish. When pain has quite ceased the drug may be stopped and quinia given its place. It merits further study in this disease,193 and Dr. Shapter of the Exeter Hospital has very recently stated that he is so convinced or the value of propylamine that salicylic acid has not fully commended itself194 to him. Senator has recently recommended benzoic acid or its sodium salt in large doses (about ounce ss in the day) in those cases of acute rheumatic arthritis in which [p. 63]the salicylates have failed, although he admits that it scarcely rivals them.195 His 22 patients were relieved in 4.4 days as the average, and no complications occurred in any of them. Benzoic acid is said not to produce the nausea, depression, or unpleasant head phenomena of salicylic acid, to which it is closely related in chemical composition.
193 On this subject see Farier-Lagrange's Essai sur la Trimethylamine, Strasbourg, 1870; Journal de Méd. et de Chirurgie, 1873, No. 2; Medico-Chir. Rev., i., 1873, 497; Lancet, ii., 1875, 675; The Practitioner, London, i., 1875; Le Progrès Médicale, Jan. 10, 1874; ibid., Aug. 9, 1879.
194 The Brit. Med. Jour., 1881, p. 1012. See also Tyson, Philadelphia Med. Times, 1879, vol. x. 359.
195 Centralb. f. d. Med. Wiss., 1st May, 1880, quoted in Practitioner, Sept., 1880. See also McEwan's experience, Brit. Med. Journ., i., 1881, 336; F. A. Flint, M.D., N.Y. Med. Gazette, 1880.
Space will not permit of any notice of lemon-juice, perchloride of iron, the mineral acids, or the blistering treatment. Of this last my experience enables me to say that it frequently relieves the pains promptly, but does not at all always protect the heart. In my opinion it deserves an extended employment in conjunction with early and full doses of the sodium salicylate. As Andrews has not by any communication made since the publication of his paper in 1874196 maintained the value of the treatment of the disease by an exclusively non-nitrogenous diet of arrowroot, and as he had then treated but eight cases in that way, it is hardly necessary to consider it as a method of treatment.
196 St. Barth. Hospital Reports, vol. x. 359.
Having spoken of the treatment of the general disease acute articular rheumatism, it remains to speak of the treatment of its visceral manifestations and of some of its more important incidental symptoms and complications. As the treatment of the various forms of cardiac inflammation will be given in extenso in the articles specially devoted to those topics, I will be very brief in my notice of them.
In every case of rheumatic fever it is our primary duty to employ those measures as early and deftly as possible which in the present state of knowledge appear to promptly relieve the pyrexia and articular symptoms, and lessen the tendency to, but do not altogether prevent, the visceral complications. Such measures have been already said to be the administration of the salicylates and alkaline salts together in full doses, and the observance of certain dietetic and hygienic details to be given hereafter. If, notwithstanding, peri- or endocarditis, or both, supervene, as it frequently happens, what is to be done? I reply that even in pericarditis active interference is seldom necessary; the general treatment previously employed may be continued in the hope that it may mitigate the cardiac inflammation by reducing the pyrexia and subduing the polyarthritis, even although it be incapable of directly controlling the pericardial inflammation. If the pain in pericarditis be really severe and the heart's action much disturbed, a dozen leeches may be applied over the heart, and be followed by anodyne fomentations or hot poultices applied, as Lauder Brunton advised, over several layers of flannel interposed between the skin and them. Leeching, however, is seldom needed, a hypodermic injection of morphia generally sufficing to relieve the pain. Should these measures not relieve the pain and allay the cardiac excitement, small and repeated doses of chloral, which Balfour observes "is not more useful as a sedative than as an antiphlogistic," may be given. If there be, as so frequently happens, but little pain or cardiac disturbance, there being only a friction sound revealing the inflammation, the hot poultices or anodyne fomentations, or even covering the front of the chest with wadding or a belladonna plaster, which I prefer, will suffice. Should pericardial effusion ensue, the diet must be improved, and if much [p. 64]debility exists, the salicylate and alkalies should be stopped, and wine may be given along with quinine alone or with pretty full doses of muriate of iron. As the strength returns absorption commonly takes place; but if it is delayed, either the iodide of potassium or the infusion of digitalis may be employed along with the quinia; or, if no special contraindication exist, a pill containing a grain each of blue mass, digitalis, squill, and quinia may be given three times a day and its effects carefully watched. Much difference of opinion obtains as to the value of flying blisters on the præcordia. Although not often required, they appear to be more useful than iodine applications. In those comparatively rare instances in which the effusion is abundant and remains unabsorbed, either because it is largely sero-purulent or purulent, it is proper to aspirate the pericardial sac, which should certainly be done if marked signs of cardiac oppression and failure coexist. Having once hesitated to aspirate in recent rheumatic pericarditis with copious effusion in a lad, and found a large amount of pus in the sac after death, I would warn against hesitancy under such circumstances. Careful employment of the instrument can hardly do harm if even no large amount of effusion exist.
Active treatment is quite uncalled for, as a rule, in acute rheumatic endocarditis unattended by pericarditis. If the valvulitis occur notwithstanding the employment of the anti-rheumatic remedies, it is very doubtful if we have any others capable of directly controlling that inflammation. Inasmuch, however, as, owing to the inflamed surface being in constant contact with the fluid, many of our remedies may be applied directly to the diseased part, it is well neither to be dogmatic on the point nor to abandon hope that agents may yet be found that will prove directly useful. While carefully treating the rheumatic fever, the main indications remaining to be filled appear to be to quiet the cardiac excitement and secure as much rest to the inflamed valves as possible. The alkaline salts, salicine, and the salicylate of sodium do usually greatly reduce the frequency of the heart, and, pro tanto, secure rest. The tincture of aconite given hourly, so as to slacken the heart's speed, is useful in the sthenic stage of endo- and of pericarditis; and the benefit of absolute rest of the body in bed and of the joints in splints during the entire course of rheumatic fever, in preventing cardiac inflammations and in treating them, has been shown by Sibson.197 When signs and symptoms of cardiac weakness arise, whether from the pressure of pericardial effusion or from myocarditis or any other cause, the employment of salicylates, alkalies, aconite, and chloral should be at once stopped and alcoholic stimulants and tonics (strychnia, quinia, iron) and good food should be freely administered. The most valuable point made of late in the therapeutics of acute inflammations of the valves is Fothergill's development of Sibson's principle—viz. that "general quietude for weeks after an attack of acute endocarditis is indicated," as the cell-growth in the valve may not be quite over in a less time,198 and the work of repair, we may add, not completed. The same principle is specially applicable in myocarditis.
197 Reynolds's System of Med., vol. iv. p. 527, Eng. ed.
198 Diseases of Heart, with their Treatment, 2d Series, 1879, 149.
The disturbances of the nervous system were divided into those [p. 65] dependent upon gross organic alterations of the nervous centres and their envelopes, and those not so related, but which we commonly speak of as functional. Were it possible generally—which it is not—to diagnosticate rheumatic meningitis from the merely functional form of so-called cerebral rheumatism, then its treatment would resolve itself into a vigorous use of the anti-rheumatic remedies, salicylates, alkalies, etc., and the active employment of ice and leeches to the scalp, purgatives, full doses of the iodide and bromide of potassium, ergot, etc. If, together with the symptoms of that often obscure and comparatively rare complication of rheumatic fever, ulcerative endocarditis, there occurred severe headache, delirium, or paralysis, we might find great difficulty in determining the cause of the cerebral disturbance, and would naturally vary our measures according as we suspected meningitis, embolism, or simple functional disturbance, and the treatment adapted to these several conditions will be found under their respective heads in this work.
Coming now to the functional disturbances of the nervous centres, which are the ordinary forms met with in acute articular rheumatism, they may be divided, for therapeutical reasons, into two groups: (1) Those unattended by hyperpyrexia, and (2) those preceded, accompanied, or followed by hyperpyrexia.
(1) When any sign of disturbance of the nervous system, delirium, restlessness, taciturnity or talkativeness, insomnia or somnolence, deafness, tremulousness, vacancy, stupor, or what not, occurs in rheumatism with but a moderate temperature, 101° to 103°, while we anxiously watch the temperature from hour to hour, prepared to combat any tendency to hyperthermia the moment it is discovered, we endeavor to control the cerebral disturbance as in other febrile affections, but with greater diligence, knowing that in this disease these nervous symptoms very often precede hyperpyrexia. We persist with the salicylates to reduce the rheumatic element of the affection, employ remedies to control the cardiac or pulmonary inflammations which are so frequent in such circumstances, sustain the general powers by food, wine, and quinia, if, as frequently happens, there are evidences of failing strength, and meet any other special indication that may arise. For example, we procure sleep and allay motor and mental excitement by opium or chloral and by evaporating lotions or the ice-cap to the head. We reduce temperature, allay restlessness, preserve the strength, and promote sleep by lightening the bed-clothes, drying frequently the entire surface of the body if it is perspiring freely, or by sponging it with tepid water hourly if dry and hot. We act on the kidneys, bowels, and if necessary the skin, if from the scantiness of the urine or other evidence we suspect uræmia. Should these means fail and the delirium and other symptoms which occur in cerebral rheumatism continue, and especially should they be severe, it would be, in the writer's opinion, proper to employ the methods that are now resorted to when hyperpyrexia accompanies those symptoms; for patients suffering from cerebro-spinal disturbance or rheumatic fever, although unattended by hyperthermia, do die if those symptoms continue. Moreover, the hyperthermia may at any moment supervene; it is itself perhaps as much a nervous disturbance as delirium, and apt to succeed the latter. It was in these very cases in which the delirium preceded the hyperpyrexia that the London committee to be presently mentioned found the highest [p. 66]mortality. If along with these nervous symptoms the articular pain or the sweating disappear suddenly, or if the pulse suddenly increase in frequency without demonstrable increase of cardiac mischief, there is reason to anticipate the supervention of hyperpyrexia.
(2) When the cerebro-spinal disturbance of rheumatic fever is followed, preceded, or accompanied by hyperpyrexia, there is one indication for treatment which dominates all others, and that is the prompt reduction of the hyperthermia. The terrible danger of this condition in rheumatic fever is known to all persons who have had much experience of the disease. Wilson Fox in 1871 had not known a case recover after a temperature of 106° unless under the use of cold, yet that is not an alarming temperature in intermittent or relapsing fever, and is often recovered from in typhoid fever. Thanks to Wilson Fox,199 Meding,200 H. Thompson,201 H. Weber,202 I. Andrew,203 Maurice Raynaud,204 Black,205 Fereol,206 and many others since, it has been established that when the hyperthermia is removed by external cold the nervous disturbances also usually at once disappear or lessen very much. And thus we are brought to the treatment of the hyperpyrexia of acute articular rheumatism. On this important topic it will be most satisfactory and convincing to give some of the conclusions arrived at respecting hyperpyrexia in acute rheumatism by a committee of the Clinical Society of London.207 I will condense some of them.
199 Treatment of Hyperpyrexia, 1871, and Lancet, ii., 1871.
200 Archiv für Heilkunde, 1870, xi. 467.
201 Brit. Med. Jour., ii., 1872; Lancet, ii., 1872; and Clinical Lectures, 1880.
202 Clin. Soc. Transactions, v. 136.
203 St. Bartholomew's Hosp. Repts., x. 337.
204 Journal de Thérap., No. 22, 1874.
205 Gaz. Hebdomad. de Méd. Sci., 1875.
206 Soc. Méd. des Hôpitaux, 8 Juin, 1877.
207 Brit. Med. Jour., i. 82, 807.
1. "Cases of hyperpyrexia in acute rheumatism prevail at certain periods;" "such excess corresponds in a certain degree, but not in actual proportion, to a similar excessive prevalence of acute rheumatism generally. The largest number of cases of hyperpyrexia arise in the spring and summer months, whereas rheumatism is relatively more common in the autumn and winter." 2. "Whilst very little difference obtains between the two sexes in regard to proclivity to rheumatism, the proportion of males to females exhibiting hyperpyrexial manifestations is 1.8 to 1." (3 omitted.) 4. "The cases of hyperpyrexia preponderate in first attacks of rheumatic fever." 5. "Hyperpyrexia is not necessarily accompanied by any visceral complications, but may itself be fatal. The complications with which it is most frequently associated are pericarditis and pneumonia." 6. "The mortality of these cases is very considerable, hyperpyrexia being one of the chief causes of death in acute rheumatism." 7. "Although present in a certain number of cases, and these of much value from their prodromal significance, neither the abrupt disappearance of articular affection, nor the similarly abrupt cessation of sweating, is an invariable antecedent of the hyperpyrexial outburst." (8, 9, 10 omitted.) 11. "The post-mortem examinations in a certain proportion elicited no distinct visceral lesions, and when present the lesions were not necessarily extensive." 12. "The prompt and early application of cold to the surface is a most valuable mode of treatment of hyperpyrexia. The chances of its efficacy are greater the earlier it is had recourse to. The temperature cannot safely be allowed to rise above 105° F. Failing the most [p. 67]certain measure—viz. the cold bath—cold may be applied in various ways: by the application of ice, by cold affusions, ice-bags, wet sheets, and iced injections."
Whatever differences of opinion may obtain as to the value of cold in the treatment of the hyperthermia of typhoid fever, there is a tolerable consensus of opinion that it is our most reliable and promptest resource in those formidable cases of rheumatic fever attended with hyperpyrexia, both when alarming delirium and coma coexist and when they are absent.208 Space will not allow of details here in the employment of cold to reduce hyperpyrexia—a subject discussed elsewhere in this work. Suffice it to say, that besides the cold bath (70° or 60°) which the committee regards as the most certain, the tepid bath (96° to 86°) is employed by Fox and regarded as the best by Andrews; it may be cooled down to 70° by adding ice or cold water to it (Ziemssen). The cold wet sheet-pack is still thought much of, like the last, in old and feeble people. Kibbie's method deserves more attention than it has received. He pours tepid water (95° to 80°) over the patient's body, covered from the axillæ to the thighs with a wet sheet and laid upon a cot, through the open canvas of which the water passes and is caught on a rubber cloth beneath the cot, and conveyed into a bucket at the foot of the bed.
208 The powerful depressing effects of high temperature on the human body, and the remarkable opposite influences of a cool temperature, have been personally experienced by the writer in the last three days. For two or three days the weather has been very hot, and he has experienced the usual feeling of exhaustion, incapacity for thought and action. After a thunderstorm last evening the temperature fell 25°, and this morning, twelve hours later, he feels vigorous, refreshed, and capable of intellectual and physical labor. The change is remarkable.
The existence of polyarthritis, of peri- or endocarditis, of pneumonia or pleurisy, does not contraindicate the cold bathing. If much weakness of the heart obtains, it is well to give some wine or brandy before employing the bath, and perhaps while in it, and the patient should not be kept in the bath until the temperature reaches the norm, for it continues to fall for some time after his removal from the bath. If the temperature fall rapidly 2° to 3° in five or six minutes, remove the patient from it as soon as the temperature recedes to 102° or 101° F. If it fall very slowly, the bath may be continued till the temperature declines to 99.5°, when he should be taken out. Should marked symptoms of exhaustion or of cyanosis arise, the bathing should be at once stopped. After it has been found necessary to employ cold in this way, the thermometer should be used every hour, and if the temperature tend to rise rapidly again, the diligent application of a succession of towels wrung out of iced water and applied to the body and limbs, or of Kibbie's method, may suffice; but should they not, and a temperature of 103° or 104° be rapidly attained again, the cold or tepid bath should be at once resumed. In severe cases of this kind a liberal administration of alcohol and liquid food is generally needed, and it is well to try antipyretic doses of quinia by mouth or rectum, although they are usually very disappointing in these cases. It is admitted that cold baths have in a few rare instances caused congestion of the mucous membrane, pneumonia, pleurisy, and even fatal syncope. This is a reason for the exercise of care and constant oversight on the part of the physician, but hardly an excuse for permitting a person to die in rheumatic hyperpyrexia without affording [p. 68]him at least the chance of recovery by the use of the cold or tepid bath.
If delirium and deafness supervene during the employment of the salicylates, it is prudent to suspend their use and take the temperature every couple of hours, as one cannot feel confident that hyperpyrexia may not be impending. Both Caton and Carter have found that the addition of bromohydric acid to the sodium salicylate mitigated or controlled the tinnitus and deafness produced by full doses of that salt.
SUMMARY OF TREATMENT OF ACUTE RHEUMATIC POLYARTHRITIS.—As a general rule, commence at once with a combination of sodium salicylate, say 10 grains, and citrate of potass. gr. xv, every hour for twelve doses, after which give the citrate alone every two hours during the rest of the day. Repeat these medicines in the same way daily until the temperature and pain have subsided, when only half the above quantities of the drugs are to be given every twenty-four hours for about a week longer, after which three 15-gr. doses of the salicylate, with a like quantity of the citrate, are to be administered every day for another week or ten days, to prevent relapses. It is in this third week that quinia is most likely to be required, and as a general rule it may be given with benefit at this period in doses of 2 grains three times a day between the doses of the salicylate. Should the above dose of salicylate not relieve the pains sensibly in twenty-four hours, increase next day the hourly dose to 15 or 20 grains; and if this free administration of the medicine afford no relief after four or five days' use, substitute for the salicylate salt the benzoate of ammonia in 15- to 20-grain doses hourly, continuing the citrate of potassium and conducting the treatment in the manner first advised. Should the benzoate likewise fail after four or five days' trial, omit it, and employ the full alkaline method together with the quinia, of which about 10 to 15 grains may be given in the day between the doses of the alkaline salt.
For the local treatment no uniform method is invariably applicable. In many cases simply painting the joints with iodine daily, or enveloping them in cotton wool, with or without the addition of belladonna or laudanum, and securing it by the smooth and gentle pressure of a flannel roller, proves sufficient. Hot linseed poultices containing a teaspoonful of nitre or of carbonate of soda often afford relief, and so does Fuller's lotion, applied to the articulations by means of spongio-piline, or lint covered with oiled silk. It consists of liq. opii. sed. fl. ounce j, potass. carb. drachm iv to drachm vj, glycerinum fl. ounce ij, aqua fl. ounce ix. It must be plentifully applied. If the articular affection be very severe and not relieved by the above measures, absolute immobility of the joints, secured by means of starch and plaster-of-Paris bandages, has been shown to be very useful, relieving the pain, shortening the duration of the local and the general disturbance, and protecting neighboring joints from invasion.209
209 See Heubner in Archiv der Heilkunde, vol. xii., and Oehme in ibid., vol. xiv., and a striking case in St. Barth. Hosp. Reports, 1876, p. 174, by R. Bridges, M.D.
We have little experience in this country of ice continuously applied to the joints until all the symptoms of acute rheumatism have disappeared (Esmarch and Stromeyer).
Circlets of blistering fluid applied above all the affected joints [p. 69]simultaneously, as practised especially by Herbert Davies,210 often afford prompt relief to the pain, but they do not invariably protect the heart, in my experience.
210 London Hospital Reports, vol. i., 1864, 292.
The hygienic and dietetic management of acute articular rheumatism demands careful attention. While the room should be well supplied with fresh air and sunlight, it should be kept at a uniform temperature and free from draughts. Feather and other very soft beds should be prohibited. Many authorities put the patient between heavy blankets, which I regard as a mistake. The bed-clothing should be light and just sufficient to keep the patient agreeably warm; the night-gown may be of thin flannel and the sheets of cotton. The excess of perspiration should be removed by gentle rubbing with a warm towel at regular intervals, and the sheets should be changed frequently before they become almost saturated with the perspiration. Fatigue and exposure of the patient's person when taking food, attending to his natural calls, or having his personal or bed-clothing changed should be specially guarded against.
The diet in the early actively febrile stage should consist of panada, corn-meal or oat-meal gruel, milk, and barley-water, or even pure milk. Where persons will not take milk the various thin animal broths to which good barley-water or arrowroot or well-boiled rice has been added, jellies, sago and other starchy puddings, may be allowed. Suitable drinks are—plain water, Seltzer and Apollinaris water, carbonic-acid water, lemonade. This low, unstimulating diet should be observed until all fever and articular inflammation have subsided, the tongue become clean, and the visceral inflammations declined, and a return to solid food, and especially to animal food, should be made cautiously. Eggs are to be regarded as of very doubtful safety in this disease. As a very general rule, ales, wines, and the stronger alcoholic liquids are objectionable, but they may be required under the same conditions as in other fevers. Should the salicylates depress the heart, old wine or whiskey may be given with advantage.
During convalescence the patient should not be permitted to leave his bed for several days after complete removal of the fever and articular pain, and for the first four days he should occupy a sofa or easy-chair. Premature walking may induce relapse. An occasional alkaline or sulphur bath, if cautiously taken, sometimes appears to complete the recovery. If endocarditis have existed, a longer rest is desirable, more especially in severe cases, in order that the reparative process going on in the lately inflamed valves may not be in the least disturbed.
synonymous with rheumarthritis chronica, rheumatisme articulaire chronique simple (Besnier), polyarthritis synovialis chronica (Heuter), is defined here as a chronic idiopathic inflammation of one or a few articulations, which is more prone to become fixed than the acute form, and which, notwithstanding its protracted duration, produces no profound structural alterations in the joints.
ETIOLOGY.—It may be the direct sequel of a single attack or more [p. 70] commonly of several attacks, of acute, or more especially of subacute, articular rheumatism. But it is generally a primary affection, occurring in persons who have not had either acute or subacute rheumarthritis, yet owning the same causation as these, and occasionally in its course exhibiting acute or subacute symptoms. The specially predisposing conditions are inheritance; repeated attacks of subacute or acute articular rheumatism, which in accordance with general laws impair the resisting power of the affected joints; prolonged residence or employment in cold, damp, or wet rooms or localities; repeated exposure to bleak, cold currents of air or to frequent wettings of the body or lower limbs. For these reasons it is most common amongst the poor, who are especially exposed to the influences just mentioned; and amongst them cellar-men and sailors, washerwomen and maid-servants, are very liable to the disease. It is chiefly an affection of advanced life, or at least of mid-age, and is rare in youth. The first attacks, and especially exacerbations, are apt to be induced by the direct action of a draught of cold air or by unusual exposure to cold and damp air, especially when the body has been fatigued or overheated. In many cases no distinct exciting cause can be traced.
The morbid anatomy of simple chronic articular rheumatism will vary with the severity and duration of the disease. The alterations are such as chronic inflammation of a non-suppurative character might be expected to produce in the joints by one who had learned those characteristic of acute rheumarthritis. In the simple chronic form the proliferating process involves chiefly the synovial membrane, the capsular and other ligaments, and the periarticular tissues; to a less degree the cartilages, and to a much less degree, and exceptionally, the osseous surfaces. The synovial membrane is thickened, slightly injected, and its fringes hypertrophied and more vascular than normally. Little fluid usually exists in the joint unless during an exacerbation, when a moderate amount of thin, cloudy serum may be present; generally only a trace of thick, turbid fluid, containing oil-globules, and in severe cases débris of the cartilages, but no pus, is found. The fibrous capsule and ligaments become thickened, dense, and stiffened by hyperplasia; and sometimes the adjacent tendons and their sheaths, the fasciæ and aponeuroses, undergo similar alterations, so that the movements of the joints become seriously interfered with. In some cases this irritative hyperplasia specially involves these periarticular fibrous structures, and these, undergoing retraction, produce marked deviations, subluxations, and deformities of the articulations very like those observed in rheumatoid arthritis, although the osseous components of the joints are unaffected. Jaccoud gave to such cases the title of chronic fibrous rheumatism.211 It is worth noting that Jaccoud's, Charcot's,212 and Rinquet's213 cases of so-called "chronic fibrous rheumatism" developed out of acute articular rheumatism, while Besnier's was primarily chronic. In simple chronic rheumatism, if protracted, the cartilages also proliferate, lose their semi-transparency and polish, and become opaque and white; they are often rough and traversed by fissures, and occasionally present erosions; and these erosions [p. 71]are either naked or covered with a layer of newly-formed connective tissue, which may occasionally produce fibrous adhesions between the articular surfaces. Points of calcification occur in the cartilages and tendons in very chronic cases. Instances are observed in which the bones exhibit, to a slight degree, the alterations found in rheumatoid arthritis, and are probably transitional between the two affections. The muscles which move the affected articulations in severe cases are often atrophied, and the wasting imparts to the joints an appearance of considerable enlargement.
211 Vide Jaccoud, Clin. Méd. de la Charité, 23e Leçon, Paris, 1867.
212 Besnier, Dictionnaire Encyclopéd., etc., t. iv., p. 680 et seq.
213 Du Rheum. Artic. Chronique, etc., par Martial Rinquet, Thèse, Paris, 1879, pp. 28-33.
SYMPTOMS AND COURSE.—Simple chronic articular rheumatism presents many varieties. In the milder forms the patient experiences trifling or severe pain in one, or less frequently in two or more, joints, more especially in the knee or shoulder, or both, attended with want of power in the member or with stiffness in the affected articulation. The pain frequently is likewise felt in the soft parts, muscular and tendinous, near the joints, and is usually increased by active or passive movement; it is not always accompanied by tenderness, and rarely with local elevation of temperature or swelling. The wearying aching in the joint is of an abiding character, but is very liable to exacerbations, especially at night; and these come on just before atmospheric changes, such as a considerable fall of temperature, the approach of rain, variations in the direction of the wind, etc., and they usually continue as long as the weather remains cold and wet. A very common symptom is a creaking or a grating which may be felt and heard during the movements of the joint.
The above symptoms may rarely prove more or less constant by night and day for years, but far more frequently, at least at first, they last an indefinite period and disappear to recur again and again, especially in the cold and changeable seasons of the year. Although in the earlier attacks, and often for a long time, no alteration of structure is perceptible in the painful joints, yet in some instances slight effusion into the articulation may be observed during the exacerbations, or the capsule and ligaments may at length become slightly thickened, or the muscles may waste and produce an apparent enlargement of the joint; and this prominence of the articular surfaces may be increased by retraction of the tendons and aponeuroses—a condition which causes real deformities (deviations, subluxations, etc.) of the articulation and impairs more or less its movements. In very chronic cases a fibrous ankylosis may be established.
These last-mentioned conditions often entail great and long-continued suffering, and may even cause some anæmia and general debility; but very frequently the general health and vigor continue good, notwithstanding the permanent impairment of the functions of one or several of the large articulations, and the liability to exacerbations often amounting to attacks of subacute rheumarthritis from changes in the weather, fatigue, or exposure.
Besides the above varieties may be mentioned a not infrequent one consisting of a series of attacks of subacute articular rheumatism recurring at short intervals, involving the same joints, and attended with slight elevation of temperature, febrile urine, perspiration, and moderate local evidences of synovitis, heat, pain, tenderness, swelling, and effusion into the affected joints. This is an obstinate variety, and is often associated with rheumatic pain in the muscles and fibrous tissues of the affected member.
[p. 72]Simple chronic articular rheumatism, like the acute form, is most apt to affect the larger articulations, knees, shoulders, etc., but it frequently also involves the smaller ones of the hands and feet. Although usually polyarticular, it is prone to become fixed in a single joint, but even then it may attack several other articulations, and may migrate from one to another without damaging any.
The course of the disease is usually one of deterioration during persistent or recurring attacks, and in many cases the intervals of relief become shorter and less marked; the joints become weaker and stiffer; and although the pain may not increase and the general health may not be seriously impaired, yet the patients may continue for many years or the rest of their lives severe sufferers, unable to work, and often hardly able to walk even with the aid of a stick. Occasionally, after several years of pain and weakness, a sudden or slow improvement may set in and the patient become free from pain and lameness, and only experience some stiffness in the movements of the joints after several hours of rest, and slight thickening of the ligaments and capsule of one or more articulations. The duration of the disease is indefinite; the danger to life trifling.
The complications of simple chronic articular rheumatism are held by many, and especially by those who regard the disease as constitutional or diathetic, to be the same as those of the acute form, and that they may precede, follow, alternate, or occur simultaneously with the articular affection. All admit that they are observed much less frequently in the former than in the latter. Other pathologists either deny the occurrence of the visceral complications (Senator, Flint) or do not mention them (Niemeyer). It is not denied that cardiac disease may be found in chronic articular rheumatism which has succeeded the acute form, and which may then be referred to the acute attack. The tissue-changes then set up may not have produced at the time the murmurs indicative of endocarditis, but these tissue-changes may have ultimately roughened the endocardium, puckered a valve, or shortened its cords, so that cases of chronic articular rheumatism having a history of an acute attack cannot be safely included when inquiring into the influence of the chronic form upon the heart or other internal organ. Attention has not been sufficiently given to ascertain the frequency of the occurrence of these complications in primary chronic articular rheumatism, and reliable evidence is not at hand. It is not unlikely that the chronic form may slowly develop cardiac changes, as the acute form rapidly does; but when the advanced age of the persons most liable to chronic rheumatism is borne in mind, it must be admitted that valvular and arterial lesions (endarteritis) are observed at such periods of life independently of rheumatism, and referable to such causes as repeated muscular effort, strain, chronic Bright's disease, senile degeneration, etc. Somewhat similar observations are applicable to the attacks of asthma, of subacute bronchitis, of neuralgia, and of dyspepsia, which are frequently complained of by sufferers from simple chronic rheumarthritis. Such affections are common in elderly people in cold and damp climates; they may be mere complications rather than manifestations of rheumatism, or outcomes of the confinement and its attendant evils incident to chronic articular rheumatism, as is probably the relationship of the dyspepsia. There is [p. 73]no doubt of the frequent coexistence of muscular rheumatism with this variety.
DIAGNOSIS.—Simple chronic articular rheumatism may be confounded with rheumatoid arthritis, with the articular affections of locomotor ataxia and other spinal diseases, with chronic articular gout, with syphilitic and with strumous disease of the joints. The reader may consult the observations made on four of these affections in connection with the diagnosis of rheumatoid arthritis. A few additional remarks are called for in distinguishing chronic articular rheumatism from chronic articular gout, which is often a very difficult problem. Both are apt to be asymmetrical in distribution, to have paroxysmal exacerbations, to recur frequently without damaging the articulations, to have been preceded by acute attacks of their respective affections, and to be uncomplicated by endo- or pericarditis. But chronic rheumarthritis has no special tendency to attack the great toe; it is more persistent than gouty arthritis; it does not, even when of long standing, produce the peculiar deformities of the articulations or the visible chalk-like deposits in the ears or fingers observed in chronic gout. The etiology of the two diseases is dissimilar. There is no special liability to interstitial nephritis in articular rheumatism, nor is urate of soda present in the blood in that disease.
In chronic strumous or tubercular disease of a joint the youth, the personal and family history, and sometimes the evident defective nutrition, of the patient; the moderate degree of local pain compared with the considerable progressive and uniform enlargement of the joint; the evident marked thickening of the synovial membrane, either early or late according as the disease has originated in the synovial membrane or in the bones; the continuous course, without marked remissions or exacerbations, of the disease; the rarity with which more than one joint is affected; and the tendency to suppuration, ulceration, marked deformity, and final destruction of the joint,—will prevent the disease from being mistaken for chronic rheumatism.
The PROGNOSIS in simple chronic rheumarthritis is unfavorable as regards complete recovery, and it is chiefly while comparatively recent, and when the sufferer can be removed from the conditions productive of the disease, that permanent improvement, and sometimes cure, may be expected. As a rule, the disease once established recurs. It does not, however, endanger life.
TREATMENT.—All are agreed that hygienic treatment constitutes an essential, if not the most valuable, part of the curative and palliative management of chronic rheumarthritis. A dry and uniform climate is the most suitable, and there is much evidence in favor of a dry and warm rather than a dry and cold climate. Protection of the body against cold and damp by means of flannel next the skin, sufficient clothing, residence in dry and warm houses, etc., is of prime importance. In fact, all the known or suspected causes of the disease should be as far as possible removed.
The direct treatment of the disease resolves itself into general and local, and is essentially the same as that recommended for rheumatoid arthritis, to which subject the reader is referred. A few observations only need be made here. Although, like everything else in chronic rheumarthritis, it often fails, no single remedy has in the writer's [p. 74]experience afforded so much relief to the pain and stiffness of the joints as the sodium salicylate; and he cites with pleasure the confirmatory testimony of J. T. Eskridge of Philadelphia,214 of whose 28 cases 75 per cent. were decidedly benefited. Jacob of Leeds also reports some benefit in 75 per cent. out of 87 cases treated by the same agent.215 It must be given in full doses, and be persevered with. Salicylate of quinia should be tried if there be much debility or if the sodium salt fail. Propylamine or trimethylamine is deserving of further trial in this disease. From 100 to 200 grains are given in the day in peppermint-water. Iodide of potassium, cod-liver oil, arsenic, iodide of iron, and quinia are all and several remedies from which more or less benefit is derived in chronic articular rheumatism. The combination of iodide of potassium with guiaiac resin—gr. ij-iij of each three times a day in syrup and cinnamon-water—is sometimes very useful. The writer has no experience of the bromide of lithium (Bartholow). When the skin is habitually dry and harsh a dose of pilocarpine every other night for a few times will often prove very useful.
214 Phila. Med. Times, vol. ix. pp. 75-77, 1878, and The Medical Bulletin, Phila., July, 1879, pp. 44-48.
215 Brit. Med. Jour., ii., 1879, 171.
Cod-liver oil, iron, quinia, etc., the various forms of baths and mineral waters, electricity, and the several local measures recommended for the treatment of rheumatoid arthritis, are all occasionally very useful in, and constitute the appropriate treatment of, simple chronic articular rheumatism. The dietetic management of the two affections should be the same.
SYNONYMS.—Myalgia rheumatica or myopathia; Fr. Rheumatisme musculaire; Ger. Muskelrheumatismus.
DEFINITION.—The affections included under this term are certain painful disorders of fibro-muscular structures. They are commonly found in persons the subjects of the rheumatic diathesis, and are characterized by pain and often spasm, and sometimes a slight degree of fever. No doubt as our knowledge increases so many attacks connected with painful states of muscles and fasciæ are eliminated from the somewhat uncertain group of muscular rheumatism. True inflammation is not believed to exist, and pathological investigation has rarely shown any morbid changes in the affected parts. The symptoms, therefore, have been attributed to some temporary hyperæmia, slight serous exudation, or neuralgic state of the sensory nerve-filaments. The strongest support is given to this statement from the absence of any marked tenderness in such affected muscles as can be sufficiently examined. In certain cases, undistinguishable clinically, it is quite probable that a periarthritis is in reality the principal factor in the case. In others, again, a subacute rheumatism affecting a joint seems to spread to the adjoining tendinous sheaths, and thus secondarily to attack the muscles themselves, the affection of which may ultimately remain the only condition present.
ETIOLOGY.—Muscular rheumatism is a very common affection. All ages are liable to its occurrence, but the part affected varies with the time [p. 75]of life, children and young adults being much more subject to torticollis, and older persons to lumbago and general rheumatism of the limbs. Amongst hospital patients the disease prevails more amongst men than women, owing doubtless to the greater exposure of the former to the cold; but amongst other classes the same difference is not seen. It is observed in all countries, but according to some writers it is unusually frequent in tropical climates, although there acute rheumatism is very uncommon. The causes of muscular rheumatism are mainly exposure to cold and strain or fatigue of muscles. If these two conditions coexist—e.g. standing in a draught of cold air or lying on the ground when fatigued—the chances of the affection coming are greatly enhanced. Strain, a twist of the body, or a false step can actively start an attack of this kind, and by the sufferers themselves it is constantly attributed to this cause. The part played by this element is difficult to determine, a very slight strain being often followed by great pain and distress from the subsequent rheumatic affection. Some individuals are specially prone to attacks, the slightest current of air, change of clothing, etc. being sufficient to determine its occurrence. These persons are often found to have suffered from rheumatism in some other form, and thus in them we must consider that the rheumatic diathesis furnishes the reason for their unusual susceptibility. It only remains to mention the fact that a disposition to gout seems to favor the development of muscular rheumatism. In gouty families, therefore, it has been observed to be common.
SYMPTOMS.—In all cases pain is the prominent, and in many cases the only, symptom present. In all except the more aggravated attacks pain is felt only when the affected part is disturbed. In such when complete rest or fixed immobility is maintained there is comfort, or at most a somewhat dull, uneasy sensation, but when any contraction of the muscles in question is produced, whether voluntary or otherwise, severe often excruciating pain is at once experienced, often giving rise to a sudden cry or causing the features to be contracted in a grimace. The suffering ceases almost at once when the muscular contraction is relaxed. In more aggravated attacks the pain is more severe, and besides persists, though to a less degree, even when there is no contraction. In rare cases when the maximum degree has been attained there is continuous pain, but the affected muscles are persistently maintained in a relaxed condition by means of true spasm in the surrounding muscles. Slow passive movement affects the subject of muscular rheumatism, and may often be accomplished with a little management without causing pain. If, at the same time, these muscles be handled by pinching and slight pressure, it will be found that they are very sensitive to the touch. When some tenderness does exist, it is slight and is not located in the district of the lower nerve-trunks. Pressure even sometimes allays pain. The constant effort to avoid pain gives rise to a feeling and appearance of stiffness, and thus characteristic attitudes and positions of the head, trunk, or limbs are voluntarily and persistently maintained. There is no spasm of the affected muscles; the distortion is the result of stiff contraction of the associated muscles, which thus forcibly fix the faulty one and hold it in a state of relaxation. Cramp or spasmodic contraction of a single muscle of a painful character does, however, sometimes occur in rheumatic subjects, and much resembles the condition above described. In [p. 76]the same persons also muscular rheumatism may occur in a much more fugitive or erratic form, frequently being nothing more than a slightly painful condition of some group of muscles which have in some way been exposed to cold. This may last but a short time, and either spontaneously disappear or be readily removed by exercise or friction. Muscular rheumatism is generally confined to one muscle or a single group of muscles. Those most liable to it are the very superficial and those easily exposed to cold (e.g. the deltoid and trapezius), powerful muscles often subjected to violent strain (e.g. the lumbar muscles), and those aiding in the formation of the parietes of the great cavities.
This affection very commonly exists without any constitutional disturbances, but sometimes there are present the symptoms of pyrexia—slight elevation of temperature and temporary disorder of the digestive organs—loss of appetite, constipation, and general malaise.
The acute forms generally last but a few days, terminating by gradual subsidence and final disappearance of the pain. The fugitive kind, already alluded to, may, however, be present more or less during several weeks.
DIAGNOSIS.—Errors of diagnosis between muscular rheumatism and a variety of other disorders are common. Laymen especially are only too apt to attribute pain felt in muscles at once to rheumatism of these muscles—a term which is badly abused. Some of these errors are of no great interest, but others are of the highest importance, for they may cause the onset of a serious disease to be overlooked. The principal affections to be borne in mind with reference to diagnosis are the following: organic diseases of the spinal cord (notably tabes dorsalis), causing peripheral pains as an early symptom; functional disorder of the same part, as hysteria or spinal irritation; intra-thoracic inflammation; the onset of an exanthem; the pains produced by the chronic poisoning of lead and mercury; neuralgia; painful spasm of muscle from deep-seated inflammation or suppuration. It is sufficient to indicate these various sources of fallacy, which, if remembered, can generally be guarded against by a consideration of the special features characteristic of each one.
TREATMENT.—The indications for the treatment are mainly two—viz. to relieve the pain and to counteract the diathetic condition generally present. The relief of the pain is accomplished in various ways, according to the seat of the trouble. In severe cases it is proper to resort to the hypodermic use of morphia, to which may be advantageously added some atropia. When the pain is seated in large muscles, the injection will produce better results if thrown not merely under the skin, but into the substance of the muscle. Sometimes perfect rest in bed is necessary to secure the required immobility; in other cases this can better be secured by plaster or firm bandages. Soothing anodynes are extremely useful locally, and counter-irritants also may be used with benefit. Liniments give us a convenient form of application. The best are those containing a considerable proportion of chloroform with either aconite or belladonna, or both. The repeated application of tincture of iodine often gives great relief. Galvanism sometimes proves a rapid cure. Continuous heat is nearly always grateful, and may be applied either in the dry form or by means of soft warm linseed poultices with or without a [p. 77]percentage of mustard. When these are discontinued, care should be taken to protect the affected muscles from cold by keeping them enveloped in flannel or woollen coverings.
Whilst these local measures are being adopted the constitutional disorder should also receive attention. A diaphoretic action should be set up. For this purpose the hot-air or Turkish bath at the outset would seem to be sometimes really abortive. Of medicinal means amongst the most reliable are liquor ammonii acetatis and Dover's powder. Pilocarpine occasionally proves useful. The fixed alkaline salts are also sometimes beneficial, such as the acetate and citrate of potassium and, at a later stage, the iodide of potassium. In a certain number of cases of muscular rheumatism the sodium salicylate acts promptly and well. This drug will succeed well in proportion as the evidence of the rheumatic constitution is well marked, as shown by the tendency on other occasions to attacks of acute articular rheumatism.
Persons who are subject to muscular rheumatism should be made to wear warm clothing, avoid draughts, guard against strains and twists, and in other respects to be careful of their general hygiene. Obstinately recurring cases will very often receive benefit from a visit to some of the natural springs known to possess antirheumatic qualities.
The chief varieties of muscular rheumatism, divided according to the locality affected, require some separate description.
1. Lumbago, or myalgia lumbalis, is that common form which attacks the lumbar muscles and the strong aponeurotic structures in connection with these. It is more frequently than any other form attributed to some effort of lifting or sudden twist of the trunk, but in many cases it owes its origin directly to exposure to cold. The pain comes on suddenly and renders the person helpless, the body, if he is able to go about, being held stiffly to prevent any movement or bending; if severe, he is absolutely compelled to observe complete rest in bed. The muscles, when handled, appear slightly sore, but no local point of acute tenderness can be found. This fact, with the characteristic shrinking from any movement, distinguishes lumbago from neuralgia and from abscess. Pain in the loins, more or less severe, is such a frequent accompaniment of disorder of several organs and parts that careful examination should always be instituted lest some serious organic disease with lumbar pain as a symptom be mistaken for a simple lumbago. The most important of these are perinephritis, lumbar abscess, spinal disease, abdominal abscess, and disease of the rectum and uterus.
2. Pleurodynia, myalgia pectoralis or intercostalis. Here the affected muscles are the intercostals, and in some cases the pectorals as well. Spasmodic pain is felt in one or other side of the chest, and is especially aggravated by the movements of respiration; it is rendered intense by the efforts of coughing or sneezing. Pleurodynia may be confounded with pleurisy, the distinguishing features being the absence of fever and the friction sound of pleurisy. Intercostal neuralgia is sometimes with difficulty known from pleurodynia, but in the former the pain is more circumscribed, more paroxysmal, and more easily aggravated by pressure than in pleurodynia, and when severe there are tender points in the course of the nerve a little outside of the middle line posteriorly (dorsal point) and anteriorly (sternal point). Now and then the hyperæsthetic [p. 78]areas become anæsthetic, and even patches of herpes may form in the course of the nerve, when doubt can no longer remain. From periostitis of a rib pleurodynia may be known by the fact that in the one the tenderness is marked in the intercostal space, and in the other in the rib itself. Pleurodynia is a frequent accompaniment of thoracic affections, causing cough, the frequent paroxysms of coughing tending to induce a painful state of the overworked muscles. The pain, which may be very great, can often be controlled by fixing the chest with imbricated plaster or a firm bandage. Dry cups sometimes answer very well; if more active measures are necessary, then hypodermic injections of morphia must be resorted to.
3. Torticollis, myalgia cervicalis, stiff neck or wry neck, caput obstipum. This term includes those cases of rheumatic idiopathic affection of one or more of the muscles of the side and nape of the neck, which fixes the head firmly in the median line or else in a twisted fashion, with the face turned toward the sound side. The disease can be recognized at a glance by the peculiar manner in which a person will turn his whole body round instead of rotating his head alone. It is much more common in children than in adults. The sterno-mastoid is the muscle chiefly affected, but any of the muscles of the neck may become rheumatic in the same way, and frequently several of them suffer at the same time. The most important point at the outset of an attack of wry neck is to determine whether we have to do with a true rheumatic (idiopathic) disorder, or whether the muscular stiffness is secondary to some spinal or vertebral lesion. The diagnosis is usually founded upon the suddenness of the onset, the absence of other symptoms of nerve disease, and the rapid course of the case, terminating in a cure in a few days. There is nothing special in the treatment of torticollis beyond what has been already said under the general heading.
Other forms of muscular rheumatism which have received special names and have been separately described are the following: myalgia scapularis or omalgia, when the surroundings of the shoulder are affected; myalgia cephalica or cephalodynia, an affection of the occipito-frontalis; and abdominal rheumatism, when the external muscles of the abdomen are involved.
SYNONYMS.—Nodosity of the joints (Haygarth); Chronic rheumatic arthritis, or rheumatic gout (Adams); Arthritis, rheumatismo superveniens (Musgrove); Goutte asthénique primitive; Arthritis pauperum; A. sicca; Usure des cartilages articulaires (Cruveilhier); Arthrite chronique (Lute); Progressive chronic articular rheumatism; General and partial chronic osteo-arthritis;216 Arthritis deformans.
216 Nomenclature of Diseases R. C. Physicians, London.
Neither my space nor time will permit of a history of this disease; it must suffice to say that Sydenham in 1766-69 appears to have first tersely described it and distinguished it from gout; that in 1800, Landré-Beauvais in his inaugural thesis made some observations upon the disease under the title of primary asthenic gout; that in 1804, Heberden, and [p. 79]more especially Haygarth, in 1805, pointed out some of the more striking clinical features of this disease, and distinguished it from both gout and chronic rheumatism under the title nodosity of the joints. The latter author, in the work mentioned, claims to have written a paper upon the subject twenty-six years previously, although it was not published; and to him belongs the merit of having so described the disease as to have given it a place in nosology. Incidental allusions were made to the affection in 1813 by Chomel, in 1818 by Brodie, and by Aston-Key in 1835; in 1833, Lobstein, and about the same time Cruveilhier, pointed out some of the more striking characters of the morbid anatomy of the affection. But it is to Adams of Dublin that we are indebted for the most complete account of the anatomy and of many of the clinical features of the disease—first in a paper read before the British Association in 1836, next in his article on "The Abnormal Conditions of the Elbow, Hand, Hip, etc.,"217 and finally in his able monogram "On Rheumatic Gout" in 1857. The contributions to this subject since that date have been very numerous as well as valuable from the leading countries of Europe, and I must not here attempt to assign to each investigator his proper portion of the work.
217 Todd's Cyclop. of Anat. and Phys. (1836-39).
It may be here remarked that Landré-Beauvais and Haygarth described more particularly that form of the disease which, beginning in the small joints of the extremities, tends to extend to the larger joints in a centripetal way, and to involve many of them—peculiarities which have given rise to the epithets progressive polyarticular chronic rheumatism, peripheral arthritis deformans, and which is the form of the disease usually described by physicians as rheumatic gout, rheumatoid arthritis, nodular rheumatism, and by the other names just mentioned. On the other hand, Key, Colles, Adams in his earlier paper, and R. W. Smith described the disease as it affects the larger joints, hip, shoulder, or knee, to one or two only of which it may be confined; and as this variety is frequently observed in elderly persons, and in them often involves the hip, it is often spoken of as senile arthritis, malum senile articulorum, morbus coxe senilis, mono-articular arthritis deformans, partial chronic rheumatism, and has been described by surgeons rather than by physicians. However, even when beginning in the hip or shoulder, the disease is apt to involve several of the intervertebral articulations, and not unfrequently to extend to other joints than the one first affected, and even to the peripheral joints. Its progressive and general nature is thus evidenced, whether it invade from the beginning a single large joint or several symmetrical small articulations. Finally, on this topic Charcot has insisted that Heberden's nodi digitorum contributes a special form of the disease under consideration, and proposes to call it Heberden's rheumatism or nodosities.218
218 Lectures on Senile Diseases, Syd. ed., 1881, p. 137.
Rheumatoid arthritis presents the clinical varieties or groupings of phenomena just mentioned, at times quite distinctly appreciable from one another, but sometimes more or less blended, yet even then manifesting in their periods of invasion and early stages an adhesion to all of these typical groupings. Charcot has especially dwelt upon these: 1st, the general or polyarticular and progressive form; 2d, the partial or oligo- or mono-articular form; 3d, Heberden's nodosities.
[p. 80]1st. The symptoms and clinical history of general or polyarticular and progressive rheumatoid arthritis. This is the most common form of so-called chronic rheumatic arthritis, the classical rheumatic gout, or rheumatisme noueux, and it may declare itself, as Garrod and Fuller pointed out, very rarely in an active or acute form, or, as it usually does, in a chronic and insidious form.
The acute form of rheumatoid arthritis closely resembles the milder varieties of acute articular rheumatism or the best marked examples of the subacute form of that disease. But it presents the following particulars, by which it may generally perhaps, but not always, be distinguished: while the temperature, the thirst, the furring of the tongue, the frequency of the pulse, the articular pains and tenderness, etc., are less developed than in acute articular rheumatism, there is wanting the profuse and continued perspiration, the early involvement of the endo- or pericardium in the inflammation, and the prompt prostration of the strength so commonly witnessed in that disease. On the other hand, while the rheumatoid affection may involve the larger joints—knees, ankles, elbows, and wrists—it almost certainly implicates the smaller joints of the fingers, and often of the toes. There is apt to be greater effusion into the synovial capsules (McLeod's capsular rheumatism) and into the synovial sheaths and bursæ about the affected joints than in ordinary acute or subacute rheumatism; further, the inflammation does not migrate from joint to joint, but obstinately persists in several of them, and more especially in the wrist and in the metacarpo-phalangeal joints of the index and middle finger, perhaps also in the ankles and in the metatarso-phalangeal articulation of the great toe. Instead of disappearing in four to six weeks, the articular inflammation continues, although the pain may abate very much, and the capsules of the joints continue swollen and rather tense. The muscles of the extremities waste, and are the seat of painful reflex spasms which interfere with the movements of the joints; and although the patient is capable of moving about, and is free from all febrile disturbance, one or several of his joints remain permanently swollen, painful, and crippled. Perfect restoration of all the affected joints seldom if ever occurs. In common with other observers, I have met with this acute form most frequently in young women twenty to thirty years of age—several times in connection with recent delivery or rapid child-bearing, or lactation; once after what was regarded by the medical attendant as an attack of acute rheumatism occurring not long after labor. It has been observed in children, and is not uncommon after forty. These patients usually suffer in their general health—become weak, pale, depressed in spirits, and lose flesh. In several cases of this form marked intervals of improvement have occurred; the local disease has ceased to progress, and tolerable comfort has been experienced, perhaps, till pregnancy, delivery, or lactation again determined a fresh outbreak of the disease. Sometimes, however, this acute form steadily advances, and in a year or two establishes changes in the cartilaginous and osseous structure of the affected joints. Such a case I met in a lady of twenty-one who had had a good deal of anxiety as a mathematical teacher, and whose illness set in during vacation while at the seaside. It proved obstinately progressive for several years, until several of the larger joints, as well as the smaller, were badly crippled.
[p. 81]The primary chronic form is much the more frequent, although between it and the acute variety there are many intermediate grades. For weeks or months the patient may experience numbness or formication and rheumatic pains in the limbs, perhaps with a sense of stiffness in the joints, especially felt after rest or the day after unusual fatigue. Then one or more joints—most frequently the metacarpo-phalangeal of the fingers—become painful, swollen, tender when touched, and inordinately hot; these symptoms may subside under rest or treatment, and after weeks or months recur, either without known cause or from exposure, fatigue, or some impairment of the health. Usually, the original joint is again affected, but frequently one or two more of the same on the other hand suffer likewise. More or less complete remissions of the pain and local inflammation now tend to take place from time to time and alternate with exacerbations or fresh attacks of the local disturbance, and the disease extends, as it were, centripetally and more or less symmetrically to the wrists, then to the elbows, and then to the shoulders, or from the toes to the ankles and thence to the knees—although there is no invariable sequence of this kind—and next to the hands; the knees are specially liable to invasion. Of Haygarth's 34 cases, in 2 the knees alone suffered, and "in all or nearly all the rest the hands, chiefly the fingers, were probably affected." In Charcot's 45 cases the début took place in the small joints of the hands and feet 29 times; in the hands, feet, and one large articulation, 7 times; in one large joint, and later in the fingers, 9 times. Even in this primary chronic form there is usually in the earlier stages some effusion into the joints; the soft parts of the articulation are thickened and swollen; obscure fluctuation in the smaller and very distinct fluctuation in the larger joints may be felt. The pain may be severe, especially at night, and during the exacerbations of the disease it varies greatly in its degree and persistency. The position and shape of the joints are altered, partly by spasmodic retraction of the muscles, and more or less by the effusion into the capsules and adjacent bursæ and sheaths, and the thickening of the soft parts covering the articulations. As the disease progresses further deformities ensue from the growth of new bone around the heads of the bones, the absorption of the articular cartilage, the development of masses of cartilage in the hypertrophied synovial processes and beneath the synovial membrane at the margin of the bones; the relaxation of the articular ligaments; and the displacements and subluxations of the unshapely bones composing the joint. The great wasting of the muscles of the member affected has some share in producing its unnatural appearance. In the advanced stage there is more or less abiding pain, soreness, and stiffness in the affected articulations, violent cramps are experienced in the course of the adjacent muscles, and pains either along the nerves or vaguely down the limbs. Crackings or creakings are to be heard, and grating is to be felt during the movements of the joints; these movements become more and more restricted, so that an immobility almost equal to that of true bony ankylosis is established, this result seldom occurring except amongst the carpal, tarsal, tibio-tarsal, and the vertebral articulations. Interlocking of the osteophites formed on and around the articular surfaces, and in other cases union of these surfaces by the interposition of newly-formed fibrous tissue, produce a spurious ankylosis [p. 82]destructive of the articular functions. In the very advanced stages the feet, ankles, and legs are often considerably enlarged and the integument thickened by a chronic oedematous infiltration, or the bones and soft parts are atrophied and the integument is pale, smooth, and attenuated, resembling parchment or the condition seen in certain stages of scleroderma and tightly drawn over the wasted rigid fingers. This primary chronic form is especially apt to progress steadily for many years, the joints earliest affected becoming gradually more distorted and crippled, and fresh joints becoming invaded until there may hardly remain a single sound articulation in the limbs, or even in the body; and at length the patient may be unable to feed himself or masticate or raise his chin from his sternum or rotate his head or stand.
The deformities of the several joints, being largely the result of muscular contraction, observe certain general types, which, however, are not peculiar to the disease, but occur in various affections of the nerve-centres, involving paralysis or spasm or both. Charcot has carefully described those met with in the hands, and I must refer to his masterly article upon chronic articular rheumatism for his account of them.
(1) It must suffice to say here that the predominant features of the hand in chronic rheumatoid arthritis are the following: The first phalanx of the fingers is either flexed upon the metacarpus or extended, and the terminal phalanx in like manner is either markedly flexed or extended upon the second, or these two phalanges are maintained in a straight line, while the first phalanx is, as usual, decidedly flexed upon the metacarpus.219 In all these varieties the hand is pronated; there is a great tendency to deviation of the fingers toward the ulnar border of the hand, although sometimes the deformed fingers stand out, not unlike a bunch of parsnips. The thumb escapes longer than the other fingers, and its metacarpo-phalangeal joint is usually flexed, rarely extended.
219 Lectures on Senile Diseases, Syd. ed., trans. 1881. Figs. 1 and 2, Pl. II., on the hand, give good illustrations of these deformities.
(2) The great toe, enlarged at the metacarpo-phalangeal articulation, is usually drawn to the outer border of the foot, across and above, but rarely below, the other toes, and the foot is usually abducted and flattened, the prominent internal border resting on the ground. The wrist, elbow, and knee-joints are generally flexed; the distal ends of the ulna and radius, more or less enlarged, project backward; the semi-flexed tibia is drawn backward on the femur and rotated outward, thus rendering the internal condyle of the femur prominent and displacing the patella toward the external condyle, and foreign bodies may frequently be felt in the enlarged knee- and elbow-joints. Finally, the extremities of the affected bones will, as a rule, be found enlarged and misshapen, and nodosities, rims, tips, ridges, and stalactiform growths of new bone may be felt on them.220
220 Figs. 12 to 18 and 22 in Adams's Treatise on Rheumatic Gout are nice illustrations of these deformities.
The general condition in this chronic form varies in different individuals, and there is no characteristic disturbance of the functions, such as obtains in chronic gout. There is no elevation of temperature, unless to a slight degree during an active crisis of the disease; the tongue may be clean, the pulse tranquil, the appetite and digestion satisfactory, and [p. 83]the urine normal or perhaps pale and of low density. Fuller, however, says that "more generally the complexion is sallow and the skin sluggish, and evidence of mischief is furnished by yellowishness of the conjunctivæ, constipation of the bowels, a pale and unhealthy character of the dejections, excessive flatulence after meals, turbidity of the urine, and fulness of the pulse." My own experience hardly harmonizes with this, and I have seen many persons suffering for years from the general and partial form in the enjoyment of excellent general health. Should, however, the disease develop in a person the subject of menorrhagia or other uterine disorder, or of repeated child-bearing, or after prolonged mental anxiety, some disturbance of the general health fairly referable to such disturbing conditions may be certainly looked for. In the advanced stages the prolonged suffering and confinement often induce anæmia, dyspepsia, and failing health.
More numerous and exhaustive analyses of the perspiration, urine, and blood in the disease are needed. There is no uniform condition of the skin; general perspirations, chiefly at night, often obtain, but I know of no authoritative report as to the chemical reaction of the sweat in this disease; Garrod221 and Charcot222 vouch for an absence of uric acid in the blood, while Marrot223 found both this acid and the urea below the normal quantity in the urine, although the acid increased notably under baths of high temperature.
221 Reynolds's Syst. Med., i. 918.
222 Loc. cit., p. 190.
223 Contribution à l'Étude des Rheum. Artic., Examen de l'Urine et du Sang, Paris, 1879, p. 42.
Certain affections other than the articular have been occasionally observed in persons suffering from rheumatoid arthritis, but many even of those authors who regard the disease as a form of rheumatism speak of these affections as coincidences, and not as essential manifestations of the disease. Charcot and Besnier, however, maintain the latter to be their true relation to the articular affection which they regard as chronic rheumatism. The two authors just named allege that all the visceral localizations that occur in acute articular rheumatism may obtain in the nodular form, but that such localizations are infinitely less frequent and serious than in the acute, subacute, or simple chronic forms of articular rheumatism—that endo- and pericarditis undoubtedly do occur in nodular rheumatism, and appear especially where there is an exacerbation of the disease and where there is some approach to the acute state.224 As Charcot has adduced these cardiac affections in proof of the rheumatic nature of rheumatoid arthritis, it is deserving of mention that he had personally met with but two instances of endocarditis and five of pericarditis, four of the latter having been discovered not during life, but in nine autopsies, and that he cites only eight other cases of endo- or pericarditis which had been either published or reported to him. He admits too that there had generally been in these cases, at some former period, an attack of acute rheumatism. Besnier, Homolle, Malherbe, Vidal, and Colombel, in their articles upon the disease under consideration, do not cite a single case in which they have seen cardiac disease in rheumatoid arthritis. On the other hand, McLeod, Garrod, Fuller, Flint, Senator, and Pye-Smith either deny or ignore the occurrence of cardiac disease as a manifestation or complication of this disease. My personal [p. 84]experience coincides with that of those authorities last cited, except in one instance, and that is open to the objection that the patient's father had had acute articular rheumatism, the mother was the subject of chronic deforming arthritis, and the patient had experienced during many winters an affection which began in the smaller joints and permanently damaged them; when first seen by me he had chronic disease of the aortic valves. He may have had true articular rheumatism as well as rheumatoid arthritis. His father had experienced the one, his mother the other. If those instances be excluded in which a former attack of acute rheumatism might be adduced in explanation of the supervention of cardiac disease, but few cases will remain to suggest that rheumatoid arthritis may develop endo- or pericarditis; and when it is borne in mind that in several ways the cardiac affections may have arisen as mere coincidences of the rheumatoid affection, it is well to wait for further evidence before accepting as proved the occurrence of cardiac affections as local manifestations of rheumatoid arthritis. Garrod's observation is still pertinent: "The form of the disease in which acute cardiac inflammation has occurred may be rather that of true articular rheumatism of a very subacute character."
224 Loc. cit., 172-175; Besnier, loc. cit., 699.
Nor is the evidence at all satisfactory in favor of any special tendency to the following affections, much less of their being local manifestations of rheumatoid arthritis: viz. pleuritis (McLeod, Fuller), asthma (Charcot), chronic laryngitis (Garrod), grave cerebral or spinal disturbances (McLeod, Fuller, Vidal), paralysis agitans, locomotor ataxia, sciatica, trifacial neuralgia, and albuminous nephritis.225
225 To mention only some of the many sources of cardiac disease other than rheumatism may be adduced scarlet and other fevers, extension of inflammation from the pleura or lung and other sources of local irritation, powerful or oft-repeated muscular efforts, Bright's disease, senile degeneration, etc.
Among the more frequent complications may be mentioned migraine, certain cutaneous affections, more especially psoriasis, prurigo, lichen, and some diseases of the eye, chiefly iritis, which is apt to be relapsing, and sometimes episcleritis. It is remarkable that iritis very seldom occurs as a complication of acute articular rheumatism.
The so-called rheumatic nodules occur also in chronic rheumatoid arthritis. It is not yet established that they are peculiar to rheumatism and to rheumatoid arthritis. Dr. Stephen Mackenzie has seen them in one instance in tertiary syphilis, the patient not having had arthritis, rheumatism, or chorea.
2d. The partial or oligo-articular form of rheumatoid arthritis, like the general or polyarticular variety, is usually a primarily chronic affection, insidious in its invasion and slow in its progress. It is chiefly observed in old persons, especially men (senile arthritis), affects frequently a single joint, and chiefly the hip, but occasionally the knee, shoulder, or spinal column, either as a consequence of special injury or of the wear and tear of life, or exposure to cold and wet, or even of what seemed to be simple acute or subacute articular rheumatism or gonorrhoeal rheumatism. When not the result of injury, two or three joints may suffer, both hips or knees, or hip and some of the vertebræ, hip, knee, and ankle of the same limb, and so on. Even in those cases in which the disease for a long time is confined to a single joint and may have been caused by an injury,226 other joints, finally, are apt to become [p. 85]affected, often in a symmetrical order. So that it may seem almost general, or at least polyarticular, just as the converse sometimes happens in the general rheumatoid arthritis of long standing, where the disease becomes greatly aggravated in one articulation and produces great deformity and destruction of it, the others remaining as they were.
226 See Ord's case, II., Brit. Med. Journal, i., 1880, 158.
The symptoms of this partial chronic form are very much those of the general form already described, but there is usually in the early stages less heat, tenderness, and swelling of the affected joint; the pain is less acute, but more abiding, and, with the exception of more or less stiffness or impeded movement in the joint, it may be the only sign of disease present, so that at this stage of the affection it may be taken for simple chronic articular rheumatism. But the disease persists; the voluntary movements become more painful and difficult; slight exercise of the joint is followed promptly by fatigue and aggravation of the pain, and yet the articular surfaces may be pressed together, and flexion and extension be practised, without causing much suffering. Slowly and continuously alterations take place in the affected articulation; with but little heat or redness it enlarges steadily, the soft parts becoming infiltrated and thickened, or effusion taking place into the capsule; the articular surfaces become irregularly depressed by the growth of osteo-cartilaginous rings, osseous nodosities, and stalactiform processes upon them, and these irregularities, together with one or several loose bodies, may be felt in the joint. The enlargement of the articulation becomes more apparent, owing to the wasting of the muscles of the limb; its movements become more and more restricted and difficult, although perhaps not more painful, and are attended with creakings and gratings perceptible to the ear and hand; and at last nearly all movement of the joint may be prevented by the alterations in the shape of the epiphyses, or by the interlocking of the osseous outgrowths, or in rare cases by actual union of the bones. This form constitutes, par excellence, arthritis deformans. In many instances there is little effusion throughout the process, notwithstanding the grave deformity in progress; hence the term dry arthritis. Even the partial form is sometimes more active in its invasion, as when it very rarely succeeds acute or subacute articular or gonorrhoeal rheumatism, or, more frequently, follows an injury.
The duration of the partial form is usually very protracted; it may be ten or twenty years. Exacerbations of the disease occur from time to time, in the intervals of which the patient may be free from pain, although the affected joints are seriously crippled.
The affection is not in itself fatal; the patient may attain an advanced age and die of some intercurrent disease, such as dysentery, pneumonia, cerebral hemorrhage, or other affection incident to old age.
A description of the features presented by partial rheumatoid arthritis affecting the hip (morbus coxa senilis), the shoulder, and other joints rather appertains to works on surgery, and only a glance at the evidences of the disease in the vertebral column (spondylitis deformans) will here be given. When the cervical vertebræ are implicated the power of rotating the head from side to side is usually preserved and is attended with a crackling noise, while the rest of the cervical region is stiff and the head cannot be bent forward; when the dorsal or lumbar vertebræ suffer the back becomes bent, the patient stoops greatly and cannot stand [p. 86]erect, and his body is shortened and more or less twisted. A careful examination will discover not only the great rigidity of the spine, and as it were fusion en masse of its joints, but in these persons the bony outgrowths may be felt. Occasionally the alteration in the vertebræ by compressing the cord or its membranes, or the spinal nerves and ganglia, may produce neuralgic pains in the cervical, dorsal, lumbar, or sciatic nerves, wasting of the muscles, more or less paralysis, and even vasomotor disturbances.
3d. Heberden's nodosities are certainly sometimes the effect of rheumatoid arthritis, implicating chiefly, often solely, the distal joints of the fingers, where it slowly forms two little hard nodules about the size of dried peas upon the side of the articulations. These are notably enlarged and their movements impaired, but pain is seldom experienced, and were it not for deviation of the end of the finger to one side or the knob-like excrescences upon the joints—appearances which much disfigure the hand—patients would not speak of the affection. In many cases these alterations likewise involve, but in a minimum degree, the first phalangeal articulations, and less frequently the metacarpo-phalangeal, and even some of the larger joints—the wrist, knee, or hip, etc. Like the other varieties of rheumatoid arthritis, this form occasionally has a more active invasion than is above mentioned, and may be attended by local pain, heat, and redness, or such symptoms may occur as exacerbations of the chronic disease.
Gout may precede these nodosities, or, as in the case of Charcot's,227 the latter may precede the former by several years. Finally, Charcot remarks that Heberden's nodosities are "often accompanied by asthma, migraine, neuralgia, especially of the sciatic nerve, and muscular rheumatism, and that these manifestations may alternate with the exacerbations of the disease."
227 Loc. cit., 198.
MORBID ANATOMY.—Every component tissue of the articulations exhibits signs of a chronic inflammatory process. In the chronic form affecting the larger joints the synovial membrane is found more or less congested, opaque, and thickened; at the point of its reflection upon the bones its fringes are thickened and injected and their villosities greatly increased in number, length, and thickness, and in extreme instances have been aptly compared to the wool on a sheep's back. The cartilage-cells normally existing in the synovial fringes likewise proliferate and develop into cartilaginous growths, many of which become infiltrated with lime salts, or even ossified, and in this way originate some of the foreign bodies, pedunculated or sessile, which are found in the joints. These may be attached to the synovial fringes, or imbedded in the membrane itself, or set free by rupture of their pedicles. In some examples these neoplasms resemble in size small melon-seeds; in others they form irregular masses, many of which are as large as hazel-nuts.
At the outset there is frequently an increase of synovial fluid, richer in mucine than natural, which lessens considerably in the later stages and becomes a turbid, viscid fluid of a dirty white or reddish-yellow color, containing no pus, but degenerating epithelium and fragments of villosities and cartilage. In many cases, more especially of the partial [p. 87]form, very little effusion into the articulations takes place (arthrite sèche).
The inflammatory irritation excites proliferating and degenerating processes in the cells and basis-substance of the cartilage covering the bones, and the changes described in connection with acute rheumatic arthritis ensue. Those parts of the cartilage covering the bones which suffer pressure in locomotion fibrillate on their surface, and either undergo mucous degeneration, resulting in ulceration and complete absorption, or are thinned and worn away by attrition. In either way the ends of the bones become laid bare. Those portions of the cartilage at the periphery of the joints which escape compression in the erect posture likewise proliferate, but, according to Cornil and Ranvier, in consequence of being covered by the synovial membrane the proliferating elements are retained in situ, instead of escaping into the articular cavity, and develop into actual cartilage, and may ultimately ossify. In this way irregular masses of cartilage (enchondromata) and bone (osteophytes) form around the heads of the bones, enlarging them considerably, altering their shape, encroaching upon the articular cavity as well as extending up the shafts of the bones, and displacing the capsules of the articulations. Similar productions of cartilage sometimes form in the thickened capsules and ligaments, especially in very protracted cases, or these parts become infiltrated with lime salts.
While these processes are going on at the periphery and the centre of the cartilages, in its deeper layers the proliferating cells are undergoing ossification and rendering the ends of the bones very dense and compact, so that under the attrition to which they are exposed by the articular movements they acquire the smoothness, polish, and white aspect of ivory (eburnated). It is probable that the articular ends of the bones participate in this proliferation and development of bone, which increases their compactness and is followed by eburnation. That the bone itself does sometimes play a part in the hyperostosis which is in progress is shown by an increase of an inch in the length of the right ramus of the maxilla over that of the left in Adams's first plate.228 Forster's229 and Ziegler's230 later investigations confirm this view. Nor is the periosteum exempted from the proliferating process which may have long existed in the several articular tissues, as is shown by the considerable enlargement of the diameter of the shaft of the long bones and by the osteophytes which form on the exterior of the vertebræ and often unite several of them together by a series of osseous splints, interfering with the mobility of the spine. Notwithstanding this development of cartilage and bone upon the exterior of the articular extremities, the interior, especially in old people or in very chronic examples of the general form of the disease, or rarely in the partial form, undergoes degeneration and atrophy. The spongy substance becomes rarefied, thinned, and friable (osteoporosis), so that it has been easily cut or crushed, and it is frequently loaded with fat. True ankylosis of the diseased joints is rare, except in the very small articulations when kept at rest; even under this condition fibrous ankylosis is not of frequent occurrence.
228 Illustrations of the Effects of Rheumatic Gout, London, 1857.
229 Forster, Handbuch der Path. Anat., p. 1000.
230 Virchow's Archiv, 1877.
Finally, the interarticular fibro-cartilages and ligaments and the long [p. 88]tendon of the biceps degenerate and are absorbed. The muscles in protracted cases suffer simple atrophy, but are sometimes the seat of an interstitial accumulation of fat. Thus far, no lesions of the nerves supplying the diseased joints nor of the spinal cord have been discovered.
ETIOLOGY.—The causation of rheumatoid arthritis is involved in much obscurity—in part, because sufficient attention has not been paid to its clinical varieties. We will examine first the general progressive form which is the more common.
In women it prevails during the child-bearing period. It is probably oftenest developed between twenty and thirty, and continues to occur frequently up to the period of the menopause, fifty, after which it develops comparatively seldom. Of Ord's 33 cases, 10 were between twenty and thirty years; 11 between thirty and forty; 9 between forty and fifty; and 3 between fifty and sixty.231 Children are not exempt. E. C. Seguin saw three children of the same family suffering from the disease at ages from two and a half to four years.232 Moncorvo233 met with an example at two years and a half, Laborde at four, and Charcot at ten. It occasionally begins in both sexes after sixty.
231 Brit. Med. Jour., 1880, 156.
232 The Med. Record, London, 1877, 797.
233 Du Rheumatisme Chronique Noueux des Enfans, Paris, 1880.
It is pre-eminently a disease of females up at least to fifty; after that it is not infrequent in men, and is then often only partial, at least at first. The most frequent progressive form, however, does often occur even in boys.
It is probably more frequently observed in cold and damp climates than in those of opposite qualities, for cold is regarded as its most common cause. However, it is met with in India and other hot climates. Besnier asserts it is almost unknown in the tropics, but new investigations are needed on this point.
Direct hereditary predisposition exercises but little influence, according to Garrod, and we certainly often see the disease confined to a single member of a large family, although Seguin saw three young children of one family affected with it, their parents being free from any disease. Trastour three times saw the children of women who were afflicted with nodular rheumatism already suffering from articular rheumatism; and Charcot once saw the grandmother, the mother, and the granddaughter successively attacked. At present I have a patient whose mother at fifty-five and maternal grandmother at sixty became subjects of a crippling polyarticular affection; another of my patients informed me that his mother and a young sister were like himself victims of the disease. This direct transmission appears to be rare, judging from my own experience and from the few instances of it mentioned by writers. But very many authorities maintain that simple acute and chronic rheumatism and gout in the parents predispose to rheumatoid arthritis in the offspring (Charcot, Trastour, Besnier). Now, the facts given in support of this opinion are not numerous. Trastour found that out of 45 cases of nodular rheumatism the father or mother were rheumatic in 10 instances, but the form of the rheumatic affection is not stated. Charcot, Besnier, and Homolle, although believers in the doctrine, do not cite an example in proof. However, in Pye-Smith's 27 cases of osteo-arthritis, five stated that rheumatism had occurred in their families. Thus, two fathers [p. 89]had had rheumatic fever, and one was rheumatic, and two sisters of different families had had rheumatic fever. Besides, the father of a sixth and the grandmother of a seventh had had gout.234
234 Guy's Hospital Reports, 3d Series, xix. 348.
The evidence in favor of the doctrine that true articular rheumatism transmits an hereditary tendency to rheumatoid arthritis does not appear to be conclusive, although it is highly thought of by those who regard the latter disease as a variety of rheumatism. Some considerations of an opposing character deserve mention. Acute articular rheumatism has very rarely passed continuously into rheumatoid arthritis, and very rarely has been followed at short interval by that disease; and in such exceptional cases the antecedent affection may have been really the acute form of rheumatoid arthritis, which closely resembles acute articular rheumatism. Trastour,235 Vidal,236 Charcot,237 and others admit that acute rheumatism can hardly be placed amongst the antecedents of the rheumatoid affection. Garrod238 with some others states that now and then acute rheumatism acts as an exciting cause of it, which appears to have been Fuller's view;239 he had repeatedly known it to commence apparently as a sequel of acute rheumatism. However, Ord met with a case in which the lesions of rheumatoid arthritis were present in a typical form in a patient who had mitral disease as a result of acute rheumatism, the arthritis having begun as a continuation of the acute attack.240
235 Thèse de Paris, 1853, p. 41.
236 Ibid., 1855, p. 9.
237 Leçons Cliniques, p. 214.
238 Reynolds's Syst. Med., 1870, i. 920.
239 Lib. cit., 333.
240 Brit. Med. Jour., 1880, i., 158.
That so common an affection as articular rheumatism should occur in the family or personal history of a patient the subject of the rheumatoid arthritis is not improbable; nasal catarrh and many other very common diseases must be frequent antecedents of the rheumatoid affection, yet are not causes of it. Much the same remarks apply to the view that gout in the parents may transmit a tendency to rheumatoid arthritis in the offspring. The experience of English physicians in this matter is hardly reliable, owing to the great prevalence of gout in England. In Canada and many parts of the United States, however, while gout is a rare disease, rheumatoid arthritis is a common one, and the writer has not found an intimate relationship to obtain between the two affections. It is not intended to deny that when the children of rheumatic or gouty parents fail in health owing to their inherited constitutional disease, they become liable to rheumatoid arthritis, for feeble health predisposes to that affection.
Finally, many of the difficulties connected with this subject are reasonably met by Hutchinson's241 doctrine that there exists a state of tissue-health which is transmissible by inheritance, which involves liability to inflammations of joints and fibrous structures, and upon this arthritic diathesis as a foundation may be built up, under the influence of special causes, a tendency to gout, rheumatism, or any one of their various modifications or combinations.
241 Trans. International Med. Congress, ii. 95; Guéneau de Mussy's chap., "De la Diathèse Arthritique," Clin. Méd., 1874, t. i. 317-338.
Hutchinson has demonstrated that gout is often followed by rheumatoid arthritis, the lesions characteristic of both affections coexisting in the same joint. Charcot and Cornil had previously observed the same [p. 90]thing.242 Acute and perhaps chronic rheumarthritis have sometimes preceded rheumatoid arthritis. If a predisposition, inherited or acquired, to rheumatoid arthritis exist, the occurrence of gouty or rheumatic irritation in the joints may suffice to induce the peculiar form of disturbance characteristic of the rheumatoid affection, just as injuries sometimes develop the partial form.
242 Mémoires de la Société de Biologie, 1864.
There is a group of conditions affecting the sexual functions and organs of women which appear to be specially connected with the general peripheral form of rheumatoid arthritis. The disease follows pregnancy, and specially frequent pregnancies, protracted lactation, and various disorders of menstruation. The latter influence obtained in ten out of eleven instances of the disease met with in girls under eighteen by Fuller.243 The frequency of the disease about the period of the menopause has been already mentioned. Todd noticed its coincidence with dysmenorrhoea. Ord in an able and original paper244 has lately dwelt upon ovario-uterine disorder or irritation as a frequent active cause of the disease, having in his opinion met with 33 instances of the kind. The relationship between these various conditions of the functions and organs of generation and rheumatoid arthritis cannot be regarded as settled. Garrod supposed that such conditions, by causing debility, predisposed to the articular disease. Todd, an ardent humoralist, held the nexus between the two to be unhealthy secretions of the uterus, leading to blood impurity; while Ord has ably defended Remak's view that a direct influence of the nervous system is the real link of relationship. It seems necessary to remark that mere coincidence may play a large rôle in the explanation of many of these cases. In 17 at least of Ord's 33 cases the conditions stated by that author cannot safely be adduced as anything more; and it is probable that they would be found present in much the same proportion in any other chronic painful disease of women.
243 Loc. cit., 335.
244 Brit. Med. Jour., i., 1880, 151-153.
Scrofula and phthisis are regarded by Charcot, Cornil, and Garrod as frequent antecedents of rheumatoid arthritis: the first had several times seen white swelling in youth, followed by nodular rheumatism in later life;245 and Fuller found that 23 out of 119 victims of rheumatic gout had lost a parent or one or more brothers and sisters by consumption.246 Chlorosis has several times preceded rheumatoid arthritis. When the prevalence of scrofula, phthisis, and chlorosis is borne in mind, it will not appear strange that they should frequently be found amongst the antecedents of rheumatoid arthritis, without inferring any other relationship between them. Gonorrhoeal rheumatism has also occasionally preceded rheumatoid arthritis, but Ord and Hutchinson are probably correct in regarding that affection as a variety of rheumatoid arthritis.247
245 Loc. cit., p. 208, foot-note.
246 Loc. cit., p. 334, foot-note.
247 Trans. International Med. Congress, vol. ii. p. 92; Brit. Med. Jour., 1881, p. 158.
Cold, especially when prolonged and associated with dampness, is commonly held to be the most common cause of general rheumatoid arthritis. A protracted residence in low, damp dwellings, deprived of the sun's rays and of a free circulation of air, is a condition thought most favorable to the provocation of this disease, perhaps years after the condition has been done away with.
[p. 91]Poverty and all that it implies are at least frequent antecedents of the disease (hence one of its epithets, arthritis pauperum), as are other debilitating influences, such as night-watching, insufficient food, mental worry, grief, anxiety, etc. Be it remembered, however, that the disease is frequently observed in the well-to-do, who live in dry climates and warm houses, are well fed, and want for nothing; so that the external conditions first mentioned are not essential causes of the disease, and many of them may act merely as adjuvants.
Direct injury of a joint from a blow, a fracture, a whitlow, etc. may sometimes induce a local rheumatoid arthritis, which may subsequently become multiple and involve several articulations more or less symmetrically.248
248 Vide Charcot's and Ord's cases, loc. cit.
The partial form presents some peculiarities of causation—thus: it occurs chiefly in advanced life (senile arthritis), much less frequently in middle life, very exceptionally in the very young. Men are much more liable to it than women. It is chiefly this variety which follows injuries, blows, dislocations, pressure, etc., and the disease may then be limited to the injured joint and be monoarticular, or it may extend and become polyarticular, or rarely, as in Ord's case, even general. This monoarticular form appears to be sometimes induced by other local irritations of the articular structures than those following traumatic influences; and as foreign growths in joints and gouty irritation may respectively induce the lesions indicative of rheumatoid arthritis, so, it is probable, may simple chronic rheumatism; and this may be the true relationship existing between these several affections. It is doubtful at present whether purely local irritation or injury of a joint can originate the alterations belonging to rheumatoid arthritis—that is, in the absence of all predisposition to that disease or of the arthritic diathesis. Cold and dampness are generally admitted to be causes of the partial form, but the evidence on this point is not altogether satisfactory. It may be that chronic articular rheumatism is induced by the prolonged operation of damp cold, and that the prolonged rheumatic irritation, aggravated by constant use of the joint and by occasional violence, ultimately superinduces the profounder alterations characteristic of arthritis deformans. It appears highly probable that if the predisposition exist, any long-abiding irritation of a joint, whether the result of violence or disease, may ultimately originate the alterations of the cartilages and bones which obtain in rheumatoid arthritis.
As regards the etiology of Heberden's nodosities, and their relation to other affections of the joints, the following summary must suffice: They obtain chiefly in advanced life, but do occur rarely in the young; they are probably somewhat more frequent in women than in men; although more frequently seen in the upper classes, the poor are not exempt from them, no doubt because they are specially exposed to slight but oft-recurring injuries of their digits, such traumatism being an exciting cause of the disease, especially when confined to a single joint. The affection is sometimes hereditary; both it and the general or the partial forms of rheumatoid arthritis may coexist in the same family and even in the same person. The alterations in the joints are identical with those found in the general variety of rheumatoid arthritis, and exist without [p. 92]deposits of urate of soda (Charcot). It resembles the general form of the disease just mentioned in its tendency to involve many symmetrical articulations at the same time, and the partial form in the rarity with which it extends beyond the joints first attacked. While Heberden's nodosities, as Haygarth taught, do occur independently of gout and the gouty habit, I believe with Begbie249 and Duckworth250 that in some persons they are evidences of gout or the gouty diathesis.
249 Contributions to Practical Med., 1802, p. 29.
250 "On Unequivocal Gouty Diseases," St. Bartholomew's Hospital Reports, vol. xvi., 1880, p. 190.
Quite recently a woman aged forty-eight consulted me with these nodosities beginning upon the last joint of the fingers, while she was the subject of vesico-renal irritation and was passing free uric acid in the urine. Hutchinson has twice seen them in combination with a peculiar insidious and painless inflammation of the iris and vitreous body, which occurs in the children of the gouty, yet such children have no deposits of lithates in their joints, nor any lithiasis, nor acute paroxysms of true gout, and he considers that "the last joint arthritis is to be regarded as in part gouty, and in part a kind of articular chilblain."251
251 Trans. International Med. Cong., ii. p. 96.
Lastly, in some instances they are no doubt the hybrid offspring of an inherited tendency to both gout and rheumatoid arthritis.
No more important principle in pathology exists than has been of late years insisted upon, especially by Jonathan Hutchinson and in his recent lecture by Sir James Paget252—to wit, that "by inherited dispositions, accumulating and combining or converging in definite proportions, new diseases may be developed and old ones be variously modified."
252 Lancet, ii., 1882, 1017-1021.
The pathogenesis of rheumatoid arthritis is the subject of differences of opinion very like those existing in regard to acute articular rheumatism. The weight of evidence is in favor of its diathetic relationship to rheumatism; and the doctrine of an arthritic diathesis and of the operation of the causes of the disease through the nervous system appears to be specially applicable to it, with less difficulty than to acute rheumatism, and the probability of a specific germ being its true cause is very remote. What seems to be necessary in addition to the preceding is, that the causes shall be more persisting and oft-recurring, so as to maintain a prolonged local irritation of the articular tissues, or that the neuro-arthritic diathesis shall be highly developed. Under these conditions the prolonged or oft-repeated application of cold and damp to the peripheral nerves, severe or oft-repeated slight injuries to joints, urethral or ovario-uterine irritation, chronic gout or rheumatism, or even, exceptionally, an attack of the acute form of these diseases, may originate rheumatoid arthritis; and all wearing influences, such as anæmia, excessive menstruation, prolonged lactation, innutrition, failing health, mental anxiety, or shock, etc., act as adjuvants in the development, aggravation, and maintenance of the articular disease.
DIAGNOSIS.—It is perhaps not possible to distinguish with certainty either the acute or the chronic form of rheumatoid arthritis from subacute or chronic rheumarthritis respectively before the characteristic deformities of the former affections have appeared. Acute rheumatoid arthritis, which is comparatively rare, may be said to exist, rather than subacute [p. 93]articular rheumatism, if the disease affect early and chiefly the smaller joints of the hands and feet alone or along with some of the larger articulations, especially the sterno-clavicular or the temporo-maxillary; if the effusion into the joints be abundant; if inflammation persist in the articulations first involved, notwithstanding the invasion of other joints; if the heart escape; if the patient be a female who is constitutionally delicate, or has borne children rapidly, or is the subject of disordered menstruation, or has been attacked soon after childbirth or during lactation;—finally, if, on cessation of the attack, one or more of the joints remain swollen and permanently enlarged and impaired in function. The coexistence of iritis, or a history of a previous attack of that disease not attributable to syphilis or gout, would strengthen the above view.
Precisely the same considerations serve to distinguish chronic general or polyarticular rheumatoid arthritis from chronic articular rheumatism, with the following qualifications: endo- or pericarditis is not of frequent occurrence in chronic rheumatism, so that this distinction is not available, and chronic rheumarthritis of long standing does sometimes impair the movements of the joints, and even produce slight alterations in them. However, it does not, as a rule, involve so many joints as rheumatoid arthritis; it is less symmetrical in its distribution, and much less prone to implicate the sterno-clavicular, the temporo-maxillary, or the vertebral articulations. Nor does it cause removal of the articular cartilage, enlargement of the heads of the bones, and the formation of osteophytes around them, and of loose bodies in the articulations, together with marked deformities and luxations of the joints. A history of a remote or recent attack of acute rheumarthritis or of chorea, or the presence of chronic valvular disease, would strongly indicate the simple rheumatic nature of the case.
The partial form of rheumatoid arthritis can with even less certainty than the general be distinguished from chronic articular rheumatism before the characteristic alterations of the joints have been developed, more especially as it is sometimes a consequence of gouty irritation and probably of chronic rheumatism. Chronic arthritis following a traumatic cause, and persisting obstinately in the injured joint is probably rheumatoid, if not strumous, gouty, or periarthritic.
But before definitely deciding it will be prudent to await the development of some of the characteristic alterations of structure appertaining to rheumatoid arthritis. An affection of the shoulder frequently occurs which resembles in many respects rheumatoid arthritis, and has been well described by Simon Duplay253 and W. Pepper.254 It usually follows an injury, such as contusion, sprain, etc., of the joint, but may be spontaneous; it is unattended by swelling or deformity. Its early symptoms are pain on pressure of the shoulder a little below the outer border of the acromion, and especially behind it and at the coracoid process, also about the insertion of the deltoid and below the acromion during movements of the joint, especially when the arm is raised from the side or rotated inwardly; early restriction of these movements, which increases till a fibrous ankylosis becomes established and scapula and humerus move together as one piece, motion between those bones no longer existing, and forcible attempts to produce it giving great pain, and sometimes producing [p. 94]crepitus in or about the articulation; sometimes early numbness and pain down the member to the hand in the course of the ulnar, internal cutaneous, or the radial nerve; vicious and painful semiflexion of the elbow; after a time wasting of the group of muscles which move the shoulder-joint. Although usually monoarticular and of traumatic origin, I have seen it affect first one and then the other shoulder in the absence of any known injury, and beginning like a neuritis or a neuralgia of the scapulo-humeral nerves. Duplay, however, regards it as a periarthritis. It may be distinguished from the rheumatoid arthritis by the absence of effusion into or enlargement of the articulation, and of deformity of the bones; by the early restriction of the movements and the rapid development of adhesions which fix the articulation; and by the curability of the disease.
253 Archives Générales de Méd., Nov., 1872, pp. 512-542.
254 Archives of Med., Oct., 1880.
The articular affection of locomotor ataxia sometimes closely resembles monoarticular rheumatoid arthritis,255 but may be distinguished by its sudden invasion, often without pain or fever; the prompt development of a general and often enormous tumefaction of the entire member, with copious effusion into the joint; the early destruction of the articular cartilages, the rapid wearing away of the heads of the bones, and the proneness to spontaneous fracture of their brittle shafts; the prompt absorption of the articular effusion, followed by a relaxed state of the ligaments and a facility of dislocation; the early occurrence of the articular affection, when motor inco-ordination is scarcely developed, and its frequent association with the crises of ataxia or the presence of some of the other symptoms of that disease. The importance of these facts will be especially evident in those examples of ataxic articular disease in which, at an advanced stage, eburnation and deformity of the articular surfaces, with the formation of loose bodies and osteophytes, are observed, just as they are in arthritis deformans.
255 Charcot's Lectures on Diseases of the Nervous System, Syd. Soc., 1877; Archives de Physiologie, t. i., p. 161, 1868; ibid., xi., 1869.
Articular disease closely allied to what occurs in locomotor ataxia is now and then observed in the early stages of progressive muscular atrophy,256 but while the large joints, more particularly the knee and the shoulder, suffer in the former affection, the phalangeal chiefly and the larger articulations more rarely are attacked in the latter. Of course the peculiar symptoms of progressive muscular atrophy coexisting with those of the articular affection would serve to distinguish the latter from rheumatoid arthritis.
256 Remak, Allgem. Med. Central. Zeitung, March, 1862; Rosenthal, Clinical Treatise on Diseases of the Nervous System, translated by L. Putzel, M.D., 1879, p. 286.
It is often very difficult to say whether a given case is one of chronic rheumatoid arthritis or of chronic gout; and there is no doubt that in England, where gout prevails, it is not unfrequently associated with rheumatoid arthritis, sometimes preceding and even causing it, much more often following it, for the one does not exclude the other.
While rheumatoid arthritis most frequently begins in the hand, and is usually symmetrical and bilateral, gout commonly begins in the lower extremities, and especially in the metatarsal joint of the great toe, and of one foot only. Chronic gout is far more frequently preceded by attacks of acute gout than chronic rheumatoid arthritis is by the acute form of that affection; a history of inherited predisposition, of indulgence in the [p. 95]use of wine, ale, porter, and of animal food, of deficient bodily exercise, with perhaps great mental occupation or anxiety, of recurring gouty dyspepsia or of a tendency to lithiasis, would indicate gout, while the absence of these and a history of frequent exposure to cold and wet, of injury to the joint, of previous exhausting disease or drain, of impaired health, debility, or poverty, would strongly imply rheumatoid arthritis. Gout is especially observed in males over thirty, and very rarely in children; general rheumatoid arthritis is chiefly a disease of females during menstrual life, and occasionally occurs in children of either sex.
The partial form is, like gout, chiefly a disease of men, but occurs generally at a more advanced age than gout. Even chronic gout is more or less paroxysmal, with distinct intermissions; chronic rheumatoid arthritis is more or less abiding and progressive, with only remissions in its course and severity; the former is frequently associated with chronic renal disease, the latter is not. The urate-of-soda deposits about the articulations in gout appear as more or less round or ovoid swellings in the close vicinity of the joints, but not observing their exact level or their general form; softish when recent, they never acquire a bony hardness, and are nearly always capable of slight lateral movement. The skin covering them is frequently stretched and glossy, and may exhibit white spots of urate of soda. The articular nodosities in chronic rheumatoid arthritis are actual osseous enlargements of, or outgrowths from, the articular surfaces, forming part of them, immovable and conserving more or less their form. The integument covering the nodosities is not glossy or dotted with chalk-like specks. The several types of deformity of the fingers previously described, and mainly produced in rheumatoid arthritis by muscular contractions and altered shape of the articular surfaces, are not seen in gout. Finally, if chalk-like concretions are visible in the ears, joints, or finger-ends, or if the blood contain uric acid, gout is present. While rheumatoid arthritis and chronic gout occasionally coexist in the same patient in England, in Canada, where the latter disease is comparatively rare and the former quite common, the writer does not remember to have observed such coexistence.
Besides the acute syphilitic disease of the joints already alluded to as occurring in children (inherited), a chronic arthritis is observed in the adult amongst the very late lesions of syphilis. It is usually monoarticular, affects the larger joints, especially the knee, and may originate either in the synovial membrane or in the bone and periosteum. In syphilitic synovitis the history of the case, the existence occasionally of soft gummy tumors in the periarticular tissues and of hydrarthrosis, the trivial degree of pain and tenderness, the insidious invasion and chronic course of the affection, and its prompt relief by antisyphilitic remedies, will indicate the nature of the case.
When it originates in the bone and periosteum, although the invasion may be prompt and the pain at first severe, the latter usually moderates greatly and becomes nocturnal, and the articular surfaces present localized rather than general enlargement (hyperostosis); nodes often coexist; effusion is moderate, unless the synovial membrane is also involved, and full doses of iodide of potassium will soon afford relief.
PROGNOSIS.—In the polyarticular form the course varies much more than is commonly believed, and the disease must not be regarded as necessarily [p. 96]progressive and incurable. When it occurs in young persons, and in children more especially, although it may suffer exacerbations and remissions for a few years, yet arrest of the disease and recovery of the functions of the joints, sometimes with very little deformity, now and then take place under suitable management. Quite recently a man of thirty-two consulted me about a vesical affection who from the age of eight had suffered every winter for twenty years from rheumatoid arthritis in his hands and feet, and finally in the knees. Yet when seen by me he had been free from pain in his joints for three years, and, although they were somewhat deformed, their movements were remarkably free and painless.
Several of my younger patients while bearing children rapidly and nursing them have had the disease in their hands or hands and wrists; exacerbations have recurred during subsequent lactations, and yet the disease has either become arrested or progressed very slowly and at long intervals. It is admitted, however, that these are all exceptional cases, and that the tendency both of polyarticular and of the monoarticular forms is to progress, and, either steadily or at intervals and by recurring attacks, to permanently deform the joints and impair their movements. Even under these circumstances, however, the patients may suffer little pain unless when forcible movements of the articulations are attempted.
On the other hand, while the disease cannot be regarded as curable under the employment of drugs, very much can frequently be done, especially in the polyarticular form, to relieve the suffering and to retard, if not arrest, the progress of the disease, and even to restore sometimes very considerably the functions of the joints. Neither of these forms of rheumatoid arthritis can be said to be dangerous to life, and they often exist ten or twenty years and more without seriously injuring the general health. Heberden's nodosities are incurable, but they are little more than deformities.
TREATMENT.—The treatment of rheumatoid arthritis is, as a rule, disappointing, and perhaps no affection requires more perseverence and self-reliance on the part of the physician or more hopeful resolution on that of the patient. Our first duty is to make an exhaustive search as to the probable cause of the disease, as its removal is an important step in the treatment of the affection, although such search is frequently futile, and many of the alleged causes may, after all, be mere antecedents or coincidences. However, inasmuch as the pathology of the disease is very obscure, any abnormal condition of organ or function that may be discovered should receive strict and prompt attention, lest it should, either through disturbed innervation or malassimilation or impaired nutrition or defective excretion, be the predisposing or exciting cause of the disease. In women the most careful inquiry should be made into the state of the ovario-uterine organs and functions, and the least departure from their norm should be at once treated. Deficient, excessive, or painful menstruation, leucorrhoea, ovarian irritations, or pain, even displacements of the uterus or ovary, should be corrected as soon as possible. Repeated pregnancy and prolonged lactation, recurring mental anxiety and physical fatigue, defects of diet, want of food, of sunlight, and of good air, residence in damp dwellings, occupations involving exposure to cold and wet, are conditions supplying important indications which too often are [p. 97]beyond the control of the physician, although they peremptorily require his attention. The general form is often met with in anæmic persons and in those of impaired health and vigor, and probably very rarely occurs under opposite circumstances; and there is a consensus of opinion that a lowering system of treatment is contraindicated in rheumatoid arthritis.
Having efficiently set about correcting or removing these various predisposing or determining causes of the disease, we next direct our care to the disease itself. The remedies which had been found most useful in rheumatoid arthritis before the introduction of salicylic acid were cod-liver oil, quinia, iodine, iron, arsenic, and various mineral waters, employed either externally or internally, usually in both ways. Judging from my own late experience and from the results obtained by Sée257 and other French physicians, as communicated by Jules Compagnon,258 sodium salicylate, given in sufficient doses, promises to be more generally useful in the more acute forms or in the actively inflammatory periods and exacerbations of the disease than any of those agents. Including Sée's cases, Compagnon has related 17 examples of rheumatoid arthritis, most of them of the general progressive form, in which great improvement as regards pain, stiffness, swelling, and even deformity, followed promptly the employment of that salt, even after the failure of other remedies. It proved signally useful recently in a rebellious chronic case of my own. Pollock has lately published an instance in which 5 grains of salicylate of quinia three times a day were in three or four days followed by great relief.259 The testimony already given of Dr. J. T. Eskridge as to the great value of this salt in chronic rheumatism will be held by some to be corroborative of its value in rheumatoid arthritis. It is hardly necessary to say that it often fails in this intractable disease, but it has frequently relieved the pain and swelling and arrested the progress of it, at least for the time, even when alkalies, iodine, arsenic, baths, etc. had failed.
257 Bullétin de l'Académie de Méd., Paris, t. v., 2d Serie, 1877.
258 De l'Utilite du Salicylate de Soude dans le Traitement du Rheumatisme, par Jules Compagnon, Paris, 1880.
259 The Lancet, ii., 1882, 141.
It is probable that less than 45 grains per diem of the sodium salt is of little value in even the most chronic forms, and that the quantity requires to be increased in proportion as the febrile symptoms are active, so that a drachm and a half or two drachms may need to be administered in the day to some persons. It should be given in divided doses at intervals of two hours, and, what is of primary importance, it should be continued for a long time, even after much improvement has resulted, and should be resorted to from time to time, especially during recurrences of the pain, heat, or swelling. It is of consequence, especially in elderly patients, to ascertain that the medicine is being promptly eliminated by the kidneys and to watch its effect upon the heart. The administration along with it of a little old rye whiskey or brandy will sometimes be necessary in feeble people. In those rather common cases in which the skin is inactive and perhaps harsh the salicylate often improves that important organ of oxidation and elimination, and should it not do so the addition of the ammonium carbonate may be tried, especially in feeble persons with weak hearts.
[p. 98]Moreover, the other drugs which sometimes prove serviceable in this disease may be given at the same time or alternately with the salicylate, or instead of it if it is not found to be of use or is not tolerated. In chronic cases a prolonged course of cod-liver oil, alone or along with malt extract, often seems to be of real service, especially when nutrition is much impaired or when the patient is the subject of acquired or inherited struma. Iodide of potassium, in combination with quinia or other tonic, will often prove signally useful in chronic cases unaccompanied by pyrexia, in which the pains are worst at night. It should be first tried in moderate doses (5 to 8 grains), and be continued for a long time with occasional intermissions, and before discarding it from disappointment—which often arises—15- to 20-grain doses may be given tentatively for a short period. Milk or coffee or Vichy water are good vehicles for its administration. Whether free iodine in the form of the tincture, so highly spoken of by Laségue,260 acts as well or better than the iodide of potassium is doubtful. He gave it at meals, in doses progressively increased from 10 drops to 5 or 6 grammes twice a day, in sherry or sweetened water, and persevered with it for a long period. Garrod has had many restorations to health in severe forms of this disease from the persevering employment of the syrup of the iodide of iron. The iron in these preparations may deserve as much commendation as the iodine, for it has often proved signally useful in this disease, not alone on account of the anæmia which so frequently attends it, but through its beneficial influence upon the nutritive functions and the circulation.
260 Arch. Gén. de Méd., 1856.
The usual rules regulating the employment of iron are to be observed, and the condition of the digestive organs will demand special attention during its employment. Although the influence of arsenic upon rheumatoid arthritis is not uniform, yet as it sometimes proves really useful261 it should be tried. Like iron, it may prove beneficial in several ways—by improving the quality of the blood, promoting the circulation in the superficial layers of the skin, or exerting some influence upon either the nerve-centres or perhaps upon the vaso-motor nerves of the cutaneous or articular tissues. The last-mentioned suggestion is favored by the circumstance noted by Charcot—viz. that the first effects of arsenic in nodular rheumatism are often intensification of the articular pains, and sometimes the production of redness and swelling where they did not exist before. That author found arsenic without effect or injurious in very inveterate cases and when the disease had appeared at an advanced age. Five to ten minims of Fowler's solution, or of the solution of the arseniate of sodium, which is perhaps less irritating than the former, should be given immediately after meals, and its effects upon the gastric and hepatic functions carefully watched. De Mussy has highly recommended arsenical baths (drachm ss-ij of arseniate of soda to 30 gallons of water), but as the arsenic is not absorbed by the unbroken skin, any improvement which may follow its employment is probably owing to the temperature of the bath or the bath itself.
261 As to the value of arsenic in rheumatoid arthritis, see Bardsley's Medical Reports, London, 1807; Begbie, Edin. Med. and Surg. Jour., 1858; Fuller, lib. cit., p. 362; Garrod, lib. cit., 3d ed., p. 534; Guéneau de Mussy, Bull. de Thérapeutique, t. lxvii., 1864, p. 24; Charcot, lib. cit., p. 222.
A similar remark has been made respecting the value of the various [p. 99]thermal mineral baths, natural and artificial, so much employed in this disease.262 It is neither the nature nor proportion of their mineral ingredients, but the degree of temperature, which constitutes the essential point in the action of a bath. This, if true, explains the almost equal reputation of the many varieties of thermal mineral springs in the treatment of rheumatoid arthritis and chronic rheumatism. It is this that permits the physician to promise the poor patient as much benefit from the employment of hot baths of simple water as of those of New Zealand, Plombières, or Arkansas.
262 Vide Niemeyer, Text-Book Pract. Med., N.Y., 1867, p. 488; Traitement du Rheum. par les bains à haute temperature, par Ch. Aug. Bouther, Paris, 1878.
The time for a resort to hot baths in rheumatoid arthritis is when the very violent pains have subsided sufficiently to allow of their employment; and while they may be hopefully used in the most chronic and advanced cases, the earlier they are employed the more curative they are. The temperature of these hot baths need not, as a rule, exceed 95 to 100° F., although some authorities approve of raising the temperature to 110° or 112° while the patient is in the water. A series of twenty to thirty such baths, taken every second day for ten to twenty minutes, is sufficient for one trial, and often effects very great improvement in the disease. The aggravation or return of pain in the joints which often follows the employment of warm baths will cease after the fifth or sixth bath. Garrod's experience of the Turkish bath is not favorable; it very often does much mischief by causing debility, and its excessive use has induced rheumatoid arthritis in persons previously free from the disease.
Now, while it may be true that simple hot-water baths employed at home are as good as mineral thermal baths taken at their source, it is generally admitted that it is best to send persons who can afford the expense to the springs themselves, where they may drink the waters as well as employ them externally, and at the same time secure all the advantages arising from change of habits, scene, and climate, from restriction to a proper diet, and from the systematic employment of the waters and baths under the direction of persons experienced in their administration, etc. No reliable rules can be laid down for the selection of the mineral waters best adapted to each case: the stronger alkaline waters perhaps had better be used with great care, such as those of Carlsbad, Vichy, Mont Doré, Weisbaden, and after a course of thermal mineral baths at such places as Aix-les-Bains, Wildbad, Bath, Aix-la-Chapelle, etc., Garrod advises resort to some place where the air is bracing and the waters tonic or chalybeate, as Buxton, Spa, Schwalbach, or St. Moritz. In this country good results are often obtained at the Hot Springs of Arkansas and the Hot Sulphur and the Lithia Springs of Virginia. The use internally and in the form of hot baths of the mineral springs of Saratoga, of Michigan, of the Licks of Kentucky, and of California, of St. Leon and St. Catherine's (Canada), is frequently very beneficial. In the selection of the mineral waters to be drunk, and of the temperature and other qualities of the baths to be employed, careful attention must be paid to the condition of the functions of the skin, liver, kidneys, and nervous system; but space cannot be afforded here for the consideration of this extensive topic. Moreover, it occasionally happens that after failure of [p. 100]sulphur or alkaline baths some other form may succeed, as the vapor or hot-air, or tepid or very hot-water bath. If decided benefit follow the first series of baths, recourse should be had from time to time to a fresh series, even for several years, in obstinate cases. Mud and peat baths are much valued in Germany, although they do not always agree with weakly or aged people.
The local treatment is of equal importance with the general, and it is not unfrequently more effective in restoring the functions of the articulations. In that rare variety, acute rheumatoid arthritis, attended with much pain and heat in the joints, perfect rest in bed is called for, together with other measures adapted to subdue the inflammation and allay the pain. Compresses wet with warm water, rendered anodyne by the addition of laudanum or belladonna, or both, and covered with oiled silk, suit some cases—light linseed poultices, applied moderately warm and extending considerably beyond the limits of the articulation and covered with gutta-percha or oiled silk, in others. As the pain and local heat subside, the tincture of iodine may be applied extensively, or blistering-fluid over limited areas above and below the affected joints, but not on them until the inflammation has very much abated and is becoming chronic. These simple methods should be employed assiduously and be aided by appliances to secure actual rest of the inflamed joints. In the chronic variety complete rest is not needed unless during the acute exacerbations, but the movements should be at first somewhat restrained and be regulated by the effects produced. But the severe pain experienced during the movements must be borne; it will subside promptly. Decided increase of pain and heat in the part, lasting many hours, would indicate more reserve in the use of the joints. It is frequently very difficult to determine when and to what extent movement may be permitted in this disease. No fixed rule can be laid down of universal application, but it may be stated that in proportion as the local disease becomes indolent and inactive may pressure and active movements of the joints be resorted to, for they then have a beneficial influence in preventing stiffness, contraction, and deformity. Indeed, in my opinion it is not wise to delay these movements long even in subacute cases. The editor of this work has especially insisted upon the importance of systematic daily movements of the affected joints as the most essential part of the treatment,263 "combined with thorough massage of all the muscles whose functional activity is impeded and impaired."
263 "Some Practical Remarks on Chronic Rheumatism," by Wm. Pepper, M.D., Archives of Medicine, Oct., 1880.
The abiding chronic inflammation indicated by local heat, swelling, and inflammation of the affected tissues may be variously treated. The joints may be thoroughly fomented with tolerably hot water or by means of the local vapor bath for half an hour, morning and night, and then be gently rubbed for ten or fifteen minutes with iodine or weak mercurial ointment or with the compound camphor or acetic turpentine liniment, or, if these are too stimulating, with some bland oil, such as cod-liver or neats' foot or cocoa oil, after which should be applied hot-water compresses or linseed poultices or a wrap of soft cotton wool covered with oiled silk and secured by an elastic, moderately tight roller. If these means prove inefficient and the inflammatory process grow more [p. 101]indolent, counter-irritants may be conjoined with or substituted for them. Small fly blisters or strong iodine paint may be applied close to the joints, or the ordinary iodine tincture may be brushed over them, or the above ointments or liniments and one of the bland oils may be more forcibly rubbed in. The prolonged rubbing of these stiff, swollen joints with oil is not valued as much as it deserves.
Compression of the thickened tissues by means of a thick envelope of cotton wool and thin flannel or rubber bandage sometimes acts very well, probably by reducing the amount of blood and interfering with cell-growth or promoting cell-degeneration. Hot sand-baths to the affected joints are sometimes useful.
These several measures should be perseveringly applied, and in proportion as chronicity prevails the active and passive movements of the articulations and massage of the muscles and adjacent tissues should be daily and efficiently practised.
Electricity will often be found an important adjuvant in this as well as in an earlier stage, not only in improving the nutrition of the muscles, but in promoting absorption, allaying pain, and subduing excitability of the peripheral structures, removing muscular contractions, and probably modifying the local inflammatory processes. It appears also in some cases to improve the general health. The constant current is generally the most useful, and should have an intensity of about ten to fifteen milliampères, and be applied daily for ten or fifteen minutes. The positive pole, terminating in a large flat moistened sponge, is applied to the spinal origin of the brachial or lumbar plexus, according as the superior or inferior members suffer, while the negative pole is immersed in a vessel of warm salt water in which the hands or feet are placed. Some apply the negative electrode to the joints and the positive to the limb higher up.264 The faradic current may also be employed on account of its action upon the muscles and small vessels. In the advanced stage attended with marked thickening of the articular and periarticular tissues, with contractions of the muscles and greater or less impairment of movement, the above measures are still our chief resources; but they may be employed more vigorously. We have little fear now of lighting up inflammation; we indeed desire to excite a more active circulation in the part with a view of removing the congested state of the capillaries and venules, so favorable to the development of fibroid growths. In this stage especially vigorous active and passive movements of the affected joints, and massage of the muscles which move them, and gymnastics, are imperatively needed, and it is sometimes almost marvellous what an amount of mobility and usefulness may thereby be restored to apparently helplessly crippled and deformed articulations and members. Persons who have not walked for years are frequently so much improved as to be able to leave their sofa or bed, and with or without crutches or mechanical aids walk about, while their abiding pains depart, and this notwithstanding the permanent deformity of the articular surfaces. (For the various mechanical appliances that are sometimes necessary in this advanced stage works upon surgery may be consulted.)
264 Homolle, lib. cit., p. 710.
The hygienic measures to be observed are probably very much the [p. 102] same as those indicated in the article upon simple chronic articular rheumatism—some of them at least—and are such as may be inferred from a review of the exciting causes of rheumatoid arthritis. Be it remembered also that acute and chronic articular rheumatism appear amongst the causes of that disease. We are hardly justified in promising arrest of the disease on removal to a warm, dry, and even climate; yet wealthy patients need not be dissuaded from trying the experiment. The use of flannel underclothing and the employment of tepid or even moderately cool baths, followed by the use of the flesh-brush or rough towel, are important means of protecting persons predisposed to this disease. The ordinary hygienic laws adapted to maintain a healthy state of all the functions, mental as well as physical, are to be observed, for in this disease the influence of the mind over the body is shown by the frequency with which rheumatoid arthritis follows closely upon mental shocks, worry, etc.
The diet, it is generally admitted, should be of a nutritious character, yet plain and digestible, and, unless specially required to meet certain indications, should not include heavy wines or fermented liquors. However, Garrod affirms that uncomplicated rheumatoid arthritis is not aggravated by the use of porter, ale, or sound wines; and his rule is to give sufficient of these alcoholic beverages to support the tone of the whole system, but not enough to excite the circulation and thereby produce subsequent reaction.
Finally, the above system of treatment must be persisted in year by year with the object of securing arrest when cure has not been effected.
SYNONYMS.—Arthrite ou Arthropathie blennorrhagique, Tripper-rheumatismus, Gonocele, Urethral Rheumatism, Urethral Synovitis.
ETIOLOGY.—As its name implies, the cause, par excellence, of the disease is gonorrhoea, as was perhaps first indicated by Selle265 and Swediaur,266 although, no doubt, an affection apparently identical is rarely observed associated with non-contagious urethral discharge and with the urethral irritation incident to catheterism and to stricture. I have seen it associated with a simple mucous urethral discharge in a man of gouty habit, married and free from the suspicion of specific infection. Such discharge has been attributed to gouty irritation, to dietetic and venereal excesses, and to the contact of non-specific vaginal secretion; and such origin is well established. More than one observer has noticed a susceptibility to urethritis on the part of persons who have had gonorrhoeal rheumatism. A gouty taint is undoubtedly often present in urethral rheumatism. These non-gonorrhoeal cases require more close investigation than they have received.267 Fournier has not met with them.268
265 Chr. Th. Selle, Medicina Clinica, oder Handbuch der Medicin, Berlin, 1781.
266 Swediaur, London Med. Gaz., 1781.
267 See Elliotson, "Non-contagious Urethral Rheum.," Med. Times, i. 60, p. 613.
268 Fournier, Nouv. Dict. de Méd. et de Chir., t. v. p. 228.
The stage of the gonorrhoea at which the articular affection may appear varies very much. It frequently sets in from the sixth to the sixteenth day of the discharge; it is common enough between the third and sixth or twelfth weeks, and may be delayed as late as the twelfth month. There [p. 103]is no constant relation between the severity of the urethral inflammation and the frequency with which, or the time at which, the articular symptoms arise; and these, once established, appear to be largely independent of the state of the urethra. On the advent of the joint affection the discharge usually continues as it was, although it often abates somewhat. Fresh attacks of gonorrhoea, even when very mild, often develop new invasions of the articular affection, as though an idiosyncrasy existed.
While the ordinary exciting causes of simple acute articular rheumatism are not necessary to the production of gonorrhoeal rheumatism, they do now and then act as adjuvants. Such are cold, fatigue, and injuries of the joints, and a severe acute arthritis is not infrequently developed during gonorrhoea under such circumstances. Other predisposing influences probably exist, the absence of which in some measure explains the infrequency of gonorrhoeal rheumatism as compared with the prevalence of gonorrhoea. Besnier holds that constitutional rheumatism, the arthritic habit, or l'héredité arthritique, is not infrequently present in the victims of gonorrhoeal rheumatism as a predisposition; Nolen269 found an inherited rheumatic predisposition in 6 out of 88 cases, and that 4 others had had rheumatism before contracting gonorrhoea; and Hutchinson maintains that it is the existence of the arthritic diathesis which enables urethral inflammation to produce gonorrhoeal rheumatism. He says: "From statistics that I have carefully collected I have no hesitation in believing that the predisposing cause of it usually is the inheritance of arthritic tendencies;" and adds, "Very often the subject of gonorrhoeal rheumatism will give a family history of gout." However, the disease often occurs in the absence of any discoverable tendency, hereditary or acquired, to simple articular rheumatism. On the other hand, persons have had one or several attacks of gonorrhoea previously that did not give rise to rheumatism. Nolen's table of 88 cases contains 12 instances of this kind. It is probable that by reducing the resisting force of the organism, scrofula, the so-called lymphatic diathesis, anæmia, and debility favor the development of the disease.
269 "Rheumatismus gonorrhoicus," Deutsches Archiv für klin. Med., Bd. xxxii., 1883.
Gonorrhoeal rheumatism, like gonorrhoea, is proportionally as well as actually much more frequent in men than in women (111 men, 7 women, Nolen); and the greater proclivity of the former has been attributed to the greater delicacy, sensibility, and complexity of the structures involved in them than in women by gonorrhoea.
MORBID ANATOMY.—The lesions of gonorrhoeal rheumatism in the early stage resemble closely those of acute articular rheumatism; and it is probable, for opportunities of ascertaining by actual dissection are very rare, that the synovial membrane chiefly suffers. In more advanced stages the joints contain serous fluid in which fibrinous flakes and numerous leucocytes are found; the cartilages may be eroded and softened; and in some protracted cases even the bones may participate in the inflammation, and the changes found in polyarticular rheumatoid arthritis may be developed. Ultimately fibrous adhesions, resulting in ankylosis, may occur. Suppuration very rarely takes place, and it is probable that in such cases pyæmia is added to gonorrhoeal arthritis.
[p. 104]SYMPTOMS.—Gonorrhoeal rheumatism may attack any of the joints; it most commonly invades the larger at first, more especially the knee; the ankle is next in order of frequency, and then succeeds the shoulder, closely followed by the smaller joints of the hands and feet, which are very seldom affected primarily and antecedently to the larger joints. The temporo-maxillary, the sacro-iliac, the sterno-clavicular, the intervertebral, do not escape gonorrhoeal rheumatism more than they do rheumatoid or pyæmic arthritis.270 The disease most frequently invades several joints simultaneously or successively, but, soon declining in many of them, it finally becomes localized in a few or rarely in a single articulation. It is monoarticular from the first in about 20 per cent. of cases, especially in the knees.
270 Vide Fournier, Nouv. Dict. de Méd. et de Chir. Prat., t. v. p. 230: in 119 cases, knee, 83; ankle, 32; fingers and toes, 23; hip, 16; wrist, 14; shoulder, 12; elbow, 11; temp.-maxillary, 6; etc.
Gonorrhoeal rheumatism presents several clinical forms: First, Arthralgic: pains of greater or less severity, sometimes increased by movement, but unaccompanied by redness or swelling, affect one or frequently several joints; they wander from joint to joint, are liable to exacerbations, and sometimes resist treatment. This form occurs either in a chronic state in the course of an old gonorrhoea, and without other rheumatic symptoms, or as an acute affection along with other rheumatic symptoms, as in the second form. Second: Rheumatic: in this the symptoms are almost identical with those of subacute articular rheumatism or the more active forms of polyarticular rheumatoid arthritis. Several joints are usually implicated, perhaps suddenly, either quite spontaneously or after chill, exertion, or strain, or rheumatic-like pains having been felt for two or three days in the soles, ankles, or loins, the painful joints become moderately swollen, tender, and hot; pyrexia supervenes with its early chilliness, malaise, and anorexia; the temperature is not high; the profuse acid sweating and the very acid, high-colored urine of acute articular rheumatism are not observed or but transiently and to a very slight degree. In a few days the moderate febrile disturbance subsides, but the local inflammation persists, and extends to other joints, without promptly leaving those first invaded; while lingering in all it often fixes itself in one or more joints, and is apt to produce a copious and rebellious intra-articular effusion. Still, it very rarely involves as many articulations as primary acute rheumatism. The periarticular tissues usually are more involved than in subacute or even chronic primary articular rheumatism. Hence the considerable swelling from oedema on the back of the hand or foot, around the knee, behind the elbow, and the copious effusion into the adjoining bursæ and tendinous sheaths, and in the case more especially of the small joints of the fingers and toes the fusiform enlargement and deformities resulting from periostitis of the articular extremities. The pain, deformity, pseudo-ankylosis, etc. produced by these periarticular processes are very persistent and rebellious, and, although they do usually disappear at last, occasionally the inflammatory irritation extends to the cartilaginous and osseous structures, and rheumatoid arthritis with its permanent deformities results. It is perhaps chiefly in this polyarticular form of gonorrhoeal rheumatism that cerebral, spinal, cardiac, pleural, and ocular complications most frequently occur. [p. 105]In the Third form, or Acute Gonorrhoeal Arthritis, after two or three days of pain wandering from joint to joint, a single articulation suddenly, and frequently about the middle of the night, becomes the seat of atrocious and abiding pain, followed in a few hours by very considerable swelling of the articulation, not due chiefly to articular effusion, but to periarticular oedema and enlargement of the bones. The pain and tenderness are most severe at the line of junction of the articular surface; the swelling begins at that point, and extends widely, especially over the dorsal aspects of the wrists and elbows, the joints most liable to this form, although any articulation may suffer. The joint is also hot, it may be pale, but is usually more or less red, and occasionally presents the appearances of severe phlegmonous inflammation, and excites a sensation of pseudo-fluctuation.271 The affection may resolve, or fibrous ankylosis may ensue, or very rarely suppurative destruction of the articulation may occur, although such issue has been denied (by Fournier, Rollet, Voelker). It is remarkable that, like the other forms of gonorrhoeal rheumatism, the acute inflammatory form is not accompanied by a general febrile disturbance at all proportionate to the severity of the local disease. A Fourth form occurs as a Chronic Hydrarthrosis. Although occasionally accompanying the polyarticular variety, it is frequently observed independently, and is then often monoarticular, and affects especially the knee; however, both knees sometimes are involved. The ankle- and elbow-joints suffer much less commonly than the knee. The effusion into the articulation takes place insidiously, although rapidly producing considerable enlargement of and fluctuation in the joint, without local heat, redness, or tenderness, and often with but little or no pain or pyrexia. It is not as often associated with inflammation of the tendinous sheaths and bursæ or of the eye as the polyarticular form, but it is apt to be very slow in resolving, and may last for two or three months, a year, or several years, and in scrofulous patients may degenerate into white swelling. The formation of pus in the joint is very rare. It occurred twice in 96 cases tabulated by Nolen; hydrarthrosis obtained 12 times; and serous synovitis 64 times; chronic rheumatism or arthritis deformans 5 times; tumor albus once.272 A Fifth form of gonorrhoeal rheumatism, like other varieties of so-called secondary rheumatism, involves predominantly the tendons and tendinous sheaths, the bursæ and periosteum, sometimes without, but far more frequently in association with, affection of the joints. Pain, sometimes severe and increased by movement and pressure and aggravated at night, with local swelling and tenderness, are the symptoms. In their fixity and persistence, their tendency to relapse, and their chronic course these periarticular affections resemble gonorrhoeal inflammation of the joints. Gonorrhoeal bursitis is often severe enough to resemble phlegmon, but it does not end in suppuration; it is most common in the bursæ covering the patella, the olecranon, and especially in that under the tendo Achillis and the deep one covering the inferior tuberosity of the os calcis; but any of the bursæ may suffer from gonorrhoeal rheumatism. The periosteum in the vicinity of the affected articulation and over the most prominent parts of the bones is sometimes the seat of small circumscribed firm nodes which [p. 106]are painful and tender, and may either resolve rapidly or very slowly (Fournier).
271 De l'Arthrite aigue d'origine blennorrhagique, par le Dr. André Felix Bieur, Paris, 1881.
272 Loc. cit., p. 133.
Along chiefly with the third form of gonorrhoeal rheumatism, or independently, the various muscles and nerves may be the seat of myalgia and neuralgia. The sciatic nerve is specially liable. In the same form are often met those ocular affections observed not infrequently in rheumatoid arthritis and very rarely in acute articular rheumatism—viz. conjunctivitis and iritis. Aqua capsulitis is more common than the others, according to Fournier. The ocular affections may precede, accompany, or alternate with the articular, and, not being due to direct introduction of the urethral contagium into the eye, are regarded as manifestations or localizations of gonorrhoeal rheumatism. The varieties of erythema sometimes present in primary acute articular rheumatism have been observed in gonorrhoeal rheumatism.
Much difference of opinion obtains as to whether inflammations of the heart, lungs, and serous membranes occur as manifestations or localizations of true gonorrhoeal rheumatism. Even those who, like Besnier, contend for the rheumatic nature of gonorrhoeal rheumatism admit that they are quite exceptional in that affection. Endocarditis is probably more frequent than pericarditis, and the aortic are more liable than the other valves to suffer. Gonorrhoeal endocarditis has been observed without the articular affection, although it is especially when several joints are involved and the pyrexia is well marked in gonorrhoeal rheumatism that the above visceral complications occur. While admitting that Morel,273 Marty,274 Pfuhl,275 and others have reported what appear to have been authentic cases of gonorrhoeal endocarditis, I would remark that it must be almost impossible at times to distinguish a polyarticular acute gonorrhoeal rheumatism from ordinary acute articular rheumatism, and that in other instances the possibility of pyæmia developing in gonorrhoea, and producing both the articular and the visceral lesions, or the latter only, cannot be denied. And the same remarks are applicable to the cerebral and spinal disturbances that Vidart and others have recorded as occurring in gonorrhoeal rheumatism.
273 Rev. des Sciences Méd.
274 Archives générales de Méd., Dec., 1876.
275 Deutsche Zeitschrift für pract. Med., No. 50, 1878.
The course, termination, duration, and prognosis need not be insisted upon after what has gone before. The duration is very variable. Many recover in four to eight weeks, many not for three to six months and longer; relapses are of frequent occurrence; complete and tolerably prompt recovery is not uncommon in first attacks and in young and healthy subjects; rebellious persistency, and even deformity, with impairment of the articular movements, and not infrequently even fibrous ankylosis of one or many joints, sometimes including the vertebral, may be observed. Indeed, the most formidable examples of spondylitis are associated with gonorrhoeal rheumatism as its exciting cause.276 These unfavorable issues are most apt to follow repeated attacks in unhealthy and especially scrofulous persons. Both rheumatoid arthritis and strumous articular disease have appeared as sequels of gonorrhoeal rheumatism. Life is not endangered, except in very rare instances in which cardiac or cerebral [p. 107]complications obtain; and to stiffened enlarged joints the functions may often be restored by efficient treatment.
276 Brodfurst cites two such cases: Reynolds's System of Med., i. 980. So does Nolen in an elaborate article upon rheumatismus gonorrhoicus in Deutsches Archiv für klin. Med., Bd. xxxii., 1883. I had not seen it before this paper was written.
DIAGNOSIS.—In some instances no doubt what appears to be ordinary gonorrhoeal rheumatism, owing to the coexistence of urethral discharge and articular inflammation, is really pyæmic arthritis. The intermediate link in the causation may be suppuration in the prostate or its veins or in the testicle or the penis or in its dorsal vein, or the urethral pus may undergo changes and become septic and be absorbed. In other instances it is highly probable that true primary acute articular rheumatism sometimes occurs coincidentally with gonorrhoea. If in addition to the presence or recent existence of gonorrhoea the case present several of the following features, gonorrhoeal rheumatism may be said to exist: moderate or mild pyrexia and articular pain; the number of joints attacked being few, with a tendency to concentration in one, either from the first or secondarily; no migration from one joint to another; no delitescence, but marked chronicity and indolence, with a tendency to hydrarthrosis and to implication of the synovial sheaths and bursæ; an absence of cardiac complications; the frequent and often early coincidence of special ophthalmic affections.
TREATMENT.—The patient should be confined to bed, so as to secure rest to the inflamed articulations, and when severe arthritis (third form) exists an efficient splint is peremptorily required, and its application is often followed by prompt relief to the pain. It should be retained until not only all pain, but all tenderness on pressing the articulation, has disappeared. In short, the principles and details of local treatment suited to gonorrhoeal rheumatism are the same as those recommended for rheumatoid arthritis, which it so closely resembles; and the reader is referred to that article for information. Although there is a greater proclivity to copious effusion into the joints in gonorrhoeal rheumatism than in rheumatoid arthritis, there is less to those deeper lesions which affect the bones, and complete recovery is usually more certain and more prompt in the former than in the latter. Measures to prevent stiffness and even ankylosis of the articulations are often an urgent indication. In the general treatment, also, almost the same remedies are indicated as have been recommended for rheumatoid arthritis. The salicylate of sodium, given freely, is sometimes signally useful, more especially when several joints are acutely inflamed. In the more chronic stages, when much articular effusion exists, a prolonged course of potassium iodide is occasionally beneficial. The local measures, however, simultaneously employed, doubtless co-operate efficiently. Iron and quinia will frequently be demanded by general debility, anæmia, and impaired nutrition; and the same may be said of cod-liver oil, extract of malt, etc. The circumstances under which the various baths are likely to be useful have been mentioned in connection with the treatment of rheumatoid arthritis.
The gonorrhoea should be treated in the same way that it ought to be if no arthritis existed. The rest, the moderate diet, and even the salicylate of sodium, favor its removal, but the frequent employment of mild astringent injections should not be omitted.
DEFINITION.—Gout, as a disease, in the traditional acceptation of the term, is a specific arthritis, characterized by the deposit of the salts of uric acid in the affected joints. Gout, as a diathesis, is a blood crasis in which there is an accumulation in the blood serum of the uric acid salts, the consequence either of the increased formation or of the defective excretion of these products of proteid metamorphosis. The manifold irritations of the different tissues, and the accompanying subjective and objective symptoms provoked by this dyscrasia, are termed gouty.
SYNONYMS.—(a) Eng., Gout; Lat., Gutta; Fr., Goutte; Sp., Gota; Ger., Gicht—derived from the nomenclature of humoral pathology and descriptive of the distillation (goutte à goutte) of the poisonous humor into the joints—arthritis uratica. (b) Gouty diathesis; constitutional gout; irregular gout.
CLASSIFICATION.—(a) Gout as a specific form of articular inflammation is classified according to its location—cheiragra, onagra, podagra, gonagra, etc. (b) Gout as a constitutional disease is classified, 1st, according to the structures affected—e.g. articular gout; tegumentary gout, embracing mucous as well as cutaneous affections of gouty origin; nervous gout; parenchymatous or visceral gout; 2d, according to the degree of the inflammatory process—acute, subacute, and chronic; 3d, according to certain irregularities manifested in the development and progress of gouty lesions as metastatic, retrocedent, and suppressed gout. This classification of constitutional gout is based upon the well-recognized clinical observation in the history of gouty persons and gouty families, that the characteristic lesions of the joint-structures are often correlated with lesions of the skin, mucous and serous membranes, vessels, nerves, and parenchymatous organs, which are marked by the same blood dyscrasia that exists in articular gout, and which are most successfully treated by the same measures which experience has suggested in the management of the arthritic disease.
Musgrave in his work1 treats of a great number of varieties of gout, as follows: De arthritide anomala; de colica arthritica; de diarrhoea arthritica; de dysenteria arthritica; de abscesse intestinorum arthritica; de melancholia arthritica; de syncope arthritica; de calculo renum arthritico; de asthmate arthritico; de catarrho, tussi, et peripneumonia arthritica; de phthise arthritica; de angina arthritica; de capito dolore et [p. 109]vertigine arthritica; de apoplexia arthritica; de paralysi arthritica; de doloribus in corpore vagis, fixis; de ophthalmia, de erysipelate et achoribus arthriticis; etc.
1 De Arthritide Anomala, sive Interna, Dissertatio, Geneva, 1715.
HISTORY.—The records of medicine furnish simple evidence of the prevalence of gout in all ancient as well as in modern civilized communities. Its origin in the perversion of physiological functions was as clearly recognized by the prophets of the old testament of the medical art as it is by the founders of the gospel of modern science. The refined processes of animal chemistry have simply revealed the materies morbi which was foreshadowed in the "peccant matters" of the humoralists, which were supposed to be distilled into the joints and other structures, provoking inflammation and tophous deposits. This is the most notable and interesting fact in the history of gout, that it has from the earliest times been regarded as a specific form of arthritis and dependent upon the circulation in the blood of peccant matter. It was not, however, until the latter part of the eighteenth century, when Murray Forbes, and a few years later Wollaston, called attention to the fact that uric acid was the chief ingredient in urinary calculi and in tophous deposits, that our knowledge of the pathology of gout may be said to have had its beginning. The demonstration by Garrod, in 1848, of the presence of lithate of soda in the blood of gouty persons, also marks an era in the history of the pathology of gout.
While the humoralistic theory of gout has prevailed almost to the exclusion of all others, it is historically interesting to note that the views of the solidists, as represented by Cullen, who maintained that "gout was an affection of the nervous system in which the primary moving powers of the whole system are lodged," have been recently revived and are attracting considerable attention.
ETIOLOGY: PREDISPOSING CAUSES.—Heredity may be regarded as the most prominent of the predisposing causes of gout. Statistics of arthritic gout show this tendency in a varying but always large proportion of cases. Scudamore observed it in nearly 60 per cent. of his cases; Garrod, in 50 per cent. of his hospital cases and, in a much larger proportion, in his private practice; Gairdner found it in 140 out of 156 cases. If all the manifestations of the gouty vice were taken into consideration in determining the influence of heredity, it would doubtless be shown in a still larger percentage of cases.
It is generally supposed that there is a greater frequency of inheritance from the male ancestors and in the male descendants. This may be explained by the fact that men are more exposed to the other predisposing and to the exciting causes of gout. My own experience leads me to suspect that if we took into consideration the irregular manifestations of this morbid inheritance, we should find it as frequently in the female, both in the ascending and descending line; of the greater frequency of acute articular gout, however, in the male, there can be no question. While it is true that acute attacks are comparatively rare in women, both before and after the menopause, it is undeniable that the subacute and chronic forms of gouty arthritis are by no means rare in them, both before and after the cessation of menstruation. The Hippocratic proposition that women enjoy immunity from gout by reason of the menstrual flux can hardly be entitled to much consideration in view [p. 110]of the fact that they are commonly less exposed to the exciting causes of the disease, and that when they subject themselves to the same vicious habits which entail the disease in men they suffer like men.
Statistics as to the age at which articular gout is most often developed show that the larger proportion of cases occurs in the decade from thirty or forty. It is rare before twenty, and the frequency diminishes rapidly after sixty. Some well-authenticated cases have been observed before puberty in children in whom the hereditary taint was strongly developed. Gairdner claims to have seen several cases in infants at the breast. Trousseau saw a case in a boy aged six, and Garrod in a youth of sixteen. At the other extreme Garrod reports a first attack at the age of eighty, and another in the ninetieth year. The cases at the extremes of age are certainly rare, and other causes of arthritic inflammation might easily be invoked to explain them. It is a significant fact that the largest proportion of attacks of acute articular gout occurs after the period of complete development is ended and before the period of degenerative changes has begun, when the necessities of growth have ceased and food is required only for the nutrition of the tissues, the maintenance of vital energies, and the demands of work.
Much stress was laid by the earlier writers on the effect of temperament as a predisposing cause of gout. The vague ideas involved in the classification of mankind according to temperament may be said to have lost their influence in the scientific conceptions of modern pathology. Gout is observed in persons exhibiting the most diverse peculiarities in physical conformation and physical disposition. The true interpretation of the facts in regard to the relations of temperament to gout, so far as those relations exist, would seem to be that the conditions which give rise to gout are responsible also for the physical and moral idiosyncrasies of gouty subjects.
A vicious hygiene may be regarded as one of the chief predisposing causes of gout. The disease is essentially one of advanced civilization, and is alike the product of the luxury and the misery which a high civilization entails. It is a common error to suppose that gout is the consequence only of luxurious living. If the essential cause of the disease is the circulation of imperfectly oxidized plasma, then there are two ways in which this defective oxidation may be brought about: either there is an excess of food ingested beyond the capacity of the individual, under the most favorable conditions, to consume, or the conditions of oxidation may be so impaired that the complete combustion of even a moderate supply of food is impossible. Perfect oxidation requires an even balance between the amount of food ingested and the oxygen inhaled. A consideration of this axiom explains several circumstances in the history of gout. As has been remarked, the disease is rare during the period of growth and development, when the processes of nutrition are active and the consumption of food in excessive quantities is rendered possible by the large demands for the needs of the growing body and for the development of active energy. It is common in adult life when the processes of nutrition are less active, when growth is complete, and when the supply of food must be regulated according to the amount of energy to be developed. It must also be observed that while the disease is most frequently caused by excesses in the consumption of [p. 111]food, it is also often the consequence of an insufficient supply of pure air; hence we find it often among those who cannot be accused of gluttony, but whose occupations or poverty compel them to live and work in a vitiated atmosphere.
The influence of alcoholic liquors in the production of gouty dyscrasia is generally acknowledged. There seems to be a striking difference, however, in the effects of the distilled and fermented preparations of alcohol in this respect. Gout is certainly more prevalent in countries where large amounts of fermented liquors are used than in those where distilled spirits are chiefly consumed. The disease is more prevalent, for example, in England than in Scotland or Ireland, especially among the lower classes; it is said also that it is rare in Russia and Poland, where spirits are more exclusively used. There is a difference also in the predisposing influence of the different varieties of fermented liquors in the production of gouty dyscrasia. The heavier wines, sherry, madeira, and port, are known to be more mischievous in this respect than the lighter wines of France and Germany, though there is abundant clinical evidence of the fact that even these wines, and especially the richer clarets. Burgundies, and Rhine wines, frequently give rise to acute gout and the gouty habit. There can be no question as to the pernicious effects of the malt liquors as gout-producers. The great frequency of gouty diseases particularly among the lower classes who consume these beverages in large quantities is undeniable. This is true especially of the stronger English and Scotch ales, and to a less degree of the lighter English, American, and German beers. The effect of cider and perry as gout-producers is also well recognized. It has been observed in certain districts of England where cider is largely consumed, and, though acute articular gout is said not to be a common disease in New England, where cider has always been much used, there can be no question that it often leads to the development of the irregular forms of gout. As one of the forms of fermented alcoholic beverages containing, in its fresh state especially, a large amount of sugar, it favors the production of the acid dyspepsia which is a common antecedent in the formation of a gouty dyscrasia.
In 1854, Garrod called attention to the fact that a considerable proportion of the gouty patients in hospital practice—at least 30 per cent.—was represented by painters and other workers in lead. This statement has since been confirmed by other observers, and the association of the characteristic symptoms of this form of metallic poisoning, such as the blue line on the gums, colic, and the different forms of paralysis, with both articular and visceral gout, especially the contracted kidney, is certainly frequent. The relation, however, of saturnine poisoning to gout in this association is not easy to determine, Garrod himself pointing out that while the women in the lead-works frequently had the colic, they but rarely had gout. The difference in susceptibility of different individuals to all forms of metallic poisoning is well recognized. It is more strikingly observed perhaps in mercurial and arsenical poisoning than in that of lead. It is well known that the internal use of lead as an astringent in cases of hemorrhage and intestinal catarrh is occasionally, though very rarely, followed by the evidences of lead-poisoning. This difference in susceptibility is perhaps explicable on the theory that persons inclined to gout have less power in eliminating the [p. 112]metal than those who are not gouty, so that it is possible that plumbism is the effect rather than the cause of gout, as has been commonly supposed.
Tanquerel des Planches found none of those changes in the kidneys as the result of plumbism such as are frequently met with in gout, and Rosenstein, who was able to produce saturnine epilepsy in dogs, found no renal changes to have occurred. Charcot and Gombault in recent experiments of feeding guinea-pigs with lead found changes in the kidneys similar to those produced by tying the ureters.
EXCITING CAUSES.—Paroxysms of acute or subacute gouty inflammation of the joints, skin, or mucous membranes, as well as the neuroses of gouty origin, are excited by a variety of causes: errors in diet, both as to quantity and as to specific articles; excesses in the use of fermented liquors—even moderate indulgence, in persons with strong gouty tendencies—are perhaps the most common exciting causes. Sudden changes in temperature, and especially sudden changes in barometrical pressure, sometimes excite and often aggravate the sufferings of gouty persons. Blows, contusions, and mechanical strain frequently determine arthritic attacks; the large proportion of paroxysms affecting the metatarso-phalangeal joint of the great toe is explained by the fact that this joint is more exposed than any other to strain and injury. Finally, nervous exhaustion, from any cause, from overwork or sexual excesses, from grief, anger, or shock, may provoke any of the inflammatory or neurotic consequences of this disease.
PATHOLOGY.—It would be impossible in the limits of this article to review the many theories that have prevailed in regard to the pathology of gout, or even to discuss fully those that may be said to divide professional opinion at the present day. Since the discovery, by Garrod, of the salts of uric acid in the blood-serum of gouty patients, the humoral pathology of gout has certainly had the largest number of adherents.
The lithæmic pathology may be said to be based primarily upon the chemical theory of digestion or food-transformation. This theory proceeds upon the idea that every atom of albuminous or carbonaceous food that enters the body, whether it goes to the construction of tissue or is destined for the direct conversion of potential into active energy, is finally eliminated, for the most part, as urea, carbonic acid, and water. This transformation, of course, is supposed to be effected by a process of oxidation, but neither the exact mode of transformation nor the share which the different organs and tissues take in its accomplishment can be said to be certainly known. Recent investigations seem to indicate that the liver is chiefly concerned, not only in the metamorphosis of the carbohydrates, but also in the formation of urea, so that the arrest in the conversion of starches and sugars which results in glycosuria, and the check in the metabolism of the proteids which give rise to lithæmia, may both have their origin in hepatic derangement. The not infrequent association of glycosuria and lithæmia in the same patient, and the frequent alternation of gout and saccharine diabetes in gouty families, are significant facts in support of the common origin of these diseases.
The purely chemical theory of gout and diabetes, that they are diseases of suboxidation—a theory most ably advocated by Bence Jones2—has [p. 113]much to commend it from the valuable suggestions which it affords in the clinical management of these maladies; but it must be acknowledged that while a defective oxidation seems to be an essential factor in the production of gout and diabetes, it is impossible to reduce the process to the simplicity of a chemical equation. It cannot be claimed that the complex chain of transformations which organic chemistry has demonstrated in the destructive metamorphosis of albumen and starch in the laboratory is represented in the vital chemistry of the body. All that can be said in the present state of knowledge is, that the metabolism of food is in its nature a chemical analysis, modified and regulated by vital force, and resulting in the building up of tissues and in the conversion of potential into active energy. Imperfect blood-elaboration must depend upon much besides a disturbance of the balance between the amount of food ingested and the oxygen inhaled, though this must unquestionably be an important factor in its production. Heredity and the mysterious influence of the nervous system complicate the problem of the malnutrition which leads to gout, in such a way that while the general proposition may be maintained that gout is a disease in which suboxidation occurs, it is not possible to affirm whether suboxidation is the essence of the disease or only one of its phenomena.
2 Lectures on Some of the Applications of Chemistry and Mechanics to Pathology and Therapeutics, H. Bence Jones, London, 1867.
It is probable, however, that the pathogenesis of the gouty dyscrasia involves a much more complex process than the simple accumulation of uric acid salts in the blood. Uric acid, like urea, is one of the normal results of the metamorphosis of the albuminous foods and tissues. In birds and reptiles it takes the place of urea as the final issue of this metabolism. It has been supposed, as one atom of uric acid can be split by oxidation into two atoms of urea and one of mesoxalic acid, that uric acid was the penultimate of urea, the result of a lower degree of oxidation. It is by no means certain, however, that it is a necessary antecedent of urea. In birds, who consume by their rapid breathing an enormous proportion of oxygen, as well as in the slow-breathing reptilia, the nitrogenous excrements are in the form of urates; and under such divergent conditions it is impossible to explain the variations in the proteid metabolism by varying degrees of oxidation. The only reason that can be assigned for the elimination of the nitrogenous waste in some animals in the form of urea and in others in that of urates is the teleological one that the urea is destined for a fluid and the urates for a solid excretion.
But apart from these physiological objections to the theory that uric acid is necessarily the offending substance in gout, it is well known that uric acid salts accumulate in the blood in febrile diseases, in disorders of digestion, and in anæmia—notably in splenic anæmia—and do not produce either the symptoms or lesions of gout. Todd maintained that gout might occur without an excess of uric acid in the blood; and it is certain that in the atonic and irregular forms of the disease uric acid may not be found in excess in the blood or appear in excess in the urine. Another significant circumstance in the history of gouty persons tending to show that uric acid may be, after all, only an epiphenomenon in the disease, and not its exciting cause, is that the power of digesting farinaceous and saccharine foods in this disease is markedly diminished. To such a degree is this true that sufferers from the gouty dyscrasia are most [p. 114]promptly relieved of their symptoms of primary indigestion by restricting their diet very largely to albuminous foods; and not only does such a diet diminish the dyspeptic symptoms, but I am persuaded by a considerable experience that it is one of the surest prophylactics against the recurrence of gouty lesions. It is well known that the fermented preparations of alcohol are among the most frequent exciting causes of acute gout, and cases are by no means infrequent in which indulgence in sweet foods and in fruits will provoke many of the well-recognized local lesions of the disease.
The explanation of this anomaly in the uric acid pathology of gout may possibly be found in the suggestion of Garrod, that the deposition of the urates is caused by their insolubility, and, as this insolubility is increased by the diminished alkalinity of the serum, that the evolution of the acids in the digestion of the carbohydrates so diminishes the normal alkaline state of the blood that the uric acid salts are more readily precipitated. But even if we accept this explanation, the fact remains that as efficient factors in the production of the gouty diathesis the carbonaceous foods may play as large and perhaps a larger part than the albuminous foods. It would seem, therefore, in view of the conflicting evidence in regard to the theory of the uric acid origin of gout, that the chemical pathology of this dyscrasia is still involved in considerable obscurity.
The recent advances in neuropathology have revived of late years the views of Cullen on the pathology of gout. Dyce Duckworth3 has lately advocated the theory that gout is a trophoneurosis. This theory grows out of the recognition of the protean manifestations of this disease, and especially of the neurotic element which is so prominently developed in its evolution. The frequency of purely nervous symptoms in gouty persons is a fact which is daily brought to the notice of those who have much opportunity to study the disease. These symptoms may be said to affect all the functions of the nervous system; among these we may mention psychical disturbances, such as hypochondriasis and hysteria; derangements of sensation, such as neuralgias and dysæsthesias of every variety; and spasms of voluntary and involuntary muscles, such as cramps, grinding of the teeth, asthma, and vesical tenesmus. Another fact which arrests attention in the history of gouty persons is the frequency with which purely nervous influences determine attacks of gout; the effect of nervous exhaustion, whether provoked by overwork or mental anxiety, or the more explosive discharges of nerve-force in rage and great emotional excitement of any kind, is well recognized as a frequent precursor of gouty lesions. The influence of certain diseases of the nervous centres also, such as cerebro-spinal meningitis, Pott's disease, and tabes dorsalis, in determining arthropathies and lesions of the skin and mucous membranes, furnishes a striking analogical argument in favor of the possible nervous origin of the lesions in gout. The recognition of these facts, however, does not necessarily militate against the commonly accepted humoral pathology of gout. The healthy action of the nervous centres must depend primarily upon a normal nutrition, and a normal nutrition depends on healthy blood-elaboration. That perverted innervation may be an important factor [p. 115]in the development of malnutrition through the accident of inheritance is doubtless true, but in the acquired disease it seems more probable that the lithæmic condition is the primary source of disturbed innervation. It may be that gouty lesions are determined as reflex phenomena through the medium of the trophic centres—if such centres there be—rather than by the direct irritation of the affected tissues by the gouty blood; and it is not unreasonable to suppose that nervous exhaustion from any cause may produce in these centres greater reflex excitability.4
3 Brit. Med. Jour., March 26, 1881.
4 Edward Liveing, in his work On Megrim, Sick Headache, and Some Allied Disorders, p. 404, thus expresses his conviction as to the neurotic theory of gout: "The view which is commonly entertained is, that the excessive generation or retention of uric acid in the system, which is regarded as the fundamental fact in the pathology of gout, exerts a toxic influence upon the nervous centres, while the particular character of the disorder is determined by the territory involved. This limited operation of a cause so general in its nature is a real obstacle to this view; on the other hand, there is much in the history of gout—its hereditary character, limitation to particular ages and sexes, periodicity, explosive character, sudden translations, and remarkable metamorphic relations with nervous disorders—which seems to stamp the malady as a pure neurosis; and even the fit itself, with its sudden nocturnal invasion, the late Dr. Todd was accustomed to compare to one of epilepsy or of asthma."
PATHOLOGICAL ANATOMY.—Blood-Changes.—Garrod's demonstration of the excess of uric acid in the blood of gouty persons constitutes the chief recognized hæmic change in this disease. That this is a constant change, and one that is essential to the existence of gout, cannot be said to be proved. The presence of uric acid in the blood is not always productive of gout, since it has often been found in the blood of healthy persons, and its temporary excess during pyrexia, and especially in the fevers and other morbid states in which spleen is congested, has already been noted. The excess of uric acid, however, in gouty blood may reach, according to Garrod, as much as 0.11 grain in 1000 grains of serum. It is probable that other excrementitious substances exist in the blood in gout which bear a closer etiological relation to this disease than uric acid, but they have not been demonstrated. The other blood-changes which are noted by Garrod—the diminished specific gravity of the serum from loss of albumen, the diminished alkalinity, and the increase of the fibrin in the inflammatory forms of the disease—are probably inconstant. In chronic gout the objective signs of anæmia which are often present would indicate a marked diminution in the red blood-corpuscles.
The tissues which are the chief seat of gouty lesions are the connective tissues. In the evolution of the disease the joints, where the connective tissue is most dense and the least vascular, suffer earliest; at a later period the connective tissue of the blood-vessels, nerves, and viscera becomes subject to gouty changes.
According to Garrod, the exudations in articular gout are rich in the urates of soda, lime, magnesia, and ammonia; they also contain some phosphate of lime and traces of organic matter. The watery portion is absorbed and the salts are deposited in crystalline forms. The location of these deposits varies: they are found on the synovial surfaces, in the cartilage-cells, and in the intercellular substance; in the tendons, ligaments, and bursæ, and in the subcutaneous connective tissue. The urate of soda occurs not on the free surface of the cartilage, and replacing [p. 116]the latter, as was formerly generally supposed, but as an infiltration into the substance of that tissue; and Garrod found that there is always a thin layer of unaffected cartilage lying between the deposit and the free articular surface—an observation which has been confirmed by Budd and quite recently by Ebstein.5
5 W. Ebstein, Die Natur und Behandlung der Gicht, Wiesbaden, 1882.
Very important are the recent investigations of the latter. After making numerous observations on the cartilages and other affected tissues of gouty subjects, besides studying the disease artificially produced in fowls, he has shown that those portions of cartilage and other tissues in which the deposit occurs are in a state of necrosis, as is evident from the fact that when the urates are dissolved out by warm water the area in which the deposit occurred, though apparently normal to the eye, refuses to be stained with aniline dyes, and lies plainly visible as a light spot in the midst of stained tissue. Since the work of Weigert we know that this is a sure sign of that peculiar form of death of a tissue to which the name of coagulation necrosis has been given. Ebstein regards this necrosis as primary and the deposition of the uratic salt as secondary. According to him, the urates circulating in the blood give rise to necrosis in parts where the circulation is sluggish (as the articular cartilages, the ears, and the extremities generally), and where, consequently, they remain a greater length of time in contact with the tissues. The necrotic portion has, however, an acid reaction, which causes a deposition, from the soluble neutral salt, of an acid urate in a crystalline form. Ebstein claims that this necrotic area, in which there is deposited a crystalline urate of soda, and around which there is a secondary inflammatory zone, is characteristic solely of gout. "I have never seen," he says, "in gout a crystalline deposit of urates occurring in normal tissue."
In addition to these so-called specific changes we find a hyperplasia of the connective tissue in the fibrous structures of the affected joints. The thickening thus induced, with the contraction of the new tissue and the atrophic changes resulting from pressure and disuse, are the causes of the deformities, subluxations, and impaired movements of gouty joints. Occasionally, the local irritation provoked by the pressure of the tophous deposits results in abscesses from which a mixture of pus and pasty urates may be discharged. These abscesses in feeble and anæmic subjects are sometimes difficult to heal. More frequently the skin undergoes gradual absorption and the chalk-like deposits are exposed.
The frequency with which the metatarso-phalangeal joint of the great toes is affected in gouty persons has always been noted. In Scudamore's tables the proportion of the first attacks in this joint was 72 per cent., and in 66 per cent. one or both great joints were affected to the exclusion of other joints. This frequency is due to the fact that this joint is the most vulnerable one in the body, bearing as it does the weight of the body and being exposed to most frequent shock. The phalangeal joints of the hands and the wrist-joints are also often the seat of acute gout, though these joints are more frequently affected by the subacute form of the disease. The larger joints may also be the seat of true gouty inflammation; indeed, no joint, not even the intervertebral, can be said to enjoy immunity, and the hip and shoulder are occasionally attacked to [p. 117]the exclusion of others. The cartilages of the ear and the arytenoid cartilages are sometimes the seat of gouty deposits.
The great frequency of arterial sclerosis, and the subsequent fatty and chalky metamorphosis in persons who have suffered from chronic gout, are well recognized. Next to syphilis, gout seems to be the most common cause of these arterial changes. The influence of these lesions in the arteries and capillaries in determining cardiac hypertrophy and cerebral hemorrhage is often seen in the accidents which terminate the lives of gouty patients.
In the heart a gouty endocarditis is of not uncommon occurrence, according to Ebstein, who cites Lancereaux as having found uric acid in concretions on the valves. Garrod, however, after examining a number of cases in which cardiac disease existed with gout, states that in his opinion the valvular changes are not due to a gouty deposit, he never having been able to demonstrate the presence of uric acid in them.
Some years ago Sir James Paget called attention to the frequency of adhesive phlebitis as a gouty lesion. This is observed in connection with articular gout, but may also occur independently of joint-lesion. It is observed most frequently in the lower limbs, is generally symmetrical, and shows a disposition to metastasis.
Neuritis and sclerotic lesions of the nerve-centres are not uncommon in the history of acquired and inherited gout. The neuralgias and other temporary dysæsthesias which constitute a considerable category in the symptoms of gouty persons are doubtless due to transient central and peripheral lesions.
The so-called gouty kidney is the most striking illustration of the effect of the gouty dyscrasia in the production of a characteristic visceral lesion. The changes which occur in the kidney as a result of gout are—a contraction of the organ, the result of interstitial inflammatory processes, and a deposition of uratic salts, occurring mainly in the papillary portion. The views as to the exact locality where these deposits occur still differ considerably. Garrod is of opinion that it occurs in the fibrous interstitial tissue. Virchow, on the other hand, regards the lumen of the tubuli as the seat of the deposit, and in this he is supported by Charcot and Cornil and Ranvier, Lancereaux and Wagner. Dickinson inclines to the view of Garrod, and believes that it is the deposition of the urates in the interstitial tissue which gives rise to the chronic inflammation which results in cirrhosis of the kidney—the granular kidney of gout. Ebstein seems to think that the interstitial connective tissue, having previously undergone a state of necrosis, as in cartilage and other connective tissues, is the seat of the deposit. As in cartilage, he regards this necrotic state as typical of gouty deposits. About the necrotic area in which the deposit has occurred a secondary inflammation takes place, leading ultimately to contraction of the new fibrous tissue formed. He calls attention to the fact that (1) the kidneys may be perfectly sound in gout; (2) the kidneys may be the seat of chronic interstitial inflammatory changes, with cirrhosis, without any urate deposits of any kind being demonstrable; (3) there may be chronic interstitial nephritis, with crystallized urates in the urinary tubules.
As regards changes in the liver, few satisfactory accounts exist. [p. 118]Portal originally called attention to the fact that in gout and rheumatism indurations of the liver caused by the deposit of a phosphatic earth occurred, and Charcot has recently referred to the fact. Ebstein cites a case in which in a gouty patient he was able to make a diagnosis of moderate hypertrophic hepatic cirrhosis, but so far he had not been able to confirm it by post-mortem examination.6
6 Gout in Animals.—Of the occurrence of gout in animals not many reliable reports exist; Ebstein has collected a few. Thus, he cites a case where in an old hunting-dog uratic concretions were found in the articular ligaments and in the periosteum of the epiphyses of many joints, but especially those connecting the ribs with their cartilages. In the toes of falcons and of parrots kept in confinement deposits of urates have been observed, and in an alligator dying in captivity deposits were found in the muscles as well as the joints which consisted of free uric acid together with sodium urate.
Experimentally, Ebstein was able to produce gouty lesions having all the characteristics of those occurring spontaneously in man by injecting subcutaneously small quantities of the neutral chromate of potash into the blood of cocks for a considerable period of time. By this method changes in the epithelial elements of the kidney were produced, preventing the elimination of the urates from the blood and causing their consequent accumulation in the system. He obtained in this way typical deposits of urates in the joints, tendons, muscular sheaths, heart, and other organs, while the birds emaciated and finally died. But these experiments, which are extremely valuable and interesting, still need confirmation.
The experiment of tying the ureters of fowls is an old one. Galvani who was perhaps the first to perform it, employed it in his investigations on the kidney, and since then Zalesky, Pawlinoff, Von Schroeder, Colosanti, and others have made use of it in their experimented studies on the site of origin of uric acid. As a result of this operation deposits of urates occur in various organs. Ebstein, however, does not regard them as analogous to the gouty deposit in human beings, as they lack the feature of necrosis, which, as mentioned above, he considers as alone characteristic of the true gouty lesion.
SYMPTOMATOLOGY.—The development of true gouty lesions, whether of the acute or subacute form, is usually preceded by a period, more or less protracted, in which characteristic derangements of the health present themselves. These derangements may be conveniently classified as disturbances of primary digestion and as manifestations of malnutrition.
The disturbances of primary digestion are repeated attacks of flatulent dyspepsia, with pyrosis, colicky pains, alternate constipation and diarrhoea, and a scanty, high-colored, and heavy urine with uratic sediments. This dyspepsia may be accompanied with a variety of reflex nervous symptoms, such as pain in the nape of the neck and occiput, insomnia, palpitation, sighing respiration, singultus, and nausea. These symptoms are commonly described as due to biliousness, and are provoked by excesses in diet, and not unfrequently by moderate indulgence in certain common articles of food, such as sweets, fruits, farinaceous foods, and the fermented preparations of alcohol.
Derangements of nutrition are shown by a disposition to erythematous and catarrhal affections of the skin and mucous membranes, to affections of the sebaceous glands, and to premature falling of the hair. There is often a more or less marked tendency to obesity. Accompanying these derangements there may be a loss of energy, both physical and mental, manifesting itself in indolence and fatigue on slight exertion, in irritability of temper, with diminished intellectual activity and hypochondriasis. Neither the primary indigestion nor the nutritive derangements invariably precede the development of acute gouty lesions, nor are they necessarily followed when they exist by the articular signs [p. 119]of gout; but they are so commonly associated with the evolution of what are regarded as the specific lesions of gout that they may fairly be described as constituting its prodromal period.
ACUTE ARTICULAR GOUT.—A typical attack of acute gout is usually sudden. It seizes its victim without warning, and often rouses him from sleep with a vicious agonizing pain in the joint assailed. Examination will reveal a slight redness, heat, and puffiness of the part affected altogether disproportioned to the intensity of the pain; the tenderness is exquisite, and the torture is often aggravated by the occurrence of reflex spasms of neighboring muscles. There is usually moderate fever, and if the surface be exposed there may be a chill. Sleep is impossible and the restlessness uncontrollable. As the morning advances slight perspiration occurs, and sleep may become possible. With the abatement of pain there is coincident increase in the signs of inflammation: the joint swells, the skin becomes red and oedematous around the joint, and the superficial veins are distended. But, though the pain subsides with the occurrence of swelling, and usually in proportion to its degree, the tenderness and pain on any attempt to move the joint continue to be extreme. The day is passed in comparative ease, but the evening generally brings an exacerbation of pain and fever, and the night another paroxysm of agony—not as severe as the first, but severe enough to make the daylight a benison. The progress of the disease after the second day, provided it is confined to one joint, is usually marked by a steady and regular decline in the severity of the symptoms. If the attack is confined to a single joint, a week may elapse before the inflammatory signs subside, and it may be a fortnight before pressure can be borne or the mobility of the joint is restored. Occasionally the sufferings of an acute attack of gout may be protracted by successive seizures for several weeks. The fever during the attack is distinctly remittent, the evening exacerbation rarely exceeding 103° F.
The urinary symptoms before, during, and after an acute paroxysm of gout are interesting and important in their bearing upon the uric-acid theory of the disease. Garrod's statements upon this point are generally accepted, and have been confirmed by other observers. He says that previous to the attack the amount of uric acid in the urine is below the average—that during the paroxysm the proportion grows smaller, and only rises to the normal standard with the termination of the seizure. The reaction of the urine is strongly acid during the paroxysm. This is due probably to the increased excretion of acid phosphates. The quantity of the urine is generally diminished, the specific gravity increased, and the color deepened.
Attacks of acute gout are generally followed by improved health and capacity for physical and mental work and enjoyment. The blood seems to be purified, the processes of digestion and assimilation are once more normally performed, the equilibrium of the nervous centres is restored, and the evolution of all the vital energies proceeds with ease and vigor. This state of well-being may continue for a year or two years, or even a longer period, after the first attack, the immunity varying according to the intensity of the inheritance or the habits of life. The subsequent attacks are apt to occur at increasingly shorter intervals, and, as a rule, the acuteness of them tends to diminish. Gradually the dyscrasia becomes more [p. 120] profound, and the constitutional symptoms and structural changes which belong to the atonic and irregular forms of the disease are developed.
ATONIC GOUT.—Though subacute, irregular, or atonic gout is often the sequence of repeated attacks of the acute disease, it is not necessarily preceded by them, nor is acute gout invariably followed by a marked gouty dyscrasia. It is not uncommon for a well-characterized gouty habit to exist, manifesting itself by many and varied gouty phenomena, without the occurrence of any acute lesions, and repeated attacks of acute articular gout may occur without the development of the progressive impairment of health and the tissue-changes which distinguish the chronic malady. The recognition of this fact is important, inasmuch as the occurrence of acute gout is commonly regarded as an essential element in the diagnosis of the gouty dyscrasia. Acute articular attacks, as already noted, are very rare in women, in whom the subacute and irregular forms of the disease are by no means infrequent. So far as acute articular gout is of value in the diagnosis of the constitutional vice, it is perhaps as significant if established in the history of a near relative as in the individual in whom the disease is suspected.
The general symptoms of atonic gout—or, as it may more properly be called, the gouty dyscrasia—are similar to those which sometimes precede the development of the acute form. The difference lies in their persistence, in the subacute character of the local lesions, and in the absence of the relief to the constitutional symptoms which follows acute attacks.
The dyspeptic symptoms are perhaps the most pronounced and uniform in the history of the evolution of chronic gout. These symptoms have been already described, but the fact which seems especially to distinguish them is that they are chiefly provoked by the acid fermentation of the carbohydric elements of the food, the sugar and starches, and especially by the fermented preparations of alcohol; the ability to digest these articles of diet appears to be deficient in the gouty dyspeptic.
The changes in the urine in the gouty dyscrasia are especially important. In the formative stages of the gouty vice the amount of urine may not vary much from the normal quantity, but the proportion of solid constituents, especially of the urea, is increased, so that the specific gravity may rise to 1.030 or 1.035. The acid reaction is intensified by the excess of the acid urates and phosphates upon which the normal acidity depends. Sometimes crystalline deposits of uric acid, urates, and oxalates take place in the tubuli of the kidney and in the bladder, and lead to the nephritic and vesical irritations which are often the source of much inconvenience and pain. Where the urine is free from these crystalline constituents as it comes from the bladder it may deposit them within a few hours after its passage. At a later stage in the development of the gouty dyscrasia the quantity and quality of the urine undergo marked changes. The quantity is increased; the color is pale, partly in consequence of dilution and partly through a diminution in the amount of coloring-matter. The quantity may be so considerable as to constitute a polyuria. The reaction is neutral or only feebly acid; crystalline sediments of uric acid and calcium oxalate may occasionally appear, and the specific gravity may be so low as to indicate not only a relative but an absolute diminution in the daily excretion of urinary solids. Traces of albumen and of sugar are not infrequently observed.
[p. 121]The articular symptoms of chronic gout are subacute. They affect the joints, as a rule, which are most exposed to strain and injury, and hence are most common in the hands and feet, but they may involve the knee and the hips, the elbow and the shoulder, and even the intervertebral joints. The pain is less severe, because the tension is never so considerable; the tenderness is often a source of great discomfort; the swelling varies with the acuteness of the inflammatory process, the joints being more or less permanently enlarged by hypertrophic changes affecting the articular structures and by tophous deposits. The deformities are increased by ankylosis, by contractions, by absorption of the cartilages, by partial luxations, and by the atrophy of disused muscles. Crepitations are often observed in the affected joints. Exacerbations of the local symptoms are often provoked by movements, by imprudence in diet, by changes in temperature or in barometric and hygrometric conditions, and not infrequently by psychical disturbances.
The frequency with which tegumentary affections, mucous as well as cutaneous, are observed as correlative phenomena of arthritic lesions in gouty persons and in gouty families justifies the inference that the same lithæmic vice which determines articular inflammations is often responsible for derangements of nutrition in the skin and mucous membranes. The French school of dermatology, which has always maintained the humoral origin of many cutaneous diseases, has long recognized the arthritic nature of a large class of affections of the skin. Bazin7 has given the most precise description of the arthritides, as he terms them. He insists upon certain functional derangements of the skin as characteristic of the gouty diathesis, such as excessive perspiration, especially in certain regions, as the head, the axillæ, the hands and feet, and the sexual organs, and also affections of the sebaceous glands, causing the different forms of seborrhoea and the premature falling of the hair. He notes the liability in gouty persons to certain neurotic affections, such as pruritus, general or localized, about the arms and genital organs. Erythematous affections, especially urticaria, erythema nodosum, and the fugitive erythema which occurs about the face, causing sudden and evanescent swelling of the eyelids, cheeks, lips, and even the tongue and soft palate, are recognized by him and other observers as arthritic in their origin. Among the erythemata which are observed in gouty persons the peliosis rheumatica should be mentioned.
7 Affections génériques de la Peau, Paris, 1862.
The more persistent inflammatory lesions of the skin, such as eczema and psoriasis, which are characterized by long-continued hyperæmia with hyperplasia, are now recognized as among the possible transformations of gout. They are certainly often observed alternating with arthritic lesions, and associated with all the characteristic derangements of nutrition which belong to the gouty habit. The frequency of the various forms of acne, the inflammatory, as well as those which result from excessive function of the glands, in persons having a strong gouty inheritance, is recognized by many dermatologists. I have noticed these lesions especially in young women belonging to gouty families. They are generally accompanied by marked dyspeptic symptoms, and not infrequently by neurotic derangements.
Garrod, in a paper read at the International Medical Congress in 1881 [p. 122]on "Eczema and Albuminuria in Relation to Gout," affirms that each year strengthens his conviction that gout and eczema are most closely allied. Since his attention was first called to this relation in 1860, he has found a gradually increasing percentage of eczema in the cases of gout that have come under his observation. Dividing all the cases from 1860 to 1881 into ten groups, he found the percentage rose from 10 in the first group to 47 in the tenth. He accounts for this rapid increase in the percentage in the fact that in the first few years the eczema was only observed when it was very patent; during the past two or three years he has had made more careful inquiries as to the presence of eczema or other skin eruption in every case of gout, and by these means has frequently discovered its presence when it might otherwise have been overlooked. Garrod believes that eczema is the special skin-lesion of gouty subjects, and does not regard psoriasis as having anything more than an accidental connection with gout. He admits that the latter is often associated with rheumatoid arthritis. It must be remembered, however, that Garrod does not admit that gout ever exists without lithatic deposits.
In regard to the location of gouty eczema, it appears to affect by preference the more tender and vascular regions of the skin. The eyelids, ears, the scalp, and back of the neck, the fingers and toes, particularly the dorsal and lateral surfaces, and in old people the legs, are especially liable to be attacked. The subjective symptoms of gouty eczema are often the source of great suffering; the burning and itching are sometimes intolerable. This is especially true of persons of highly neurotic constitution.
It is not possible to affirm that there are lesions of the mucous membranes which are strictly analogous in their transient character to the erythematous affections of the skin, but it is not unreasonable to suppose that many of the temporary disturbances of indigestion to which gouty patients are subject are caused by an evanescent hyperæmia corresponding to the vaso-motor derangements which are observed in the external integument. In regard, however, to the more persistent catarrhal lesions, there can be no question as to their analogy with those which affect the skin. The continuity of these lesions at the orifices of the mucous tracts, and the frequent association of external eczemas with catarrhs of mucous membranes, are facts of common experience. Greenhow8 of London first called attention to the frequency with which chronic bronchitis is associated with the gouty dyscrasia. In an analysis of 96 cases of chronic bronchitis he elicited the fact that in 34 out of the 96 a distinct gouty history attached either to the patients themselves or to some of their immediate relatives. In 14 of the cases the patients were subject to attacks of acute regular gout as well as to bronchitis. He also noted the association in a number of cases of bronchitis and psoriasis with gravel and gout. My own experience confirms these observations, and also the alternations of catarrhal and parenchymatous tonsillitis, of pharyngeal and laryngeal catarrh, and of asthma and chronic bronchitis, with the more common manifestations of regular and irregular gout.
8 On Chronic Bronchitis, E. Headlam Greenhow, M.D., London, 1869.
The occurrence of subacute gastro-duodenal and intestinal catarrhs [p. 123]with hemorrhoidal complications is even more common that the catarrhal affections of the respiratory tract. The lesion, in fact, which gives rise to the manifold dyspeptic symptoms in gouty subjects is doubtless a catarrhal one.
The genito-urinary tract exhibits also the tendency to catarrhal affections in sufferers from the gouty dyscrasia. It is certain that gouty persons are especially liable to vesical catarrh, and it is generally admitted that rheumatic and gouty persons are particularly susceptible to gonorrhoea. My own experience leads me to suspect that chronic urethral discharge resulting from acute urethritis is more common in rheumatic persons than in those not having this taint. The etiological relations of gonorrhoeal rheumatism and kerato-iritis are still involved in obscurity, though I am inclined to believe that a careful examination of the personal and family history in cases of these diseases would establish the opinion that has been maintained as to their gouty origin.
The presence of albumen in the urine of persons suffering from acute gout is occasionally observed. Under these circumstances it is transient, and has probably no more significance than is usually attached to this symptom in the course of any acute febrile disease. In chronic gout it is by no means infrequently observed as a more or less persistent symptom. It is associated under these circumstances with a copious discharge of urine of pale color and low density, and with the general signs of what Rayer first described as the néphrite goutteuse.
The importance of this symptom is very great when we consider the insidious development of this form of disease and the difficulty of its early diagnosis. Recent investigations point to the value of the changes in the urine in the progress of the gouty dyscrasia as bearing upon this question. It has already been noted that in the early history of gouty persons the urine is often scanty, high-colored, excessively acid, of high specific gravity, occasionally albuminous and saccharine, and frequently depositing sediments of urates and calcium oxalate. McBride of New York9 has recently called attention to this condition of the urine and its association with high arterial tension as the functional stage of the granular kidney—as the stage, that is to say, during which the necessity of eliminating large amounts of imperfectly oxidized nitrogenous material maintains a constant state of renal hyperæmia, which finally induces the changes in the tubular and intertubular structures which constitute the anatomical features of this form of disease.
9 The Early Diagnosis of Chronic Bright's Disease, T. A. McBride, M.D., New York, 1882.
The occasional presence of sugar in the urine of gouty persons has already been noted. I have repeatedly observed this symptom in the urine of gouty dyspepsia. It occurs more commonly in obese subjects, and is usually intermittent and easily controlled by dietetic restrictions. In these cases it is not necessarily associated with a very large amount of urine. In chronic gout and in connection with the granular kidney a more serious form of glycosuria is occasionally observed. Under these circumstances it increases largely the polyuria which is characteristic of gouty nephritis, and is sometimes overlooked because it occurs in a urine of a low density, often not more than 1.010. It is not controlled by diet [p. 124]to the same extent that it is in the cases previously described, and is in my experience a prognostic sign of bad import.
Some of the most distressing symptoms to which gouty persons are especially liable are those connected with the passage of gravel from the kidney to the bladder. Where gravel alone passes, it may cause little uneasiness, and the fact is only recognized through the discovery of blood in the urine in connection with uric acid or calcium oxalate crystals. When, however, the sand forms concretions in the pelvis of the kidney, their dislodgment and passage through the ureter are accompanied by the well-known agonies of renal colic.
Dysuria is a symptom from which gouty persons often experience much inconvenience and suffering. It is usually associated with extremely acid urine of high density containing crystalline sediments. It may manifest itself only in frequent and painful micturition, or it may be associated with such a degree of vesical tenesmus as to cause retention and necessitate the use of the catheter.
DIAGNOSIS.—If the term gout be restricted to that form of arthritis in which an excess of urates is found in the blood with tophous deposits in the affected joints, the cartilages of the ear and nose, and in the subcutaneous connective tissue, then the diagnosis of this disease is a simple one. It is a disease with a pathognomonic sign. But if the pathology of gout consists rather in a more complex morbid condition of the blood, of which an excess of urates in the serum is only one of a number of phenomena, and not necessarily the sole and essential cause of the local lesions, then the question of diagnosis involves a consideration of all the correlated morbid conditions which are so frequently associated in gouty persons and gouty families as to justify the inference that they have a common origin in a perverted nutrition, the essential nature of which is imperfectly understood.
The very existence of the terms gouty rheumatism and rheumatic gout which are in common use shows that what is regarded by many excellent authorities as the confounding of distinct entities must have some foundation in clinical experience. If we consider gout, in its strictest pathological sense, acute inflammatory rheumatism, rheumatoid arthritis, or gouty rheumatism, and senile arthritis or the arthritis deformans and gonorrhoeal rheumatism as separate and distinct diseases, we shall find ourselves compelled to ignore certain common clinical facts which indicate a bond of union between them. Heredity, for example, is common to them all, and more than this, there appears to be a tendency to a differentiation of the taint in families. It is well known, for instance, that the children of gouty parents are especially liable to acute rheumatism, and acute rheumatism in youth is often followed by gout in later years. It is also a fact of common experience that while the men in gouty families are the victims of true gout, the women are apt to be the subjects of rheumatoid arthritis. The arthritis deformans which develops with the degenerations of advancing years is not infrequently associated with a family history of genuine gout. Gonorrhoeal rheumatism also, according to the experience of many trustworthy observers, often recognizes an inheritance to gouty lesions. But it is not alone in heredity and the differentiation of the type of the disease in families that the unity of these affections displays itself. The same disturbances of digestion which [p. 125]characterize the history of true gout are observed in those who are liable to acute rheumatism, to rheumatoid arthritis, and to arthritis deformans. It is true that excesses in food and fermented liquors do not determine, as in gout, attacks of acute rheumatism nor of the chronic forms of arthritis, for these latter diseases are commonly due to causes operating upon the nervous system, as exposure to cold and dampness or to physical or emotional shock of some kind; still, there is in the subjects of these diseases a more or less marked tendency to the same dyspeptic disorders, and especially to the diminished capacity in digesting the carbohydrates, which the subjects of true gout exhibit. In the diagnosis of gout, therefore, it would seem that the question of differentiating this disease from those which simulate it is not one in which we are called upon to distinguish one morbid entity from another, as typhus from typhoid fever or syphilis from cancer, but rather to determine, first, the presence of a recognized constitutional vice; and, secondly, to differentiate the variety of the lesions by which this vice manifests itself.
In the diagnosis of the gouty dyscrasia the first point to determine is that of heredity. This requires a careful inquiry into collateral as well as direct descent, and does not necessarily involve the discovery of arthritic diseases in the ancestors, though these are doubtless the most striking and trustworthy proofs; but the tradition in the family of persistent dyspepsia, or what is commonly called biliousness, of chronic catarrhal affections of the skin and mucous membranes, and of the chronic forms of renal disease, are significant indications of this dyscrasia. In the personal history the evidences of the lithæmic tendency, as indicated by the characteristic dyspeptic symptoms which have been described, and especially by the feeble capacity for the digestion of carbohydrates, are of great diagnostic value.
The diagnosis of gouty joint-lesions, whether acute or chronic, depends partly upon the determination of the gouty dyscrasia, and partly upon the differential distinctions which separate gouty inflammations from acute rheumatism, rheumatoid arthritis, and from the arthropathies which result from traumatism and from lesions of nerves and nerve-centres.
Gouty arthritis may be distinguished from acute rheumatism by the fact that it is more often hereditary—that it occurs in older subjects, attacking generally the smaller joints, and, as a rule, in the acute form, localizing itself in one or two joints. It is also noteworthy that the constitutional symptoms are not as severe as in rheumatism. Gout deforms the joints, while acute rheumatism leaves no traces of the inflammatory process. In addition to these distinctions there is, according to Garrod, the crucial test of an excess of urates in the blood-serum.
From rheumatoid arthritis or rheumatic gout, gout in its acute and regular form is distinguished by the more acute local and constitutional symptoms. Gout is periodical in its attacks, while rheumatoid arthritis is progressive. It attacks the smaller joints or those most exposed to strain, while rheumatoid arthritis occurs in the large as well as the small joints, and appears to be more independent of traumatism as an exciting cause. Gout is more common in men, rheumatoid arthritis in women. According to Garrod and other excellent authorities, deposits of urates are never found in the joints in rheumatoid arthritis, and there is no excess [p. 126]of urates in the blood. This statement is denied by Hutchinson. Ulcerations of cartilages, contractions of tendons, atrophies of muscles with subluxations of joints, are more common in rheumatoid arthritis than in gout.
While these local distinctions are undeniable, it is proper to observe that in rheumatoid arthritis the constitutional symptoms of the gouty dyscrasia, especially the dyspeptic derangements and the nervous disturbances, are often well marked; and it should also be noted that the principal distinction, the absence of urates in the blood and in the diseased joints, is one that is based on the exclusive theory that uric acid is the materies morbi of true gout. If, as is still maintained by some excellent authorities, uric acid is not essential to gout, then it must be confessed that the other distinctions are purely lesional, and that the common constitutional symptoms suggest that these diseases are divergent branches of a single trunk.
Gouty arthritis is not always easily distinguishable from traumatic inflammation of the joints, inasmuch as traumatism plays so important a part as an exciting cause of gouty attacks. The history of previous seizures and the presence of predisposing causes of gout are the points upon which the determination of the gouty nature of the inflammation would depend. A termination in suppuration would exclude the idea of the gouty nature of an arthritis.
With the arthropathies of purely nervous origin, such as occur in paralyzed limbs, in Pott's disease, and in tabes dorsalis, gout can hardly be confounded, although the arthritic complications in these diseases have been used to illustrate the neurotic theory of both gout and rheumatism.
The diagnosis of irregular gout—i.e. of gouty affections of the skin and mucous membranes, of the structures of the eye, and of the parenchymatous organs—must be based more upon the hereditary history and upon the correlated phenomena recognized in the personal history than upon any specific character in the lesions themselves. In the gouty form of nephritis there are, it is true, in the urinary symptoms, in the anæmia, in the arterial fibrosis, and in the cardiac hypertrophy, diagnostic signs of great value.
PROGNOSIS.—Acute, regular, articular gout is probably never a fatal disease where it occurs in a robust person without visceral complications. In rare instances the first attack may never be repeated, or only two or three attacks may occur in the course of a long life. In the majority of instances, however, frequent repetitions are the rule, the intervals between the attacks growing progressively shorter; occasionally repeated seizures go on through a long life, the attacks becoming milder with advancing years, and, save the crippling effects of the disease, the patient may enjoy in the intervals a fair degree of health. This, however, is the exception. With the increased frequency of the arthritic attacks the signs of the constitutional vice become more marked. The dyspeptic disorders become more persistent and rebellious to treatment, various transformations of the disease manifest themselves, and tissue-changes make insidious and inevitable progress. When this stage of the gouty disease is reached, the prognosis becomes more grave because of the complications and accidents to which the sufferer is liable. These complications and accidents are the result of the nervous, vascular, and visceral lesions which have been [p. 127]described. Vaso-motor instability gives rise to a great variety of painful functional derangements resulting from serious cerebral, pulmonary, gastric, and renal congestions. Glycosuria is not an uncommon complication in chronic gout, and seriously affects the question of prognosis. Arterial degenerations may cause thrombotic accidents, and the formation of miliary aneurisms in the brain may determine a fatal issue by softening or hemorrhage. Anginal attacks due to cardiac muscular degeneration may also imperil life.
The principal visceral lesion which leads directly or indirectly to a fatal issue in gout is that of the kidney. This involves danger either through the induction of a hopeless anæmia and its consequences in dropsical effusions, or by determining inflammatory accidents of the gravest nature.
That gout shortens life in the majority of cases is unquestionable—a fact which is sufficiently attested by the care with which life-insurance companies exclude risks in which a well-pronounced inherited tendency or existing manifestation of the disease can be substantiated.
The prognosis varies of course with the rapidity with which the constitutional dyscrasia is developed, and this rapidity will depend on the intensity of the inheritance and the mode of life. Some gouty subjects escape the vascular and visceral complications of the disease for a long period, although crippled and deformed by its articular ravages, and attain advanced age; others may succumb in comparative youth to its most profound lesions. It is a happy circumstance that under wise hygienic management and judicious medication acquired gout may be checked in its progress, and even a strong inherited tendency may be largely controlled.
TREATMENT.—A logical consideration of the treatment of gout embraces, first, the treatment of the constitutional vice, based, as far as possible, on the nature and causes of the disease; and, secondly, the treatment of the lesions which the disease determines. If we regard the accumulation in the blood-serum of the salts of uric acid as the essential cause of the gouty lesions, then the origin of the constitutional vice is in the conditions which bring about this accumulation. As we have urged, none of the theories of the production of the lithæmic state harmonize all its phenomena. It is impossible to represent the complex processes of nutrition by chemical formulæ, and equally impossible to divorce chemical reactions from a share in their production. We can trace the metabolism of the azotized and carbonaceous foods through many changes to their ultimate disintegration into urea, carbonic acid, and water, but we do not know all the steps by which this conversion is effected, nor the organs or tissues in which it is accomplished. We may reasonably assume that the agent through which the potential energy of the food is evolved is oxygen, and that the process of nutrition is hence partly, at least, a process of oxidation. This chemical view of the digestion and assimilation of food may be said to be the rational basis of the treatment of the lithæmic state. To control the accumulation of azotized matters in the blood, and to secure their thorough combustion and conversion into urea, carbonic acid, and water are the recognized aims of the treatment of the vice upon which gout depends.
DIET.—The prevention of the accumulation of azotized matters in the [p. 128]blood involves, first, a consideration of the question of the diet appropriate to the gouty dyscrasia. The almost uniform counsel upon this point of all the authorities from Sydenham to the present time is, that albuminous foods should be sparingly allowed in the diet of the gouty patient, and that vegetable foods, especially the farinaceous, should constitute the principal aliment. This counsel is based upon the theory that uric acid is the offending substance, and, this being the outcome of a nitrogenous diet, the nitrogenous element in diet must be reduced. My own observation has led me to believe that while this may be a legitimate deduction from the uric-acid theory of gout, it is not supported by the results of clinical experience. If there is one signal peculiarity in the digestive derangements of gouty persons, it is their limited power to digest the carbohydrates, the sugars and starches. In whatever form these foods are used, they are more commonly the source of the dyspeptic troubles of sufferers from gout than the albuminous foods. They provoke the acid and flatulent dyspepsia which so generally precedes the explosion of the gouty paroxysm; and it must have attracted the attention of every observer who has studied the dyspeptic disorders of sufferers from inherited gout, who have sought to control their unhappy heritage by abstemious habits, that these disorders are especially provoked by over-indulgence in saccharine and amylaceous foods.
It is not possible to explain satisfactorily why the lithæmic condition should be induced by the carbonaceous aliments, but we believe there can be no question as to the fact. If, as modern physiological investigations tend to show, the liver is the organ in which urea as well as glycogen is formed, it may be that the overtaxing of its functions manifests itself more readily in the conversion of the albuminous than in that of the carbonaceous foods; or it is possible that the carbonaceous foods are destined chiefly for the evolution of mechanical energy, and that when this destiny is not fulfilled through indolence and imperfect oxygen-supply, they escape complete combustion, and so vitiate the blood. But whatever may be the cause of this anomaly, the clinical fact remains that in gouty persons the conversion of the azotized foods is more complete with a minimum of carbohydrates than it is with an excess of them—in other words, that one of the best means of avoiding an accumulation of lithates in the blood is to diminish the carbohydrates rather than the azotized foods.
The diet which a considerable experience has led me to adopt in the treatment of the gouty dyscrasia is very similar to that which glycosuria requires. The exclusion of the carbohydrates is of course not so strict. Abstinence from all the fermented preparations of alcohol is perhaps the most important restriction, on account of the unfermented dextrin and sugar which they contain. This restriction accords with the common experience respecting the part which wine and beer play as predisposing causes of the gouty disease and as occasional exciting causes of gouty lesions.
Next to the fermented liquors, the use of saccharine food in the diet of gouty persons needs to be restricted. This limitation also is one which common experience confirms. Sweet foods cannot be said to be as provocative of the dyspeptic derangements of the lithæmic subjects as wine and beer, but they are certainly often responsible for the formation of [p. 129]the dyscrasia and for perpetuating many most distressing ailments. Their more or less strict prohibition may constitute the essential point of treatment not only in controlling the progress of the constitutional vice, but in subduing some of the most rebellious lesions. It is important to observe that this prohibition sometimes involves abstinence from sweet and subacid fruits, in the raw as well as in the preserved state. Paroxysms of articular gout have been known to follow indulgence in strawberries, apples, watermelons, and grapes, and the cutaneous and mucous irritations which follow even the most moderate use of these fruits in some gouty persons are certainly not uncommon.
Next in order to the saccharine foods as the source of indigestion in gouty persons come the amylaceous aliments. These constitute, necessarily, so large an element in ordinary diet that the limitation of them in the dietary of gouty persons applies, in the majority of cases, only to their excessive use. This excessive use, however, is often observed. There is a popular prejudice in favor of this class of foods, and a corresponding prejudice against the too free indulgence in animal foods. The purely starchy aliments, such as potatoes and the preparations of corn and rice, and even those which contain a considerable portion of gluten, like wheat, oatmeal, and barley, often provoke in gouty subjects a great deal of mischievous and painful indigestion. This feeble capacity for the digestion of farinaceous foods is most frequently observed in the children of gouty parents, and especially in persons inclined to obesity, and in those whose occupations are sedentary and whose lives are passed for the most part in-doors, and they are least common in those whom necessity or pleasure leads to much active muscular exercise in the open air.
The fats are as a rule easily digested by gouty dyspeptics. This is a fortunate circumstance, for the reason that in the anæmia which is frequently one of the consequences of chronic gout the fatty foods are of inestimable value. In cases of persistent and rebellious lithæmia an exclusively milk diet constitutes a precious resource.
The succulent vegetables, such as tomatoes, cucumbers, cauliflower, cabbage, and the different varieties of salads, constitute for the gouty as well as the diabetic subject agreeable and wholesome additions to a diet from which the starchy and saccharine vegetables have to be largely excluded.
The quantity of food proper for gouty persons to consume can only be determined in individual cases by the age, the habits, and the occupation. It is fair to assume that in adults, in whom there is no longer any provision to be made for growth, the daily quantity of food must be regulated according to the amount of energy which is expended. In this energy must be reckoned the amount necessary for the maintenance of animal heat and the other vital functions, and the amount which is necessary for the operation of every variety of nervous force. In other words, the potential energy latent in the food must correspond to the active energy exhibited in the daily evolution of vital, intellectual, and mechanical work. The more nearly this balance is maintained the more closely the physiological standard of health is preserved. That an excess of food is a most frequent cause of the gouty dyscrasia among the well-to-do classes is undeniable, and it is possible that regulation of the quantity according to the rule above mentioned [p. 130]may, after all, be the most important point in the management of many gouty patients. It may be, also, that the reason why the withdrawal of the carbohydrates produces its good effects upon these patients is that we thereby exclude a large amount of force-producing foods which do mischief because they are imperfectly consumed.
EXERCISE.—Next in importance to diet as a hygienic regulation in the management of gouty patients is enforced exercise. The axiom of Abernethy, "to live on a shilling a day, and earn it," comprises the philosophy of the true relations of food to work, and of both to the highest development of physical health. Exercise is to be enforced not simply as a means of securing an active respiration, and thereby an abundant supply of oxygen, but also as a means of converting the potential energy of the food consumed into vital energy. The essential condition, moreover, of healthy nutrition in every organ and in every tissue is the maintenance of a vigorous functional activity. Over-use is not more productive of tissue-degeneration than disuse. Hence the question of exercise in its largest sense involves not only muscular work, but work of all kinds, which tends to promote a healthy activity of the psychical as well as the physical functions. Muscular exercise in the open air has a special value for the victims of this gouty dyscrasia by equalizing the circulation, quickening the respiratory movements, and stimulating the elimination of effete matters from the skin and lungs, but mental work and wholesome diversions are not less important as antagonizing the evil effects of indolence and over-feeding, which are among the common predisposing causes of acquired gout. In persons who are incapacitated by neuræsthenia or by excessive corpulence, the result of long indulgence in indolent and luxurious habits, it may be necessary to resort to passive exercise by rubbing, massage, and electrical excitation in order to secure the good effects of voluntary work.
BATHING.—Another hygienic regulation of great value in the treatment of gouty dyscrasia is the promotion by bathing and friction of the eliminative function of the skin. Daily sponging with cold water, where it is not contraindicated by a feeble circulation and a slow reaction from the shock, is a practice to be commended. Where, for the reasons mentioned, it is not practicable, tepid baths and frictions may be substituted. In cases where the arthritic lesions are progressive and advanced much benefit may be derived from hot baths. It is doubtful whether the thermal alkaline and sulphur spas owe their renown in the treatment of chronic gout so much to the mineral ingredients of their springs as to their high temperature. The Russian and Turkish baths furnish most efficient means for increasing the functional activity of the skin, but they often have a depressing effect on the action of the heart, producing faintness and dyspnoea, and should always be advised with caution.
CLIMATE.—In rebellious forms of the gouty dyscrasia a warm climate is unquestionably a hygienic condition of great value. The geographical distribution of gout, which shows that the disease is much less common in warm than in temperate and cold climates, while it may not perhaps be wholly explained by temperature alone, is very certainly largely due to it. The possibility of out-door life and the increased functional activity of the skin which warm climates favor are circumstances more or less antagonistic to the development of the gouty diathesis.
[p. 131]MEDICINAL TREATMENT.—The objects to be aimed at in medicinal treatment of the gouty dyscrasia are—
1st, the improvement of the primary digestion.
2d, the relief of the gastro-intestinal catarrh, which is the cause of the direct and reflex dyspeptic symptoms which belong to this diathesis.
3d, the augmentation of food-oxidation, so as to secure its thorough combustion.
4th, the promotion of the elimination of the waste products of nutrition.
1. The improvement of primary digestion—or, as it has been aptly called, exterior digestion—often requires very strict attention beyond the proper selection of alimentary substances. The distressing symptoms that indicate primary gastric and intestinal indigestion are certainly often relieved by the rigid exclusion of certain articles of diet, but in many cases it is necessary to assist the preparatory processes which are essential to perfect food-absorption by artificial methods based upon the knowledge derived from physiological experiment. To no one is the knowledge of these methods more largely due than to Roberts of Manchester. Preparations of pepsin and pancreatin, by which the proteids and starches are peptonized and the fats emulsified, are often of inestimable value in the treatment of gouty dyspepsia. Pancreatin, especially, which by means of its trypsin, diastase, and emulsive ferment possesses the threefold property of aiding the digestion of the azotized, amylaceous, and fatty elements of food, is certainly the most valuable of the artificial means for augmenting the efficiency of primary digestion.
2. The relief of the gastro-intestinal catarrh in gouty dyspeptics may often be accomplished solely by dietetic restrictions and by the aid which may be given to primary digestion. It is often necessary, however, to direct some special medication toward the relief of the catarrhal lesion. The circumstances which demand this special medication are the existence of portal congestion, the result of functional derangement, or of chronic atrophy of the liver, or of chronic diffuse or interstitial nephritis, or of cardiac disease. The hydragogues, such as calomel, podophyllin, colocynth, and other vegetable cathartics, with the salts of sodium and magnesium, constitute the most common and efficient means of relieving portal congestion, whether it arise from temporary functional derangement or from organic disease. The renown of some of the more famous mineral springs in relieving the miseries of gouty sufferers is due mainly to the relief of portal congestion and the washing away of the catarrhal mucus which obstructs the process of primary food transformation and absorption. This is especially true of the sulphate of sodium waters, like those of Carlsbad, Marienbad, Friedrichshall, Pullna, and Hunyadi János. While the value of these waters in chronic gout is unquestionable where their use is properly regulated, there is good reason to believe that their long-continued employment is often harmful by relaxing the mucous membrane, and thereby tending to aggravate the condition they are given to relieve. This is markedly true of their use in weak and anæmic persons. For these the milder magnesian waters, such as those of Kissengen, Hombourg, Wiesbaden, and Saratoga, are to be preferred.
3. The augmentation of food-oxidation may be accomplished in a large degree by regulation of the diet and by out-door exercise. The [p. 132] regulation of the diet according to the occupation and habits of life is a point of primary importance in securing proper blood-elaboration. My experience leads me to believe that the evil consequences of in-door occupations and sedentary habits are most common in those who live upon a diet composed largely of starchy and saccharine foods, and that a diet in which animal foods and fats predominate is best suited to indoor workers, whether they be engaged in mechanical or intellectual labor.
The medicines which help to promote the oxidation of the food-elements, especially the carbohydrates, are alkalies and iron. Clinical observation establishes this fact as strongly in the treatment of gout as in that of glycosuria. The relative power of the salts of potassium and sodium in augmenting oxidation is not clearly determined. The salts of sodium appear to be most useful in aiding the process of primary digestion, and the potassium salts in improving the process of sanguification. It is well known that potash predominates in the corpuscles and soda in the serum of the blood. The efficacy of the combinations of iron with the salts of potassium, as in Blaud's pills and in the citrate and tartrate of iron and potassium, in the treatment of anæmia, is well known. In the most renowned ferruginous springs, however, such as those of Schwalbach, Spa, Pyrmont, and St. Moritz, the iron is combined with salts of sodium, calcium, and magnesium. It would appear, therefore, that the increased energy of iron in augmenting hæmatosis, when combined with alkalies, is not relatively greater with potash than with either of the other alkaline bases.
4. The promotion of the elimination of the waste products of nutrition is to be accomplished by remedies which act as solvents of uric acid and as diuretics. As solvents of uric acid the salts of lithia and potash have been shown to be superior to those of soda. The urate of lithia is the most soluble of the uric-acid salts, and the low chemical equivalent of the metal lithium makes the neutralizing power of the oxide much greater than that of equal proportions of the other alkalies. It is used in the forms of carbonate and citrate, and is generally combined with potash and soda. It exists in some of the mineral springs of Europe and of this country, but in such minute proportion as probably to be of little value. In administering the salts of potash and soda it is generally admitted that the carbonates and the neutral salts of the organic acids are to be preferred to solutions of the caustic alkalies. They have less power in neutralizing the acid of the gastric juice, and enter the circulation as neutral salts, where they are decomposed into alkaline carbonates by the oxidation of the organic acids, increasing the alkalinity of the serum and acting as diuretics. The combinations of the alkalies with sulphur, with iodine, and with mineral acids, as in the alkaline springs, are frequently used in the treatment of gouty lesions of the subacute variety. The sulphur salts probably owe their chief value to their alkaline bases when they are used internally; and in sulphuretted baths, as before remarked, the good effects are probably due to the high temperature at which the bath is usually administered.
The salts of iodine are generally supposed to have a special action in removing the consequences of chronic fibrous inflammation in gout and rheumatism. They often disturb the digestion and provoke troublesome irritations of the skin and mucous membranes. In removing the sclerotic [p. 133]effects of gouty inflammation they do not exhibit the same sorbefacient power which they show in their action upon the granulation tissue of syphilitic origin. It must be admitted, however, that in certain catarrhal affections of a gouty nature the iodides of potassium and sodium are almost specific in their good effects. In the pharyngeal, laryngeal, and bronchial catarrhs from which some gouty persons suffer, where there is a dryness and irritability of the mucous membrane, the administration of these salts produces the most prompt and beneficial result. As solvents of uric acid they do not appear to equal the salts of the organic acids.
As to the mode of administering salines in the treatment of the gouty dyscrasia, it is hardly necessary to observe that it must vary with the effect desired. As antacids in acid dyspepsia they should be given soon after meals, and for this purpose the salts of soda are to be preferred, for the reason that they not only neutralize excessive acidity, but they increase the efficiency of the peptonizing process. Where it is desired to introduce these salts into the circulation for their solvent action, as diuretics or to assist the process of sanguification, they should be given three or four hours after meals and largely diluted with water.
Before concluding the consideration of the treatment of the gouty dyscrasia it should be remarked that the ability of water as a solvent, as a means of stimulating tissue-changes and eliminating waste, is not generally estimated at its true value. The use of copious libations of hot water in the treatment of gout, recommended by Cadet de Vaux in 1825, has been revived from time to time, and is at present attracting considerable attention.
TREATMENT OF ACUTE ARTICULAR GOUT.—There are three distinct methods of managing an attack of acute gout—the antiphlogistic, the expectant, and the abortive.
The antiphlogistic method, in the strict application of the term, is practically obsolete. Bloodletting, both general and local, brisk catharsis and diaphoresis, with low diet, were formerly advocated as the natural and imperative antagonists of gout as well as of all other acute inflammatory affections. Carried to its extreme degree, this method was deprecated by Sydenham and his disciples as tending often to prolong the attack and precipitate the manifestations of atonic gout. The natural reaction from the vigorous antiphlogistic practice was what has been termed the expectant method.
The expectant method may be said to be founded upon the aphorism of Mead that "gout is the cure of gout." The discovery of the salts of uric acid in the blood-serum and in the affected tissues gave a scientific basis to the humoral pathology of gout and led to the formulation of definite principles in the application of the expectant method of treatment. These principles are the prevention of the further accumulation of the urates in the blood and the promotion of their oxidation and elimination. The first principle involves restriction to a rigid diet during the attack, excluding albuminous foods and the fermented preparations of alcohol, and allowing only milk and farinaceous gruels. The oxidation of the urates is encouraged by the administration of alkalies and by an abundant supply of air, the inhalation of oxygen even having been recommended. The elimination of the urates is accomplished chiefly [p. 134]by diuretics and moderate catharsis. The local treatment commonly used with this medication consists in the application of alkaline and anodyne fomentations or of dry flannel or cotton. Local bloodletting and blistering are now rarely commended. Under this treatment the intensity of the inflammatory process is abated, the suffering is allayed, but the progress and duration of the disease are not materially modified. The recovery, however, is satisfactory, and it is claimed that the chances of early recurrence of the attack are diminished. This method has many advocates, though it cannot be said to represent the common practice of the present day. It is becoming traditional, and may be said to be gradually giving place to the specific or abortive method.
The abortive method consists in cutting short the attack by the administration of colchicum, veratria, or the salicin compounds.
The value of colchicum in joint affections is a tradition of the earliest records of medicine. It shares its curative effects in acute gout with veratria, and, though the active principle of the meadow saffron and the veratrum album are not isomeric, their effects are similar. They constitute the basis of the famous nostrums so extensively patronized by sufferers from gout. Colchicum is the active agent in the eau médicinale de Husson, in Wilson's and Reynolds's specifics, and in the pills of Lartigue and Blair, while veratria is supposed to be that of Laville's remedy. The action of these substances is not understood. The physiological action of colchicum is that of a local irritant and a cardiac depressant of great energy. It purges violently when given in large doses, causes nausea and vomiting, and may produce collapse. In therapeutical doses in a gouty paroxysm it acts as a diuretic and an antipyretic, and allays, sometimes in a most magical manner, the objective and subjective symptoms of the disease. As simple purging by other cathartics does not abort the gouty seizure, the value of colchicum cannot be ascribed to its purging effect, and, besides, purging is by no means necessary to its efficiency. Nor can its utility be ascribed to its diuretic property. There is some question in regard to its claims as a diuretic, and there seems to be no doubt that it often does good where this effect is not observed. Its influence upon the heart does not explain its marvellous action upon the local process, for the same influence obtained by other drugs has no such result. We are driven, therefore, to the conclusion that colchicum has a specific action in gout as certain and as inexplicable as that of quinia in malarial fever, or iodide of potassium in constitutional syphilis. For those who accept the theory that gout is a tropho-neurosis the therapeutical action of colchicum is a strong confirmation of its neurotic origin, for the reasons that colchicum has no influence upon arthritic lesions which are not gouty, and that its physiological effects point to its action on the nervous system.
It is useless, however, to speculate on the way in which colchicum and allied substances affect gouty inflammation; the practical question to be determined is: Are they the best and safest remedies to control it? Upon this point there is a wide diversity of opinion. The objections to the colchicum treatment are based upon humoral pathology, and upon the idea that the attack is an effort of nature to cast out the poison and purify the blood. Colchicum, it is claimed, arrests this process; the poison is retained, diffuses itself through the tissues, and lays the [p. 135]foundation of vascular and visceral lesions. It shortens the intervals between the attacks, and tempts the patient to continued indulgence in the habits which perpetuate and exaggerate the disease. The advocates of the abortive treatment, on the other hand, claim that these arguments have no real force as applied to its therapeutical value. The cure accomplished is, to all appearances, complete, and the patient is saved the suffering and exhaustion which result from the expectant method. The fact that he is so easily and speedily cured, and that he resumes his vicious habits and suffers recurring attacks in consequence, proves only that the treatment lacks the quality of moral discipline which belongs to prolonged suffering and the penance of vigorous medication. It is an acknowledged fact that the great majority of sufferers from acute gout decide sooner or later in favor of the abortive treatment; and as professional opinion has heretofore generally advocated the expectant or eliminative treatment, they commonly resort to the use of some one of the quack remedies which contain colchicum or veratria.
In view of the present uncertainty of our knowledge of the true pathology of the acute gouty arthritis, as to whether it is a tropho-neurosis or the result of the local irritation caused by the salts of uric acid, the specific treatment seems to be justified by a regard for the comfort of the patient and as a means of protecting him against falling into the reckless use of quack remedies. A speedy relief of the acute symptoms, followed by the treatment appropriate to the gouty habit, would seem to be the most rational and safest mode of managing the acute articular attacks of gout.
The selection of the preparation of colchicum in the treatment of an acute paroxysm is a matter of individual experience and preference. The acetous extract and the wine of the seeds are most commonly used, and many practitioners are not scrupulous in prescribing the proprietary preparations of Reynolds, Laville, and Blair. The wine of colchicum may be given in doses varying from 20 to 40 minims, alone or combined with Epsom salts in drachm doses, with small quantities of opium, every six or eight hours. Under this medication the pain, tenderness, and swelling rapidly abate, and sometimes with an abruptness that is magical. As soon as the acute symptoms subside, the colchicum should be continued in smaller and less frequent doses until the fever and local tenderness subside. The use of quinia with small doses of colocynth after the colchicum has been discontinued helps to re-establish the strength and regulate the digestive functions. The patient should always be warned against the possible demoralizing effects of a speedy recovery from a serious disease. Recurrence after the colchicum treatment is certainly more common than after the expectant method, but this should not be ascribed so much to a defective cure as to the temptation which the antidote offers to trifling with the poison. The accidents which have been ascribed to colchicum through its causing heart-failure are probably to be explained by its injudicious administration in large doses where acute gout is complicated with cardiac or renal degeneration.
Next in importance and value to colchicum in the abortive treatment of gout are salicin, salicylic acid, the sodium salicylate, and the oil of wintergreen. Unlike colchicum, which has no marked effect upon acute rheumatism, these medicines appear to act with similar energy on [p. 136]gout and rheumatism. The rapidity and the almost uniform way with which they allay the inflammatory symptoms in rheumatic fever are well known; their value as specific remedies in both acute and subacute gout is not so generally appreciated. Whether the specific action of colchicum in gout differentiates this disease from rheumatism, or whether the similar action of the salicin compounds indicates that these diseases are allied in their etiology, are questions yet to be solved. The good effects of salicin and the sodium salicylate in many of the forms of irregular gout, and notably in the dyspeptic disorders and the erythematous tegumentary lesions, are especially worthy of notice. In acute attacks of articular gout the salicylic acid or the sodium salicylate, in 15 or 20 grain doses repeated every three or four hours, will often cut short the attack, and will very certainly allay within twenty-four hours the acuteness of the symptoms. As in rheumatism, the medicine should be continued in smaller doses after the acute symptoms have subsided for several days, the tendency to relapse being marked if the drug be discontinued too soon. In subacute articular gout and in the irregular forms of the disease, where the medicine has to be continued for some time, salicin and the oil of wintergreen are to be preferred to salicylic acid and the sodium salicylate. They are less liable to disturb the stomach and to produce toxic effects.
It is unnecessary to describe the treatment of the different forms of irregular gout, inasmuch as the general principles described in the treatment of the gouty dyscrasia involve the most important considerations in the management of these affections.
1 There is a difference of opinion as to the correct spelling of this word, and strong reasons exist to regard the form rhachitis as the proper one. It is true that this spelling of the word has been remarked upon as unorthographical by many, mostly modern, authors. Even Virchow writes "Rachitis," claiming that Glisson took the term from "the then popular rickets." This is a mistake, as H. Rohlffs points out (Deutsches Arch. f. Gesch. d. Med., 1883, p. 294). Rachitis is a Greek word, and was used in the classical time of Hellenism. It has, however, seemed best to preserve here the usual spelling, rachitis, which has become sanctioned by general usage.
DEFINITION.—Rachitis is a general nutritive disorder, almost always of long duration, usually with an introductory stage of weeks or months and a course mostly extending over months or years. Its beginning is mostly gradual, its final recovery slow. It is complicated with or dependent on disorders of the digestive or respiratory apparatuses, which are preceded by a disposition probably created by an undue width of the arteries. It exhibits amongst its prominent symptoms muscular debility; perspiration; anomalies of the subcutaneous tissue, which is either very much infiltrated with fat or deprived of it; disturbances of the intellectual and moral functions, and of those of the large thoracic and abdominal viscera and lymphatic glands; changes in the latter may outlive all others. Its most perceptible symptom, however, consists in an inflammatory disease of the primordial cartilage of the epiphyses, a copious deposit in that region and also under the periosteum of the bones; curvature of the diaphyses, and, while absorption remains intact, softening and retarded ossification of the bone. Without these affections of the osseous system the diagnosis of rachitis is not complete.
ETIOLOGY AND PATHOLOGY.—The nature of rachitis has been considered to be inflammatory by F. A. Walter.2 Renard looked for that inflammation in the periosteum. Guérin emphasizes the vascular increase in periosteum, bone, and marrow; Trousseau and Lasègue the congestive character of the local tumefaction, besides fever and pain. Virchow also3 inclines to the opinion that the rachitical process is of an inflammatory nature, though it be impossible to state the exact cause of the process. Still, he claims that we are no better off in regard to other inflammations of unknown character—for instance, those of the skin—and that we have to look for a future increase of our knowledge of such constitutional predisposition of the organism and of such specific qualities of the blood as will produce the local irritation of the osseous tissue in rachitis. Last, and mainly, it is Kassowitz who seeks the [p. 138]essence of the rachitical process in a chronic inflammation originating in the points of apposition of the growing bones of the foetus or infant. During the chronic inflammation blood-vessels are formed in large numbers, and a morbid congestion takes place in all blood-vessels, but mainly in those of the localities in which new bone is forming; thus in the chondro-epiphyses, in the perichondrium and periosteum, and the sutural substances. Faulty introduction or elimination of lime has nothing to do with this process. It cannot be deposited in the current of a copious circulation; in fact, it is not deposited in the immediate neighborhood of blood-vessels to any extent. Even in otherwise normal bone hyperæmia produced by the experimenter softens the bone, which was fully formed before. If the relative percentage of lime were of any account in the etiology of rachitis, the periosteal and cartilaginous proliferations would find no explanation. But why is it that this peculiar process takes place at an early age only? and in the bone only? Kassowitz urges the fact that the growth of the bone differs in this from the development of all other tissues: that the latter grow uniformly through their whole mass; that the circulation in them is more uniform and carries material through and into every particle simultaneously, while in the bones the only places in which the whole circulation can contribute to their growth—the few blood-vessels distributed in the interior not adding to their growth at all—are the periosteum and the places of apposition between epiphysis and diaphysis. Every morbid irritation, whether resulting from bad air, habitation, and food, or from either chronic or acute ailment, acts on the whole mass of other tissues and organs, but in the bones only on the growing ends or surface.
2 Anatom. Museum, Berlin, 1796, vol. ii.
3 Arch. f. Path. Anat., vol. v.
The results of the pathologists and experimenters are confirmed by chemical analyses. Fat has been generally found somewhat increased in the rachitical bones, and water largely so; chondrin is diminished according to Marchand and Lehmann, but was found unaltered in the later analyses of A. Baginsky. The latter found, after having deprived the bone of fat, the organic and inorganic material to be in a proportion of 100 to 563 in the normal, and of 100 to 160 in the rachitical osseous tissue; and in 100 parts of dry bone, Gorup-Besanez found in the
Ossein. | Phosphoric Acid. | Lime. | Manganese Oxide. | |
Healthy adult | 34 | 26 | 34 | 0.3 |
Infant of six months | 34.9 | 27 | 35 | 0.5 |
Rachitical femur | 72 | 7 | 9 | 0.3 |
Rachitical tibia | 60 | 12.9 | 17 | 0.3 |
Defective calcification of the forming bone is one of the principal characteristics of rachitis. In it lime cannot either enter into the composition of the osseous tissue or remain in it. Its elimination must take place either through the kidneys or the intestinal tract. In the feces Ad. Baginsky, and many before him, have found an abnormal quantity. In regard to the urine, modern investigations do not agree with former analyses. Thus, Baginsky concludes that there is no increase of lime in the urine of rachitical as compared with that of healthy children; Seemann found even a diminution of the percentage of lime. Amongst modern writers only Rehn found an occasional increase of lime in the urine of rachitis.
[p. 139]In regard to the elimination of phosphoric acid, the analyses of different periods do not agree any better. The conclusions of previous researches, pointing to a quadruple elimination of phosphoric acid in the urine of rachitis, are refuted by Seemann, who found no increase, and by Baginsky, according to whose researches the phosphoric acid of the healthy urine compares with that of rachitical urine as 40:12-37.
As far as the elimination of nitrogen is concerned, there appears to be but little difference between normal and rachitical urine. Chlorine was found to be diminished in rachitis by Baginsky. Lehmann and Von Gorup found lactic acid several times. Several times albumen was met with; in a case of Ritchie's, blood; in one of Von Gorup's, fat.4
4 E. Salkowski und W. Leube, Die Lehre vom Harn, 1882, p. 536.
The etiology of rachitis must be studied from two points of view. It has its predisposition and its direct and proximate causes. The former has been studied by F. W. Beneke5 upon an anatomical basis. He finds that the arteries of rachitical patients are large all through the body. This is so particularly in the carotids; it seems probable that the changes taking place in the head are due to this anomaly in the size of the arteries. Three cases in which the width of the arteries of the neck was unusually large terminated fatally—one by hydrocephalus, one with a very large skull, and one suddenly. This width of the arteries is most marked, under ordinary circumstances, from the second to the fourth year; that is, the exact time in which (except the cases of early rachitis) the rachitical process is at its height. It is considered by Beneke to be the cause of the local increase of vascular irritation, particularly in the epiphyses with their retarded circulation; and also of the increase of nutritive development which is so often noticed during recovery from rachitis; and, finally, of the many pulmonary complications of an inflammatory nature.
5 Die Anatomischen Grundlagen der Constitutions Anomalien des Menschen, 1878, p. 75, etc.
There is another interesting consideration in regard to the effect of wide arteries on the relations between the blood and tissues. A great many more blood-cells are required to fill the arteries when wide than when narrow. Now, the formation of blood-cells is hindered by any disease of the digestive and blood-preparing organs, so that the tissues are liable to show the relative increase in the percentage of water, which is uniformly confirmed for rachitis by the biochemists.
The pulmonary artery of the healthy infant is larger than the aorta by not more than four millimeters. In the majority of cases of rachitis examined by Beneke this difference in size was very much more favorable to the pulmonary artery; it is abnormally large in rachitis. This anatomical fact is suggestive of the pathological processes so frequently found in the lungs and in the neighboring lymphatic and large abdominal glands. For, while the amount of blood introduced into the lungs through its wide artery is unusually large, particularly so in a chest which is contracted in consequence of the rachitical process in the bones, the exit from the lungs is relatively impeded. Not only, however, the narrowness of the chest is a cause of this disproportion. For even in rather normal chests the lungs of rachitical children are relatively small.
The liver of almost all rachitical children is large. In but one-half [p. 140]of the cases this enlargement is accompanied with a large heart. In pure cases of scrofula, on the contrary, Beneke found a small heart, rather narrow arteries, and usually a small liver, the size of the lungs offering but few anomalies.
The spleen also is large in the majority of cases. Its size is not dependent on the large size of the liver or the small size of the lungs. For these conditions are found in the majority of cases only, not in all of them, and the large spleen is not always found with a large liver and small lungs. The variability of the anatomical conditions permits of various degrees of combination; so that varying combinations of rachitis with other constitutional disorders may correspond with the different sizes of the principal organs. After all, as there is a great deal of independence of these organs, as to size, of each other, the conclusion is justified that those differences are not the result of the disease, but that they are congenital and stand in some causal relation with it.
The kidneys are large in the majority of cases, like the spleen and liver, while the lungs are small. This disproportion is apt to result in a hyperæmic condition of all the organs of the abdominal cavity, and especially of the kidneys. To what extent this undue amount of volume interferes with, or increases, renal secretion, it is difficult to say. The amount of urine secreted by rachitical children is about normal, though, as already stated, the percentage of lime in it is rather diminished, contrary to the opinions held formerly.
For the direct cause of rachitis Glisson looked to the inequality of nutrition by the arterial blood, and for that of the curvature of the long bones to their superabundant vascularization. He found the disease mainly amongst the well-to-do classes, not unlike a modern American writer, who declares infantile paralysis to be the result of the nervousness of the better classes of the American people! John Mayow (1761) held a disturbance of the innervation responsible; Zeviani (in the same year), improper food in general, and particularly prolonged lactation; and Selle (1791), a peculiar diathesis (acrimonia rachitica). About that time a defective nutrition with abnormal function of the lymph-ducts was looked upon as the cause of rachitis by many—by others, an undue production of acid, and the softening of the osseous tissue thereby. This result was attributed by some to the influence of milk (Veirac, De Krzowitz). Attention was directed at an early time to phosphoric acid and lime, with the view that variations in the elimination of these substances might explain the occurrence of rachitis. A large quantity of both was found in some urines (Malfatti); a superabundance of phosphoric acid was presumed to prevail in the whole system (Wendt, Fourcroy); while symptoms resembling rachitis were found in animals fed upon small doses of phosphoric acid by Caspari (1824). Chossat fed young animals on food deprived of lime, and claimed to produce softening of the bones and death, a result which was denied by Friedleben. Guérin claimed to produce rachitis by feeding young animals on meat in place of their mother's milk, a result equally denied by Tripier, who, like Friedleben, found the bones under such circumstances more liable to fractures, but not rachitical. Wildt and Weiske found the bones uninfluenced by withholding lime from food; Forster, however, and Roloff claimed to notice a marked influence, and the latter [p. 141] stated that animals, after having been rendered sick by depriving them of lime, recovered when they were again supplied with it. Wegner, in his numerous experiments with phosphorus, found that in growing animals it increases the growth and firmness of both long and flat bones; after the growth of the animal has been completed it renders epiphyses and vertebræ denser. There is no change, however, in the relative chemical composition of those parts. He found at the same time that results similar to those caused by the administration of phosphorus were obtained when food deprived of its phosphate of lime was given. But he met with no rachitical changes proper during these several procedures. Teissier having found an increase in the urine of rachitis after the administration of lactic acid, and lactic acid having been frequently found in the urine of rachitical patients by Ragsky, Morehead, Simon, and Lehmann, C. Heitzmann fed with lactic acid both carnivorous and herbivorous animals, found the cortical layer of the bones softened and the medullary substance hyperæmic, and claimed to produce rachitis in the former and osteomalacia in the latter. Both of these assertions were denied by Tripier and Toussaint, who insist upon Heitzmann's having selected animals which have a peculiar disposition to suffer from rachitis. Again, Milne Edwards and Boussaingault found the bones softened when they withdrew both phosphoric acid and lime from the food, without restoring the bone's consistency by administering powdered bone. But, lately, Ad. Baginsky states that he produced rachitis by withholding lime, and increased the effect by introducing lactic acid. By so doing, however, he changed only the relation of the mineral to the organic substances, without interfering with the normal proportions to each other of the constituents of the ashes. Beneke, finding oxalic acid in the urine in many cases of rachitis, attributes to it the want of calcification in rachitis, and Senator suggests that what impedes the deposition of bone might be formic, acetic, and lactic acids, which are also found in the young osseous tissue.6
6 L. Fürth, Path u. Ther. d. Rachitis, Wiener Klinik, 1882.
Of these statements many are uniform, others contradictory. Thus far, they are not convincing except in one way—viz. that both withholding and introducing certain ingredients, mainly lime, influence the growth of the bone considerably. This may prove nothing else but that lime is of paramount importance in the building up of bone, and that bone in the period of rapid development is amenable to a great many influences.
It is in the period of rapid development that rachitis is observed. Thus it occurs in every stage of intra-uterine and infant life. It is met with in the foetus in very early intra-uterine life; it is found as a congenital affection, continuing to develop after birth when it has originated in the latter half of foetal existence; there is, thirdly, the rachitis of early infancy; and, lastly, that of advanced infancy and childhood. Of 624 cases of rachitis enumerated by A. Baginsky, there were 256 less than a year old, 313 in the second, and 63 in the third year. After this time rachitis is rare, as far as the active symptoms of the disease are concerned. But still, a retarded form of rachitis (r. tardiva) has been described by some authors. It is said to occur about puberty, and to exhibit local changes in the bones of genuine rachitical character, but to be wanting in all the other symptoms required for the diagnosis of general rachitis. Such cases have been described [p. 142]by C. Lucas.7 He found it complicated, now and then, with albuminuria. The occurrence of the latter at that time of life had been referred to by Moxon.8 The principal symptoms described by Lucas are scoliosis, talipes valgus, and genu valgum. The epiphyses were slightly thickened; there were pain in the limbs, languor, and pallor. In some of the cases there were also rachitical deformities dating from infancy. He believes rachitis of adolescence to exhibit more symptoms belonging to relaxation of the ligaments than to softening of the bones.
7 Lancet, June 9, 1883.
8 Guy's Hosp. Rep., 1878.
A case of rachitis of undoubtedly congenital nature has been reported by Chiari. There were but twelve teeth. There were no other alveoli, nor was there any intimation of the formation of alveoli in the shape of the jaw, which resembled very much the usual senile form of retrograde metamorphosis.
Twenty years ago I described the lesions in part of a rachitical cranium removed from an infant who lived up to her eleventh day. She was born at full term with hernia of the brain, about one-sixth of which protruded through the small fontanel. Only the cranium could be studied with regard to rachitis, and but small portions of the frontal and the anterior half of the parietal bones surrounding the large fontanel could be removed. In these few square inches of bone there were between twenty-five and thirty openings of the usual craniotabic nature, nothing but a transparent membrane being left. The bony edges of these thin portions were partly sloping off gradually, partly very steeply, and somewhat thickened. They were distributed over the whole part of the skull removed; some were found in the immediate neighborhood of the points of ossification. No recent deposits of soft rachitical bone had taken place under the periosteum. Thus, evidently, the process was of rather an early date of intra-uterine life, and had at birth run the full course of its usual development without having had an opportunity to terminate in the restitution of the normal bone.9
9 Amer. Jour. Obst., Nov., 1870.
In a case reported by Dr. F. A. Burrall10 the infant (female) was cyanosed at birth, and had a small head and feeble general development. The respiration was shrill and piping from birth, as though from congenital laryngismus; in a few days it became raucous. The post-mortem examination proved the larynx normal, with no obstructive growths. She was pigeon-breasted, and the last phalanx of her right finger wanting.
10 Trans. N.Y. Path. Soc., vol. i. p. 81.
In the meeting of June 27, 1883, of the Société de Chirurgie of Paris, Guéniot presented a newly-born baby with well-pronounced rachitis of the extremities which had healed at the time of birth. The bones had recovered their firmness, and the characteristic deformities remained. In the meeting of December 19th he could report that the child had exhibited neither symptoms of rachitis nor of syphilis since. In regard to the latter, a very rigorous examination of the baby's whole family, made by Guéniot and Fournier, resulted in the existence of no trace of syphilis.11
11 Rev. Mens. des Mal. de l'Enfance, Janv., 1884.
Kassowitz has examined many still-born infants, and also children dying at an early age, at the foundling hospital of Vienna. In a large majority of the cases he found rachitical changes in the ends of the bones. In [p. 143]many of those who lived several weeks he found rachitis developed to such an extent that the presumption of its intra-uterine origin became conclusive. Here nothing is left but the conclusion that the cause of congenital rachitis has to be looked for in the condition of the maternal blood.
Thus, the foetal and congenital occurrence of rachitis cannot be doubted. Both forms are represented in literature. Neither requires the presence of rachitis in one or both of the parents. But the cause of the intra-uterine disease has not been found. Perhaps a disease of the mother with considerable nutritive disorders or a defective placentar supply may be found responsible. The foetal form runs its course long before the normal termination of pregnancy; the congenital may have run its full course at birth or complete it afterward. The bones are found of characteristic nature, the diaphyses suffering more than the epiphyses; even a rachitical pelvis has been met with by Fischer. Early foetal rachitis is probably dependent upon a defective development of the very first cartilaginous deposits and the first osseous nuclei; thus, many of the congenital synostoses find a ready explanation. Besides these, abnormal circulation is accounted for. For periosteal proliferation at that early period contracts the foramina carrying the blood-vessels, and, while interfering with the size of the bones, the foramen magnum also. Thus, a certain class of cretinism appears to be due to foetal rachitis, mainly of the base of the cranium, which results in early ossification of the synchondroses, particularly of the sphenoid bone. But lately I have seen a case of this description, which, however, had not terminated at the time of birth. For after birth the rachitical process developed further, and in addition to the rachitical deformity of the base of the cranium there were afterward thickening of the epiphyses, pigeon breast, and thoracic grooving and flattening.
Rachitis is found in city and country, less on mountains than in valleys. Still, it is met with at elevations of two thousand feet. In the tropical regions it is almost unknown. Why it should have been considered quite a new disease in England but a few centuries ago, or whether it did not exist before that time, it is difficult to say. It is certain, however, that deformities have been described in antiquity which we are accustomed to attribute to rachitis.
As the disease is one that occurs during the period of rapid growth, and is a developmental disease, everything that interferes with normal growth and development is apt to change physiological functions into pathological conditions and to produce rachitis. In the pregnant mother her ill-nutrition and the defective cell-material used in the building up of the embryo and foetus, or a defective placenta, may come in for the explanation of foetal and congenital rachitis, although the case of Klein's, who reports twins, of which one was normal and one rachitic, is rather difficult to explain on that basis only. Even rachitis of early infancy is not easily accounted for otherwise, for its first symptoms show themselves at a very early period; thus constipation, adiposity, and afterward craniotabes and thoracic grooving.
The common form, and that which is the usual subject of the text-books and monographs, has, however, in most cases a well-marked preparatory stage in the shape of diseases or ailments reducing sanguification [p. 144]and nutrition. Some cases are ushered in by, or follow the course of, acute exanthems or acute gastric disorders or the presence of entozoa. A larger number appear to result from insufficient oxygenation resulting from lung diseases, with a long chronic ailment following the acute stage. Even acute pneumonia, with its direct influence on general nutrition, stands often for the proximate cause of rachitis. Bad air alone, even swamp air, does not appear to be a sufficient cause. When it seems so, it is complicated with the main cause of rachitis; that is, bad, insufficient, improper food, with its immediate result—viz. intestinal catarrh. Cow's milk, particularly when acid, starchy food administered too early or in too large quantity or too exclusively, early weaning followed by improper artificial food, insufficient mother's milk or such as is either too thin or too caseinous, lactation protracted beyond the normal limit,—may all alike be causes of intestinal disturbances and rachitis.
Is rachitis hereditary? A number of women who were rachitical themselves have been known to have rachitical children. But it has been said that the process runs its full course during infancy, and that therefore a direct inheritance of rachitis from mother to child is an impossibility. Still, we must not forget that the consecutive conditions of the parents may, or will, influence the general condition of the infant and result in similar disturbances. No rule, however, exists. Dyscrasic parents may have healthy children, and healthy parents sickly or dyscrasic ones. But the probability is greater that diseased children should come from dyscrasic parents than from healthy ones. Tuberculosis in the parents has frequently been accused of being the cause of rachitis in the infant—not directly, but in consequence of general impairment of the tissues. Gout has also been accused of being the cause of rachitis, but it is a peculiar fact that the poor have but little gout and a great deal of rachitis. In all of these cases it is better to look upon rachitis as only one of the forms of general mal-nutrition, and to speak of inheritance of the disposition rather than of the disease. Thus it was that about the end of the eighteenth century Portal spoke of scrofulous, syphilitic, scorbutic, rheumatic, arthritic, and exanthematic rachitis. Particularly has syphilis been accused of being the main cause of rachitis by some, and even the only cause by others. Thus it was looked upon by Boerhaave. In modern times Parrot maintained, from 1872 up to the time of his death, which occurred recently, that every case of rachitis is of syphilitic origin. As his proof he relied mainly on the condition of the teeth and the bones. But those appearances in the teeth, the thin and ragged edges, the friability and the grooving, either horizontal or vertical, which have been considered characteristic of syphilis by Hutchinson and others, have no such dignity, and moreover they are not observed in the temporary teeth at all, but in the permanent only; the rachitical softening of the bones also is not found in syphilis at all. Particularly are there no curvatures in syphilis and no infractions. It is true that marasmus is found in both rachitis and syphilis, but it is met with in all sorts of diseases. The changes in the bones of syphilis are found at birth; in rachitis they usually develop in later months. When a baby is syphilitic and rachitic at the same time, the syphilis may last very much longer than the rachitis, which meanwhile has healed. The internal organs in rachitis do not exhibit any such [p. 145]changes as are known to occur in very many cases of syphilis. No gummata are ever found in rachitis, and the interstitial inflammation of the internal organs in syphilis is not met with to the same degree in rachitis. What Parrot claimed as a desquamative syphilide of the tongue—that is, red insulated spots, denuded of their epithelium, small in the beginning, later extending backward and increasing in size—is by no means always syphilitic, but is found in a great many cases where there is no suspicion of syphilis. It is mainly Kassowitz and Bouchut who have taken the stand against Parrot. The former, taking rachitis for a peculiar inflammatory process, admits that syphilis can be one of the causes. The latter directs attention mainly to the fact that by changing food in certain ways rachitis may be produced in dogs, but that they cannot be made syphilitic. There is no doubt, however, that syphilis may give rise to rachitis by its general influence on nutrition, and in this fact lies the key to the connection of great nutritive disorders with each other. Syphilis will undoubtedly change nutrition to such an extent as to result in rachitis. Rachitis will affect the glands; the caseous and suppurative degeneration of the glands will lead to metastatic processes, to acute tuberculosis, and so on.
Malaria been claimed as the main cause of rachitis by Z. Oppenheimer,12 or, rather, rachitis is presumed by him to be the form in which malaria makes its appearance in young infants. After disposing of other alleged causes of rachitis, none of which is proved to give rise to every case, and referring to the anatomical belief that the peculiar hyperæmia and inflammation of rachitical bones is created by the embryonic condition of the growing osseous tissue, he points to the prodromi, amongst which he emphasizes chronic diarrhoea and the nocturnal crying. The latter, with its perspiration and subsequent sleep, he claims as evidence of malaria, and as a substitute for the intermittent neuralgia of adults, the more so as he believes he finds the spleen tumefied. The persistent diarrhoea of these infants is said to be paroxysmal—to take place in the morning, contrary to what is seen in the usual form of intestinal catarrh; the discharges are said to be serous, not tinged with bile; the appetite to be good through the rest of the day; the weight of the body not to be lessened, but anæmia to develop gradually, and fever to occur occasionally. In other cases infants have cold hands and feet and blue lips toward evening; the skin is pale, the spleen enlarged; otherwise there are perhaps no symptoms, but the infants try to get uncovered, and have an increase of temperature of from 1° to nearly 3° F., and a perspiring head in the morning. After a while the rachitical symptoms belonging to the bones and the general system become apparent. After all of the author's ingenious and emphatic assertions and deductions, it becomes evident that malaria—in the severe forms in which it has been found by Arnstein, Browicz, and Henck to cause bone diseases—may give rise to rachitis, but it is also clear that he tries to prove too much. The long series of attempts at proving that every form and case of rachitis depends upon a single and uniform cause have proved futile. The physiological hyperæmia of the bones and the rapid growth of all the infant tissues are shaped into the complex ailment which we call rachitis by more than a single disease or a single nutritive disturbance.
12 D. Arch. f. klin. Med., xxx., 1881.
[p. 146]SYMPTOMS.—Before entering upon a more accurate and elaborate enumeration of the symptoms of rachitis, I mean to dwell upon peculiar differences which take place according to the age in which the disease makes its appearance. Very young babies—that is, infants of a month or two—develop rachitis in such a manner that many cases are overlooked until it is too late to relieve them in time. This occurrence takes place when there are no prominent causes, such as diarrhoea or other nutritive disorders, nor any premonitory symptoms. Such infants appear to be perfectly well; they have the average weight, and even more; they have plenty of adipose tissue, and look well. The only anomaly appears to be an undue degree of paleness. Without pain or flatulency they are constipated. This constipation is not congenital, as it always is when the colon is unusually long even for an infant, and when the sigmoid flexure is of double or even treble length, but makes its first appearance about the end of the first or the beginning of the second month. It is relieved only when the increasing muscular power of the intestine results in more effective peristalsis. The second symptom is the thoracic groove, to which I shall allude later, and a gradual thickening of the costo-cartilaginous junctures, with or without periosteal pain on pressure. About the same time the cranial softening, craniotabes, with its hyperæmia and perspiration of the entire scalp, and baldness, and the first symptoms of maxillary rachitis, become perceptible. During all this time the epiphysial swellings and the diaphysial curvatures develop but very slowly; but at a very early time chronic bronchial catarrh, with a loose cough, begins to be troublesome. When rachitis begins at a late period—say, about the sixth or eighth month—the aspect of the case is different. The infant has suffered before either from bronchitis and broncho-pneumonia, or in most cases from indigestion and intestinal catarrh. There is some degree of emaciation; the skin does not fit the limbs, as it were—is loose, thin, flabby, and rather dry. The tendency to diarrhoea continues to prevail. The epiphyses, particularly of the lower extremities, are thickened at an early time, curvatures of the tibiæ become apparent, and all the rest of the bones participate in the process, with the exception, sometimes, of those of the head.
The head, however, is liable to exhibit symptoms of rachitis at a very early period of life. It is large, or appears to be so,13 mostly for the reason that the face is proportionately small. The forehead is large, the frontal protuberances very prominent, as are also those of the parietal bones. Thus, the head is more or less square. Dilated veins are visible in and through the pale skin; there is but little hair, on the occiput less than on the rest of the head. Sometimes the occiput is quite bald, the hair having been rubbed off on the pillow. The scalp feels warm, except during perspiration. The latter is very copious, particularly on the occiput—to such an extent, indeed, that the pillow is drenched—and will remain so for months. The sebaceous follicles are often still larger and more numerous than they normally are at that age, and seborrhoea is [p. 147]often, though not always, met with. The sutures are wide, sometimes one or two centimeters; the posterior fontanel remains open; the large anterior fontanel is very large, being sometimes several inches long and wide. The pulse is felt very distinctly through it. The systolic cerebral murmur, which was first found by Fisher of Boston in 1833, and considered to be a positive symptom of rachitis (which certainly it is not, as it is found in almost every healthy baby with a patent fontanel), is very audible. The fontanel and sutures remain open for a long period. Instead of closing, as they do normally at the fourteenth or fifteenth month, the former ossifies about the end of the second or third year, or later. Gerhardt reports a case in which it persisted to the ninth year. The cranial bones appear to be thin, and give way under the pressure of the finger. Ordinarily, it is true, the cranial bones of every baby, even if perfectly healthy, are movable under pressure, but they are so only along the sutures, where they may retain this mobility, in some instances, a long time. Indeed, it appears that sometimes about the middle of the first year the occipital bone becomes thinned out in apparently quite healthy children. Moreover, even in the skulls of infants who were taken to be in good health small defects in the bones were found (Friedleben), with no uncomfortable symptoms at all. Therefore it is rather difficult to draw the exact boundary-line between the healthy and the morbid condition; thus it is possible that some of those cases which exhibited apparently morbid local changes without morbid symptoms may not have been diseased after all. In those, however, in which rachitis is really developed in the cranium a peculiar condition is found. In the posterior half or third of the parietal bones, either the right or the left side being more marked, there are in the tissue of the bone distinct spots in which the osseous material is not only thinned out, but has entirely disappeared. In fact, the bone is perforated, the edges of the holes being rather steep, sometimes slightly thickened, and the scalp separated from the brain only by a thin, transparent membrane, the remnant of the periosteum. These holes can be easily found through the integument. The finger, though ever so gently pressing down upon it, moves the cranium, if any be left, before it; the bone feels like paper, and the sensation as if it could be easily broken through is quite distinct and embarrassing. Such perforations are usually quite numerous; from five to twenty or more can often be counted. They are surrounded by normally hard bone, and thereby can be recognized from the flexible part of the cranium extending along the sagittal and lambdoid sutures. Where these results of rachitical softening, craniotabes, are most prominent—that is, on the part on which the infant is mostly reclining—the bone is flattened, and may remain so for life, though in the majority of cases the asymmetry will disappear. The flattening and perforations result from the same causes—viz. softening of the bones and pressure upon the bone between the pillow outside and the brain inside. With it go, hand in hand, thick rachitical deposits under the hyperæmic periosteum of other portions of the skull. Where craniotabes is largely developed on the occipital portion, the frontal and the parietal bones (in their anterior halves) are usually thus thickened. A cross-section with a knife will reveal a diameter of the new osteoid material between the periosteum and bone of one-half to one centimeter in thickness. It is very hyperæmic—even more so than the bone itself, [p. 148]which, when cut into, exhibits an unusual amount of blood. Sometimes the deposits are still larger, and are apt to change the appearance and weight of the skull considerably after recovery has taken place and eburnation and sclerosis have taken the place of the normal osseous tissue.
13 Boötius (1649), quoted by Haller (Bibl. Med. pract., 1779): "Infantibus caput grandescit, reliquum corpus contabescit, ossa in articulis tument, dextrum hypochondrium tumore æquali prominet; hoc malum multis millibus infantum molestum est" ("The infant head grows large, the rest of the body emaciates, the articular bones swell, the right hypochondrium is raised by a uniform tumor; this malady is a sore affection in many thousands of infants").
Such a case of rachitical cranial sclerosis I have described in the Amer. Med. Monthly of 1861. It was, however, by no means a mate of the case related by E. Huschke. The latter skull was that of a girl of seventeen years, and weighed 4117 grammes instead of the normal weight of 600 grammes. The medullary (Havers') canaliculi were large and very numerous on the surface, narrow and very few in the interior of the sclerotic bones, and the osseous canaliculi were more spherical and irregular in site and shape. The chemical composition was also abnormal, phosphate of lime being 65.59, carbonate of lime 11.12, sulphate of magnesia 1.14, cartilage and fat (very little), etc. 22.15 per cent. No fluorate of lime was found. Most of the bones were exceedingly hard, but fragile when tried in small pieces; very white inside, yellowish on the surface, the latter color being the remnant of extravasated blood or other pigmentous matter. Another skull, in Huschke's possession, and moderately sclerotic, weighed (lower jaw excluded) 1075 grammes; a third, in the museum of the University of Jena, is that of a young baboon,14 in which all the bones covering the hemispheres had become sclerotic.
14 Baboons suffer from rachitis very extensively. In the Transactions of the Pathological Society of London (xxxiv., 1883, pp. 310, 312) I. B. Sutton gives the description of two baboons, one of which was six months, the other one year and six months old, when they died. The careful description of the specimens exhibited leaves no doubt as to the rachitical nature of the changes in both the periosteum and the tissue of all the bones of the body.
Of undoubted total cranio-sclerosis Huschke reports but ten cases—those of Malpighi (1697), Cuvier (1822), Ribalt (1828), J. Forster and Bojanus (1826), Ilg (1822), Kilian (1822), Otto (1822), Vrolik (1848), Albers (1851), Huschke (1858). The disease does not affect the auditory bones, the condyles of the maxillary and occipital bones, nor the styloid process of the temporal bone. It is recognizable in the posterior part of the cranium and basis cranii, but affects mostly the bones of the face and the frontal, parietal, and cribroid bones. Thus, the disease takes its origin in the anterior portion of the skull, particularly in the superior maxilla, and proceeds upward and backward, terminating in the basis cranii in the neighborhood of the infundibulum and appendices. But two of all the cases were observed during life. In all the disease was traced back to early life. The chemical composition of the bones was greatly changed in all. Instead of the normal proportion of earthy to organic material = 2.1 (or 1.5):1, it was from 3.5 to 4.4:1. Particularly the carbonate of lime was greatly increased.
The brain and its meninges participate, in many respects, in the changes worked by rachitis, and mainly in the abnormal vascularization of the bones. They are very much congested, and succulent. A section through the brain shows a great many large and small blood-points. This hyperæmia may give rise to over-nutrition, which assumes the character of real hypertrophy of the brain. When that hyperæmia, however, becomes excessive, effusion will take place into the cavities, the tissue of the arachnoid, and the substance of the cerebrum, which latter looks [p. 149]peculiarly brilliant, elastic, and sometimes white, in consequence of the blood-vessels being emptied by the pressure on the part of the enlarged mass of the cerebrum upon the blood-vessels. Thus, instead of cerebral hyperæmia there may be anæmia. Every form of hydrocephalus may follow the rachitical process. Afterward, when the craniotabes has healed, the secondary effusions will generally also disappear, but not a few cases of hydrocephalus may be traced to rachitis occurring during the first half year of life. When that occurs, the intellectual faculties may suffer, while, on the contrary, complete recovery not infrequently exhibits an unusual degree of mental development, for the same reason which improves the chances of the development of the bone. The degrees of physiological and pathological nutrition and over-nutrition are very variable in their nature and results.
This condition of the cranial contents is not the only one brought about by rachitis. The softness of the cranial bones permits a direct pressure on the brain. The side on which the infant for the most part reposes gets flattened, and the brain is also compressed. The skull consequently bulges out in the opposite direction. This anomaly, as stated above, is sometimes visible through life, though in the large majority of cases after recovery from rachitis has taken place this asymmetry will gradually disappear. Before that can occur, however, the infant is liable to suffer from the rachitical changes. Convulsions are by no means rare. Vogel has, however, been able to produce an attack of convulsions by pressing upon the softened spots of the cranium. Permanent or temporary contractures of the fingers and toes I have seen in several instances. Gerhardt looks upon rachitis as one of the causes of tetany.
A frequent symptom of the cerebral changes which take place during, and in consequence of, craniotabes is the crowing inspiration, or laryngismus stridulus, of infants. It may be mild or severe. The mild form is very frequent, and consists in the occurrence of a shrill inspiratory sound while the baby is either quite placid or excited or crying. It is frequently overlooked entirely, is usually overcome after a number of months, and gives rise to serious trouble in but very few instances. The severe form is of a different nature. While the baby is awake or asleep, without any premonitory symptoms, while playing or crying, placid or excited, all at once respiration will cease. This will take place, usually, after expiration. The limbs are hanging down, as it were lifeless, the face turns pale, then purple, and slight convulsive twitching may set in for ten or twenty seconds. There appears to be a complete paralysis, and death from apnoea seems to be imminent. All at once, a long, deep crowing inspiration will be heard, respiration will commence again, and the whole terrible attack is overcome. It may return a number of times every day, or sometimes not for several days, during a period of many weeks or several months. The attacks which set in after inspiration are apt to be more dangerous. In such an one, but also in the other kind which sets in after the expiratory movement, death may occur suddenly, or the attack may be followed by a convulsion which may terminate fatally like any other eclamptic seizure. In this manner it is that the majority of cases of rachitis perish which terminate fatally during the active progress of the morbid process. In this connection, however, it may be well to add that craniotabes is not the only cause of laryngismus, particularly when the [p. 150]latter is found in the second year of life, or even later. But almost every case, without any exception, which is observed during the first eight or nine months is due to that very cause; and a good many cases occurring later, when the craniotabic bones have become normal, arise from the effects, either meningeal or encephalic, of the rachitic process. Still, complications of craniotabes with a large size of the thymus gland may occur, and enlargements of the tracheal and bronchial lymphatic glands are quite frequent, as we shall see below.15
15 Z. Oppenheimer prefers the name rachitic asthma in place of laryngismus, and suggests an explanation of the symptoms from a strictly anatomical point of view. If not correct, it is at all events interesting, as everything this ingenious writer proposes. He points to the ligament situated between the spinæ intrajugulares of the temporal and occipital bones, which, as long as it is of normal consistency, separates the jugular vein from the pneumogastric nerve. As it is covered with periosteum and dura, it is apt to ossify, and forms an osseous partition in the foramen jugulare, which participates in all the changes taking place in the periosteum. As this becomes softened and succulent, so will the ligament, either on both sides or on either. Its influence on the neighborhood depends on its size or succulence (as also on the difference in width of the foramen jugulare or lacerum, which corresponds with the difference in size of the transverse sinuses). The irritation of the pneumogastric is perhaps easily explained thereby, but in very exceptional cases only the accessory nerve would be affected. As, however, the latter controls the sterno-cleido mastoid and trapezius, and also the laryngeal muscles, and is apt to provoke cardiac paralysis during diastole, the occurrence of sudden death would be best accounted for.
While the size of the cranium is normal, or sometimes more than normal, the face undergoes some changes which result in absolute or relative diminution of size. These depend mostly on a reduction in the volume of the jaws. Glisson knew of it, and therefore looked for the cause of rachitis in the process of dentition. Now, both maxillæ are liable to become rachitical at an early date, as early indeed as the bones of the cranium. Rachitical deposits and softening take place in them very generally. The lower maxilla is flat anteriorly, it loses its rounded outline, is shorter in longitudinal direction, while the rami are thick and clumsy; the whole bone is shorter than normal, and sometimes asymmetric. Its changed appearance is greatly due to the effect the muscles, with their powerful insertions, produce on the softened bone; mainly the masseter, also the mylohyoid, which draws the lateral portions inward, and the geniohyoid, which pulls at the central portion. Of the latter, the lower portion is drawn out, the inner and the alveolar part inward. Thus, the teeth, mainly the incisors, of the lower jaw are turned inward to such an extent that, as those of the upper look outward, the two rows of teeth do not touch but cover each other. Besides, the periosteal proliferation around the alveoli is excessive, sometimes so much so as not only to crowd the teeth into irregular positions, but even to absorb and annihilate alveolar processes in the course of the morbid changes. The cases in which the number of teeth are actually diminished by rachitis are not at all rare. In the superior maxilla the last-described anomaly is also observed. Periosteal thickening is mainly noticed about the intermaxillary bone—sometimes to such an extent that above and behind it a considerable impression takes place. The shape of the upper jaw is more spherical than normal, and the cheek-bones become very prominent.
The belief that maxillary rachitis is now and then met without any other symptom of rachitis I do not share. What I said of craniotabes is also valid in regard to this form.
[p. 151]Irregular teething is a constant companion of maxillary rachitis, but is also present where the latter is not well, or not at all, marked. As a rule, the first teeth protrude late, about the ninth or tenth or twelfth month. That the first year and more should elapse without any tooth is of frequent occurrence in rachitis. Cases in which the first teeth do not come before the second year is completed are not very uncommon; in some there are none even when the child is much older. In most cases the retardation of dentition goes hand in hand with very marked retardation in the development of the rest of the bones and in the closure of the cranial fontanel. But not in every case of rachitis is there a retardation in the process of teething. In some a few teeth appear at the regular period (at the completion of the seventh or eighth month), or even at a very early age (in the fourth or fifth month); after which there is an interruption in the protrusion of teeth for an indefinite period. Evidently, the period in which rachitis is developed exerts its influence on the teething process. When it exists at a very early age, it will retard teething until recovery takes place. Still, it is possible that a moderate amount of periosteal and osteal hyperæmia and over-irritation matures the teeth abnormally. In all those cases, however, in which rachitis does not occur before the second half of the first year, the first teeth will appear at the normal time, and a long period will follow in which no teeth at all will make their appearance. Then, again, when the whole process comes to a standstill, and recovery takes place with solidification of the bones, and even eburnation, the teeth will come in rapid succession. Whether they will, as is frequent, decay almost as soon as formed, or whether they will be unusually hard, solid, and yellowish, depends on the stage of the disease in which they made their appearance, and on the complications aggravating the case. Of very grave import in this respect are digestive disorders before and during the course of the disease.
The vertebral column suffers also. In the normal infant it is straight, but in the rachitic it exhibits a kyphotic deformity very soon. When such a baby of three or six months is sitting up, the middle portion of the back is protruding, as in Pott's disease. In almost every case, however, this kyphosis is but apparent and the result of muscular debility. In order to arrive at a diagnosis at once, it is sufficient to place the patient on his face and support the head, and raise the lower extremities and pelvis in the air. If the kyphosis is but functional, the prominence disappears at once. By nothing can the muscular insufficiency of early rachitis be better demonstrated than by this little experiment. But actual deformity is also found in rachitis. It softens both the vertebræ and intervertebral cartilages, and either their anterior or posterior portion may be irregularly developed, and be either too high or too low. Besides, the articulating surfaces are sometimes too convex. Thus the causes of both kyphosis and scoliosis are amply furnished, and complications of the two are quite frequent, and the deformities resulting therefrom quite formidable. Scoliosis is mostly to the left; kyphosis generally complicated with lordosis, and sometimes the vertebral column exhibits a spiral shape.
The ribs of the convex half are prominent and divergent, those of the concave side flattened and parallel. The two halves of the chest are therefore very unequal indeed. Muscular traction, atmospheric pressure, [p. 152]the elastic traction of the lungs, the presence of pulmonary complications, and the pressure from below on the part of the enlarged viscera of the abdominal cavity, come also in for a considerable share in the completion of the deformity.
The ribs and the sternum aggravate it considerably. Even without any affection of the vertebral column they suffer seriously from the general affection. The manubrium is thickened and drawn inward, the ensiform process protuberant, the sternum often swelled and painful to the touch. The ribs are sensitive to the touch on one or both sides. The child cries when taken up or when fearing to be taken up. The costo-cartilaginous junctures are thickened, mainly so from the fourth to the eighth ribs. The insertion of the diaphragm becomes soon perceptible by a deep groove around the chest. The anterior portion of the ribs is flattened, posteriorly they are inserted at acute angles. Thus the intrathoracic space becomes narrow, the sternum with the costal cartilages is pressed forward (pigeon breast, pectus carinatum), the thorax is deprived of its elliptical shape and becomes triangular, the dorsal aspect being flattened, and the distance between the vertebral column and the sternum increased. Below the diaphragmatic groove the thorax expands, the liver and other abdominal organs crowding the ribs outward. All sorts of changes are experienced by the ribs in these conditions. Parts of them are flattened, parts undergo infraction, parts are even concave; they are bent and twisted, now and then to such an extent as to turn the concave side out, the convex surface in. In addition to all this, the scapula is big and clumsy and protuberant, the clavicle considerably bent and frequently infracted, and not rarely covered with genuine callus.
That the respiratory and circulatory organs must suffer from such anomalies, though they be not excessive, is certain. The heart is crowded by the flattening of the ribs and the contraction of the thoracic cavity. Its beat is visible over a large surface, and its percussion dulness is extended over its normal space, though no enlargement have taken place. This, however, is very apt to occur after some time by overexertion. The latter is increased by the condition of the respiratory organs. The ribs being flexible, the chest contracted and compressed, the diaphragm raised, the respiratory muscles feeble, respiration is insufficient, even without the presence of any further complications; thus dyspnoea and a certain amount of cyanosis are frequently met with in consequence of the anatomical changes only. In addition to this, there is from the beginning a tendency to catarrhal and inflammatory conditions. Even without any deformity the rachitical process is accompanied from an early time with bronchial and tracheal catarrh. A chronic cough in an infant, with very little or no fever, disappearing and returning, mostly with copious secretion—which, however, is swallowed as soon as it reaches the pharynx—rouses the suspicion of general rachitis. It is often complicated with extensive dulness over the manubrium sterni, due (to rachitical thickening of this bone and) mostly to the persistence of a large size of the thymus gland; and also with enlargement of the bronchial and tracheal glands, the latter of which are often accessible to recognition by percussion. They are to be looked upon as a frequent occurrence in rachitis, though no associated diseases leading to their enlargement have been noticed. They and the chronic tracheo-bronchial [p. 153]catarrh are closely dependent upon each other. They are each other's both cause and effect. Neither of them, however, remain uncomplicated. Catarrh grows into broncho-pneumonia, with frequent returns. Atelectasis, interstitial pneumonia, dilatation of bronchi, and pulmonary consumption are often traceable to such apparently slight catarrhs, which, when not recognized as depending on their constitutional cause, cannot be removed. Nor are the cases of miliary tuberculosis, resulting from caseous degeneration of rachitical glands, very exceptional.
The anatomical changes in the abdominal viscera may be due to the preparatory diseases or the complications of rachitis; but, at all events, the abdomen yields a number of changes visible through the whole duration of rachitis. It is very large; its size is due to the contraction of the thoracic cavity and the downward pressure of the chest-wall upon the contents of the abdominal cavity. It is also due to the changes wrought by rachitis in the pelvis. Softening of bones and synchondroses, torsion, the weight of the trunk, and the pressure of the femora from below produce the change of the pelvis so well known and much feared in the parturient female. The promontory and sacrum are pushed in, the arcus pubis is large, the pelvis asymmetric; the small pelvis is contracted, the large pelvis broader. Thus, the small pelvis has no room for viscera, which, then, are crowded upward. The digestive disorders which gave rise to, or formed the first stage of, rachitis result in the accumulation of gas; the scrobiculus cordis is greatly expanded. The liver16 is large, congested, and in fatty degeneration. The latter is the more frequent the more a certain degree of fatty condition is a normal attribute of every infant liver. When the liver is found but small in post-mortem examination, it is so because of the general anæmia and emaciation. Sometimes it is amyloid, as are also the spleen (mostly hyperplastic only), the kidneys, and the arteries of the intestines in many instances.
16 Dr. Norman Moore presented a cast and drawing to the Pathological Society of London (Trans., vol. xxxiv., 1883, p. 185) showing how considerable may be the digressions of the diaphragm and local pressure upon the liver in a case of rickets. Three large beads caused as many projections from the under side of the diaphragm, and corresponded with local thickenings of the capsule of the liver, probably produced by the continued pressure through the diaphragm of the beads, which were on the seventh, eighth, and ninth ribs, and the largest of which was equal in size to a hazel-nut.
The alimentary tract is the seat of many changes recognizable during life. The tonsils are often large. The tongue is seldom coated to an unusual degree. On it are found little islands, red, marginated, deprived of epithelium. They will increase in size and number and extend backward. They will heal and reappear. They are by no means syphilitic, as Parrot would have it, and correspond exactly with the erosions near the solitary glands and those of Lieberkühn in the intestinal part, which mean nothing else but a nutritive disorder of the epithelia, and give rise to nothing worse than incompetency of absorption in that locality and abnormal secretion. The stomach is in a condition of chronic catarrh, sometimes dilated. Acid dyspepsia is frequent. Anorexia and bulimia will alternate. Feces contain an abnormally large amount of lime. Diarrhoea and constipation will follow each other in short intervals. The former owes its origin to faulty ingesta or chronic catarrh; the latter, sometimes to improper food, but more generally to muscular insufficiency. [p. 154]This condition has not been estimated at its proper value. Besides myself,17 nobody but Bohn has paid the attention to it which it deserves. Here, again, I have to insist that rachitis is a disease of the whole system, and not exclusively of the bones. Indeed, the muscular system is amongst the first to suffer. In the same way in which the voluntary muscles are not competent to raise and support the head or to allow a baby to sit up without a functional kyphosis, the involuntary muscles of the intestine are too feeble for normal peristalsis. The infant of a month or two months of age may have had normal and sufficiently numerous evacuations; gradually, however, constipation sets in; the feces become dry, but are perhaps not much changed otherwise. If no other cause be apparent, the suspicion of rachitical constipation is justified. Seldom, however, after it has lasted some time—and only after some time has elapsed relief will be sought—it will remain alone. Other symptoms of rachitis will turn up and the case be easily recognized. This constipation is an early symptom, as early as thoracic grooving or craniotabes. Very often it precedes both—is, in fact, the very first symptom—and ought therefore be known and recognized in time.
17 Jour. Obst., etc., Aug., 1869.
The kidneys have been mentioned above. They are often found rather large. Though the fact has been alluded to before, I will here again state that it has always been the general impression that the amount of lime eliminated in the urine of rachitic children is excessive. The reverse of that is true. Seemann and Lander have proved beyond dispute that in most stages of rachitis there is less than the normal amount of lime in the urine. Thus, the theory that lime is eliminated by an excess of acids in the blood is proven to be incorrect. But it is a fact that the rachitical bone contains a proportionately small amount of lime. The conclusion is, then, that its introduction must have been diminished. On the other hand, every article of food contains a large amount of lime, which might be introduced into the circulation if digestion be not at fault. The fact is, that a large amount of lime introduced is not utilized, and is eliminated with the feces.
In connection with these facts the following will be found very interesting. It has been found by Bunge that when potassium, with the exception of chloride of potassium, meets chloride of sodium, the two will exchange their acids, so as to form chloride of potassium and phosphate of sodium. They will be found in the blood also, will be eliminated as such, and result in a comparative absence of chloride of sodium from the serum of the blood. Now, comparative absence of chloride of sodium diminishes the possibility of the development of hydrochloric acid. Thus, it is not a surplus of acid, but a lack of hydrochloric acid, which results from such chemical combinations. If such be the case, calcium salts are not absorbed sufficiently. Thus, they will appear in the feces, and not even be absorbed in the intestines, because of the alkalinity of the intestinal secretion, by which the lime cannot be dissolved. The more lime, then, is introduced under these circumstances, the greater the incumbrance to digestion.
The correct proportion between chlorine, phosphorus, potassium, and sodium is certainly exhibited in woman's milk. There is lime enough in even the poorest article of that kind. But indigestion brought on by [p. 155]woman's milk in a disordered condition or by any other cause will prevent the absorption of lime when a superabundance of phosphorus and potassium disturbs the formation of hydrochloric acid. In these cases not only the development of the bones, but also that of the muscles, is disturbed. The latter is of great importance in regard to circulation, because a large part of the circulation depends on the pressure on the part of the muscular fibres exerted on the small blood-vessels. These facts have been the reason why I insist upon the addition of chloride of sodium to the food of infants and children, particularly those who are fed on cow's milk; for cow's milk and vegetables contain a relative superabundance of potassium compared with sodium. Even adults will find cow's milk very much more digestible by adding table-salt to it.
The extremities begin to suffer at a later period than the ribs and cranium. The opinion of Guérin, that the rachitical process begins in the lower extremities and ascends gradually, is erroneous. It cannot even be stated that the lower extremities are affected sooner than the upper. There is no regularity at all; it is not even necessary that all the osseous tissue should fall sick. But this can be taken as a fact, that hands and feet, and particularly the phalanges, are the latest to undergo the rachitical change. First in the line of morbid alteration of the bones are the epiphyses, mainly of the tibia, fibula, radius, and ulna. Their integument appears to be thin; now and then the cutaneous veins are dilated. The periosteum of the diaphysis becomes thick, softened, and painful to the touch and pressure, its compact layer thin, the medullary space large, the whole bone flexible, at the same time that the ligamentous apparatus of the joint becomes softened and flabby. At this time babies are greatly admired and applauded for the facility with which they introduce their feet into their mouths. For at the same time the bones begin to curve under the influence of the flexor muscles, which are always stronger, as they do in later months under the weight of the body when the child begins to walk. The curvature is not always a mere arching, but sometimes the result of infraction (green-stick fracture), a complete fracture not being accomplished because both of the softness of the osseous tissue and the resistance on the part of the thickened and softened periosteum. Both the legs and forearm bend on the external side, the resulting concavity looking inward. The humerus bends in a direction opposite to that of the forearm; the thigh, usually outward and forward.
The attempts at locomotion are often the causes of quite preposterous anomalies; creeping, sliding, walking, turn the extremities in such unexpected directions that talipes valgus, genu valgum, and now and then double curvatures, are the results. These, however, may not always be very marked, but there is one change in the rachitical bone which is constant—viz. the impairment of longitudinal growth. In every case the diaphyses remain abnormally short, and the proportion of the several parts of the body are thereby disturbed. Chiari measured parts of the skeleton of a rachitical woman of twenty-six years who was nine years old before she could walk. Her height was 116 centimeters, the length of the lower extremities 42, femur 23, tibia 15, fibula 20, humerus 16, right radius 12.5, left radius 11, right ulna 15, left ulna 14 centimeters. In a second case the parts of the skeleton were measured after they had [p. 156]been extended with great care. The right arm from the acromion to the middle finger (incl.) was 39 centimeters, the left 38; the right lower extremity from the trochanter to large toe (incl.) 39, the left 41.
The skin participates in the general nutritive disorder. It is soft and flabby. In those infants who become rachitical gradually while proving their malnutrition by the accumulation of large quantities of fat, it exhibits a certain degree of consistency. When rachitis develops in the second half of the first year or later, with the general emaciation the skin appears very thin, flabby, unelastic. The veins are generally large. Complications with eczema and impetigo are very frequent; where they are found the glandular swellings of the neck and below are still more marked than in uncomplicated cases. Circumscribed alopecia is sometimes found (not to speak of the extensive baldness of the occiput). It is not attended with or depending on the microsporon Audouini, but the result of a tropho-neurosis. In the hair Rindfleisch found fat-globules between its inferior and central third. Then it would break, the axial evolution would cease, and the end become bulbous by the new formation of cells.
There is a form of rachitis which may be, and has been, called multiple epiphysitis or multiple periostitis of the articular ends of the long bones. The changes which in the usual form of rachitis require months to develop take place in a very short time. Not infrequently the children were quite well before they were taken with this peculiar affection. Cases have been known to occur between the fourth and twenty-fourth months of life, and to last from two to six weeks, or just as many months. They have been known to get well, or a few of them terminate fatally. They are accompanied with fever and rapid pulse, perspiration, now and then with diarrhoea, with eager or reduced appetites. At the same time the epiphyses swell very rapidly, and are painful. The same is true of the diaphyses and the flat bones of the head. Many authors do not recognize this form as an independent variety. Some call it an acute initial stage of certain cases of rachitis, as they are not infrequently found in infants which exhibit a very rapid growth. Some have taken it as an independent disease, developed on the basis of a constitutional disposition; some look upon it as a very intense acute form of rachitis; others, as an intense growth of the osseous tissue only. Others call it an inflammation of the bone. Some refer it to hereditary syphilis, and a few to the influence of malaria. That the disease is epiphysitis and periostitis there is no doubt. I do not hesitate to claim it as rachitis, for epiphysitis and periostitis of early age not of rachitical basis are not apt to run such a favorable course as this form frequently does. The cases complicated with subperiosteal hemorrhages are claimed as scurvy by Th. Barlow.
The differences of opinion would probably not have been so great if every author had seen all the cases of the other observers. It will not do to judge of unobserved cases by the light shed by a single case under one's own observation. I have seen cases of acute rachitis which were [p. 157]the initial stages of general rachitis, and have observed those of local or multiple epiphysitis, mainly after infectious fevers, which were diagnosticated as such. They are, however, very uncommon. But even without a preceding infectious fever, such as scarlatina or more frequently typhoid fever, there are unexplained cases of rachitis and deformity. Thus, R. Barwell had some before the Pathological Society of London,18 which are positive proofs that some forms of ostitis may occur and result in the most formidable deformities without being rachitical. A girl of seventeen years was perfectly well formed up to the age of two and a half or three years. After that time the deformities began to develop, and did not change after she was thirteen, at which time the author saw her the first time.
Her | left humerus measured | 7¾ inches | from shoulder to elbow; | distance 6¼ |
right humerus measured | 7½ inches | from shoulder to elbow; | distance 4¼ | |
left tibia measured | 10 inches | from knee to ankle; | distance 7¼ | |
right tibia measured | 9½ inches | from knee to ankle; | distance 4½ |
Her bones were always very brittle. When she was between nine and thirteen she broke her arms four times and her lower limbs on several occasions. A male patient of twenty-two years, who was born healthy and well formed, continued thus until five years of age, when he was attacked with a fever, after which his bones became soft and bent. Osteotomy was performed on him, and the femora were found to be mere thin shells of bones surrounding cavities containing great quantities of medulla, which flowed out of the wound as oil; five ounces were discharged at once. In both cases there appeared to be a hypertrophy of the medulla at the expense of the bone-substance—a condition which Barwell proposes to call eccentric atrophy. "While these subjects are still youthful very little bone-earth is deposited, or at least remains in the very thin layer of osseous tissue that subsists. The relationship between infantile ostitis and extreme development of the intraosseous fat, though well known, is still occult; neither should we lose sight of the possibility that the softening process of ostitis may be due to a fatty acid. Now, fatty ostitis usually occurs in epiphyses. In these cases the shafts were affected."
18 Trans., xxxiv., 1883, pp. 203-208.
PROGNOSIS.—The course and the prognosis of rachitis are, as a rule, favorable, but they change according to the degree and locality of the affection and the age of the patient. Generally there is neither fever nor rapid exhaustion. But the process lasts for months and even years. In favorable cases, when recovery takes place the teeth will grow faster, the bones become firmer, the epiphyses will diminish in relative size, the bowels become regular. But the length of the bones is, and remains, reduced, and the head remains large as compared with the length of the body. Not only are the bones of normal firmness, but the compact substance undergoes a process of hardening called eburnation by Guérin. The internal organs also become very active, perhaps because the total amount of blood has to supply only a body less extended in length. Nor does the brain suffer after complete recovery has taken place. On the contrary, it appears that the somewhat more than normal vascular dilatation, which under unfavorable circumstances leads to effusion, is [p. 158]frequently apt to nourish the organ of intellect up to a higher standard. In all cases of rachitis, however, the curvatures of the extremities will not disappear altogether, while mild ones, it is true, are hardly recognizable in advanced age. Curvature of the ribs and of the vertebral column, however, will remain, and interfere with the expansion and the normal functions of the lungs and heart. In regard to the lungs, it appears that in many cases they do not find sufficient space to expand. As far as the heart is concerned, it touches the flattened, no longer elliptic, chest-wall over a larger surface, and is very apt to give rise to the suspicion of enlargement in consequence of extended dulness on percussion. The rachitic pelvis is well known to the obstetrician for the difficulties it gives rise to during parturition.
Thus, the prognosis would, as a general thing, be sufficiently favorable if it were not for the number of complications or severe symptoms. The chronic catarrh of the lungs accompanying rachitis, the enlargement of the tracheal and bronchial glands and the lymphatic glands in general, are apt to lead to inflammatory disease of the lungs, which, after having returned several times, leads to infiltration of the lungs with caseous deposits, and not infrequently results in phthisis. The nervous symptoms accompanying craniotabes may prove very dangerous. Spasm of the larynx and laryngismus stridulus may prove fatal in a single attack by suffocation, or general convulsion may set in during an attack of laryngismus or without it, in which the child may perish. Therefore the prognosis in every case of laryngismus and in every case of craniotabes has to be very guarded. It is my rule to wait from six to eight weeks before giving expression to a decided prognosis, because during that time medicinal and dietetic treatment will probably have resulted in such an improvement of the symptoms and condition as to render the prognosis more favorable. Under no circumstances, however, ought we to lose sight of the fact that, though rachitis may disappear, the causes leading to it may still linger on. Defective nutrition, diseases of the lungs, and intestinal affections which gave rise to or accompanied rachitis will complicate the prognosis, though rachitis itself, as far as the bones were concerned, be no longer in existence.
TREATMENT.—To meet the cause of a disease by preventive measures is the main object and duty of the physician. He thus either obviates a malady or relieves and shortens it. Now, if the original disposition to rachitis, as has been suggested, is to be looked for in early intra-uterine life, when the blood-vessels begin to form and to develop, we know of no treatment directed to the pregnant woman or uterus which promises any favorable result. But the more we recognize an anatomical cause of the chronic disorder, the more we can appreciate the influence upon the child of previous rachitis in the mother, and are justified in emphasizing the necessity on the part of the woman to be healthy when she gets married, and to remain so while she is pregnant. After the child is born the most frequent cause of rachitis is found within the diet or the digestion of the patient. To attend to the former is in almost every instance equal to preventing disorders of the latter; for most of the digestive disturbances during infancy and childhood are the direct consequences of errors in diet. It is, however, impossible to write an essay on infant diet in connection with our subject. I have elaborated the subject in my [p. 159] Infant Diet (2d ed. 1876), in the first volume of Buck's Hygiene, and of C. Gerhardt's Handbuch d. Kinderk. (2d ed. 1882). Still, the importance of the subject requires that some points should be given, be they ever so aphoristic.
The best food for an infant, under ordinary circumstances, is the milk of its mother. The best substitute for the mother is a wet-nurse. Woman's milk ought not to be dispensed with when there is the slightest opportunity to obtain it, particularly when the family history is not good and nutritive disorders are known to exist, or to have existed, in any of its members. When it cannot be had, artificial food must take its place, and it is in the selection of it where most mistakes are constantly made. This much is certain, that without animal's milk no infant can or ought to be brought up; as ass's milk can be had only exceptionally, and dog's milk, which has been said to cure rachitis, is still less available, the milk of either goat or cow must be utilized. The former ought not to be selected if the latter is within reach, mainly for the reason that it contains, besides other objectionable features which it possesses in common with cow's milk, an enormous percentage of fat. Cow's milk differs in this from woman's milk, that it contains more fat, more casein, more potassium, and less sugar than the latter, and that its very casein is not only different in quantity, but also in chemical properties. Even the reaction of the two milks is not the same, woman's milk being always alkaline, cow's milk often either neutral or amphoteric, and liable to acidulate within a short time. Thus, the dilution of cow's milk with water alone yields no equivalent at all of woman's milk, though the dilution be large enough to reduce the amount of casein in the mixture to the requisite percentage of one, and one only, in a hundred. The addition of sugar (loaf-sugar) and of table-salt, and sometimes alkali (bicarbonate of sodium or lime-water, according to special circumstances), is the least that can be insisted upon. Besides, the cow's milk must be boiled to prevent its turning sour too rapidly, and this process may be repeated to advantage several times in the course of the day. Instead of water, some glutinous substance must be used for the purpose of diluting cow's milk. As its casein coagulates in hard, bulky curds, while woman's milk coagulates in small and soft flakes, some substance ought to be selected which keeps its casein in suspension and prevents it from curdling in firm and large masses. Such substances are gum-arabic, gelatin, and the farinacea. Of the latter, all such must be avoided which contain a large percentage of amylum. The younger the baby, the less is it in a fit condition to digest starch; thus arrowroot, rice, and potatoes ought to be shunned. The very best of all farinacea to be used in diluting cow's milk are barley and oatmeal. A thin decoction of either contains a great deal of both nutritious and glutinous elements, the former to be employed under ordinary circumstances, the latter to take its place where there is, on the part of the baby, an unusual tendency to constipation. The decoction may be made of from one to three teaspoonfuls of either in a pint of water; boil with a little salt, and stir, from twelve to twenty minutes, and strain through a coarse cloth. It ought to be thin and transparent. Then mix with cow's milk in different proportions according to the age of the baby. Four parts of the decoction, quite thin, and one of milk (always with loaf-sugar), for a newly-born, equal parts for an infant of six months, [p. 160]and gradual changes between these two periods, will be found satisfactory. Whenever there is a prevalence of curd in the passage the percentage in the food of cow's milk must be reduced, and now and then such medicinal correctives resorted to as will improve a disturbed digestion. Care ought to be taken lest for the newly-born or quite young the preparations of barley offered for sale contain too much starch. The whiter they are, the more unfit for the use of the very young, for the centre of the grain contains the white and soft amylum in preference to the nitrogenous substances which are found near the husk. Thus, it is safest to grind, on one's own coffee-grinder, the whole barley, but little deprived of its husk, and thus secure the most nutritious part of the grain, which is thrown out by the manufacturer of the ornamental and tidy packages offered for sale. But very few cases will ever occur in which the mixtures I recommend will not be tolerated. In a few of them, in very young infants, the composition recommended by Meigs19 has proved successful. It consists of three parts of a solution of milk-sugar (drachm xvij¾ in pint j of water), two parts of cream, two of lime-water, and one part of milk. For each feeding he recommends three tablespoonfuls of the sugar solution, two of lime-water, two of cream, and one of milk: mix and warm. The baby may take all of it, or one-half, or three-fourths.
19 In Med. News, Nov. 28, 1882.
The recommendations given above are based on a long experience, and the simplicity, cheapness, and facility of preparation of the articles. The substitutes offered for sale under the title of infant foods are in part worthless, all of them expensive when compared with the simple articles recommended by me, and not recognizable as to their uniformity and compounds. But no matter how appropriate my mixture may be, it is always for the young infant to be considered as a makeshift. It is to be used as a representative of mother's milk only when this cannot be had. Therefore it is better to alternate with breast-milk when this is secreted in but an insufficient quantity. Some good breast-milk is better than none at all; but with this proviso, that it is good. There are some milks either too watery or too dense and white. The former will produce diarrhoea, the latter hard and dense curd. The former may be improved by feeding and strengthening an anæmic and overworked mother; the latter, by giving the baby, before each nursing, a tablespoonful of a mixture of barley-water and lime-water, or, when it produces constipation, lime-water and thoroughly sweetened oatmeal-water. The cases in which breast-milk, such as can be had, is not digested by the infant are rare, but they will occur. In them the proper substitute will yield a better result than mother's milk; for mother's milk will not always be a boon, and must then be dispensed with. Particularly is this so when it is too old. Weaning ought to take place when the first group or the first two groups of teeth have made their appearance. After that time mother's milk is no longer the proper food, and instead of preventing indigestion and sickness it is a frequent cause of them and of rachitis. Instead of muscle, it will then give fat, and the large fontanels and big head, the paleness of the rotund cheeks, the flabbiness of the soft abdomen and thighs, will tell the story of rachitical disease slowly engendered by the persistent employment of an improper article of food. I cannot insist too often on this, that rachitis may develop with increasing weight, [p. 161]and that the use of the scales alone is no means of ascertaining the healthy condition of a baby. As much harm, therefore, can be done by weaning too late as by so doing too early or too abruptly.
At that early age we treat of here, digestive disorders are more frequently the results of improper diet than of a primary gastric disturbance. But when the latter is once established it furnishes its own indications. A frequent occurrence, together with a general gastric catarrh, is the presence of fat acids in the stomach, such as an improper amount of lactic, acetic, butyric, etc. acids. Before digestion can be anything like normal they must be neutralized. For that purpose calcined magnesia, carbonate and bicarbonate of sodium, prepared chalk, and lime-water have been found useful. The latter, as it contains but a trifle of lime, in order to neutralize must be given in larger doses than is usually done; a tablespoonful contains but a quarter of a grain of lime. And all of the alkalies must not be given in the food only, but also between meals. For when given in the former way alone it neutralizes the abnormal and injurious acids, together with the normal digestive secretion, the lactic and muriatic. Not infrequently, when the infants have suffered for some time, general anæmia will set in, and result in diminishing the normal secretions of the mucous membranes (and glands). In those cases which do not produce their own gastric juice in sufficient quantity or quality pepsin and muriatic acid may be given to advantage. In these cases the plan suggested by me is particularly favorable—viz. to add a fair amount of chloride of sodium (one-half to one drachm daily) to the infant's food. Also that of I. Rudisch referred to by me previously,20 who mixes one part of dilute muriatic acid with two hundred and fifty of water and five hundred of milk, and then boils (one-half teaspoonful of dil. mur. acid, one pint of water, one quart of milk). Again, there are the cases in which wine and the bitter tinctures, which are known to increase the secretion of gastric juice, render valuable service. The addition of bismuth to any of the proposed plans is quite welcome. As a disinfectant and a mild cover on sore and eroded mucous membranes it has an equally good effect.
20 Am. Jour. Obstet., July, 1879.
Under the head of roborants we subsume such substances, either dietetic or remedial, which are known or believed to add to the ingredients of the organism in a form not requiring a great deal of change. Rachitical infants require them at an early period. Meat-soups, mainly of beef, and of mutton in complications with diarrhoea, ought to be given at once when the diagnosis of rachitis becomes clear or probable. Any mode of preparation will prove beneficial; the best way, however, is to utilize the method used by Liebig in making what he called beef-tea. A quarter of a pound of beef or more, tender and lean, cut up finely, is mixed with a cup or a tumbler of water and from five to seven drops of dilute muriatic acid. Allow it to stand two hours and macerate, while stirring up now and then. This beef-tea can be much improved upon by boiling it a few minutes. It may be given by itself or mixed with sweetened and salted barley-water or the usual mess of barley-water and milk which the infant has been taking before. Older infants, particularly those suffering from diarrhoea, take a teaspoonful of raw beef, cut very fine, several times a day. It ought not to be forgotten, however, [p. 162]that the danger of developing tænia medio-canellata from eating raw beef is rather great. Peptonized beef preparations are valuable in urgent cases.
Iron must not be given during any attack of catarrhal or inflammatory fever. The carbonate (cum saccharo) combines very well with bismuth; a grain three times a day, or less, will answer well. The citrate of iron and quinine (a few grains daily) can be given a long time in succession. The syrup of the iodide of iron (three times a day as many drops as the baby has months up to eight or ten), in sweetened water or in sherry or malaga, or in cod-liver oil, acts very favorably when the case is, as so frequently, complicated with glandular swelling.
Cod-liver oil, one-half to one teaspoonful or more, three times a day, is a trusted roborant in rachitis, and will remain so. Animal oils are so much more homogeneous to the animal mucous membrane than vegetable oil that they have but little of the purgative effect observed when the latter are given. The former are readily absorbed, and thus permit the nitrogenous ingesta to remain in store for the formation of new tissue, but still affect the intestinal canal sufficiently to counteract constipation. As the latter is an early symptom in a peculiarly dangerous form of rachitis, cod-liver oil ought to be given in time (in craniotabes). Diarrhoea is but seldom produced by it; if so, the addition of a grain or two of bismuth or a few doses of phosphate of lime (one to four grains each) daily, may suffice to render the movements more normal. There are but few cases which will not tolerate cod-liver oil at all. The pure cod-liver oil—no mixtures, no emulsions—ought to be given; the large quantities of lime added to it in the nostrums of the wholesale apothecaries embarrass digestion and bring on distressing cases of constipation. These mixtures have been prepared and are eulogized on the plea of their furnishing to the bones the wanting phosphate of lime. The bones, however, as we have seen before, are not grateful enough to accept the service offered. But only a certain amount of phosphate of lime is useful in rachitis and in digestive disturbances. In small doses it neutralizes acids like other alkalies; its phosphoric acid combines with sodium very easily, and gives rise to the formation of glyco-phosphoric acid, which is of very great importance in the digestive qualities of the upper portion of the small intestines.
Plain malt extracts will be well tolerated by some older children. The preparations which are mixed with a goodly part of the pharmacopoeia by generous manufacturers are to be condemned.
Craniotabes requires some special care in regard to the head. The pillow ought to be soft, but not hot; no feather pillow is permitted. The copious perspiration of the scalp requires that it should be kept cool, the perspiration wiped off frequently to avoid its condensing into water, and the flattening side of the head may be imbedded in a pillow with a corresponding depression. Copious perspiration indicates the frequent washing with vinegar and water (1:5-6). The muscular debility commands great caution. The baby must not be carried on the arm, but on a pillow which supports both back and head, or in a little carriage. No sitting must be allowed until the back will no longer bend to an unusual degree. No walking must be encouraged at any time. The patients will walk when their time has come. The bones are so fragile that great care [p. 163]is needed sometimes not to fracture or to infract them and to avoid periosteal pain in lifting. The skin must undergo some training by gradually accustoming the little patient to cool water. It can be readily, but gradually, reduced to 70° for a bath at any season. The addition of rock- or table-salt to the bath is a welcome stimulant. And fresh air ought to be granted freely.
Laryngismus stridulus shares the indications for treatment furnished by craniotabes. The general treatment remains the same. Prominent symptoms and complications ought to be treated besides; constipation requires the more attention the more convulsive attacks of any description may arise from reflex action. The general nervous irritability may be relieved by bromide of potassium, sodium, or ammonium. One gramme daily (15 grains) of either, in three doses, is well tolerated for a long period. When there are symptoms of an imminent convulsion, or to soothe the convulsibility which may break out any moment, chloral hydrate, eight or ten grains in from one hour to four hours, two grains in a dose, will be convenient. If the stomach refuses or is to be spared, from four to eight grains may be given in an enema of warm water. A severe attack of convulsions ought to be checked with inhalations of chloroform. When a warm bath is to be had, care should be taken that the child be not tossed about. Hold the baby in a small sheet or a large napkin, and immerge it thus into the water, raising the head and cooling it with cold cloths or an ice-bag. Genuine attacks of laryngismus with well-developed stages—the first paralytic, the second spasmodic—give but little time for any treatment. The proposition to apply the electrical current is well meant, but the attack has passed by, or terminated fatally, or resulted in a general convulsion, before the apparatus can possibly be in operation. I can imagine, however, that a Leyden flask kept ready might be used to advantage during the stage of apnoea for the purpose of bringing on inspiration. Sprinkling with cold water, beating with a wet towel, shaking by the shoulders, may certainly contribute to awake respiratory movements. The advice to wait quietly until the attack has passed by is more easily given than carried out. Marshall Hall's direction to perform tracheotomy will, I hope, soon be forgotten.
Nothing is more gratefully appreciated by the little patients than air. May it never be forgotten that night-air is better than foul air, and that furnace-air means air greatly modified by injurious additions. More than twenty years ago I was in occasional attendance upon a male baby—now a medical man of some promise—with craniotabes and a number of general convulsions. No treatment would remove, or even relieve, the attacks, until, without the physician's advice, the father took the baby into the street in the hardest winter weather. After the first long absence from his furnace the baby was well of his convulsions, and the physicians profited by their involuntary experience.
In the same way that salt-bathing is beneficial, so is sea-air. A summer at the seaside is a great blessing to rachitical children. Sea-baths have been arranged for them in France (Berx-sur-mer), in Italy (San Ilario di Nervi, Viarreggio, Livorno, Volti, Fano), in England (Margate), in Germany (German Sea, by Prof. Beneke), and for some little time past in the neighborhood of our own large cities.
[p. 164]Complications command great attention in rachitis, particularly where there is danger from the affection of the nerve-centres, for the slightest irritation in some distant part of the body may give rise to an outbreak. Thus, in craniotabes it is desirable to watch even the gums. Not sharing the etiological superstition which attributes so many diseases of infancy to dentition, I still know that a slight irritation of the gums may suffice to exhaust the slim resisting power of the infant. If there be local swelling and congestion of the gums over a growing tooth, it may become necessary, or at least advisable, to lance. An otitis which under ordinary circumstances would give rise to no symptoms at all besides some inconvenience or slight pain will prove the source of great danger in a rachitical (craniotabic) infant. The chronic bronchial catarrh and frequent broncho-pneumonia of such patients require early attention, for they and the neighboring lymphatic glands stand too much in the relation of a vicious circle of cause and effect.
Rachitical constipation, depending on incompetency of the intestinal muscle, must not be treated with purgative medicines. Now and then, when a great deal of abnormal acid is formed in the stomach, calcined magnesia, a grain or two given before each meal, will control that disorder and at the same time keep the bowels open. But, as a rule, every purgative after it has taken effect will leave the intestinal muscular layer less fitted to perform its functions than before. Its place may be taken by a daily enema of tepid water. Further indications are—such a change in the food as will contribute to keep the bowels moist and slippery, but principally such a modification of food and such medical treatment as are known to prove beneficial when all the symptoms of rachitis are fully developed. When the cause of the infant's rachitis can be traced back to the mother or to an insufficient quality of her milk, she must give way to a wet-nurse, or the nurse must be changed for similar reasons. When neither mother nor wet-nurse prove competent, or either be dangerous, artificial food will take their place to advantage in the manner I have stated above. Beef-soup or beef-peptone is to be added to the baby's food daily. Of the two best farinacea, barley- and oat-meal, the latter is preferable as an addition to cow's milk, because of its greatly laxative effect. The percentage of cow's milk in the food ought to be more carefully watched than in other conditions. Pure cow's milk or cow's milk mixed with water only is borne worse in no other condition. Half a drachm or more of table-salt and a few drachms of sugar ought to be added to the daily mess. The general indications require the administration of iron, which has no constipating effect in this ailment. Particularly is that the case with the iodide of iron. Cod-liver oil, in three half-teaspoonful or teaspoonful doses daily, acts very satisfactorily both for its general rachitical and for its local effect on the mucous membranes. Now and then massage, repeated many times a day a few minutes each time, practised with the palm of the hand only, or gentle friction, with the dry or oiled hand, of the abdominal surface, will prove effective in bringing about peristalsis and strengthening the intestinal muscle. An obstinate case may also require two daily doses of one one-hundred-and-fiftieth or one one-hundred-and-twentieth of a grain of strychnia for the same purpose, or such other improvements on the above detailed plan as the judgment of the attending physician may direct. At all events, the diagnosis of [p. 165]any case, and the appreciation of the cause of any ailment, are, to a well-balanced and educated mind, of infinitely greater value than any number of specified rules and prescriptions.21
21 Jour. Obstetr., Aug., 1869.
It is not impossible that phosphorus, in substance, not in any of its compounds, may prove of great utility in the treatment of rachitis. Minimal doses of phosphorus render the newly-formed tissue at the points of apposition of the bones more compact in a very brief time. The new formation of blood-vessels in the osteogenous tissue gets retarded by it. Larger doses of phosphorus, however, increase vascularization, and osseous tissue is either less rapidly formed or even softened. When the doses are still larger, vascularization and softening may rise to such a point as to separate the epiphysis from the diaphysis. Thus the administration of the drug results in an irritation which, according to the doses employed, may give rise either to normal condensation or to inflammatory disintegration. This experience, arrived at by Wegner in a great many experiments made on animals, Kassowitz has confirmed. For its therapeutic effect he tried phosphorus in 560 cases of rachitis. Employing doses of one-half milligramme (one one-hundred-and-twentieth of a grain) several times daily (less will suffice), he soon found the skull to become harder, the fontanel smaller, the softening of the bones of the thorax and extremities to disappear, and all the other symptoms of rachitis to improve. This result was obtained though no particular change in the feeding of the patients was resorted to. To what extent this experience will be verified by others we shall soon learn. My own is already sufficiently extensive to base upon it a strong recommendation of the plan of treatment I have detailed. My therapeutical results in other diseases of the bones also encourage me to believe that phosphorus will accomplish much in the treatment of rachitis. Ever since Wegner's publications—viz. these thirteen or fourteen years—I have utilized phosphorus in cases of chronic and subacute inflammations of the bones, mainly of the vertebral column and the ankle-joint and tarsus. After having taught the method for many years in my clinic and otherwise, I made a brief communication on the subject to the Medical Society of the State of New York.22 Since that time, again, I have followed the same plan in many cases of the same description, and feel sure that the prognosis in this serious class of bone diseases has become more favorable and recovery speedier. Infants of a year or more were given a dose of one-eightieth or one one-hundredth of a grain of phosphorus daily. One grain, dissolved in an ounce of oil or cod-liver oil, is a convenient mixture, four or six drops of which may be administered daily in two or three doses.
22 Trans., 1880.
From what I have seen of phosphorus in bone disease, and what is thus far known by experience in rachitis, it appears to me that it will be of decided advantage in that form of acute rachitis which is apt to destroy rapidly with the symptoms of acute epiphysitis, rapid pulse, diarrhoea, rapid diminution of strength, and scorbutic gum. In the few cases I have seen these last years it appeared to me to act satisfactorily, together with immobilization of the whole body.
Rachitical curvatures are very apt to become less marked while growth is increasing and the limbs extending. But many of them are so marked [p. 166]that they remain for life. Many of these might have been benefited by timely orthopædic interference. That the application of Sayre's jacket is indicated in every form and stage of spondylitis, though it be not equally valuable in all forms, goes without saying; and that infractions ought to be straightened and supported by splints when observed and when practicable, is self-evident. But, as a rule, while the chronic rachitical process is developing in the long bones the use of mechanical apparatuses is of doubtful merit; they ought not to be resorted to before the healing has at least commenced. Nor is it advisable to postpone mechanical interference so long that eburnation of the bones has time to take place. Surgical operations for the purpose of removing the curvature are of different nature according to the different types to be treated. Mere straightening of the curvatures is indicated, and successful with children under two years. Osteoklasy—that is, fracturing of the curvature while the periosteum is left intact—is successful in children of three (or four, according to Volkmann) years. The fracture does not injure the periosteum, and is always transverse. In later years osteotomy has proved successful to an almost unexpected degree, and is one of the happiest achievements of modern surgery.
Partly as a preventive, partly as a curative measure, Gramba of Turin and Pini of Milan point to well-directed gymnastics as a requisite in the treatment of rachitis. For older children they have established schools in which systematic exercises are brought to bear on chronic deformities.
SYNONYMS.—French, Scorbut; Spanish, Escorbuto; Italian, Scorbuto, are the various terms in the Romance languages used to designate this disease, derived from the Middle-Latin word scorbutus, which is evidently an offspring of one of the early Gotho-Teutonic dialects, perhaps of the Low German word Schärbunk, Danish Scorbuck, or the Old Dutch Scheurbuyck, from scheren, to separate or tear, and bunk, the belly. These terms originally denoted rupture of the belly, and afterward scurvy, or scorvy, as it is found in the English dialect. It has also been traced to the Sclavonic word scorb, disease. The first is now believed to be the true etymology.
DEFINITION.—Scurvy is an acquired condition of the body whose essential feature is a perversion of nutrition, which gradually arises from prolonged employment of food deficient in succulent or fresh vegetable matter, and progresses uniformly to a fatal issue, in a longer or shorter time, if the dietetic errors remain uncorrected. This condition becomes manifest by a change in the complexion to a dull yellowish or earthy tint, lassitude, marked decrease in the muscular power, depression of spirits and mental hebetude, breathlessness on the slightest exertion, minute flecks at the roots of the hairs, especially those of the legs; and, later, hemorrhagic effusion into the skin, forming blotches and spots of varying sizes and aspects, which may finally slough and lead to obstinate ulceration; sponginess of the gums, which bleed easily and break down into a detritus that impresses a malodorous taint upon the breath; ecchymotic staining of mucous and serous surfaces, and, in advanced stages of the disease, effusions of bloody serum or of blood into the cavities and tissues of the body.
HISTORY.—Obscure passages in certain of the ancient medical classics (Hippocrates, Celsus) and historical works (Pliny, Strabo) have been considered as descriptive of scurvy, but the earliest trustworthy accounts are to be found in the writings of the thirteenth century. Jacob de Vitry describes an epidemic which occurred among the troops of Count Saarbrücken besieging Damietta in 1218, and Sire de Joinville another epidemic among the troops of Louis IX. lying before the same town in 1249. On both occasions the sufferings of the men were inexpressible and the mortality fearful. The disease was directly traceable to defective supplies of fresh vegetable food, aided by exposure to wet and cold weather, fatigue, and mental depression.
[p. 168]The almost total neglect of horticulture in Europe during the Middle Ages, especially in its more sterile northern portions, the habitual diet of salted, smoked, and dried flesh and fish, and the prolonged spells of cold and damp weather of this region, were conditions most favorable to the development of scurvy, and these regions were the very first in which its devastating effects were early observed and recorded. In the first half of the fifteenth century it prevailed epidemically in the north of Europe and almost everywhere endemically, more especially in the countries bordering on the Baltic and North Seas, although the largest and richest cities were frequently afflicted in the severest manner in consequence of imperfect food-supplies and the wretched sanitary conditions under which the inhabitants lived (Fabricius). The long voyages and imperfect diet of crews of ships furnished a large quota of harrowing nautical experiences with the scurvy, commencing with Vasco da Gama's voyage to India in 1497, and running up to 1812. In this interval it was all but universal on long voyages, both on single ships and in fleets, in the mercantile marine and in the navy.
In 1798, through the better insight into the causes of the disease, and especially through the exertions of Dr. James Lind in ameliorating the dietary of British sailors, it was practically stamped out of the navy or restricted to isolated occurrences. The influence of the success thus achieved was not lost upon the navies of other nations nor upon the growing fleets engaged in commerce, as the disease has become less and less frequent, constituting at present but a very trifling proportion of the diseases incident to seafaring people. This remarkable result is in part attributable to the fact that the chief maritime nations have enacted beneficent laws intended to compel the owners and masters of merchant vessels to observe certain sanitary and hygienic measures that protect the crews from scurvy. The number of cases returned in the English navy for 1881, in an aggregate of 52,487 cases of all diseases, was 4; in the Prussian navy, 3 in 8659; in the Austrian navy, 27 in 8096; in the U.S. navy, none in 13,387. Thus, in a grand total of all diseases in the chief naval services of the world of 82,629 there were only 34 cases of scurvy—a ratio of .41 per 1000. In the mercantile marine 62 cases occurred in 32,613 cases of all diseases, of which 43 were on the Pacific coast: this gives a ratio of 1.9 per 1000. Altogether, the 115,242 cases produced only 96 of scurvy—a ratio of .83 per 1000. The difference in favor of the naval over the marine service is accounted for by the greater attention paid to the health and comfort of the men in the former.
The U.S. steamer Jeannette spent two winters in the Arctic region, and had a single case of scurvy. The U.S. steamer Rodgers was wrecked, and the crew, during its sojourn of six months among the Siberian tribes, suffered severely.
The operations of armies in recent times have not furnished the frightful mortality which, from neglect of sanitary precautions, formerly afflicted them. During the rebellion of 1861-64, out of 807,000 cases there were but 47,000 of scurvy, or 5.8 per cent., with a death-rate of 16 per cent. The French army1 of 103,770 men during the Crimean struggle had 27,000 cases of scurvy, or 26.0 per cent., with a death-rate of 1.5 per [p. 169]cent. In the Bulgarian campaign of 1877-78, in an army of 300,000 strong, there were, according to Pirigoff,2 87,989 cases of disease, of which 4234, or 4.8 per cent., were frankly-expressed cases of scurvy. This gave a proportion of only 1.4 per cent. of the entire force—a result entirely due to the maintenance, both before and during the war, of a high standard of health.
1 Scrive, Rélation Médico-Chirurgicale de la Campagne de l'Orient, Paris, 1857.
2 Krieg Sanitäts-Wesen, Leipzig, 1882.
ETIOLOGY.—Perhaps no disease has furnished a more fertile field for etiological conjectures than scurvy. The father of medicine ranked the disease in one place among those presenting enlarged spleens, and in another with the twisted bowels. He recognized a putrescence of the humors as the underlying factor—a theory that held sway until the beginning of the nineteenth century. The disease attracted wide attention in the seventeenth and eighteenth centuries from its frequent epidemic and endemic occurrence in various parts of the north of Europe, and was believed to be restricted to cold and particularly wet districts—a view that has been long since abandoned with a better knowledge of its habitats. It has been encountered alike in high latitudes north and south, amidst sterile wastes covered with eternal snows and ice, in the temperate zones and in the burning plains of the equatorial regions of America and Africa.
Sex has no predisposing influence, and the fact that more males than females are affected during an epidemic simply indicates that the former are more exposed to the ordinary determining causes. During the siege of Paris, according to the tables of Lasègue and Legroux, there was a very large excess of male cases, and Hayem's figures show only 6 women in 26 cases.
Scurvy has been observed at all ages from infancy to advanced periods of life; it is believed by certain writers that adolescence is less predisposed than adult age.
The epidemic feature of the disease led many to the opinion that it was contagious—a view that retained its hold for many years. It was also considered to be of a miasmatic character, which, with the previous feature, seemed to assimilate it in nature with typhus fever and other diseases of the miasmatic contagious group. This view had a vigorous advocate in Villemin, who in 1874 read a lengthy paper before the Royal Academy of Medicine in its support. His arguments were specious, inconclusive, and inaccurate, the weight both of facts and authority being decisively against his view. Its occurrence among members of the same family led a few to regard it as hereditary, and it was thought to be transmissible from the mother to the recently-born as well as to nursing infants. The depressing influence of certain emotions, fear, anxiety, and nostalgia, upon the functions of nutrition has, as might have been anticipated, been noted as contributing indirectly to the manifestation of epidemics of scurvy in the presence of the essential determining dietetic causes.
Scurvy cannot be regarded, as Lhéridon-Cremorne3 has argued, as the last term of nostalgia, the other alleged causes being secondary; nor as the immediate result of mental depression, as Gueit4 believed from his experience in the ship Henry IV. during his service on the blockade in the Black [p. 170]Sea in 1858, because the disease first invaded those laboring under nostalgia. The currency of such opinions may be readily explained by the fact that ordinarily depressing mental influences occur under the same conditions as those associated with scurvy—viz. during sieges, after defeat, in prisons, and in workhouses; and, further, the mental phenomena ordinarily occur as prodromes of the disease long before the pathognomonic phenomena present themselves. Out of these facts grew the mistake of regarding the mental change as causative instead of consecutive. Murray went farther and regarded mental despondency as at once cause and effect, and long ago scurvy was compared to hypochondriacal diseases.5 It may be concluded from the recorded epidemics that no degree of mental exhilaration could ward off the disease in presence of the determining causes, nor any degree of mental despondency induce it with proper alimentation.
3 Thèse de Paris.
4 Thèse de Montpellier, 1858.
5 Dolée, 1684.
The various qualities or changes in the atmosphere were regarded individually or collectively at various times as the determining causes. It was supposed that the air might become impregnated with putrid exhalations from various sources, as the holds of ships, or rendered impure by the vapors of the sea. The foul air of crowded habitations, vessels, or cities was appealed to, or the common cause was sought either in its temperature or humidity, or in both. The earlier observers gave prominence to cold as a determining cause of scurvy, and especially when combined with dampness, and hence its frequency in the north of Holland, Brabant, Belgium, Russia, and Germany. This was the current view in the seventeenth century. On the other hand, with equal confidence the disease has been supposed to be determined by excessively high temperatures, and its occurrence in India, South Africa, and the equatorial regions has been alleged in support.
Personal habits have been in the eyes of earlier observers an all-sufficient cause, and thus excessive exertion attended with fatigue and exhaustion has been considered the cause of several severe outbreaks on shore and at sea. In contrast with this opinion we find the English physicians placing great stress upon indolent habits and lack of exercise as a predisposing if not a powerfully determining cause.
The use of tobacco was inveighed against by Maynwaring and Harvey as a powerfully morbific cause, while to the lack of the same narcotic its occurrence was ascribed by Van der Mye. More recently it has been referred by Fabre6 to vaso-motor disturbance due to a miasm.
6 Des Rélations Pathogéniques des Troubles Nerveux, etc., Paris, 1880.
In the drink and food, however, most observers have sought the exciting causes of scurvy. Instances have been reported where the disease seems to have depended upon the use of impure water, etc. The imagination has been tortured to seek in some quality or sort of food the specific origin of scurvy. With regard to quantity, it may be stated that in severe famines scurvy may or may not occur according as the food, though scant, is in due proportions of animal and vegetable, though it is true that the ordinary conditions of a famine preclude the procurement of succulent vegetables. The quality of the food has nothing further to do with the production of scurvy than by impairing the general health, for it has often happened that putrid food has been long used without scorbutic symptoms arising. The kind of food is equally [p. 171]innocent, although various special articles have been charged with specific activity. The frequency of scurvy in Brabant was attributed by Ronseus to the use of aquatic birds; Sherwin and Nitsch assigned the same peculiarity to a free use of fish; and Henry Ellis to the too free use of spirits. Even the generally widespread and much-esteemed article of diet sugar was in disrepute with Willis. The too free use of salted meats has been often accused of causing the trouble. The fat rising on water in which salt provisions were boiled was considered by Cook and Vancouver to be of particular pernicious effect, and even the copper vessels in which they were cooked were condemned by Travis as able to communicate the scorbutic poison to the food. To the milk of animals browsing on verdure upon which pernicious dew had fallen was referred an epidemic which occurred in Silesia in 1591. Diseased potatoes were considered sufficient to determine scurvy in Ireland and Scotland by O'Brien.
The scurvy occurring on land was deemed to be different from that occurring at sea, and its frequency afloat brought into unmerited disrepute the sailor's salt diet, and its saline materials were even considered the chief offending cause. This idea was rejected by numerous observers, who assigned as the chief causative rôle in scurvy deficiency in vegetable food, especially of the fresh, succulent variety. The particular constituent of this sort of food, so powerful in warding off scurvy and of curing it when prevention has failed, has baffled discovery. Dr. Aldridge attributed it to mineral elements generally. Dr. Garrod singled out the potassic salts as the particular one to which the specific action must be attributed; but neither of these views has gained in credit. From all the facts, both positive and negative, we may reasonably assume that the essential dietetic error leading to the development of scurvy, in the immense majority if not in all cases, consists in a deficiency in the variety of food; that is to say, there is not the requisite proportion of animal matter with a diversity of vegetable substances. No single natural order contains plants that supply all the elements essential to the nutrition of the body and the right composition of the blood. The graminaceous and leguminous articles of food, for instance, are numerous, but not various; they all afford the same or analogous albuminous elements, which have about the same nutrient value as the corresponding substances in animal food, and hence health and vigor cannot be sustained on a diet of flesh, combined with wheat, rice, and oatmeal or with beans and peas, or with all of them together. Outbreaks of scurvy have occurred on shipboard, where the ration is made up principally of these articles; as in Anson's ship, when supplied with an abundance of fresh animal, farinaceous, and leguminous foods. It is clear, therefore, that in order to obtain a variety of materials required in nutrition, we must resort to several of the natural groups, those particularly which comprise the succulent vegetables and fruits.
MORBID ANATOMY.—The bodies of persons dead of scurvy are, in most cases, much emaciated, because the quantity as well as the quality of the food has usually been defective. When the food-supply is abundant and only lacking in the elements indispensable in warding off scurvy, the bodily weight is not noticeably decreased, although the characteristic tissue-changes of scurvy are present. This was noticeable in the cases recorded by Trotter of negro slaves dying of scurvy while their bodies [p. 172]presented a fat and sleek appearance. Rigor mortis usually sets in early, and chemical decomposition invades the tissues speedily. The skin presents the discolorations and blotchings observed during life. The subcutaneous connective tissues are soaked with serous exudations, especially in the lower extremities, and in various localities are infiltrated with bloody or fibrinous extravasations. The same changes occasionally affect the muscles, the infiltration occurring beneath the fibrous sheaths and into the intermuscular spaces, and the fibres are more or less torn. These effusions occur most frequently about the knees, the elbows, and the pterygoid muscles of the jaw.
The bones are sometimes necrosed by the mechanical influence of copious effusion beneath the periosteum, forming nodes of varying sizes and obstructing the supply of blood. The joints are occupied by serous or bloody transudations; their synovial investment is destroyed in part, so that the cartilage is exposed; and the latter not infrequently is softened, and even separated from the subjacent osseous connections. Sometimes the morbid changes occurring in the joints are the results of disease in the subcutaneous connective tissues surrounding them.
The muscular system presents marked changes. The muscles undergo fatty degeneration in a remarkable degree. The changes begin first in the lumbar muscles, the fibres losing their striations and sarcolemma, and finally being replaced by granular and fatty matter.
The brain has been found in rare instances the seat of softening and infiltration, and the ventricles may contain serous or bloody fluid. Similar effusions have also been noted in the arachnoid. Most frequently, however, the brain and its membranes present an anæmic appearance, there is less blood than natural in the vessels, and the tissues are pale. Very often no changes whatever are observed.
The heart is smaller than normal, relaxed, and flabby, its fibres easily broken, and a cut surface presents the yellowish aspect of fatty degeneration in certain parts, with occasional extravasations located in the cardiac walls. The valves of the heart are relaxed and illy adapted to accurate closure. In certain recent cases soft coagula or dark fluid blood, and in others firmly coagulated blood, are found in the cavities; in those which have been prolonged the blood is more likely to be found fluid and the coagula diffluent. The endocardium is often blotched to a greater or less extent by sanguineous imbibition. The pericardium often contains serum, and in the worst cases is inflamed, lacerable, and contains bloody effusions. The inner surface of the great vessels at the base of the heart is stained by imbibition.
The respiratory organs are variously affected. The mucous membrane lining the nose, larynx, and trachea is generally pale and flecked with extravasations of a dark-red color; more or less frothy fluid, tinged with blood, is present in these passages, and occasionally oedema of the glottis is encountered. The lungs are, as a rule, infiltrated with a bloody serosity, particularly in those cases with renal complication, or with a fibrinous or bloody exudation. The posterior portions of the lobes often present evidences of hypostatic congestion, or even of gangrene, and in the latter case the tissue is easily friable and emits a disagreeable odor. Their surfaces are mottled with superficial discolored patches of varying size and outline. The lungs may, on the other hand, be found pale, [p. 173]with empty collapsed vessels and with little or no effusion. The pleural cavities commonly contain a serous fluid, or, in rare cases, a copious effusion of blood. Traces of inflammation and discoloration by sanguineous staining are traceable on the pleural surfaces.
The digestive organs furnish strongly marked lesions. The mouth presents the most constant scorbutic feature, a stomatitis in which the gums are infiltrated, spongy, livid, and the seat of fatty degeneration; the teeth are loosened or have already fallen out. The stomach and small intestines are thin-walled, and the mucous membrane is often softened, and in places ulcerated; similar changes have been noted in the solitary glands. Follicular ulceration of the large intestine occurs, with softening and infiltration of the mucous membrane. Hemorrhagic effusions into the mucous membrane, forming stippling, flecks, or patches, occur in various degrees along the whole extent of the alimentary canal. The pancreas is occasionally found softened and containing hemorrhagic effusions.
The kidneys are, as a rule, found in the normal condition in cases in which albumen has been observed in the urine. Occasionally they are engorged, with infarction of the cortical substance, and the mucous lining softened and thickened and covered with blood-tinged mucus, or they may present various degrees of parenchymatous degeneration. The ureters and bladder sometimes present ecchymotic spots, and the contained urine is mingled with blood.
The liver is always more or less altered by fatty degeneration, and at times replete with blood and softened, and its surface ecchymotic. The spleen is occasionally greatly enlarged, and its tissues very lacerable, laden with blood, and infarcted.
PATHOLOGY.—The essential character of scurvy consists in perverted nutrition, in which the blood undergoes such peculiar and profound changes that its fitness for the maintenance and renewal of the various tissues and organs is impaired; hence the nervous depression, loss of muscular power and tonicity of tissues, and the transudation of the blood or of its constituent parts.
The processes of secondary assimilation are chiefly at fault, leading to the blood-changes, and through these to the textural lesions. Primary assimilation remains intact, as the bodily weight is little altered as long as the food is in sufficient quantity. This loss of nutritive balance between the blood and tissue is due to the absence of certain elements furnished by fresh vegetable matter. What these are, and how their absence acts in inducing this disturbance, have not yet been determined; we only know that the mysterious harmony of the vital, chemical, and physical relations which exist between the blood and tissues in health is deranged by their absence.
Endless explanatory surmises and assumptions have been proffered. The earlier explanations involve either the Galenical theory of putrefaction of the fluids and humors, a breaking down of the blood-corpuscles, or the later chemical theories of superabundance or absence of certain salts, sulphur, etc., and hence there were an acid scurvy, an alkaline scurvy, a muriatic scurvy, etc.
The frequent effusions of blood in scurvy led Andral to suspect that the chief peculiarity in scorbutic blood was the decrease of fibrin; which was [p. 174]in perfect accord with a theory that he had formed that this change was the uniform cause of passive hemorrhage. Magendie had already given experimental support to this conjecture by inducing in animals phenomena analogous to those of scurvy by the injection into the veins of defibrinated blood or of alkaline solutions. Andral7 believed his views confirmed when in 1841 he analyzed on two occasions the blood of scorbutic patients and found the fibrin reduced to 1.6 parts per 1000. Similar results were obtained by Eckstein and Frémy. On the other hand, the blood was analyzed by Busk, about the same time, in three well-marked cases of scurvy that occurred on the Dreadnaught hospital-ship, and in all of them the fibrin was in excess of the normal amount, the least being 4.5 and the greatest 6.5 parts per 1000. In perfect accord with Busk's results were the analyses of the blood of five scorbutic females, communicated in a note to the Academy of Sciences in 1847 by Becquerel and Rodier. In no case was the fibrin diminished, but in some it was sensibly increased. In a subsequent case Andral found that the fibrin, instead of being less, exceeded the physiological mean, reaching 4.4 parts, and he concluded that a diminution of this element was not a necessary and common occurrence, but only an effect—a result of prior morbid modifications, and a consequence which was produced more or less frequently according to the severity and duration of the disease. Parmentier and Déyeux found the blood of three scorbutics to resemble inflammatory blood in respect to fibrin, while Frick obtained in one analysis 7.6 parts of fibrin and Leven 4.3 parts.
7 Essai d'hématologie pathologique.
In mild cases of scurvy neither the color, the alkalinity, nor the coagulability of the blood differs from that of blood in health, though Wood alleges that the clot is loose and cotton-like, and Canstatt that its coagulability, in consequence of the large proportion of saline matters, is diminished. In Busk's cases the separation of the clot and serum was as perfect, and took place as rapidly, as in healthy blood, and in two of them the blood was both buffed and cupped, as it was also in Leven's cases. In two of the most severe of Becquerel's cases the blood coagulated firmly, and in a slight case the clot was dark and loose. The albumen of the blood shows no marked change as regards its quantity. The five analyses of Becquerel and Rodier showed the average amount of organic matters of the serum to be 64.3 parts in 1000, the smallest being 56.2 and the largest 69.2 parts. 1000 parts of the serum of the same cases gave an average of 72.1 parts of organic matter. Frick's single case gave 87.045 parts per 1000, and the average of Busk's was 78.2 parts, while Chotin and Bouvier obtained only 62.3 parts. The last-mentioned writers have recorded a fact in connection with the physical characters of scorbutic blood that deserves notice: the blood in one case did not coagulate at the usual temperature (about 158° F.), but required a temperature some degrees higher for that purpose. The red corpuscles in all the foregoing cases were notably diminished, the largest amount given being 117.078 parts per 1000, while the lowest was 47.8 parts. In Andral's second case the globules had decreased to 44.4 parts per 1000, the lowest amount yet recorded.
The alkalinity of the blood seems not to be changed, although Chotin and Bouvier noticed a slight increase. The saline constituents do not [p. 175]vary greatly from the normal standard. The average amount in the cases of Becquerel and Rodier and Busk was 8.1 per 1000, the smallest being 5.5 parts and the largest 11.5. In Ritchie's two analyses the proportion of saline matters is given as 6.44 and 6.82 parts per 1000. Opitz and Schneider have found less than the physiological mean. In Frick's case the amount was 8.8, the iron being 0.721 parts per 1000, and 0.782 to 127 parts of globules; lime 0.110, chlorides 6.846, and phosphates 1.116 parts per 1000. The iron was in excess of that in the normal blood, but in Becquerel's cases the mean was 0.381—less than the normal. The proportion of iron in Duchet's cases was respectively 0.393, 0.402, and 0.476 parts, giving a mean of 0.423 parts per 1000, which nearly approximates the normal. Garrod in one analysis of the blood found a deficiency of the potassium salts, upon which he erected his well-known theory of the etiology of the disease. It is an interesting fact that in the physiological state the quantity of sodium chloride is not subject to variation, any excess introduced with the food being thrown off by the kidneys. The quantity in the urine bears a relation to the amount introduced as food, but the proportion in the blood is constant.
The quantity of water in the blood has been found to be increased in all the analyses which have been made. Chotin and Bouvier estimated water and loss at 831.1; in Frick's case it was 791.69 parts per 1000; and in Becquerel's five cases it was put at 807.7, 810.9, 811, 813.7, and 854.0 parts per 1000, respectively. In Busk's three cases the lowest amount was 835.9 and the highest 849.9 parts per 1000. The specific gravity of the defibrinated blood was in all cases low in comparison with the normal standard (1057), the average in Becquerel and Rodier's cases being 1047.2, the lowest 1083.3, and the highest 1051.7. In the single observation of Chotin and Bouvier it was 1060. The specific gravity of the serum was also less than normal (1027), the average of four of Becquerel's analyses giving 1023.8, the lowest 1020.8, and the highest 1025.5. Busk gives 1025 in one case and 1028 in another.
The results of the most recent analyses, those of Chalvet, are shown in the following table, in which scorbutic blood is contrasted with that of a healthy, robust female:
Scorbutic blood. | Healthy blood. | |
Water | 848.492 | 772.225 |
Solid matters | 151.508 | 220.775 |
Dry clot | 140.194 | 209.000 |
Albumen | 72.304 | 68.717 |
Fibrin | 4.342 | 2.162 |
Globules | 63.548 | 138.121 |
Extractive matter—by absolute alcohol | 10.312 | 8.013 |
Extractive matter—by ether | 1.002 | 1.300 |
Ashes of clot | 3.000 | 5.691 |
Peroxide of iron of globules | 1.060 | 2.259 |
Potassium of globules | 0.329 | 0.625 |
From the conflicting statements of various observers the following conclusions may be drawn: that in scorbutic blood water is in excess; that there is, on the one hand, a marked increase of the fibrin, and in a less degree of the albumen and extractive matters, while on the other hand there is a marked decrease of the globules and in a less degree of the mineral matters. On the authority of Chalvet it may be also stated [p. 176]that demineralization of the muscular tissue is a notable chemical feature in scurvy.
So far, microscopic examination has been entirely negative. Hayem8 found no appreciable alteration from healthy blood, and in this view Leven9 concurs; while Laboulbène10 notes the occurrence of an unusual number of white globules.
8 Mém. de la Société de Biologie.
9 Communication to the Académie des Sciences, 1871.
10 Epidemie de Scorbut.
Petrone Luigi11 injected scorbutic blood into the connective tissue of rabbits. In three instances the animals died, presenting on the ears distinct evidences of the formation of petechial extravasations. The viscera revealed everywhere bloody effusions of larger or smaller size. The spleen was enlarged and its parenchyma and capsule distended. In the blood were found oval, shining, spontaneously-moving corpuscles, which he regarded as the bearers of the specific poison of scurvy.
11 Annali Univers. di Med. c. Chir., 10, 1880.
SYMPTOMS.—The symptoms of scurvy are insidiously and usually slowly developed under the influence of the efficient causes, and the disease runs a chronic course, often extending over five or six months, especially in cases in which the hygienic surroundings of the patient have been imperfectly or not at all rectified. In light cases the course is much shorter. A gradual alteration of the nutritive processes first occurs, until what might be called a scorbutic cachexia is established in a period varying from a few weeks to several months. The initial symptoms consist in the skin losing its color and tone and assuming a yellowish or earthy hue: it is relaxed, dry, unperspiring, and rough; in the legs particularly this roughness is very marked, and the skin, when rubbed, sheds an abundance of furfuraceous scales. The cutaneous follicles, markedly on the extensor aspect of the lower extremities, are prominent, similar in appearance and feel to the condition known as goose-flesh. Rouppe12 calls this the signum primum pathognomonicum. Dark-red or brownish flecks, of a circular outline and of varying but small size, not unlike flea-bites, appear on the face and limbs. The cutaneous circulation is feeble and the superficial warmth less than natural; slight depression of the atmospheric temperature produces a sensation of chilliness, and the feet and hands are cold. On assuming the erect posture the patient complains of headache and dizziness. The muscles are relaxed and soft to the feel, and a corresponding loss of vigor and strength is experienced by the patient, who is indisposed to exert himself in the performance of his customary duties and seeks repose and freedom from feelings of fatigue and languor in recumbency. This prostration is occasionally so extreme that the slightest efforts in attempting to stand or walk are attended with rapid action of the heart, accelerated respiratory movements, and a sense of suffocation and breathlessness. The general circulation is impaired; the heart acts feebly; the arteries are contracted; and the pulse is slow, small, and compressible.
12 De morbis navigantium.
The mental powers are equally impaired. The face wears a haggard appearance and depressed expression; gloomy forebodings of evil and disinclination to turn the attention to the usual mental pursuits are markedly present—a disinclination that may subsequently merge into complete apathy or indifference to passing events, or even into somnolency.
[p. 177]Pains in the legs, joints, and loins are early manifestations: they closely resemble those of rheumatism, for which they are often mistaken. The pains are not exacerbated at night, but, on the contrary, are often more severe by day. Not unfrequently lancinating pains in the muscles of the chest are complained of. The sleep is not disturbed until the disease has made some advance, when it becomes broken and is no longer refreshing.
The appetite is usually unimpaired in the early periods of the disease, and even throughout its course the condition of the mouth alone prevents the patient from indulging his desire for food, even, as is occasionally noticed, to voracity. There may be a yearning for certain articles of diet, principally those of an acid character; but, on the other hand, some cases present exactly the reverse condition—a disgust for food in general or for particular varieties; or the appetite may be vacillating, at one time craving and at another repelling nourishment. There is no noticeable change in the normal thirst, except on the occurrence of febrile complications, when it is increased. The gums do not, at this stage of the disease, present the livid, swollen appearance of fully-developed scurvy, but, on the contrary, are generally paler than usual, with a slight tumid or everted line on their free margins, and are slightly tender on pressure. The breath is commonly offensive, and the patient complains of a bad taste in the mouth. The tongue is flabby and large, though clean and pale, and the bowels are inclined to be sluggish.
This preliminary stage is followed, after varying intervals of time, by certain local phenomena which are quite characteristic of the disease. There is a marked tendency to extravasation of blood into the tissues, either causelessly or upon the infliction of slight injuries or wounds. Fibrinous exudations occur sooner or later into the gums, which become darkened in color, inflamed, swollen, spongy, and bleed upon the slightest touch or even spontaneously, and finally separate from the teeth. These results are due, in part, to the considerable amount of pressure to which these parts are subject in mastication, and it is a conspicuous fact that the gums of edentulous jaws remain free from these changes. In a few cases the gums are but slightly altered, perhaps oedematous only or pitting upon pressure, or they become the site of bloody extravasations. In severer examples, in later stages of the disease, these various alterations progress to an extreme degree, and the extravasation is so voluminous that the gums present great, fungous, lacerable excrescences, which may finally break down into a suppurating, brownish, and very fetid mass, communicating to the breath an odor of a most offensive character. In certain epidemics of scurvy, notably in that of Florence described by Cipriani, the lesions of the gums were absent. The rest of the mucous membrane of the mouth remains unaltered, or at most slightly ecchymotic. Samson and Charpentier13 in a large number of cases saw this but once, and in one of Leven's14 cases the fungous growth invaded the palatal mucous membrane, extending to the anterior pillars of the fauces. The salivary glands are enlarged and swollen; the tongue is imprinted with the form of the teeth, while the latter become encrusted with tartar and more or less concealed by the exuberant gums, or, becoming gradually loosened from the alveoli, finally drop out. The morbid process may extend to the bone itself, and necrosis and extensive [p. 178]exfoliation follow. Mastication is more or less painful, and often impossible, so that the patient is reduced to the necessity of prolonging life by the use of fluid or semi-solid food. Under the influence of appropriate treatment it is remarkable how rapidly (in from two to four weeks) these marked changes recede and the parts resume their normal condition, yet it occasionally occurs that permanent, callous thickening of the gums results.
13 Étude sur le Scorbut, 1871.
14 Une épidémie de Scorbut, p. 28, 1872.
In the progress of the disease effusions of blood under the skin are of early occurrence. They are at first located in the superficial stratum of the cutis or just beneath the epidermis, especially around the roots of the hair, and present themselves as roundish, bluish-red flecks, varying in size from that of a pin's head to that of a split pea, not effaceable by pressure with the tip of the finger, but slightly, if at all, elevated above the surface, and enduring for weeks together. The nutrition of the hair-follicles is impaired, so that the hairs are often either lost, broken, or distorted. These petechiæ fade in color with progressive improvement in the case, and finally disappear, leaving brownish-yellow discolorations. They first appear on the extremities, particularly the lower limbs, then on the face, and lastly on the trunk. At a later period extravasations of a larger size and more irregular form occur in the deeper layers of the derma. They vary in size from that of a finger-nail to blotches two or three inches in diameter; at first reddish in color and subsequently of a bluish red. When recession occurs under appropriate treatment, the color passes through various shades of violet, blue, green, and yellow, as in ordinary traumatic ecchymosis. Outpourings of blood also occur into the subcutaneous connective tissue, notably that of the legs, and in localities where connective tissue is particularly abundant and loose, as in the ham and axilla. The dispersion of blood in this tissue may be so considerable as to cause the legs from the knees down to present a uniform dark-blue coloration that in form may not inaptly be compared to a stocking. The upper extremities also suffer, usually on their inner side from the armpit down, the extravasation rarely reaching, however, to the hand. These extravasations may take place after the infliction of very slight injuries, as from blows or the pressure of hard bodies, or even from the mechanical effects of prolonged dependency of the limbs, as in riding on horseback. Extravasations of a similar nature are occasionally present in the connective tissues of the muscles themselves or between them, giving rise to swellings of various forms and dimensions. Nearly always along with the sanguineous effusions there is more or less oedema, usually beginning at the ankles and gradually extending upward; in some cases there are puffiness of the face and general anasarca, so that deep pits remain on pressure.
This profound impairment of nutrition of the skin continuing, in the worst cases blood is effused beneath the cuticle, forming blebs of varying size, which finally break and leave superficial ulcerated surfaces, that ultimately become covered with flabby, exuberant granulations, pouring out a purulent often offensive sanies and bleeding upon the slightest touch.
In some cases the ulceration begins in the petechiæ at the hair-roots, and a number of these, running together, form a large ulcer. The destruction of tissue by ulceration is disposed to spread more widely and deeply, and is often of a most intractable character. Old cicatrices [p. 179]are the first tissues in these cases to take on the ulcerative action. Certain muscles, chiefly those of the legs, and notably the gastrocnemii, the abdominal and pectoral muscles, the psoas magnus, and pterygoids, may become the seat of fibrinous extravasations, which finally change, by lapse of time, into hard, firm tumors, impairing the functions of those parts and leading to contractions of the limbs.
The symptoms in certain epidemics of extraordinary severity have displayed alterations in still deeper structures. Effusions occur between the periosteum and the bone, forming painful, hard, and resisting nodes of varying dimensions, especially along the course of the tibiæ, upon the scapulæ, and upon the maxillæ. In young persons the epiphyses are separated from the shaft of the long bones, and in other cases the ribs become necrosed and disarticulated from the sternum, producing a creaking noise during respiratory movements, as related by Poupart.15 This occurs mostly on one side and about the middle of the series, yet it has been noted to occur on both sides, so that the sternum and attached cartilages, deprived of support, were perceptibly sunken. Oserctzkowski16 reports two fatal cases of scurvy attended with spontaneous fracture of the ribs. There was extravasation into and beneath the periosteum, and subsequent destruction of the continuity of the bone. In one case the ribs on both sides were affected, so that the anterior wall of the thorax sunk in and embarrassed the respiration, which was chiefly maintained by the diaphragm. Inflammation of the lungs succeeded, and the patient died in agony.
15 Mémoires de l'Académie des Sciences, p. 237, 1699, and Philosophical Transactions, vol. xv.
16 Wratsel, No. 51, 1881.
Recently-repaired fractures have been known to recur under the influence of scurvy from the destruction of the callus.17
17 Anson's Voyage Around the World, edited by Walter.
The articulations as well as the bones in very severe cases of scurvy present evidences of disease, consisting in periostitic effusions which involve the surrounding soft parts, producing impairment of motion, enlargement, and false ankylosis, and even destroying the normal anatomical relation of the osseous surfaces, so as to determine deformities. These changes are usually attended with severe pain, and most commonly occur in the ankle-, knee-, shoulder-, and hip-joints, and disappear tardily, requiring perhaps months for their recession, if indeed this takes place at all.
The symptoms manifested by the circulatory organs are prominent from an early period of the disease. The pulsations of the heart are slower, feebler, irregular, and often intermittent; its impulse is decreased or becomes quite imperceptible; and when the associated anæmia has progressed to a certain extent a systolic murmur may be audible. The arterial and venous channels are of diminished calibre; the pulse becomes soft, of less volume, and tardier; and a venous murmur may sometimes be heard in the cervical veins. The remarkable nutritive changes in the capillary walls in part account for the numerous hemorrhages which occur both by rhexis and diapedesis. The most frequent is epistaxis; the slightest blows, sneezing, or blowing the nose will often determine it, or it may occur spontaneously, and in severer cases with such profuseness as to threaten impending dissolution, requiring nothing less than timeous introduction of the tampon to rescue the victim. Hemorrhage from the [p. 180]lungs is of rare occurrence, and when it does happen is rather indicative of pre-existing pulmonary disease, such as phthisis, or of the approach of a complication, such as infarction or gangrene, than a constituent feature of scurvy. Hæmatemesis is less uncommon, but is by no means frequent; the blood ejected from the stomach is usually small in quantity, but in isolated examples the bleeding is profuse, producing great exhaustion and a sense of cardiac depression which preludes speedy death. Hemorrhage from the bowels is also an ill-omened feature, completely blanching the patient and presaging early exhaustion and death. Blood may also appear as a product of a complicating dysentery which determines abundant, offensive discharges that may run on for several weeks before the patient is finally exhausted. Hæmaturia sometimes occurs, especially in broken-down and cachectic subjects and in an advanced stage of scurvy. All of these forms of hemorrhagic effusion, now mentioned as localized in the mucous membranes, are to be deprecated as exercising a pernicious influence, seriously aggravating ordinary cases and fatally jeopardizing the issue of severe ones.
Effusive and inflammatory complications are also encountered in the serous structures, and usually in cases of great severity, though they occasionally present themselves when the more common localized phenomena of scurvy are not particularly prominent. These complications may be marked by a gradual accession, or they may rapidly arise and involve the patient, just before in apparent security, in the greatest peril. These incursions are almost always attended by febrile exacerbations and the usual grouping of clinical characters denotive of the same pathological conditions arising under ordinary circumstances. The local complications may either affect the pleura or pericardium, or both. In Karairajew's18 60 autopsic examinations pericardial effusions were noticed in 30, pleural in 30, pericardial and pleural in 6, peritoneal in 7, and arachnoidal in only 1. The exudations are sero-sanguinolent or fibrinous in character, and sometimes reach the inordinate quantity of four or five pounds, occasioning the patient the utmost distress and embarrassing the respiratory and circulatory functions. Although these augment in a high degree the risk to life, yet under prompt and appropriate treatment recovery may take place and the effusions vanish with surprising rapidity.
18 Himmelstiern, Beobachtungen über den Scorbut, S. 50, Berlin, 1843.
Hemorrhagic extravasation into the nervous centres is a very rare occurrence. It has not been as yet recorded as having occurred in the brain-substance itself, but has in several instances been noted between the meninges, producing headache, dizziness, vertigo, and sometimes somnolence, delirium, and coma. Opitz19 relates an interesting case in which convulsions suddenly occurred with unconsciousness, followed by hemiplegia of the left side of the body and the corresponding side of the face. After twenty-four hours consciousness returned and the paralysis disappeared. There were, however, headache and hyperæsthesia of the upper extremities present; twelve days later these also receded, and the patient finally recovered. The same author records paralysis as occurring in one case from extravasation into the spinal meninges. Samson observed an instance in which a fibrinous effusion formed upon the sciatic nerve, with consequent pain.
19 Prag. Vierteljahrschrift, S. 153, 1861.
[p. 181]In the circulatory system symptoms always of threatening and often of fatal import may arise: embolism may occur at various points, particularly in the lungs and spleen, occasioning hemorrhagic infarctions, which have undoubtedly been the occasion of the sudden deaths sometimes observed in scorbutic cases not apparently of a very dangerous form nor attended with an excessive degree of exhaustion.
The urinary system supplies no prominent symptoms; the statements as to the condition of the kidneys and the composition of the urine are contradictory. The urine not infrequently contains albumen, particularly in severe cases, but this is by no means indicative of corresponding changes in the renal structure: on the contrary, this may be found after death to be apparently free from disease.
The conclusions that would seem to be authorized by the statements of various authorities are that the quantity of urine passed is decreased, as well as that of the urea, while the amounts of the albuminoid and mineral matters are increased.
Physical examination will reveal the frequent occurrence of enlargement of the spleen, independent of malarial influences, and Krebel has encountered one case in which the liver was involved in inflammation.
Some derangement of the visual organs is present in many cases. Foltz, in the epidemic on the Raritan, reported four cases of nyctalopia and two of hemeralopia, and other affections of the eye, such as conjunctivitis, induration and irritation of the ciliary margins of the lids, with a copious and acrimonious discharge, these conditions being obviously due to the scorbutic diathesis. Medical Director J. Y. Taylor, U.S. Navy, in a private communication to me states that hemeralopia was a frequent premonitory symptom of scurvy that occurred in the U.S. sloop-of-war Decatur in 1854 during a laborious and tedious passage of three months through the Straits of Magellan. The men were overworked and much exposed to cold and wet, and part of the time were on diminished rations. The hemeralopia was at first erroneously attributed to the reflection from the snow and glaciers—a species of snow-blindness—but other phenomena speedily appeared in a majority of the causes: a subacute inflammation, with considerable pain and swelling of the small joints, especially those of the toes; sore and tender gums, although only a few progressed so far as to exhibit sponginess or bleeding; and debility, depression, anxiety, and insomnia. In a few cases the blindness was so complete as to render their subjects almost helpless after sunset. This was the most pronounced and remarkable symptom and the one most complained of. These incipient scorbutic symptoms were promptly arrested by the free use of wild celery (Apium graveolens), which was found growing abundantly in sheltered places. The short rations were also supplemented advantageously by mussels (Mytilus edulis) whenever they could be obtained. A few weeks later the crew appeared to be in ordinary health.
Hemorrhage may occur under the conjunctiva, raising it into small pouches; into the anterior chamber, causing iritis and adhesions; and, finally, into the choroid and vitreous humor, exciting a general inflammation of the entire organ.
Dulness of hearing and buzzing in the ears have also been signalized as occasional symptoms of scurvy.
[p. 182]The phenomena of fever are always absent during the course of uncomplicated scurvy, the temperature of the mouth sometimes falling as low as 92° F., and being always one or two degrees lower than normal. It is only in the later periods of the disease, when pathological processes most often supervene in the internal organs, that an elevated temperature and the other ordinary symptoms of fever are manifested. The lowered vital resistance of scorbutic subjects particularly disposes them to the incursions of fevers, especially those of malarial and typhoid types: hence in the low, marshy districts of Northern Europe and in sections of country afflicted by famine and overcrowded dwellings these complications are very common.
DIAGNOSIS.—Little or no difficulty will be encountered in discriminating scurvy from other diseases under the circumstances that usually surround its development and prevalence. These circumstances are altogether peculiar and characteristic, and involve the absence of succulent vegetable food as the prime factor, and exposure to cold, fatigue, mental despondency, or other depressing influences as accessory in its production. This combination of causes has been usually witnessed in all the outbreaks of scurvy in camps, besieged towns, on shipboard, particularly on ships in Arctic service.
Sporadic cases may escape immediate identification in the absence of some of these circumstances, but a close attention to the symptoms will surely lead to a correct conclusion. The scorbutic cachexia denoted by the sallow or earthy hue of the skin; the spongy gums; the discoloration of the surface; pains in the limbs and joints; the sense of weariness, and, later, the exhaustion, dyspnoea on the slightest exertion; the bloody and fibrinous effusions into the connective tissues and muscles about the joints, and into the pleuræ, pericardium, and peritoneum; the stiffness and contraction of the legs,—furnish a complexus of phenomena not met with in any other disease than scurvy. The discoloration of the skin in purpura, leucocythæmia, anæmia, chlorosis, and hæmatophilia, or other conditions involving hemorrhagic extravasation, are easily discriminated from those of scurvy when taken in connection with the other symptoms and the history of those diseases. In the beginning of scurvy the pains in the back and limbs might divert the attention to rheumatism, but an examination at this early stage will, in all likelihood, disclose the peculiar gingival and cutaneous lesions of scurvy.
The rapid improvement of scorbutic cases under a fruit and vegetable diet is also a noticeable feature not witnessed in any of the foregoing diseases.
PROGNOSIS.—The prognosis of scurvy is always favorable in the early stages, and even in the very worst recovery occurs under improved hygienic surroundings with remarkable promptness and certainty. It must not be overlooked, however, that sudden death may occur in seemingly light cases from failure of the heart's action or from embolism. There is a ready disposition to the recurrence of the disease under slight causes, and it may so impair the health as to lead to the development of other fatal maladies. The gravity of the case is to be gauged not so much by its seeming severity as by the accessibility of proper food-supplies, for without these the worst results may be expected. Where the case is embarrassed with complications of the respiratory and circulatory [p. 183]organs, involvement of the bones, and intercurrent diseases, the outlook becomes correspondingly grave.
Throughout the world, in recent times, greater areas of territory are devoted to agriculture and horticulture, and the products are distributed over wide extents of country by the increased facilities of communication by the highways and railroads, so that it would now be impossible for an epidemic of scurvy to devastate a region of country so provided as it did a century ago, or might do and has done in regions of country where tillage is neglected and communications are cut off by an absence of roads from more productive centres, as in Southern and Eastern Russia.
Hygienic improvements that have almost stamped out scurvy on shore have also done good service for mariners, and thousands of ships now cross the ocean on long cruises with perfect security from the disease. In the naval services of the world, as has been already shown, the disease is rarely encountered, and it is greatly diminished in the merchant marine, from which, it is hoped, in a few years, by a more rigid enforcement of existing laws for the protection of sailors, it may also entirely disappear. Even in exceptionally long and arduous cruises, as in the Arctic regions, the disease may be arrested, as was the case with the Jeannette, which was drifted about, locked up in ice, for sixteen months, yet only a single case of scurvy appeared.
It is of the first importance to enlist a healthy crew for long voyages, free from previous syphilitic, scorbutic, or other constitutional taint; then, by observing proper hygienic precautions, to maintain their health. One of the prime factors in securing this result is a suitable dietary. The improved methods of preserving food afford facilities for storing up adequate quantities of both kinds, animal and vegetable, to last the cruise. To economize these stores it will be well to start with a stock of live animals and recent vegetables, such as can be now had in almost any quantity in any considerable maritime city, and not until these are consumed are the canned and preserved supplies to be opened. All the ordinary meats, as beef, mutton, veal, and lamb; most vegetable products, as asparagus, beans, peas, potatoes, and a great variety of fruits, as peaches, plums, berries, etc., are obtainable at moderate expense, and should form an integral portion of the ration. Eggs can be easily preserved so as to keep for months by simply packing them in plaster or in salt, and they furnish a valuable and acceptable article of diet. Among articles of great nutritive value milk takes high rank, and it can be preserved sweet and pure indefinitely. Sauer-kraut is an antiscorbutic of considerable virtue, and should not be overlooked in laying in stores for a distant cruise. Cheese and oatmeal will be found useful additions to the ordinary ration.
It may be proper to state in the event of the occurrence of scurvy and the exhaustion of the fresh vegetable stores that various quickly-growing vegetables, such as mustard, radishes, turnips, and cresses, could be cultivated on shipboard if seeds are provided.
With such a varied dietary, comprehended in the above enumeration, it would be impossible for scurvy to invade the ship's company, especially when aided by other wholesome agencies, as cleanliness, well-ventilated and dry sleeping rooms, and clothing adapted to the weather. The antiscorbutic virtues of lime-juice were known long ago, being mentioned by [p. 184]Albertus in 1593, but it was not until many years later that it became an integral part of the English navy-ration. The law requires it to be carried on board all merchant vessels, and to be served out ten days after the crew has been living on salt rations. The juice keeps well if properly prepared and preserved from contact with air, especially when fortified with a small quantity of alcohol, the usual strength being about 10 per cent. It should be carried in vessels containing just enough to furnish a few days' rations to the whole crew, by which plan only a small amount need be exposed to the decomposing influence of the air. The juice can be reduced by evaporation to a very small bulk. This method was adopted in supplying the Arctic cruiser Rodgers.20 The juice was reduced to a paste, each pound of which represented one gallon of the solution of the ordinary strength. It has also been used in the form of lozenges and biscuit. It may be stated that great reliance has been placed upon malt, the acid wines, and cider as good antiscorbutics.
20 Report of the Surgeon-General of the Navy for 1880.
In connection with the food-supplies it is proper to mention those influences of a depressing character which have a tendency to favor the development of scurvy. The first is dampness in the sleeping apartments of the men. This should be prevented by ventilation, drying stoves, and taking care that no wet garments are permitted to remain in the apartments. They should be taken off immediately and hung outside to dry, and under no circumstances should the men be permitted to sleep in them, as is sometimes done.
Exposure to cold is unavoidable under certain conditions, and the men should then be protected by proper clothing adapted to the weather. Protracted fatigue is a third favoring circumstance, and the crew should be spared all the strain of hard work possible, especially in high latitudes. The apartments should also be kept well ventilated and scrupulously clean; and, lastly, depressing mental emotions, which are so apt to arise from exposure to danger and want, should be dispelled by cheering assurances, constant occupation, and whatever amusements can be had. These are the chief influences which are to be considered in adopting measures to prevent the occurrence of scurvy in communities, armies, on shipboard, or in persons confined in houses of detention.
The therapeusis of scurvy presents no intricate problems for solution. Its origin in dietetic errors is admitted by almost common consent, and it is surprising with what rapidity patients apparently beyond hope of recovery gather health and strength with a change in the character of the food. This is indispensable in the treatment, as drugs have little or no curative influence without it; and, therefore, the first object should be to supply the patient with lemon-juice or acescent fruits and fresh vegetables, as garlic, mustard, cresses, sorrel, nasturtium, taraxacum among the wild plants, and potatoes, onions, turnips, beets, radishes, etc. among the domesticated plants. And in conjunction with these fresh meats, in the form of soups if the solids cannot be masticated, may be used with advantage. Ordinarily, the dietetic treatment alone will suffice to re-establish the health. Should, however, convalescence be delayed, the vegetable bitters with the mineral acids and ferruginous tonics and quinia will furnish useful adjuvants. These are the standard remedies; others have been recommended at various times, as the juice [p. 185]of the maguey, a Mexican plant, potassium nitrate alone or combined with vinegar, tincture of cantharides, etc.
Attention will often be required to the various scorbutic complications, especially stomatitis, which is always a source of discomfort and suffering. One of the best local applications for this is pencilling the parts with a solution of nitrate of silver, which often affords marked relief. Mouth-washes, composed of solutions of chlorinated lime, potassium permanganate, carbolic acid, are beneficial by suppressing foul odors, exercising local stimulative action upon the gums, and promoting healing. Should ulceration attack the legs, as is often the case, the application of mild astringents and stimulative ointments will be all that is required. The parts should, of course, be kept clean and protected from irritation by protective dressings.
Hemorrhages from the nose, gums, stomach, bowels, or into the serous cavities should be treated upon the general principles applicable to their character, as the local use of cold, astringents, and the internal administration of hæmostatic agents—lead acetate, ergot, tincture of iron, and other remedies, vegetable and mineral, of this class. In desperate cases effusions into the chest, threatening death by interfering with the respiratory and circulatory organs, may render operation necessary as the last resort for their removal.
During the treatment it is important to obviate any sudden or severe strain upon the heart by premature movements or exercises, as this is fraught with danger.
It has been customary with authors to describe under the general heading Purpura a number of affections presenting as a common symptom the extravasation of blood into the tissues, more especially of the skin and mucous membranes, quite irrespective of etiological or pathological considerations. Thus, the tiny ecchymoses caused by the bites of fleas have been denominated purpura pulicosa; the larger bruises resulting from external violence, purpura traumatica; the extravasations occurring in the course of scurvy, purpura scorbutica; those encountered in malignant small-pox, purpura variolosa; and so on. These affections, differing widely in nature, possess as a common symptom the escape of blood from the vessels into the tissues. It is evident, therefore, that in the sense often employed the term purpura is used to describe a symptom or symptoms common to a variety of non-related maladies.
If there be a peculiar morbid process having for its constant and characteristic symptom the spontaneous escape of the blood from the blood-vessels, it is plain that interstitial hemorrhage from external violence or from the action of a definite poison circulating in the blood and disorganizing it and its containing vessels, as in phosphorus-poisoning, or from the influence of certain zymotic diseases, should not be designated by the title properly belonging to a substantive malady. The question, therefore, is: Are there groups of symptoms indicating morbid action of definite character, but of varying intensity, to which the name purpura may with propriety be applied?
In the present light of pathological science it is impossible to answer this question in the affirmative without considerable qualification. It must be confessed that we do not possess a knowledge of any definite chain of morbid processes constituting a distinct disease that may be designated as purpura. And yet we are able to recognize a set of symptoms varying greatly in intensity, from the most trivial petechial eruption to profuse and fatal hemorrhages, accompanied by a train of manifestations which we are unable to connect with any of the causes already spoken of, and which, indeed, depend upon no fixed exciting cause with which we are acquainted. It may be eventually proven that purpura, even as we understand it, is merely a set of phenomena due to widely-differing influences acting upon the blood and blood-vessels, and that the term will disappear from our nomenclature as indicating a disease, but will be preserved as denoting a symptom. For the present, [p. 187]purpura is understood to be a group of symptoms characterized by the effusion of blood into the tissues of the body, or upon its free surfaces, or into its serous cavities, which seem to arise spontaneously, and for which we are unable to assign a definite cause. With this view of the nature of purpura it becomes necessary to exclude from present consideration blood-extravasations from internal or external violence, the action of the specific principles of contagious or infectious fevers, the dyscrasia of scurvy, the influence of poisonous substances, and, in a word, any of those affections of which the escape of blood from the vessels constitutes an epi-phenomenon.
Purpura may be conveniently considered as presenting three varieties: 1, purpura simplex; 2, purpura hæmorrhagica; 3, purpura rheumatica.
These three forms of the disease are not distinguished by sharply-outlined differences, but merge the one into the other, now one, now another set of symptoms predominating. To these may be added, likewise for convenience, three sub-varieties—purpura urticans, purpura papulosa, and purpura nervosa. The difference between these forms of purpura should not be considered as of more than clinical import. Whatever variations present themselves may with probable propriety be ascribed to complicating influences.
PURPURA SIMPLEX.—This is the mildest form of purpura, and may in many cases readily escape observation. It may begin abruptly, in the midst of health, without the slightest subjective symptom, or the extravasations may be preceded for several days by some discomfort, aching of limbs, sluggishness, anorexia, even a small amount of fever. The eruption usually appears first upon the lower extremities, preferably the flexor surfaces of the thighs (Duhring), but frequently upon the legs. It extends from these points to the upper extremities and trunk, usually sparing the face. The lesions vary in size from that of a pin-head to that of a fingernail (petechiæ), or they may be linear (vibices). They remain discrete, and do not increase in size throughout their course. Each spot of hemorrhage will endure for from one to two weeks. At first the lesions are of a livid red color, and declare their extra-vascular nature by remaining unaltered when subjected to pressure. The color of these spots changes, as in ordinary ecchymosis, in consequence of the metamorphoses of the hæmatin preparatory to its final absorption, from crimson to purple, to blue, to green, to yellow, and finally fades away. When recent, the spots appear sharply outlined, with sometimes a faint encircling zone of hyperæmia, but as they become older their margins grow indistinct. While the early lesions slowly disappear, others continue to develop, and the affection may thus be protracted for weeks. At times the petechiæ appear in crops, recurring every few days, the patient at one time apparently nearly well, at another time worse than ever. Finally, the symptoms definitely disappear, to return no more, or they pass into those of other forms of purpura. During the course of purpura simplex the blood-vessels of the skin alone are affected, the deeper tissues and mucous membranes probably remaining unchanged.
Throughout the attack the general health may—usually does—remain good. As an occasional symptom there will be observed a few vesicles or blebs, containing blood, upon the skin. The extent of the general eruption may vary from a few scattered petechiæ to a copious and startling [p. 188]number of purpuric spots. The maintenance of the upright position tends to perpetuate the evolution of the lesions.
In elderly persons purpura simplex is sometimes observed, and has been described by many writers as purpura senilis. Hillier, following Bateman, describes it as occurring in old women "upon the outside of the forearms in successive dark, purple blotches of an irregular form and various magnitude."1 Aged men as well as women are liable to the affection, which may quite as well appear upon the lower extremities of either sex. It is altogether likely, however, that in such cases degenerations of the vascular walls alone may cause the extravasations.
1 Reynolds's System of Medicine, vol. i. p. 792.
PURPURA HÆMORRHAGICA (MORBUS MACULOSUS WERLHOFII).—In this form of purpura there are added to the symptoms of purpura simplex hemorrhages into and from the various mucous tracts, the nasal, faucial, pharyngeal, gastric, intestinal, renal, uterine, rarely the pulmonary mucous membranes, and exceptionally into the various serous membranes and cavities. It may begin abruptly, in the midst of apparently vigorous health, or after premonitory symptoms extending over several days, vague sensations of discomfort—headache, pains, anorexia, indisposition to exertion, and the like—or it may occur as a transition from other forms of purpura. Usually there is no fever.
The hemorrhagic spots upon the skin appear much as in purpura simplex, though the lesions are larger, acquiring the size of coins or even of the palm of the hand. Spots soon appear upon the visible mucous membranes, and free hemorrhages occur; indeed, the latter may be the first symptom observed. Epistaxis is of most common occurrence, but bleeding from the mouth, stomach, and intestines almost as frequently results. The gums are almost constantly affected, and upon inspection these may be found covered with blackish scabs, upon removal of which the mucous membrane will be found pale and not swollen—an important point in diagnosticating this affection from scurvy. Vesicles and blebs filled with blood form both on the skin and mucous membranes. They quickly rupture and discharge their contents. Bleeding from the stomach and intestines is revealed—in the former case by the vomiting of a brownish material resembling coffee-grounds; in the latter case by the passage of black, tar-like evacuations. Pulmonary hemorrhage is to be distinguished from hæmatemesis by the frothy and arterial character of the blood. Hæmaturia may proceed from any part of the urinary tract. Bleeding from several parts may occur at the same time, and may be very copious.
In the mucous membranes extravasations of greater or less extent may occur, as in the derma. Into the serous membranes they may take place with or without effusion into serous cavities. It is only, however, in cases that will almost certainly end fatally that the effusions into these cavities are encountered. Hemorrhages into the substance of the lungs, into the brain and other viscera, as well as into the tissues generally, are occasionally observed.
At the outset of these bleedings the general health of the patient may appear unimpaired, and if they be few in number and moderate in extent but slight evidences of debility may be shown throughout the attack; but it is often the case that the loss of blood is excessive and long continued, and symptoms of profound anæmia supervene. The [p. 189] patient becomes greatly exhausted; intense pallor is developed, shortly followed by general oedema. Attacks of syncope appear, and in fatal cases—which are not common—death results from asthenia. This result may occur after a few days from the profuseness of the hemorrhage; usually, however, only after several weeks. Throughout the attack the cutaneous lesions continue to develop, either irregularly or in successive outbreaks, scattered over the general surface, involving the face less frequently than other parts. These spots undergo the color-changes peculiar to extravasated blood, and may be seen in all the stages of involution in the same patient. Fever, usually absent throughout the attack, may appear at the height of the affection, but does not run high. Local inflammations are exceedingly rare. In favorable cases recovery follows the gradual mitigation and disappearance of the symptoms, but relapses frequently occur, and convalescence may be retarded for months.
PURPURA RHEUMATICA (PELIOSIS RHEUMATICA).—Schoenlein in 1829 described as peliosis rheumatica an affection in which the symptoms of purpura simplex were associated with pain and often with effusion into the joints, especially those of the knee and ankle. He considered it as an independent malady. This opinion has been shared by Fuchs, Hebra, Kaposi, Neumann, and many others. Kaposi2 regards it as related to erythema nodosum, with which affection, indeed, it possesses some features in common. It probably, however, constitutes a complication of ordinary purpura. That it is not primarily rheumatic is shown by the almost invariable absence of many of the symptoms characteristic of rheumatism; that it cannot be an independent affection appears from its intimate relations with other forms of purpura.
2 Hautkrankheiten, 1880, p. 277.
Purpura rheumatica commonly begins with malaise, anorexia, debility, sometimes with mild fever. The patient is soon attacked with pains, of a more or less acute character, in the joints, especially the knees and ankles. There may be some effusion into the joint and cutaneous oedema. After a few days the nature of the complaint will be revealed by an eruption of petechiæ, first near the painful joints, but soon extending, involving in many cases even the head and trunk. The eruption may be at first slightly elevated and surrounded by a fine halo of hyperæmic injection.
The pains usually subside upon the appearance of the eruption, and the malady may be completed after a single outbreak. More commonly new joint-pains are experienced, fresh crops of petechiæ appear, and the trouble may be prolonged for weeks, even months, the patient meanwhile suffering not very greatly in general health. The lesions may be cutaneous only; rarely bleeding from mucous surfaces will occur (Scheby-Buch). Albuminuria may be present (Kaposi). An annual type is said by Kaposi, Neumann, and others to be sometimes observed, the spring and autumn being the usual seasons for the outbreaks. This is supposed to indicate a relationship with erythema nodosum and multiforme. Cardiac murmurs have been detected in the course of purpura rheumatica,3 but these were probably anæmic or antedated the purpuric symptoms. Purpura rheumatica never seems to result in endo- or pericarditis.
3 Kinnicutt, Archives of Dermatology, i. p. 193; Mollière, Ann. de Dermatol., v. p. 44.
SUB-VARIETIES.—Henoch4 and Couty5 have described a form of [p. 190] purpura mostly observed in children, in whom rheumatoid pains occur along with colic and vomiting of greenish or bilious matter, tenesmus, and sometimes with loss of blood from the bowels. The disease may be protracted throughout months by relapses. Cutaneous oedema frequently occurs. Couty regards it as a form whose peculiarities justify its assignment to a position of its own. The cause of the associated train of symptoms is supposed (Couty) to reside in the sympathetic system, and the name purpura nervosa is proposed for it. So many features of ordinary purpura are manifested in these cases that it seems better to consider them as examples of ordinary purpura complicated with gastro-intestinal derangement. It has been suggested that the nausea, vomiting, and abdominal pains may result from extravasation of blood into the peritoneal tissue.6
4 Berl. Klin. Wochenschr., 51, 1874.
5 Gaz. Hebd., 36 et seq., 1876.
6 Immermann, Ziemssen's Cyclopæd., vol. xvii. p. 265.
In the course of purpura there is frequently observed, more especially in purpura simplex, a wheal-like arrangement of the eruption—such, indeed, as occurs in urticaria. The term purpura urticans has been given to this sub-variety, which may or may not be accompanied by itching. Scheby-Buch has suggested that the urticaria may, with more propriety, be attributed to the gastric disturbances that so often accompany the forms of purpura presenting it.7 The wheals are usually seen upon the lower extremities, but may appear elsewhere. A considerable degree of oedema may be present, particularly in lax tissue, such as that of the scrotum, eyelids, etc.
7 Deutsche Arch. f. Klin. Med., B. xiv. p. 490.
Purpura papulosa (lichen lividus, Willan) is a form of purpura where, in the midst of ecchymoses, livid papules appear. These probably depend upon a large amount of hemorrhage occurring within a limited space, most often surrounding the orifices of hair-follicles, because these are supplied with a capillary network that comes directly from the deeper layer.8 They are formed most abundantly on the legs of scrofulous, cachectic persons who have purpura. Care must be taken to distinguish this form of purpura from erythema multiforme and erythema nodosum, where blood is usually extravasated secondarily into the tissues. Those cases only where the purpura is primary should be recognized as purpura papulosa.
8 Hebra, Skin Diseases, New Syd. Soc. Transact., ii. p. 425.
The purpuric effusion appears to act as an irritant upon the tissues, and to excite inflammation. Gangrene of the mucous coat of the intestines has resulted from extensive hemorrhagic extravasations, and from a similar cause cutaneous gangrene has been known. These complications, however, are rare.
ETIOLOGY.—The immediate causes of purpura are quite unknown. Both sexes and persons of every age are affected by it. While it is most often seen in debilitated subjects, those in vigorous health possess no immunity. It has often been observed during convalescence from other maladies. It cannot be said that those who are miserably clothed, fed, and lodged are especially predisposed to attacks of purpura. Between purpura and hæmophilia, etiologically, there are many points of difference. Purpura is not hereditary, nor is there a purpuric diathesis in the strict sense of the term. Some persons, indeed, seem to possess a [p. 191]predisposition to the disease, and some authors claim for purpura rheumatica a distinct annual type. This, however, is not at all certain.
Recently it has been claimed that purpura hæmorrhagica depends upon the presence of a minute organism in the blood. Petrone9 injected blood drawn from patients with this disease under the skin of rabbits, producing widely-distributed hemorrhages. In the blood of these individuals and of the injected rabbits micrococci and bacilli were detected. Watson Cheyne10 also describes a plugging of the capillaries with bacilli. These were 1/7700 of an inch in length and 1/20000 of an inch in diameter, and were arranged in colonies. In another case there were found micrococci arranged in chains. These swarmed in the capillaries and some larger vessels, and sometimes completely blocked them. Although an origin in infection has thus been claimed for purpura hæmorrhagica, the fact that more than one variety of micro-organism was observed cannot fail to excite suspicion of, possibly, erroneous observation.
9 Lo Sperimentale, 51, 1883.
10 Lancet, i., 1884, 344.
PATHOLOGY.—In the foregoing description those extravasations of blood due to simple mechanical violence, as from flea-bite, and sudden increase of blood-pressure, as in the effort of coughing in whooping cough, also from the deleterious influence exerted upon the blood-vessels and blood by certain drugs, the specific fevers, Bright's disease, and the like, have been excluded. Only those have been considered where the effusion of blood seemed to occur spontaneously, and the symptoms to result from some peculiar but not understood morbid process. The hemorrhage is but a symptom; the process by which it is brought about depends upon some change in the blood or blood-vessels. We do not know what these subtle changes are. The blood of purpuric patients has been carefully examined, but, with the exception above mentioned, no definite changes have been discovered. Immermann11 found during the first stage of the disease the blood-corpuscles perfectly normal in appearance, the white corpuscles subsequently slightly exceeding the red in number—a simple result of copious hemorrhage. No stated chemical changes in the blood are known in purpura, nor is it known how the blood escapes from the vessels. It undoubtedly escapes through alterations in the vascular wall, but it is also true that red blood-corpuscles, as well as the pale ones, may find their way in considerable numbers through the unruptured wall of the vessels, per diapedesin, as was first suggested by Velpeau, but definitely determined by Stricker. The causes of this migration are obscure. Immermann12 asserts that a fatty degeneration of the vascular tissues and of the muscles takes place. This, however, is manifestly a result of the loss of blood, and not its cause. Dr. Wilson Fox13 found extensive albuminoid disease of the muscles and capillaries of the skin; but the albuminoid degeneration involved several organs of a patient with syphilis, and the purpura was certainly secondary to the morbid conditions. Rigal and Cornil14 think that the hemorrhages are a result either of sympathetic irritation or of diminished action of the vaso-motor centre. It is indeed altogether likely that the cause will ultimately be found to reside in the vaso-motor system.
11 Ziemssen's Cyclop., xvii. p. 258.
12 Loc. cit.
13 Brit. and Foreign Med.-Chir. Review, Oct., 1865.
14 L'Union Méd., 5, 6, 7, 1880.
[p. 192]DIAGNOSIS.—The affection bearing the closest resemblance to spontaneous purpura is scurvy; indeed, its supposed relationship to this disease has given purpura one of its synonyms, land scurvy. The two affections, however, are probably without the slightest relationship. They possess in common the hemorrhagic symptoms, both in the tissues and from free surfaces, but the resemblance does not extend much beyond this. Scurvy depends upon deprivation of fresh vegetable food and the use of unsuitable and insufficient food generally, and upon bad hygienic surroundings. Purpura may—frequently does—appear in broken-down constitutions, but it equally attacks the strong and vigorous, while the character of food exerts no special influence on its production. Scurvy only follows long-continued privations and as a culmination of a train of distressing symptoms. Purpura appears in the midst of health, or after brief premonition, or during convalescence from totally unrelated diseases. In scurvy there is a decided tendency toward ulceration, which is absent in purpura. In scurvy the mouth and gums inflame and ulcerate, the latter becoming swollen, spongy, and of a bluish-red color. In purpura, ulceration of the buccal mucous membrane does not occur, and the gums are pale and intact. The curative influence of fresh vegetables, lime-juice, etc. in the treatment of scurvy is not observed in purpura. It has been claimed that purpura is but a mild degree of scurvy: this cannot be so, for we may have a mild scurvy or a severe, even fatal, purpura.
The hemorrhagic diathesis, or hæmophilia, presents points of analogy with purpura. Here, however, is found the almost constant history of heredity and the implication only of persons of the male sex. The disposition to bleed at all times upon the receipt of the smallest injury is quite unlike the suddenly-developed and transitory hemorrhages of purpura, which are also more generally distributed.
With the secondary hemorrhagic effusions and ecchymoses that occur in conditions of profound alterations of the blood and blood-vessels in cases of malignant small-pox, scarlatina, typhus fever, etc., and in some cases of poisoning, as from phosphorus, spontaneous purpura presents identities, but the history of the complaint and the condition of the patient will prevent error. A knowledge of the circumstances will serve to distinguish purpura simplex from the petechiæ and small ecchymoses produced by fleas, by diminished atmospheric pressure, by coughing, in the course of Bright's disease, etc.
Purpura rheumatica presents, as has been shown, many points of resemblance to erythema multiforme and erythema nodosum. The mild fever, the joint-pains, the extravasations of the latter affections, are much like the symptoms of this form of purpura. The nodular, inflamed, tender condition of the lesions, their location—frequently upon the extensor surfaces of the extremities—their course and duration, usually serve to identify erythema nodosum, while with erythema multiforme it is usually not difficult to observe its essentially inflammatory character. Scheby-Buch has shown the difficulties often opposed to the differentiation of purpuric lesions and ecchymoses due to violence.15 Where the petechial eruption of purpura simplex is well marked, where the internal hemorrhages of purpura hæmorrhagica are copious, the inquiries of the observer will usually lead him to correct conclusions. Where the [p. 193]ecchymoses are larger and upon exposed parts of the body, the diagnosis from the lesions alone becomes impossible, and due consideration of all concomitant circumstances is essential. It should be remembered that in purpura very slight violence may call forth extensive ecchymosis. This circumstance has important medico-legal bearings.
15 Viertelj. f. Dermatol. und Syph., 1879, p. 99.
PROGNOSIS.—Purpura usually terminates favorably. Its course runs from two to six weeks, rarely longer. Relapses and remissions are frequent. Purpura simplex is of very little gravity, and need excite little apprehension. Purpura rheumatica almost always ends in recovery; fatal terminations, however, have been known. Purpura hæmorrhagica is of much more serious import. Even here, however, though the patient may fall into profound debility from loss of blood, recovery is the rule, the symptoms gradually diminishing in severity until health becomes re-established. In fatal cases death ensues after prolonged and profuse losses of blood. Purpura may subside after a single outbreak or many relapses, and recrudescences may occur extending through months. Anæmia may persist long after the disappearance of purpuric symptoms. A tendency to purpura may be shown at irregular intervals for years, and even throughout life.
TREATMENT.—Very mild cases of purpura simplex require no treatment, not even confinement within doors. The patient is often first made aware of his disease by accident; doubtless it frequently escapes detection altogether. It has been observed that purpura often appears upon the lower limbs of convalescents from other diseases when they first essay the upright position. Relapses of purpura also frequently appear as the patient leaves his bed. We have here an important indication for treatment—viz. the maintenance of the recumbent posture in cases of any degree of severity. Fresh vegetables and vegetable acids do not have the same happy influence as in scurvy. It is manifestly important that appropriate food should be administered in sufficient quantity, both to improve the general health and to repair the exhausting losses of blood. Milk is an exceedingly valuable article of diet in these cases, being but little apt to irritate the mucous membrane of the alimentary canal.
The patient should be guarded against violence. Injuries that may be of no consequence to healthy persons may excite in the purpuric profuse hemorrhage, free or interstitial. Violent emotions and physical efforts should be avoided, as in stimulating the heart's action a condition of increased blood-pressure ensues that may readily result in extravasation.
There are no remedies that exert a specific influence over purpura, and yet quite a number have enjoyed, and still enjoy, high reputation in controlling the symptoms. Probably the most frequently employed remedy against purpura is sulphuric acid, preferably the aromatic sulphuric acid, in doses of from 15 to 20 drops, diluted well with water and administered every third or fourth hour. It is certainly an agent of value, though some authors maintain that it has no efficacy (Immermann). Acetate of lead undoubtedly exercises an influence over the course of the disease. More recently, ergot has been employed. Its use has been highly extolled by Buckley and others. Very large doses may be given. The hypodermic use of ergotin has been followed by results most gratifying to those employing it. Oil of turpentine has enjoyed considerable reputation. A remedy that undoubtedly has a good effect is iron, both as [p. 194]exercising a controlling action over the bleeding and as assisting to repair the resulting anæmia. The tincture of the chloride is the most suitable preparation, and may be given in large doses (from minim xx to fluidrachm ss), well diluted, every fourth hour. Care must be exercised to avoid irritating the digestive organs with it. Formerly, venesection was employed to prevent the occurrence of hemorrhage, but its efficacy in this direction is at least doubtful, and cannot but help to intensify the disastrous consequences of severe and protracted attacks.
The various complications that may arise, as well as the general results of purpura, must be treated symptomatically. For the mucous membranes astringent washes should be used, and in favorable situations the tampon may sometimes be employed with profit. In purpura rheumatica the arthritic pains will be alleviated by anodyne liniments and plasters, and the often accompanying abdominal pains and colic by anodynes internally administered. Hæmatemesis, hæmaturia, etc. must be treated upon general principles. The results of profuse hemorrhage must be combated with stimulants. Transfusion of blood has been proposed and practised for the extreme anæmia that sometimes occurs, but without encouraging results. If necessary, the bowels may be kept free by mild aperients. In severe cases rest in bed should be rigidly enforced until after the establishment of convalescence. Quinia, iron, and nux vomica are indicated above all other remedies for the anæmia resulting from an attack of purpura.
Diabetes mellitus is a term applied to a group of symptoms more or less complex, of which the most conspicuous is an increased flow of saccharine urine—whence the symptomatic title. It is associated with a derangement of the sugar-assimilating office of the liver, as the result of which an abnormally large quantity of glucose is passed into the hepatic vein and thence into the systemic blood, from which it is secreted by the kidneys. The condition is sometimes associated with alterations in the nervous system, at others with changes in the liver or pancreas, while at others, still, it is impossible to discover any structural alterations accompanying it.
FIG. 1. |
To show the position of the punctures required to produce glycosuria, the lobes of the cerebellum are separated. Below are seen the restiform bodies, the divergence of which circumscribes the apex of the calamus scriptorius and the fourth ventricle. The puncture p' produces glycosuria; the puncture p, glycosuria with polyuria; and a puncture a little higher up than p, albuminuria. |
PATHOLOGY AND PATHOGENESIS.—Notwithstanding that this disease has been recognized for two centuries and a half, that abundant opportunity has been furnished for its post-mortem investigation, and that experimental physiology has contributed much information bearing upon the subject, its pathology is still undetermined. Experiment has, however, rendered it very likely that all cases of essential glycosuria—that is, all cases in which saccharine urine is not the direct result of over-ingestion of sugar or sugar-producing food—are accompanied by a hyperæmia of the liver. This hyperæmia, with its consequent glycosuria, can be induced by puncturing or irritating the so-called diabetic area1 in the medulla oblongata. This area corresponds with the vaso-motor centre, and with the roots of the pneumogastric or vagus nerve in the floor of the fourth ventricle; whence it was at first inferred that this nerve is the excitor nerve of glycosuria. It was soon ascertained, however, that when the pneumogastric was cut, glycosuria ensued only when the central end was stimulated, while [p. 196]stimulation of the peripheral portion was without effect. Whence it became evident that this nerve is not the excitor, but the sensory nerve concerned in glycogenesis.
1 The diabetic area, as marked out by Eckhard, and which corresponds with the vaso-motor area, as defined by Owsjannikow (Ludwig's Arbeiten, 1871, p. 21), is bounded by a line drawn four or five mm. above the nib of the calamus scriptorius, and another about four mm. higher up.
It was also learned in the course of continued experiment that glycosuria resulted upon transverse section of the medulla oblongata, of the spinal cord above the second dorsal vertebra, of the filaments of the sympathetic accompanying the vertebral artery, upon destruction or extirpation of the superior cervical ganglion, and sometimes, but not always, after division of the sympathetic in the chest (Pavy); also after section or careful extirpation of the last cervical ganglion, section of the two nerve-filaments passing from the lower cervical to the upper thoracic ganglion around the subclavian artery, forming thus the annulus of Vieussens,2 and after section or removal of the upper thoracic ganglion.
2 Cyon and Aladoff, reprint from the Mélanges biolgiques and Bullétin de l'Académie Impériale de Petersbourg, vol. xiii. p. 91; cited by Dr. Brunton in the Lectures named in note on p. 198; also British Medical Journal, Dec. 23, 1871, p. 731.
FIG. 2. |
The last cervical and first thoracic ganglia,
with circle of Vieussens, in the rabbit, left side. (Somewhat
diagrammatic, many of the various branches being omitted.) Trach., trachea; Ca., carotid artery; n. vag., the vagus trunk; n. rec., the recurrent laryngeal; sym., the cervical sympathetic nerve ending in the inferior cervical ganglia, gl. cerv. inf. Two roots of the ganglion are shown—rad., the lower of the two accompanying the vertebral artery, A. vert., and being the one generally possessing accelerator properties; gl. thor. pr., the first thoracic ganglion. Its two branches, communicating with the cervical ganglion, surround the subclavian artery, forming the annulus of Vieussens. sym. thor., the thoracic sympathetic chain; n. dep., depressor nerve. This is joined in its course by a branch from the lower cervical ganglion, there being a small ganglion at their junction, from which proceed nerves to form a plexus over the arch of the aorta. It is this branch from the lower cervical ganglion which possesses accelerator properties, hence the course of the accelerator fibre is indicated in the figure by the arrows. (Modified from Foster's Physiology.) |
All these operations paralyze the vaso-motor nerves by which, in health, the blood-vessels of the liver are kept in a state of tonic contraction; hence these vessels dilate when the nerves are cut. From the facts named we also learn the path of the glycogenic influence, which must be from the medulla oblongata into the spinal cord, thence by the filaments of the [p. 197]sympathetic which accompany the vertebral artery into the lower cervical ganglion; thence through the annulus of Vieussens into the first dorsal ganglion; and thence through the prevertebral cord of the sympathetic, and branches not precisely determined, to the hepatic blood-vessels as shown by the dotted line in Fig. 3.
FIG. 3. |
Diagram showing the course of the vaso-motor nerves of the liver, according to Cyon and Aladoff. These nerves are indicated by the dotted line which accompanies them: a, vaso-motor centre; b, trunk of the vagus; c, passage of the hepatic vaso-motor nerves from the cord along the vertebral artery; d, fibres going on each side of the subclavian artery and forming the annulus of Vieussens; e, first dorsal ganglion; f, ganglionated cord of the sympathetic; g, the spinal cord; h, the splanchnic nerves; i, coeliac ganglion, from which vaso-motor nerves pass to the hepatic and intestinal vessels; k, the lungs, to which fibres of the vagus are seen distributed; l, the liver; m, the intestine; n, the arch of the aorta. |
I say, by branches of the sympathetic not precisely determined, because our power to produce artificial diabetes fails below the first thoracic ganglion; for section of the sympathetic between the tenth and twelfth ribs, and of the splanchnics, is not followed by glycosuria, although the vaso-motor nerves to the liver are known to pass through them.
According to Eckhard,3 the phenomena of artificial glycosuria are irritative and not paralytic. This view he believes sustained by his own experiments, according to which if the splanchnics, through which [p. 198]the vaso-motor nerves of the liver pass, are cut prior to the diabetic puncture, not only does this operation fail to produce glycosuria, but it even renders ineffectual the puncture itself as well as the section higher up. But Cyon and Aladoff remind us that it is not mere dilatation of the hepatic vessels, but increased velocity in the movement of the blood, which deranges the sugar-assimilating function and causes glucose to appear in the urine. The vaso-motor nerves of the intestinal blood-vessels also pass through the lower part of the sympathetic and the splanchnics, and section of the latter must cause these blood-vessels to dilate. Now, in rabbits, in which this experiment is usually performed, the digestive canal is very long, and the blood-vessels so capacious that when dilated they hold as much blood as all the rest of the vascular system together, so that when the lower sympathetic and splanchnics are cut, so much blood goes into the intestines that the increased velocity required in the blood-vessels of the liver to produce glycosuria is impossible. But if the vessels of the liver be first dilated by puncturing the floor of the fourth ventricle, section of the sympathetic or of the splanchnics may then be made without arresting the formation of sugar; whence it would appear that the glycogenic influence may still pass through the lower sympathetic and splanchnics.
3 Beiträge zur Anat. und Physiologie, iv., 1859, p. 1; vii., 1873.
In view of the fact that Eckhard4 has failed to confirm the results of Cyon and Aladoff, but has traced the glycogenic influence down the spinal cord as far as the fourth dorsal vertebra in rabbits, and even a little lower, and that Schiff5 has shown that diabetes sometimes results after section of the anterior columns of the cord between the medulla and the fourth cervical vertebra, Dr. Brunton6 suggests that the vaso-motor nerves of the liver may not always leave the spinal cord to join the sympathetic by the branches accompanying the vertebral artery, but sometimes pass farther down the cord, leaving it by the communicating branches to some of the dorsal ganglia, as indicated in Fig. 4.
4 Beiträge zur Anat. u. Physiologie, viii., 1877, p. 79.
5 Untersuchungen über Zuckerbildung in der Leber, 1859, S. 108.
6 Lectures on the Pathology and Treatment of Diabetes Mellitus; reprinted from the British Medical Journal, 1874, p. 12.
FIG. 4. |
Diagram showing another course which the vaso-motor nerves of the liver may take. The letters indicate the same parts as in Fig. 3. The hepatic vaso-motor nerves are here represented as passing lower down the cord than in Fig. 3, and leaving it by communicating branches to the second dorsal ganglion. It is possible that they may sometimes leave by the branches to the first, and sometimes by those going to a lower, ganglion. In such cases any irritation to the third or one of the other cervical ganglia may cause diabetes by being conveyed along the vertebral artery and up the cord, as indicated by the dark line, to the vaso-motor centre, where it may cause reflex inhibition in the same way as any irritation to the vagus. |
It is evident that an agency involving any part of this tract in such a way as to paralyze the vaso-motor nerves of the liver is capable of producing glycosuria. Such cause may operate upon the central ganglia whence the nerves emanate, as the vicinity of the oblongata and upper parts of the spinal cord or the coeliac ganglion and its branches, including those to the pancreas. Or the irritation may be peripheral and its effects reflex. We have seen that irritation of the central end of the cut vagus will produce glycosuria. Any irritation, therefore, involving the peripheral distribution of this nerve may produce it. Hence embarrassed respiration, whether due to disease of the respiratory passages, strangulation, or inhalation of irrespirable gases and anæsthetics, produces glycosuria in dogs and rabbits; and this symptom has been known to attend these conditions in the human subject. So, too, glycosuria may be produced by such substances as woorara, strychnia, morphia, and phosphoric acid, introduced into the blood and irritating the terminal filaments of the pneumogastrics, or it may be brought about secondarily through the embarrassed respiration these drugs produce. Such peripheral [p. 199]irritation may reside also in the stomach, intestines, liver, or any organ to which the pneumogastric is distributed.
It is not unlikely that irritation of the extremities of sensory nerves other than the pneumogastric may become the cause of reflex glycosuria. Even puncture of the floor of the fourth ventricle itself may be reflex in its operation, the roots of the pneumogastric being thus irritated. The effect of the irritation conveyed to the glycogenic centre is to inhibit the usual tonic influence of the vaso-motor nerve upon the vessel walls. Among the experimental irritations, in addition to puncture of the floor of the fourth ventricle, which produce glycosuria by reflex action, are injuries of the cerebral lobes and cerebellum, optic thalami, cerebral peduncles, pons varolii, middle cerebellar peduncles, and even of the sciatic nerve and brachial plexus; whence it may be inferred that pathological irritation in the same situations may result in a glycosuria, which is temporary or permanent according as the irritation is temporary or permanent.
Finally, there is no reason why an inhibitory reflex action should not originate in the sympathetic itself. When we remember that this nerve is both sensory and motor in function, and that the inhibitory influence to which the heart's action is subject is accomplished through the sympathetic as a sensory nerve and the pneumogastric as a motor, there is no reason why similar results may not be brought about by the sympathetic alone. This being the case, we need not ascribe glycogenic phenomena to irritation in Eckhard's sense—that is, to a direct stimulant action of the irritant upon the vaso-motor nerves of the liver—but may suppose a sensory influence to ascend one set of sympathetic filaments and an inhibitory influence to descend through another.
Dr. Pavy has recently put forward some chemical theories which explain the action of the hyperæmia in producing glycosuria, but they do not account for the hyperæmia itself. In healthy digestion the carbohydrates (starch and sugar) are converted, not into glucose, but into maltose, C12H22O11, dextrin being intermediate in composition. Maltose is absorbed and assimilated, converted into glycogen. So, too, when glucose is ingested as such, it is converted by the glucose ferment into maltose in the stomach and intestines. For the proper production of maltose and its assimilation a good venous blood, producing a maltose-forming ferment, is necessary. In diabetes, in consequence of the dilatation of the arteries of the chylopoëtic viscera, the blood enters the liver too little deoxygenated, and a glucose-forming ferment is produced. The glucose thus formed is not assimilable, but passes off into the circulation and the urine.
MORBID ANATOMY.—Such are some of the facts bearing upon the pathology of diabetes mellitus. Throwing out the milder type of cases, in which glycosuria is the result of an over-ingestion of saccharine and sugar-producing food—and these can scarcely be called instances of essential diabetes—it is evident that glycosuria may be produced in a variety of ways operating through the nervous system; and accordingly we may infer that there is scarcely an organ in close relation with the sympathetic system derangement of which is not capable of producing it. Among these we would naturally expect to find conspicuous alterations in the nervous centres, and yet I have never found changes in these centres after death. At the same time, others have noted meningitis, tubercular [p. 200]and traumatic, apoplectic effusions, and tumors of the brain, especially in the neighborhood of the medulla oblongata. The alterations in the nerve-centres described by Dickinson as the essential morbid anatomy of diabetes I have looked for in vain. These changes are described as a cribriform or porous condition of the white nervous matter, said to be visible to the naked eye. The spaces thus produced are partially occupied by dilated blood-vessels, which, in turn, are surrounded by dilated perivascular sheaths and broken-down nervous matter, into which extravasations of blood have taken place, as evidenced by the presence of pigment-granules. The changes are found in the white matter of the convolutions of the brain, but fewer and larger in the central portions. The corpora striata, optic thalami, pons, medulla, and cerebellum are favorite seats for the largest and most striking holes. In rapidly-fatal cases the cavities are sometimes filled with a translucent, gelatinous substance, containing, besides vascular elements, the globular products of nervous disintegration. In the more chronic forms of the disease, as it occurs in elderly persons, the excavations are usually empty, although the elements of nervous decay are still to be found fringing the margins or collected as an irregular sheath upon the dilated or shrunken artery. There are changes in the cord similar to those in the brain, but less decided. But the most striking alteration in the cord, according to Dickinson, although not always present, is dilatation of the central canal, which in the dorsal and lumbar regions is sometimes expanded to many times its normal diameter, and forms a conspicuous object immediately after the cord is divided.
These alterations have eluded the vigilance of other pathologists who have sought for them in well-determined cases of diabetes mellitus, while they have been found, on the other hand, in the nervous centres when no diabetes was present. In the recent discussion on diabetes at the Pathological Society of London, Douglas Powell7 seemed to be the only one who was convinced that most of Dickinson's specimens were examples of positive lesions.
7 London Lancet, May 5, 1883, p. 776.
A hyaloid thickening of the blood-vessels of the brain has been noted by Stephen Mackenzie8 and Seymour Taylor9 in some cases, and miliary aneurisms of the retina in one.
8 Discussion on Diabetes, Path. Soc. of London, London Lancet, April 7, 1883, p. 593.
9 Ibid., Lancet, May 5, 1883, p. 774.
Of other organs, one of the most frequently found diseased is the pancreas, and, according to Senator, it is fair to assume that disease of the pancreas is present in about one-half of all cases of diabetes. As the result of increased experience, I am inclined to attach much more importance to pancreatic disease as a cause of diabetes than I did a few years ago. Among the changes found is a pseudo-hypertrophy, which consists chiefly in a hyperplasia of the connective tissue, fatty degeneration of the gland-cells, and atrophy of the glandular structure; cancerous disease; calculous concretions in the ducts with or without obstruction; and cystic dilatation.
Facts bearing upon the relation of pancreatic disease to diabetes have been accumulating since Cowley first discovered calculi in the pancreas of a diabetic, and Bright pancreatic cancer in a similar case. Since then [p. 201]instances have multiplied to such extent that it would be unprofitable to enumerate them. But in 1877, Lancereaux10 communicated to the French Academy of Medicine specimens of profound lesion of the pancreas from cases dying of diabetes mellitus. This, he alleged, constitutes a special and distinctive variety of diabetes, characterized by sudden onset, emaciation, polydipsia, polyphagia, and peculiar alvine dejections. More recently, Depierre11 has confirmed these observations, apparently establishing this variety of diabetes mellitus, of which a very rapid course—six months to three years—and the habitual presence of diarrhoea are characteristic; while the presence of greasy or creamy stools, and the appearance in them of undigested nitrogenous substances, may aid in the diagnosis. Precisely such a case, running the same rapid course—less than one year—with emaciation, uncontrollable diarrhoea, creamy stools, jaundice, and pancreatic disease, came under the writer's care in 1882. At the autopsy the pancreas was found enlarged, and numerous gritty particles were disseminated through it.
10 "Notes et réflexions à propos de deux cas de diabète sucre avec altération du pancréas," Bull. Acad. de Méd., Paris, 1877, 2d Serie, vi. 1215-1240.
11 Med. News and Abstract, vol. xxxix., June, 1881, p. 344, from Jour. de Méd. et de Chir. pratiques, Dec, 1880.
Supposing such pancreatic disease to be primary, it is evident that it must operate through the coeliac plexus, which, with its ganglion, is gradually encroached upon. On the other hand, it is also possible that the disease of the coeliac plexus may be primary, and the coexisting pancreatic disease and diabetes mellitus both secondarily dependent upon it. This can only be settled by more careful study of the coeliac plexus after death from diabetes, but up to the present time facts would seem to support the view of primary pancreatic disease.
The liver is frequently enlarged—sometimes but slightly, at others decidedly. It has been known to reach three times the size of the normal organ. Again, it may be darker and harder—hyperæmic. By minute examination the acini are found enlarged, the capillaries dilated and distended; the liver-cells are enlarged, distinctly nucleated, rounded, and indistinct as to their outline, appearing to fuse into each other. A weak solution of iodine strikes a wine-red color, which, according to Rindfleisch, is confined to the nucleus, but, according to Senator, may extend to the whole cell. This reaction Klebs ascribes to post-mortem changes in the glycogenic substance. They are more striking in the portal or peripheral zone of the lobule, while the intermediate or hepatic artery zone is often fatty, and the central part, surrounded by the rootlets of the hepatic vein, is nearly normal. Stockvis and Frerichs ascribe the enlargement of the liver partially to a new formation of liver-cells—in other words, to a true hypertrophy. At other times the organ has been found reduced in size.
Dickinson, Trousseau, and Budd describe an overgrowth of connective tissue, as well as of the cells of the liver, producing a hypertrophic cirrhosis.
According to Beale, Frerichs, and Folwarczny, the fat which is found in small proportion in the liver-cells in health is often diminished, and even absent, and quantitative12 analysis by the last-named observer [p. 202]confirms this view. Such diminution may be the forerunner of an atrophy of liver-cells which has been noted, and which, as the disease continues, leads to the atrophy referred to as occasionally present. On the other hand, intense fatty degeneration of the entire organ, similar to that found in phosphorus-poisoning, has been met by Gamgee, associated with a lipæmic state of the blood and symptoms of acute acetonæmia.
12 Folwarczny, "Leberanalysen bei Diabetes Mellitus," Wiener Zeitschr., N. F., 1859, ii. 6.
The kidneys, in cases which have continued some time, are apt to be hyperæmic and enlarged, although primarily they are uninvolved. It would seem that the long-continued hyperæmia which is a necessary condition of the copious secretion of urine, results, sooner or later, in an over-nutrition of the renal epithelium, a widening of the tubules, and consequent enlargement of the whole organ. The changes are mainly of a parenchymatous or catarrhal rather than an interstitial nature, the epithelium being disposed to shed. These changes may reach a more advanced stage of cellular degeneration, and may be attended by albuminuria. The cells may become very large, present a yellowish-brown color, their nuclei indistinct and non-responsive to ordinary staining solutions, but may take a red stain with a weak solution of iodine, similar to that described in the case of the liver-cells. Mackenzie describes a hyaline degeneration of the intima of the arterioles and a skeleton condition of the epithelium of the collecting tubes.13 There may also be a catarrh of the pelves of the kidneys and ureters, due to irritation of the saccharine urine.
13 Loc. cit.
Atrophy of the testes has been noted by Romberg and Seegen in young men, and recently Hofmeier14 has reported the case of a young diabetic woman, aged twenty, who came under observation for pruritus vulvæ, in whom the uterus was found small, scarcely 5 cm. (2 inches) long, and the ovaries very much atrophied. As this young woman had no other ailment, the atrophy was ascribed to the diabetes.
14 Berliner klin. Wochenschr., 1883, No. 42.
Among the most constant secondary lesions is the aggregate of changes known as those of pulmonary phthisis. But a few years ago, when our ideas on this subject were more definite than they are to-day, and when it was thought we had three distinct varieties of phthisis—the tubercular, the catarrhal, and the fibroid—the phthisis of diabetes was regarded as typically catarrhal.15 At the present time, however, when the tendency at least is to regard all phthisis as tubercular, diabetic phthisis must be consigned to the same category. At the same time, if the tubercle bacillus is to be regarded as the essential criterion of tuberculosis, it must be stated that the diabetic patient is subject to two different lung processes—at least if the observations of Riegel of Giessen16 are to be regarded as correct. In two cases of diabetic phthisis studied at his clinic, the sputum of one contained numerous bacilli, while the other, although the case presented the most distinct signs of infiltration of the apex, and although more than fifty preparations were investigated, revealed none. The sputum was also said to present some unusual physical characters. So far as I know, no autopsies of cases showing these clinical differences have been reported, although there have been found in diabetes, distinct from the usual cheesy foci, fibroid changes with small smooth-walled cavities. In such cases [p. 203] tubercle bacilli would be absent, while the physical signs of consolidation would be present.
15 See the writer's work on Bright's Disease and Diabetes, Philada., 1881, p. 256.
16 Medical News, Philada., May 19, 1883, from Centralblatt f. klin. Med., Mar. 31, 1883.
As a part of the phthisical process in diabetes, cavities of various sizes are found and gangrene of the lungs has been observed.
ETIOLOGY.—The problem of the etiology of diabetes mellitus is as unsatisfactorily solved as is that of its pathogenesis. Certainly, a majority of cases of diabetes cannot be accounted for. A certain number may be ascribed to nervous shock, emotion, or mental anxiety; a few to overwork; some to injury and disease of the nervous system; others to abuses in eating and drinking. Among the injuries said to have caused diabetes are blows upon the skull and concussions communicated to the brain, spinal cord, or vaso-motor centres through other parts of the body. Hereditation is held responsible for a certain number of cases. Malarial and continued fevers, gout, rheumatism, cold, and sexual indulgence have all been charged with producing diabetes.
Diabetes mellitus is most common in adult life, although Dickinson reports a case at six years which was fatal, Bence Jones a case aged three and a half, and Roberts another three years old; and in the reports of the Registrar-General of England for the years 1851-60 ten deaths under the age of one and thirty-two under the age of three are included. This statement, in view of the experience of the difficulties of diagnosis in children so young, seems almost incredible. I have never myself met a case in a child under twelve years. At this age I have known two, of which one, a boy, passed from under my notice, while the second, a girl, recovered completely. The disease is most common between the ages of thirty and sixty. The oldest patient I have ever had died of the disease at seventy-two years, having been under my observation for three and a half years.
It is decidedly more frequent in men than in women, carefully prepared statistics of deaths in Philadelphia during the eleven years from 1870 to 1880, inclusive, giving a total of 206 deaths, of which 124, or three-fifths, were males, and 82, or two-fifths, females. This is the experience of all.
My own experience has been singular and interesting. Up to April, 1881, I had never met a case in a woman. Of 18 cases outside of hospital practice which I have noted since that date, 9 were men and 9 women. But I still do not recall an instance of a woman in hospital practice, although I have constantly cases among men.
Not much that is accurate can be said of the geographical distribution of the disease. It seems to be more common in England and Scotland than in this country, at least if the statistics of New York and Philadelphia are considered. In the former city, statistics extending over three and a fourth years show that out of 1379 deaths, 1 was caused by diabetes; in Philadelphia, in eleven years, 1 out of 875; in England and Wales, according to Dickinson from observations extending over ten years, 1 out of 632; and in Scotland, 1 out of 916. According to the same authority, the disease is more prevalent in the agricultural counties of England, and of these the cooler ones, Norfolk, Suffolk, Berkshire, and Huntingdon. According to Senator, it is more common in Normandy in France; rare, statistically, in Holland, Russia, Brazil, and the West Indies, while it is common in India, especially in Ceylon, and relatively very frequent in modern times in Wurtemberg and Thuringia. Seegen says it is more [p. 204]frequent among Jews than among Christians, but I have never seen a case in a Hebrew.
SYMPTOMS, COURSE, AND DURATION.—The earliest symptom commonly noted by the diabetic is a frequency of micturition and the passage of larger amounts of urine than is natural. Coincident with or immediately succeeding this is an undue thirst and dryness of the mouth, which soon becomes the most annoying symptom the patient has, the freest draughts of water giving but partial or temporary relief. To this succeeds dryness, and sometimes itching, of the skin and absence of perspiration. A good appetite with fair digestion accompanies this stage of the disease, but notwithstanding this the patient loses in weight. If a male, his attention is sometimes called to his urine by the white spot left after the evaporation of a drop of urine on his boot or clothing or by the stiffness of his linen due to the same cause. To these symptoms are sometimes added an intolerable itching of the end of the urethra in males and of the vulva in females, probably due to the irritation caused by the saccharine urine in passing over and drying upon these parts.
As the disease progresses muscular weakness supervenes. This, however, comes on at varying periods after the incipient symptoms make their appearance. Sexual inclination grows less. The muscular weakness gradually increases, if the disease is not checked, until the patient can barely walk: he totters in his gait, and reminds one of a case of Duchenne's disease. Even before this he sometimes gives up and goes to bed. Often harassing cough ensues, adding its exhausting effect to that of the essential disease. Percussion and auscultation discover consolidation at one apex or over larger areas of the lungs. Dyspepsia and indigestion replace the good appetite which attended the onset of the symptoms, and all efforts to increase the latter are unavailing. The heart begins to flag, and its action is irregular. It finally ceases to act, and the patient dies suddenly, sometimes unexpectedly. Or coma may supervene before death. This coma, known as diabetic coma, is generally ascribed to the accumulation of acetone or acetone-producing substance in the blood. It is supposed to be a product of the decomposition of the sugar in the blood, and the phenomena resulting from its presence are known as those of acetonæmia. Some further account of it will be given in the section on changes in the urine. It is sometimes recognizable by a fruity odor of the breath, which may even pervade the atmosphere of the room in which the patient lies, and may be recognized on entering. It has been compared to the odor of a room in which apples have been kept, again to sour beer, and again to chloroform.
During all this time the thirst and discomfort arising therefrom, continue, although it sometimes happens that toward the end the quantity of urine and its contained sugar diminish and the urine becomes darker in hue.
Such is the course of a typical case of diabetes mellitus. Other symptoms, less conspicuous, are a lowered temperature of the body, from 1° to 2½° F. or even more; cataract, dilatation of the retinal vessels, intraocular lipæmia, functional derangements of vision, including amblyopia, presbyopia, and loss of accommodating power; and occasionally total blindness from atrophy of the retina may be present. I have known almost total blindness to appear very early in the disease, and [p. 205]subsequently to disappear. Derangements of the other special senses, as impairment of hearing, roaring in the ears, and disorders of smell and taste, also occur. Boils and carbuncles are occasional symptoms; although usually late in occurrence, the former are said to be sometimes the first symptoms recognized. Numerous skin affections may occur. Ulcerated surfaces are slow to heal, and gangrene supervenes sometimes spontaneously, but more often as the result of some trifling injury. It may start from a blister produced by cantharides, although such instances are scarcely frequent enough to justify interference with treatment demanding blisters. More frequently surgical operations do badly. Allied to this tendency is a spongy state of the gums, with recession and excavation, resulting, in asthenic cases, in absorption of the alveolar processes and falling out of the teeth. Eczema of the labia and vicinity in females, and a similar irritation about the meatus urinarius in males, are annoying symptoms. A purulent-looking discharge has been seen issuing from the urethra, in which the spores of penicilium glaucum have been recognized by the microscope.
The term diabetic coma is applied to a form of coma which is apt to occur late in the disease, indeed most frequently to terminate it; while it is also used to indicate a train of nervous symptoms of which coma is the terminal one. To this train of symptoms the word acetonæmia is also applied, and should alone be used, while the term diabetic coma should be restricted to the terminal symptom. The coma, as well as the previous nervous symptoms, is considered due to the accumulation in the blood of a product of the decomposition of sugar, formerly believed to be acetone, but now thought to be an acetone-producing substance, probably aceto-acetic acid. It is likely that in all cases of diabetes a small quantity of this substance exists in the blood, from which it is separated by the kidneys and lungs, while it is only when these channels are insufficient for its removal that it accumulates and produces the symptoms described.
Usually, the coma comes on gradually, deepening until it terminates in death. In other instances it is preceded by various symptoms, including dizziness, drowsiness, cephalalgia, delirium, mania, muscular pains, gastric and intestinal symptoms, including epigastric pain, vomiting—sometimes of blood—and even purging; also dyspnoea, with short, panting respiration like that of an animal with both vagi cut, and a fluctuating pulse-rate which continues until coma is established, after which it remains rapid and small. Both the breath and urine may exhale the peculiar odor of acetone, or it may be absent, and the urine strikes the peculiar burgundy-red reaction with perchloride of iron to be again referred to.
These symptoms may be sudden in their occurrence, whence acute acetonæmia, or they may ensue slowly. Ralfe,17 who has studied the subject of acetonæmia very thoroughly, has called attention to the parallelism between the phenomena of acute acetonæmia and those of acute yellow atrophy of the liver and of phosphorus-poisoning. The sudden, sharp epigastric pain, with gastric disturbance and vomiting, often of blood; the peculiar panting dyspnoea referred to; the short, [p. 206]noisy delirium, followed almost suddenly by deep coma; the fall in temperature as the nervous symptoms develop; the irregular, and finally rapid, pulse,—are all symptoms common to the two conditions.
17 Clinical Chemistry, 1883, p. 98; also Discussion on Diabetes before Pathological Society of London, Lancet, April 7, 1883, p. 592.
Although acknowledged to be a grave complication, and the most frequent cause of death in diabetes,18 yet it does not follow that a fatal termination is inevitable when diabetic coma sets in. I have now a patient, a woman, who considers herself in perfect health, but in whom there remains a trifling glycosuria, who at one time was supposed to be dying of diabetic coma.
18 Of 400 cases of diabetes which passed under the observation of Frerichs, the majority died of acetonæmia (Frerich's "Ueber den plötzlichen Tod und über das Coma bei Diabetes," Zeitschr. für klin. Med., 1883, vi. 3-53). Of 53 persons dying of diabetes at Guy's Hospital, London, during the last ten years, 33 died comatose (Dr. Fred. Taylor, Discussion on Diabetes, Pathological Society of London, Lancet, May 5, 1883). In my own experience acetonæmia has not been so frequent a cause of death as phthisis, acute pneumonia, and heart-failure.
Crampy pains in the legs and facial paralysis are among the nervous symptoms sometimes present, and the term diabetic neuralgia has been applied to a special form of neuralgia peculiar to this disease. It is characterized by its acuteness, stubbornness, and symmetry. Its favorite seats are the inferior dental nerves and the sciatics. Greisinger referred to the frequency of sciatica in 1859, Braun again in 1868, and others still later; but Worms in 1881 established the close relation between the two conditions and the features described. Most recently (1884), Cornillon19 collected 22 cases of diabetic neuralgia, and has further elaborated the study. Believing that diabetes affects particularly those persons who have had serious attacks of rheumatism and gout, he is inclined to think the neuralgia as much due to uricæmia as to hyperglycosuria, and that these conditions cause, not neuritis, but transitory lesions in the nerve-centres, but whether in the membranes or gray or white matter is undetermined.
19 "Des nevralgies diabétiques," Revue de Médecine, 1884, iv. 213-230.
That the phenomena of acetonæmia are those of a toxic agent or agents in the blood derived from the sugar there present is generally conceded, although Sanders and Hamilton,20 after a study of the clinical histories and the result of autopsies in several cases, are disposed to ascribe diabetic coma to slow carbonic-acid poisoning due to fat embolism of the pulmonary vessels. So far as I know, these conclusions have not been reached by any other observers. R. H. Fitz21 and Louis Starr22 have each reported cases of diabetic coma with lipæmia, carefully studied with this point in view, without finding any facts to sustain the carbonic-acid theory.
20 Edinburgh Med. Journal, July, 1872.
21 "Diabetic Coma; its relations to Acetonæmia and Fat Embolism," Boston Medical and Surgical Journal, vol. cvi. p. 24, Feb. 10, 1881.
22 "Lipæmia and Fat Embolism in Diabetes Mellitus," New York Medical Record, vol. xvii., 1880, p. 477.
Alterations in the Blood.—The blood of diabetics is variously charged with sugar, which may be in such quantity as to impart a viscidity and higher specific gravity to the plasma, which has reached 1033, the normal being 1028. On the other hand, analyses have sometimes failed to discover sugar in the blood after death, the result, probably, of the tendency of the sugar to rapid disintegration. Alcohol and acetone, or [p. 207]acetone-producing substance (aceto-acetic acid), are occasionally present as the products of such decomposition, to which are ascribed the symptoms of acetonæmia already discussed.
The presence of fat in the blood of diabetics was noted by the earliest students of the disease. It is sometimes sufficient in amount to produce a milky appearance of the serum, while the analyses of Simon revealed a quantity of 2 to 2.4 per cent., the normal being 1.6 to 1.9 per cent. The fat thus present is said to be sometimes sufficient to cause fat embolism in the capillaries of the lungs, and cases of this condition have been reported by Sanders and Hamilton,23 Louis Starr,24 and Rickards.25 Ralfe ascribes the lactescent appearance of the blood to the action of the aceto-acetic acid, since acetic will give a milky appearance when agitated with a dilute and slightly alkaline mixture of fatty matter at 100°, and the injection of acids into the blood of animals leads to the increase of fatty matter in the blood and fatty infiltration of tissues.
23 Loc. cit.
24 Loc. cit.
25 Birmingham Med. Review, Jan., 1882.
It must be admitted that the mode in which this lipæmic state of the blood is brought about is imperfectly understood, and whether it be by some chemical agency of the kind described by Ralfe, or by rapid absorption of the subcutaneous fat, or from an imperfect oxidation of absorbed fat, is undetermined. Possibly all may contribute.
Albert G. Heyl26 has described an altered appearance of the retinal vessels recognizable by the ophthalmoscope, which he ascribes to the fatty blood-plasma at the periphery of the blood-current, the normal plasma being invisible on account of its transparency.
26 For a detailed description of this appearance, with a colored lithograph depicting it, see the author's work on Bright's Disease and Diabetes, p. 262.
The red blood-discs are diminished and their ratio to the white corpuscles altered. In a count by F. P. Henry, in Louis Starr's case, the number of red discs was 4,205,000 to a cubic millimeter, the normal being at least 5,000,000; the white were 50,000 to a cubic millimeter, or 1 white to 84 red, instead of 1 to 350 or 500.
Changes in the Urine.—The most important changes in the urine are its increase in quantity and the presence of sugar. The variations in the former are extreme, being from an amount which but slightly exceeds the normal to as much as 50 pints (23.65 liters) in twenty-four hours, and even more. The quantity is of course limited by the fluid ingested, and although it may exceed this amount for a day or more, it cannot do so for any length of time. It is generally a little less. The more usual quantity in the twenty-four hours is from 70 to 100 ounces (210 to 300 cc.).
The quantity of sugar varies greatly in different cases and at different times in the same case. The maximum quantity reported by Dickinson was 50 ounces, or 1500 grammes, in twenty-four hours. The proportion may reach as much as 15 per cent., but the more usual amounts are from 1 to 8 per cent., or from 5 to 50 grains (.324 to 3.24 grams) to the fluidounce, or from 300 to 4000 grains (19.44 to 260 grams) in the twenty-four hours.
It is important to know that intercurrent febrile disease may produce a decided diminution in the daily quantity of urine, and of the sugar contained in it. A similar decrease, and even disappearance, is said to take place sometimes toward the fatal termination of a case.
[p. 208]The effect of exercise upon the sugar secretion is not uniform. Bouchardat and Kuelz have noted a diminution, and even disappearance, of sugar from urine as its result, and it is reasonable to suppose that judicious exercise is at least without harmful effect, while it is certain too that muscular exercise, if excessive, will increase glycosuria.
Changes in diet of course modify the secretion of sugar, starches and saccharine foods increasing it, while nitrogenous and oily foods diminish it. So, too, the urine secreted on rising in the morning has almost always less sugar in it than that passed on retiring; and it is not rare to find no sugar in urine passed on rising, when that passed on retiring at night may contain a small amount of sugar—from ¼ to 1 per cent. On the other hand, I have found a small amount of sugar in the morning urine when the evening urine contained none. Anxiety and excitement both increase the proportion of sugar.
Inosite, or muscle-sugar, is sometimes associated in urine with diabetic sugar, and occasionally replaces it. So, too, in experiments upon animals puncture of the fourth ventricle is sometimes followed by inosuria instead of glycosuria, and in corresponding organic disease of the brain the same thing is observed. The substitution of grape-sugar by inosite in the course of diabetes is considered by Laboulbène27 a favorable change.
27 "Note sur l'Inosurie, succédant au diabète glycosurique, et paraissant avoir une action favorable," L'Union Médicale, Oct. 14, 1883.
As would be expected, the specific gravity of saccharine urine is usually high—most frequently from 1025 to 1040—and Bouchardat noted a specific gravity of 1074 in one instance. On the other hand, I have found sugar easily detectable in urine with a specific gravity as low as 1010. Pavy records an instance of the same specific gravity, and Dickinson one in which the specific gravity was as low as 1008. It is to be remembered that the sugar is rapidly destroyed when fermentation sets in. A coincident diminution in the urea and other solids of the urine will reduce the specific gravity of a saccharine urine otherwise heavier.
The depth of color of diabetic urine is inversely as the quantity passed. Hence, when this is very large the urine is pale, and even almost colorless, but it may still contain considerable amounts of sugar and possess a decided color, quite as deep as that of urine passed in smaller quantity. When exposed to the air, diabetic urine becomes rapidly turbid from the growth of fungi, including the yeast fungus and penicilium glaucum.
The odor of diabetic urine just passed is usually in no way peculiar, but as fermentation progresses an acetous odor is developed, which is ascribed to acetic acid. At other times the odor is quite peculiar, being spoken of as vinous or compared to that of sour beer, stale fruit, alcohol, chloroform, or, as by one of my patients, to sweetbrier.
Diabetic urine has almost invariably an acid reaction, which becomes more decided as fermentation progresses. As a consequence of this increased acidity, and sometimes independent of fermentation-changes, the urine deposits a sediment of uric acid, but with this exception diabetic urine is generally free from sediment. Diabetic patients on a meat diet sometimes have a good deal of uric acid from this source.
Albuminuria may coexist with glycosuria, but is not generally found until late in the disease, after changes in the kidney begin to make their [p. 209]appearance, unless, as may happen, glycosuria supervenes upon primary renal disease.
Alcohol and acetone, or an acetone-yielding substance—aceto-acetic acid—are sometimes found in diabetic urine. They are products of the breaking up of sugar, but chemists do not explicitly agree as to the exact method in which acetone originates in the organism. First recognized in the distillate of urine and blood of a diabetic patient by Petters28 through its physical properties, odor, combustibility, etc., rather than by actual isolation, it was further investigated by Kaulich,29 Gerhardt,30 Rupstein,31 and Markownikoff,32 who obtained it in an impure state from urine; by Deichmüller and Tollens,33 whose isolated substance was pure, and finally most recently by Jaksch34 and Penzoldt.35 The former found it not only in diabetic urine, but also in that of fever, and even of carcinoma. The latter found it by the indigo test in but 18 out of 22 diabetics, and by the iodoform test, either decidedly or feebly, in 20 out of 20; in 3 out of 11 cases of typhoid fever, in 6 out of 7 cases of pneumonia, in none of 6 cases of phthisis, in 1 out of 3 cases of measles, and in 1 case of cerebro-spinal meningitis. Finally, v. Jaksch has been led to believe, from his extensive investigations, that acetone is a constant and normal product of tissue-change, although Penzoldt considers such conclusion scarcely justified.
28 Prager Vierteljahrschrift, xiv. 3, 1857, S. 88.
29 Ibid., xvii. 3, 1860, S. 59.
30 Wiener Med. Presse, No. 28, 1865.
31 Centralbl. für d. med. Wiss., No. 55, 1874.
32 Liebig's Annalen, Bd. 182, S. 362.
33 Ibid., Bd. 209, S. 25.
34 Zeitschrift für physiol. Chemie, vi. 6.
35 "Beiträge zur Lehre von der Acetonurie und von verwandten Erscheinungen," Deutsch. Archiv für klin. Med., xxxiv., 2 Oct., 1883, S. 127.
Gerhardt early discovered a substance in the urine of diabetics and habitual drinkers which struck a deep-red reaction with chloride of iron. This he considered was the source of acetone, and was probably ethyl diacetate or diacetic ether, which by decomposition yields equal molecules of acetone and alcohol; thus:
This view is still held by some, but others, in view of the recent discovery of Deichmüller and Tollens,36 that diabetic urine when distilled yields decidedly more acetone than alcohol, have suggested that the substance is derived from aceto-acetic acid.
36 Loc. cit.
The first test suggested for acetone was Gerhardt's chloride-of-iron test. A solution of chloride of iron added to urine containing acetone strikes a burgundy-red color. But this reaction occurs with so many substances that it cannot be considered entirely reliable. Ralfe's modification of Lieben's iodoform test37 is made as follows: About a fluidrachm (3.7 c.c.) of liquor potassæ, containing 20 grains (1.2 grams) of iodide of potassium, is placed in a test-tube and boiled; a drachm (3.7 c.c.) of the suspected urine is then carefully floated upon the surface. When the urine comes in contact with the hot alkaline solution a ring of phosphates is formed, and after a few minutes, if acetone or its allies are present, the ring will become yellow and studded with yellow dots of iodoform, which, in turn, will sink through the ring of phosphates and deposit itself at the bottom of the test-tube. A number of other substances [p. 210]produce the iodoform reaction, but only one of these, lactic acid, is likely to be met in urine.
37 Clinical Chemistry, Philadelphia, 1884, p. 100.
The perspiration, saliva, exudations, and effusions in diabetic cases have all been found, at times, to contain sugar.
DURATION.—Diabetes is a disease of which the duration is measured by months and years, and although cases are reported in which death supervened in from six days to six weeks after the recognition of the disease, it is evident that such periods do not necessarily measure its actual duration. The disease may have existed some time before coming under observation. On the other hand, a case is reported by Lebert which lasted eighteen years; another, under the successive observation of Prout and Bence Jones, sixteen years; and a third, under Bence Jones and Dickinson, fifteen years. The younger the patient the shorter usually is the course run and the earlier the fatal termination. Yet I have known a girl of twelve recover completely. After middle age the disease is usually so easily controlled by suitable dietetic measures, if the patient is willing to submit to them, that its duration is only limited by that of an ordinary life, while carelessness in this respect is apt to be followed by early grave consequences.
COMPLICATIONS.—The almost sole complication of diabetes mellitus is the tubercular phthisis which so often terminates it. Indeed, it is doubtful whether this complication should not be regarded as a consequence, as should also the boils, gangrenous processes, and ophthalmic conditions which have been mentioned under Symptomatology. Jaundice has occurred three times in my experience up to the present time. Senator says that when not an accidental complication due to a catarrh of the duodenum it may result from compression of the biliary capillaries by the overloaded blood-vessels and enlarged gland-cells of the liver. In one of my cases, in which jaundice appeared to be the initial symptom, but which disappeared some months before death, the autopsy revealed atrophy of the liver. It is well known that pancreatic disease, especially cancer, is apt to be accompanied by jaundice, and as pancreatic disease is often at the bottom of diabetes, it will similarly account for the jaundice, while the presence of jaundice may also suggest a pancreatic diabetes.
DIAGNOSIS, INCLUDING THE TESTS FOR SUGAR IN THE URINE.—The diagnosis of diabetes mellitus, the disease being once suspected, is easy. The passage of large amounts of pale urine of high specific gravity, the presence of thirst, dryness of the mouth, fauces, and skin, and progressive emaciation even while the appetite is good, can scarcely be misinterpreted. In the urine from such a case the application of any of the tests for sugar will produce prompt response. The urine is not always so much increased as to attract attention, while its color is also sometimes but slightly changed; but the symptoms of thirst and dryness or clamminess of the mouth are seldom wanting. On the other hand, the discovery of a glycosuria without these symptoms is, as a rule, accidental. It is a question how far such degrees of glycosuria as do not produce the usual symptoms of diabetes in an appreciable degree are signs of positive disease. At the same time, its detection is important, in that there is always danger of the simple glycosuria becoming a diabetes—a danger which its recognition and suitable treatment may avert. Accordingly, the urine of all persons having unusual appetites without evident cause, [p. 211]and of those who are fond of eating and drinking, should be tested for sugar. This should also be done for those who have passed through severe mental or physical strain, have suffered shock or concussion of the nervous system, blows upon the abdomen, etc.
Testing for Sugar.—Under the head of Diagnosis I prefer to include the testing for sugar, which requires some detailed consideration. Unless it be that the indigo test recently revived by George Oliver of London prove more delicate, that form of cupric test known as Fehling's solution is, with suitable precautions, all things considered, the most satisfactory for general use.
Fehling's volumetric solution, suitable for both qualitative and quantitative purposes, is made as follows: Dissolve 34.639 grams of pure crystallized cupric sulphate in about 200 cubic centimeters of distilled water; 173 grams of chemically pure crystallized neutral sodio-potassic tartrate and 80 grams of potassium hydrate in 500 or 600 c.c. of distilled water. To the latter add the copper solution slowly, and dilute the clear mixed fluid to 1 liter. One cubic centimeter of this solution will be decolorized by 0.005 grm. of sugar, or 200 grains will be decolorized by 1 grain of sugar. Or the copper may be dissolved in 1 liter of water, and the tartrate and potassium hydrate in another, and a cubic centimeter of each mixed at the moment they are to be used.
For qualitative testing, put a cubic centimeter of Fehling's solution into a test-tube (or if the copper and the alkaline sodio-potassium tartrate solutions are kept separate, a cubic centimeter of each), and dilute with distilled water to 5 c.c. Boil, and if, after the lapse of a couple of minutes, the solution remain unchanged, it is fit for testing. If it becomes turbid or a red sediment falls, it is spoiled, and a new solution should be obtained.38 A cubic centimeter of the suspected urine is then measured out and added drop by drop to the solution kept hot. If there is much sugar, the first drop will throw down a yellow precipitate of suboxide of copper, which becomes rapidly red. If no reaction takes place after adding the entire cubic centimeter of urine, the addition should be continued until 4 c.c. are added, when, if, after the mixture has cooled, there be no response, it may be concluded that the urine is free from sugar. By operating with a cubic centimeter of the test-fluid and the same quantity of urine or multiples thereof, we may roughly estimate the proportion of sugar. Thus, if the cubic centimeter of undiluted urine just decolorizes the cubic centimeter of Fehling's solution, sugar is present in the proportion of one-half of 1 per cent.; or if a half cubic centimeter of the urine removes all the color, the quantity is 1 per cent. If the urine is highly charged with sugar, it may be diluted, and the degree of dilution being remembered, a rough quantitative estimation may be similarly made.
38 Should this not be possible, a little more soda may be added and the fluid filtered, when it is again ready for use.
If the urine contains very minute quantities of sugar, the reaction is less satisfactory. The copper is reduced, but the suboxide is so small in quantity that it is obscured by the excess of copper solution, and a mixture results which is greenish or greenish-yellow or yellow or milky, and on standing a small yellow sediment falls to the bottom. Now, it dare not be said that it is sugar which produces such reaction. It may be [p. 212]sugar, but it may also be uric acid. Uric acid is really more frequently a source of error than is commonly supposed. I have myself seen the reaction due to it so vivid that I did not suspect it could be due to any reducing agent excepting sugar; but, noting the next day a copious sediment of uric acid which had fallen during the night, a testing of the supernatant fluid then revealed no reaction whatever. Such a urine, after being treated by the lead process to get rid of the uric acid, fails also to respond. But this process is very tedious,39 and cannot be conveniently carried out by the busy practitioner. The same thing is, however, accomplished by treating the urine with hydrochloric acid, which in twenty-four hours precipitates all of the uric acid. Simple precipitation by lead acetate solution and filtration does not answer, because all of the uric acid is not thus removed. Other substances, as hippuric acid, urates, hypoxanthin, etc., are said to act similarly, but they produce no practical interference with the test. On the other hand, a small amount of sugar may be present and yet fail to show the reaction, because the cuprous oxide is held in solution by certain substances. Such are ammonia and nitrogenous matters, including albumen, creatinin, pepsin, peptones, urinary coloring matters, etc. The latter probably produce their effect through the ammonia which is given off while heating them in the presence of an alkali. Hence all albumen should be precipitated and filtered out of urines suspected to contain sugar, and the heat applied should not be too great. Finally, excess of glucose will also hold in solution cuprous oxide, so that the suspected urine should not be added in too large a quantity at a time, but rather drop by drop.
39 The details of this process will be found in the writer's work on the Practical Examination of Urine, 5th ed., 1883, p. 63.
But qualitative testing is not sufficient during the treatment of a case of diabetes. The percentage of sugar and the quantity discharged in twenty-four hours should be determined occasionally. The process is done as follows: Place 10 cubic centimeters of Fehling's solution in a porcelain capsule, and dilute it with 40 c.c. of distilled water. Fill a Mohr's burette with the urine, which, if it contain more than 1 per cent. of sugar, should be diluted with nine times its bulk of distilled water. Slowly heat the contents of the capsule to boiling, and then allow a little of the diluted urine to run in from the burette; continue the cautious addition of urine and the gentle heating until the blue color is completely removed from the Fehling's solution. To determine the exact moment at which this takes place requires a little experience, but its recognition is facilitated by carefully tilting the capsule after each addition and stirring, so that its clear white surface may be seen through the edge of the fluid and contrasted with the latter. The number of cubic centimeters of urine used should now be read off from the burette, the number of c.c. of undiluted urine calculated therefrom, and each c.c. multiplied by .005 grm. The result indicates the quantity of sugar in grams in the urine employed, whence the percentage of sugar is determined, and also the twenty-four hours' quantity, the amount of urine passed in that period being known.
The Fermentation Test.—A very simple and easy method of determining the proportion of sugar is by Roberts's fermentation method, which, although not so precise as the volumetric process, is still [p. 213] sufficiently so for clinical purposes. A small piece of German yeast or a teaspoonful of liquid yeast is added to about four ounces (120 c.c.) of the urine, which is kept lightly stopped, at a temperature of 20° to 30° C. (68° to 80° F.), for about twelve hours; at the end of this time the sugar will have been converted into alcohol and carbonic acid. The latter will have passed off, and the urine lost in weight because of the destruction of sugar; while the difference between the specific gravity before and after the fermentation indicates the number of grains of sugar per fluidounce. Thus, suppose the specific gravity before fermentation to have been 1040, and afterward 1025; there will have been 15 grains of sugar to the fluidounce, whence, again, the twenty-four hours' quantity can be calculated. If the metric system is used, each degree of specific gravity lost will correspond to .2196 grams of sugar in every 100 c.c. of urine.
The specific gravity of the fermented urine should be compared with that of the urine soon after it is passed, because saccharine urine under suitable circumstances undergoes fermentation without the addition of yeast; and, the specific gravity being thus lowered spontaneously, the reduction in the urine fermented by yeast would appear less than it actually is. At the same time, care should be taken that the urine is of the same temperature when the specific gravity is taken before and after fermentation.
The Picric Acid and Potash Test.—Although attention was called in 1865 by C. D. Braun,40 a German chemist, to a reaction between grape-sugar and picric acid, as the result of which the latter is converted into picramic acid, very little attention seems to have been paid to this announcement. Quite ignorant of it, George Johnson rediscovered this reaction in 1882, and published it in 1883.41 It is applicable to both qualitative and quantitative purposes. In order to make use of it, a standard comparison-solution is made as follows: Take 1 fluidrachm of a solution of grape-sugar, 1 grain to the fluidounce; mix it in a long test-tube with half a drachm of liquor potassæ (U. S. P. or B. P.) and ten minims of a saturated solution of picric acid; dilute the mixture to 4 fluidrachms with distilled water, to facilitate which a tube used for the purpose may be marked at 4 fluidrachms. Raise the mixture to the boiling-point, and continue the boiling for sixty seconds, to ensure complete reaction between the sugar and picric acid. During the boiling the pale-yellow color of the liquid is changed to a vivid claret-red. Cool the liquid by cautiously immersing the tube in cold water, and if it is not then at the level of the 4-drachm mark, raise it to this by adding distilled water. The standard color thus obtained is that which results from the decomposition of picric acid by a grain of sugar to the ounce, four times diluted, or by a solution of sugar containing one-quarter of a grain per ounce. But the picramic solution rapidly becomes pale on exposure, so it becomes necessary to make a more permanent solution to use as a standard. This may be accomplished by combining liquor ferri perchloridi drachm j, liquor ammonii acetatis drachms iv, acidum aceticum (glacial) drachms iv, and water enough to make ounces iiss. The color of this is identical with that of the picric acid reduced by a one-grain solution diluted four times, and, [p. 214]according to Johnson, it will retain its color unchanged for at least six months. At the same time, whenever a new solution is made it should be compared with that of the one-quarter grain per ounce solution of sugar, boiled with picric acid and potash.
40 "Ueber die Umwandlung der Pikrinsaüre in Pikramminsaüre, und Ueber die Nachweisung der Traubenzucker," Zeitschrift für Chemie, 1865.
41 British Medical Journal, March, 1883.
For qualitative testing Johnson directs: To a drachm of urine in a test-tube add a few drops, enough to give a distinct yellow color, of a saturated solution of picric acid. Add about 10 drops of liquor potassæ and boil. If sugar is present, the mixture becomes promptly red in hue.
FIG. 5. |
Johnson's Picro-Saccharimeter. |
The shading of the side tube indicates the ferric-acetate standard. The darker shading at the bottom of the graduated tube shows the saccharine fluid, darkened by boiling with picric acid and potash, and occupying ten divisions between dilution. |
The quantitative estimation is based upon an accurate approximation, by dilution, of the color of the tested fluid with that of the standard solution. Johnson recommends the picro-saccharimeter figured in the text. This is a stoppered tube twelve inches long and three-quarters of an inch in diameter, graduated into ten, and each of these again into ten other equal divisions. By the side of this tube, and held in position by an S-shaped band of metal, is a stoppered tube of equal diameter and about six inches long, containing the standard solution corresponding to the reaction of the one grain of grape-sugar with picric acid and potash diluted four times.
It has been found that ten minims of a cold saturated solution of picric acid are rather more than sufficient for decomposition by one drachm of a solution of grape-sugar in the proportion of one grain to the ounce. A drachm of the solution will therefore contain one-eighth of a grain of sugar, which is the strength of the solution used in making the standard-color liquid. In making the analysis, while the quantity of liquor potassæ used is always the same and the dilution is always to four drachms, the picric acid must be added in proportion to the amount of sugar present, so that if the urine contains as much as six grains to the fluidounce, sixty drops or a fluidrachm of the picric-acid solution would have to be used; and when the proportion of sugar is higher than this, the urine should be diluted with distilled water five or ten times before commencing the analysis, and the degree of dilution remembered in the computation.
If, now, a drachm of a solution of grape-sugar, containing two grains to the ounce, be mixed with the same quantity of liquor potassæ and picric acid and increased by the addition of distilled water to four drachms in the boiling tube, and boiled as before for sixty seconds, the result will be a mixture of much darker color than will be produced by the one-grain solution; but if the dark liquid be diluted with its own volume of water, the color will be the same as that of the one-grain solution or the standard.
It is plain, then, that if a given quantity of the dark saccharine fluid produced by boiling—say, enough to cover ten divisions of the graduated tube, as shown in the figure—has to have added to it an equal bulk of distilled water in order to produce [p. 215]the color of the standard solution, the tested fluid will be of the strength of two grains to the ounce; if three times, three grains; and so on; while fractional additions, as indicated by the graduated markings, would show fractional additions to the proportion of sugar.42
42 A more exact comparison of the saccharine liquid with the standard is made by pouring into a flat-bottomed colorless tube six inches long and an inch in diameter as much of the standard solution as will form a column about an inch in height, and an exactly equal column of the saccharine fluid in a precisely similar tube. The operator then looks down through the two tubes at once, one being held in each hand, upon the surface of a white porcelain slab or piece of white paper. In this way slight differences of tint are easily recognized; and if the liquid to be analyzed is found darker than the standard, it is returned to the graduated tube and diluted until the two liquids are found to be identical in color, when the final reading is made.
The presence of albumen, even in considerable amount, has but little effect upon the test, nor does the coloring matter of normal urine, according to Johnson; but he says there is a coloring matter associated with ser-albumen in albuminous urine, and with egg-albumen as well, which has a reducing action on picric acid. This is partly separated by filtering off the precipitated albumen, and entirely removed by repeated filtration through animal charcoal. So, too, the albumen removed by coagulation and filtration, if thoroughly washed, does not give any red reaction if boiled with picric acid and potash diluted in the same proportion as when testing for sugar. Neither do any other unoxidized sulphur compounds found in urine decompose the picric acid and render the test fallacious.
Johnson and his son, G. Stillingfleet Johnson, claim that the picric-acid test is as accurate as any other, and that it is even more accurate than either Fehling's or Pavy's process, because the picric acid is not acted upon by uric acid or urates, which do reduce the oxide of copper. The method of analysis by the picro-saccharimeter, they claim, is at least as speedy and as easy as any other. The materials and apparatus required are easily prepared, inexpensive, and not, like Fehling's copper solution, liable to undergo rapid changes.
But while Johnson claims that neither coloring matters of normal urine nor uric acid reduce the picric acid, he admits that he has tested with picric acid and potash a large number of specimens of normal urine with the almost uniform result of a depth of color indicating the proportion of .6 of a grain of sugar to the fluidounce, the indication varying between the limits of .5 to .7 grain. The ammonio-cupric method used at the same time gave results of from .7 to .9 grain to the fluidounce, or an excess of .1 to .3 grain. Now, if my own views, the grounds for which are announced elsewhere,43 are correct, strictly normal urine contains no sugar, and any reducing action upon oxide of copper is due to uric acid, either picric acid is reduced to a degree by uric acid or by some other constituent of normal urine. This, in the light of Oliver's44 recent investigations, may be kreatinin. For he has shown that kreatinin strikes in a few seconds a red color with the cold alkaline picric solution, which is quickened by heat. From this it would seem that the exact value of the picric-acid test has as yet to be determined.
43 Tyson, Practical Examination of Urine, 4th ed., Philadelphia, 1884.
44 On Bedside Urine-Testing, including Qualitative Albumen and Sugar, by Geo. Oliver, M.D., London, Member of the Royal College of Physicians of Lond., etc., 2d ed., London, 1884.
[p. 216]The Indigo-Carmine Test.—The fact that indigotine, the coloring matter of commercial indigo, is converted into indigo when heated with an alkali in the presence of glucose and certain carbohydrates, has recently been applied by George Oliver of London in the construction of a test-paper. Carmine of indigo is the sulph-indigotate of sodium, an intensely blue salt, soluble in 120 parts of water. Sulph-indigotic acid is made by heating indigo with sulphuric acid, and when combined with a base, sodium, produces indigo-carmine. When sodium carbonate is mixed with a solution of indigo-carmine, the latter is precipitated in a minute state of division, but is redissolved on heating, when there results a greenish-blue solution. A freshly-made mixture of the indigo solution and sodium carbonate furnishes a fluid not unlike Fehling's solution, which gives the reaction to be described with glucose. Unfortunately, such a mixture will not keep, and the reagent would be useless but for the happy idea of Oliver of making the test-paper. In doing this bibulous paper is immersed in a solution of indigo-carmine with carbonate of sodium.45 The paper is then cut into strips an inch long and one-quarter of an inch wide.
45 No more precise directions than this are given by Oliver, either in his papers in the Lancet for 1883 or in his little book just published, On Bedside Urine-Testing. The sugar test-papers, as well as the entire series of albumen test-papers, suggested by Oliver, are now made by Parke, Davis & Co. of New York, and by Wilson & Son, Harrogate, London.
Mode of Testing.—One of the test-papers and a sodium carbonate paper46 are dropped into a half-inch test-tube, and water added until the upper end is just covered; a column of fluid one inch in height and half an inch in diameter will thus be produced, so that the solution of carmine obtained on boiling will always acquire the same concentration. Heat is now applied, the tube being gently shaken, and boiling kept up for a second or two. A beautiful blue solution will result. The test-paper may now be removed or allowed to remain.
46 Test-papers of the same size, charged with a saturated solution of sodium carbonate.
Not more than one drop of the suspected urine is let fall into the tube from a pipette held in an upright position. Drops of equal size are thus secured. The contents of the tube are again freely boiled for a few seconds, after which the tube should be raised an inch or more from the flame and held without shaking, while the solution is kept quite hot, but not boiling, for exactly one minute. If glucose be present in abnormal amount, the soft rich blue will be seen first of all to darken into violet; then, according to the quantity of sugar, there will appear in succession, purple, red, reddish-yellow, and finally straw-yellow. When the last-named color has been developed the slightest shaking of the tube will cause red streaks to fall from the surface and mingle with the pale yellowness of the solution, while further agitation will cause the return of purple and violet and the restoration of the original blue.
The time required for the commencement of the reaction after the boiling of the test liquid is in inverse proportion to the amount of glucose present. When the latter is large, over 20 grains to the ounce, it will be but a few seconds; but when small, 2 or 3 grains, from thirty to sixty seconds may elapse. If the urine do not contain more than the normal amount of sugar47—i.e. under half a grain to the ounce—the color of the solution [p. 217]at the end of the heating for one minute will be unchanged. The test is available by artificial light as well as by daylight.
47 It will be noted from this that Oliver accepts the view that there is a small amount of sugar in normal urine.
Precautions.—1. Care should be taken during the testing not to shake the tube or to permit free ebullition. 2. While keeping the contents of the tube hot, the latter should not be held up between the eye and the sky, for then the early color-changes will probably escape observation. The tube should be kept below the eye-level and its contents viewed by the reflected light of some bright object, such as a sheet of white paper propped up an inch or two beyond the tube as a background. 3. Oliver is not aware that the presence of earthy carbonates will prevent the carmine reaction, but as a precautionary measure he suggests the use of a soda-paper whenever the water is exceptionally hard. 4. The acids of the urine rob the carmine-paper of much alkali, so that the addition of more than a certain number of drops of urine—varying of course with the degree of acidity—will at first retard and then prevent the reaction. The addition of the soda-paper will prevent any such interference, although Oliver says that by invariably submitting only one drop of saccharine urine to the test-paper, and keeping up the heating for not less than two minutes, he has never failed to obtain the characteristic reaction without using a soda-paper. It is well to remember, however, that an excessively acid urine may thus interfere, and that the soda-paper will prevent it. 5. The blue color of the carmine is discharged by caustic alkali—liquor potassæ or sodæ. The only chance of being misled by this reaction lies in using an imperfectly cleansed test-tube which may have contained Fehling's solution or the alkaline picric solution. The caustic alkali converts the blue carmine into a green solution, which, on heating, disappears; nor does it return by again shaking the contents of the tube.
Critical comparison of this test with Fehling's solution and picric acid by Oliver has shown that of sixty-four substances experimented upon, normal and abnormal constituents of urine or medicines which after ingestion are eliminated in the urine, Fehling's was reduced by fifteen, picric acid by eleven, and indigo-carmine by eight. The only substances producing the characteristic play of colors with indigo-carmine test-papers reacted with both picric acid and Fehling's solution. They were unoxidized phosphorus, ammonium sulphide, milk-sugar, dextrin, inosit, gallic acid, tannic acid, and iron sulphate. Both the carmine and picric acid were reduced by inosit, which merely turned Fehling's solution green. On the other hand, uric acid and urates, which reduce Fehling's solution, do not react with the carmine test, while kreatinin, which reacts with picric acid also, does not respond to the carmine. Albumen, if abundant, interferes with Fehling, but not with the indigo-carmine.
Detection of Inosit.—It has been said that inosit sometimes accompanies, and even substitutes, grape-sugar in the course of diabetes. It has been mentioned that it does not reduce Fehling's solution, but turns it olive-green. It reduces the carmine and alkaline picric acid solution, and is therefore not recognizable by these. The methods recommended for its recognition in the books are troublesome, and as its presence in the absence of sugar indicates a favorable change, it is not likely that a more precise recognition than is furnished by the olive-green reaction will be needed for clinical purposes.
PROGNOSIS.—The prognosis in diabetes depends upon the organ whose [p. 218]involvement is responsible for the symptoms, upon the stage at which the condition comes under observation, and upon the age of the patient. It has appeared to me that the cases of diabetes depending upon pancreatic disease are the most intractable, that their progress is scarcely checked by treatment, and that they are comparatively rapidly fatal in their termination. In the others, where the symptom is one of a central nervous lesion, it has always seemed to me to be of secondary importance that the glycosuria is itself less marked, that it is unattended by the other distinctive symptoms of diabetes, and that its issue is that of the nervous malady.
Again, it is well known that the later in life diabetes occurs the more amenable it is to treatment, and that if a proper diabetic diet be adhered to by the patient his life need scarcely be shortened. On the other hand, diabetes mellitus is a disease in which the expectant plan is dangerous. If it does not improve it usually gets worse; and many a patient has fallen a victim to his own indifference and indisposition to adhere to a regimen under which he could have lived his natural term of life. This is especially the case when the disease appears after middle life.
If, on the other hand, the condition becomes thoroughly established before twenty-five years of age, it is less amenable to treatment; but even in such cases a promptly vigorous treatment is sometimes followed by recovery. I have already mentioned the case of a child twelve years old in which complete recovery took place.
If tubercular phthisis supervenes, recovery is not to be expected, while intercurrent disease, as pneumonia, which is rather prone to occur, is very much more serious and apt to terminate fatally.
TREATMENT.—The treatment of the aggregate of symptoms known as diabetes mellitus is conveniently divided into the dietetic, the medicinal, and the hygienic, of which the first is by far the most important. The efficiency of this treatment depends upon the successful elimination from the diet of all articles containing grape-sugar, cane-sugar, beetroot-sugar, and starch, it being universally recognized that in the early stages of the disease these foods are the sole source of the glucose in the urine. The normal assimilative action of the liver, by which the carbohydrates are first stored up as glycogen, and then gradually given out as glucose or maltose to be oxidized, being deranged, such foods not only become useless as aliments, but if continued seem to aggravate the glycosuria, and the excretion of sugar steadily increases. There is, therefore, a double reason for excluding them from the food. This is easiest accomplished by an exclusive milk diet. The exclusive milk treatment of diabetes was suggested by A. Scott Donkin in 1868. That he is correct in his assertion that in the early stages of diabetes lactin or sugar of milk is quite assimilable, and does not in the slightest degree contribute to the production of glycosuria, I cannot doubt; that it is in this respect even superior to casein, as claimed by Donkin, I am not prepared to state from actual knowledge; but that casein itself resists the sugar-forming progress immeasurably greater than any other albuminous substance, so that in all but the most sure and advanced or complicated cases its arrest is complete, I am also satisfied. Certain it is that in a large number of diabetics the use of a pure skim-milk regimen results in a total disappearance of the sugar from the urine. That in a certain proportion of these cases a [p. 219]gradual substitution of the articles of a mixed diet may be resumed without a return of the symptoms is also true. In other more confirmed cases the use of skim-milk results in a decided reduction in the amount of sugar, with an abatement of other symptoms, which continues as long as the diet is rigidly observed. In still other cases, while the skim-milk treatment makes a decided impression upon the quantity of sugar, it still remains present in considerable amount, while the disease progresses gradually to an unfavorable issue. These three classes of cases represent, ordinarily, different stages of the disease, so that it may be said that as a rule cases recognized sufficiently early may be successfully treated with skim-milk, although it may occasionally happen that cases pursue a downward course from the very beginning despite all treatment. Yet I have never seen a case which, when taken in hand when a few grains of sugar only to the ounce were present, failed to yield to this treatment.
As to the method of administration, my practice with adults is to give eight ounces (an ordinary tumblerful) every two hours, beginning at seven or eight o'clock in the morning, and continuing to the same hour in the evening. Sometimes it is well to begin with half as much at first, but rapidly to increase to the required amount. This method ensures the ingestion of three to four quarts daily—a quantity generally sufficient to maintain the body-weight of an adult person of average size and taking moderate exercise, although a slight reduction may take place at first. But if the individual is very active or of large size, it will not be found sufficient. In such event the quantity must be increased as demanded by a feeling of unsatisfied hunger. I have known fourteen pints to be taken in twenty-four hours. But when the quantity becomes thus large, the inconvenience in ingesting it is very great, and it is much more convenient to coagulate the casein of a part of the milk and use the curd thus obtained, while the second part is drunk. Curd may be seasoned with salt to make it more palatable, and should be thoroughly masticated before it is swallowed.
The milk should not be taken too cold, especially if the amount ingested is large, else it is likely to reduce the temperature of the stomach below the point necessary for gastric digestion. The temperature should not be less than 60° F., nor much over 100°. Something depends upon the idiosyncrasies of the patient, which must be the guide as to temperatures intermediate between those named.
The chief advantage of the skim-milk over the unskimmed is simply that it is more easy of digestion. Many persons who cannot take unskimmed milk for any length of time without its deranging the digestion, or, as is commonly said, making them bilious, can take with impunity milk from which the cream is removed. Although Salomon48 claims to have shown that glycogen is produced in the liver of rabbits fed upon pure olive oil, it is at least probable that fat is among the last of the substances undergoing this conversion, and in ordinary cases of diabetes it is rather its indigestible nature which renders it prudent to remove from milk the greater proportion of fat by skimming it off.
48 Virchow's Archiv, Bd. 61, Heft 3, 1874, 18.
Still more easily assimilable is the peptonized milk, in which the casein is at least partially digested, and it should be employed where there is any [p. 220]difficulty in the way of using the ordinary milks. Either skimmed or unskimmed milk may be used for peptonizing, the latter peptonized being quite as easy of digestion as the former unpeptonized. I have found the extractum pancreatis of Fairchild Brothers & Foster most successful in the peptonizing of milk, and according to the following directions: Into a clean quart bottle put 5 grains of extractum pancreatis, 15 of bicarbonate of sodium, and a gill of cool water; shake, and add a pint of fresh cool milk. Place the bottle in a pitcher of hot water or set the bottle aside in a warm place, usually for three-quarters of an hour. When the milk has acquired a slightly bitter taste, it has been completely peptonized—that is, the casein has been completely converted into peptone. After the process is complete the milk must be immediately put on ice.
It is not always necessary to completely peptonize the milk, and if the bitter taste is unpleasant the process may be stopped short of this by putting the milk on ice, the degree of digestion depending upon the length of time the milk is kept warm.
While I am confident that the promptest and most effectual method of eliminating sugar from the urine is by a milk diet, it occasionally happens that a patient cannot or will not submit to so strict a regimen. In other instances, again, it is not necessary to resort to it, because a less restricted diet answers every purpose.
A suitable diabetic diet would also be obtained by eliminating from the bill of fare all saccharine and amylaceous and other sugar-producing substances. Such a diet is, strictly speaking, impossible. For, apart from the fact just mentioned that even fats, as well as albuminous substances to a degree, are capable of producing glycogen, the monotony of a pure meat diet soon becomes unbearable, to say nothing of other derangements it may produce. Fortunately, it is not necessary that such an exclusive diet should be maintained, for certain saccharine foods seem capable of resisting the conversion into sugar more than others. Sugar of milk, or lactin, has already been mentioned as one of these, and to it may be added the sugar of some fruits, and probably also inosit or muscle-sugar, mannite or sugar of manna, and inulin, a starchy principle abundant in Iceland moss. It is found also that there are many vegetable substances containing small quantities of sugar and sugar-producing principles which may be used with impunity in at least the milder forms of diabetes. This being the case, a bill of fare for diabetics may be constructed quite liberal enough to satisfy the palate of most reasonable persons by whom it is attainable.
FOOD AND DRINK ADMISSIBLE.—Shell-fish.—Oysters and clams, raw and cooked in any way, without the addition of flour.
Fish of all kinds, fresh or salted, including lobsters, crabs, sardines, and other fish in oil.
Meats of every variety except livers, including beef, mutton, chipped dried beef, tripe, ham, tongue, bacon, and sausages; also poultry and game of all kinds, with which, however, sweetened jellies and sauces should not be used.
Soup.—All made without flour, rice, vermicelli, or other starchy substances, or without the vegetables named below as inadmissible. Animal soups not thickened with flour, beef-tea, and broths.
Vegetables.—Cabbage, cauliflower, brussels-sprouts, broccoli, green [p. 221]string-beans, the green ends of asparagus, spinach, dandelion, mushrooms, lettuce, endive, coldslaw, olives, cucumbers fresh or pickled, radishes, young onions, water-cresses, mustard and cress, turnip-tops, celery-tops, or any other green vegetables.
Fruits.—Cranberries, plums, cherries, gooseberries, red currants, strawberries, apples, without sugar. Or they may be stewed with the addition of bicarbonate of sodium instead of sugar. (See below.)
Bread and cakes made of gluten, bran, or almond flour, or inulin, with or without eggs and butter. Griddle-cakes, pancakes, biscuit, porridges, etc. made of these flours. Where especial stringency is required these should be altogether omitted.
Eggs in any quantity and prepared in all possible ways, without sugar or ordinary flours.
Nuts.—All except chestnuts, including almonds, walnuts, Brazil-nuts, hazel-nuts, filberts, pecan-nuts, butternuts, cocoanuts.
Condiments.—Salt, vinegar, and pepper in moderate quantities.
Jellies.—None except those unsweetened. They may be made of calf's-foot or gelatin and flavored with wine.
Drinks.—Coffee, tea, and cocoa-nibs, with milk or cream, but without sugar; also milk, cream, soda- (carbonated) water, and all mineral waters freely; acid wines, including claret, Rhine, and still Moselle wines, very dry sherry; unsweetened brandy, whiskey, and gin. No malt liquors, except those ales and beers which have been long bottled, and in which the sugar has all been converted into carbonic acid and alcohol.
Vegetables to be especially Avoided.—Potatoes, white and sweet, rice, beets, carrots, turnips, parsnips, peas, and beans; all vegetables containing starch or sugar in any quantity.
The following list, including essentially the same articles, but arranged in the shape of a true bill of fare, by Austin Flint, Jr.,49 will be found very convenient:
BILL OF FARE FOR DIABETES.—Breakfast.—Oysters stewed, without flour; clams stewed, without flour. Beefsteak, beefsteak with fried onions, broiled chicken, mutton or lamb chops; kidneys, broiled, stewed, or devilled; tripe, pigs' feet, game, ham, bacon, devilled turkey or chicken, sausage, corned-beef hash without potato, minced beef, turkey, chicken, or game with poached eggs. All kinds of fish, fish-roe, fish-balls, without potato. Eggs cooked in any way except with flour or sugar, scrambled eggs with chipped smoked beef, picked salt codfish with eggs, omelets plain or with ham, with smoked beef, kidneys, asparagus-points, fine herbs, parsley, truffles, or mushrooms. Radishes, cucumbers, water-cresses, butter, pot-cheese. Tea or coffee, with a little cream and no sugar. (Glycerin may be used instead of sugar if desired.) Light red wine for those who are in the habit of taking wine at breakfast.
Lunch or Tea.—Oysters or clams cooked in any way except with flour; chicken, lobster, or any kind of salad except potato; fish of all kinds; chops, steaks, ham, tongue, eggs, crabs, or any kind of meat; head-cheese. Red wine, dry sherry, or Bass's ale.
[p. 222]Dinner.—Raw oysters, raw clams.
Soups.—Consommé of beef, of veal, of chicken, or of turtle; consommé with asparagus-points; consommé with okra, ox-tail, turtle, terrapin, oyster, or clam, without flour; chowder, without potatoes, mock turtle, mullagatawny, tomato, gumbo filet.
Fish, etc.—All kinds of fish, lobsters, oysters, clams, terrapin, shrimps, crawfish, hard-shell crabs, soft-shell crabs, (No sauces containing flour.)
Relishes.—Pickles, radishes, celery, sardines, anchovies, olives.
Meats.—All kinds of meat cooked in any way except with flour; all kinds of poultry without dressings containing bread or flour; calf's head, kidneys, sweetbreads, lamb-fries, ham, tongue; all kinds of game; veal, fowl, sweetbreads, etc., with curry, but not thickened with flour. (No liver.)
Vegetables.—Truffles, lettuce, romaine, chicory, endive, cucumbers, spinach, sorrel, beet-tops, cauliflower, cabbage, brussels-sprouts, dandelions, tomatoes, radishes, oyster-plant, celery, onions, string-beans, water-cresses, asparagus, artichoke, Jerusalem artichokes, parsley, mushrooms, all kinds of herbs.
Substitutes for Sweets.—Peaches preserved in brandy without sugar; wine-jelly without sugar, gelée au kirsch without sugar, omelette au rhum without sugar; omelette à la vanille without sugar; gelée au rhum without sugar; gelée au café without sugar.
Miscellaneous.—Butter, cheese of all kinds, eggs cooked in all ways except with flour or sugar, sauces without sugar or flour. Almonds, hazel-nuts, walnuts, cocoanuts. Tea or coffee with a little cream and without sugar. (Glycerin may be used instead of sugar if desired.) Moderately palatable ice-creams and wine-jellies may be made, sweetened with pure glycerin; but although these may be quite satisfactory for a time, they soon become distasteful.
Alcoholic Beverages.—Claret, burgundy, dry sherry, Bass's ale or bitter beer. (No sweet wines.)
Prohibited.—Ordinary bread; cake, etc. made with flour or sugar; desserts made with flour or sugar; vegetables, except those mentioned above; sweet fruits.
49 "On the Treatment of Diabetes Mellitus," a paper read before the American Medical Association at its meeting in Washington, May, 1884, and published in the Journal of the association July 12, 1884. I have so far modified the bill of fare as to permit the use of milk, which Flint excludes.
One of the foods the omission of which is most illy borne by the diabetic, however great his previous indifference to it, is wheaten bread, while the substitutes which have been at different times suggested for it very imperfectly supply its place. Perhaps the best known of these is the bread made of gluten flour. It was suggested by Bouchardat in 1841, and is made by washing the ordinary wheat flour to free it from starch.50
50 The Health Food Company, of 74 Fourth Avenue, N.Y., prepare a gluten flour by first removing the five bran-coats, pulverizing the cleaned berry by the cold-blast process, stirring the powder into iced water, and precipitating the gluten, cellulose, and mineral matters, siphoning off the water holding in suspension the starch, and drying out the precipitate. In this manner the salts of the wheat are retained. A purified gluten made by the Health Food Company is deprived of the cellulose walls of the cells in which the gluten granules are held. Directions for making gluten bread and cakes of various kinds are furnished by the company on application.
Gluten flour, however prepared, contains some starch, as indeed it must if bread is to be made out of it; and I confess to having been a good deal disappointed in its use. I have known the sugar absent in a [p. 223]selected diet to return when gluten bread was permitted, and again disappear on its withdrawal. Of course gluten flour contains less starch than the ordinary wheat flour, and there may be cases where the starch in the former can be assimilated when the quantity in the latter cannot be. The gluten may be made into porridge.51
51 Gluten porridge is made by stirring the gluten into boiling water until thick enough, and then keeping up the boiling process for fifteen minutes. A little salt and butter are added at the close to improve the flavor, and it may be eaten with milk or cream.
A method of getting rid of the starch and sugar in bread, suggested by Liebig and tried by Vogel, consists in converting the starch into sugar by the action of diastase and dissolving out the sugar thus produced. This is accomplished by treating thin slices of bread with an infusion of malt. The bread is then washed, dried, and slightly toasted.
Another substitute for wheaten flour is the bran flour whence the starch is removed by washing.52 The bran itself, according to Parkes,53 sometimes contains as much as 15 per cent. of nitrogenous matter, 3.5 per cent. of fats, and 5.7 per cent. of salts. It is therefore not wholly innutritious, although the salts are washed out in removing the starch. It is considered especially useful when there is constipation, the slightly irritant properties of the bran aiding in maintaining a proper peristalsis and action of the bowels. These irritant properties are, however, inversely as the degree of comminution. The bran flour may be made with milk and eggs into a variety of cakes, of which the best known are those made according to Camplin's directions.54
52 A very carefully prepared bran flour, as well as a wheat-gluten flour, is prepared by John W. Sheddon, pharmacist, corner of Broadway and Thirty-fourth street, New York City.
53 Practical Hygiene, 5th ed., Philadelphia, 1878, p. 222.
54 The following are Camplin's directions for making biscuit of bran flour: To one quarter of a pound of flour add three or four fresh eggs, one and a half ounces of butter, and half a pint of milk; mix the eggs with a little of the milk, and warm the butter with the other portion; then stir the whole together well; add a little nutmeg or ginger or other agreeable flavoring, and bake in small forms or patterns. The cake, when baked, should be about the thickness of an ordinary captain's biscuit. The pans must be well buttered. Bake in rather a quick oven for half an hour. These cakes or biscuits may be eaten by the diabetic with meat or cheese for breakfast, dinner, or supper; at tea they require rather a free allowance of butter, or they may be eaten with curd or any soft cheese.
Where extreme restriction of diet is not required the ordinary bran bread of the bakers may be used. The unbolted flour of which this is made of course contains the starchy principles, but in consequence of the retention of the bran the proportion of starch is less. The cold-blast flour of the Health Food Company is said to contain the nutritious, but not the innutritious, parts of the bran.55
55 It is made by pulverizing the carefully cleaned wheat by a compressed, cold air blast, which strikes the wheat and dashes it to atoms.
The almond food suggested by Pavy is another substitute for bread. The almond is composed of 54 per cent. of oil, 24 per cent. of nitrogenized matter known as emulsin, 6 per cent. of sugar, and 3 per cent. of gum, but no starch enters into its composition. Theoretically, therefore, the food should be everything that can be desired if the gum and sugar can be removed. The latter is done by treating the powdered almonds with boiling water slightly acidulated with tartaric acid, or soaking the almonds in a boiling acidulated liquid which may form a part of the process for blanching. The boiling and acid are necessary to precipitate [p. 224]the emulsin, which would otherwise emulsify the oil of the almond. Pavy speaks well of biscuit made of almond flour and eggs, which he says go very well with a little sherry or other wine, although he admits they are found too rich by some for ordinary consumption. One person only under my observation has used the almond food, and found it unpalatable.
Seegen recommends an almond food made as follows: Beat a quarter of a pound of blanched sweet almonds in a stone mortar for about three-quarters of an hour, making the flour as fine as possible; put the flour thus obtained into a linen bag, which is then immersed for an hour and a quarter in boiling water acidulated with a few drops of vinegar. The mass is thoroughly mixed with three ounces of butter and two eggs; the yolks of three eggs and a little salt are added, and the whole is to be stirred briskly for a long time. A fine froth made by beating the white of the three eggs is added. The whole paste is now put into a form smeared with melted butter and baked by a gentle fire.
Biscuits made of inulin, the starchy principle largely contained in Iceland moss, were suggested by Kuelz. Although a starch, it is one of the assimilable ones alluded to, of which small quantities at least may be taken as food without appearing in the urine as sugar. The biscuits are made with the addition of milk, eggs, and salt, and are inexpensive.
To some persons sugar is almost as imperative a necessity as bread, although to many it is not a very great sacrifice to omit it from ordinary cooking, if not from tea and coffee. For the latter it is just as well to dispense with sugar altogether. But where patients feel that they must have some substitute for sugar, glycerin has been suggested for this purpose, at least for sweetening tea and coffee. But Pavy has noted56 that under the use of glycerin the urine increased from three and three and three-fourth pints to between five and six pints, and the sugar from 1100 grains to 3000 grains per diem, in the course of three days. Its withdrawal was followed by a prompt fall in both the urine and sugar, a return to it by a second increase, and subsequent withdrawal by another decline. Along with the increase of urine and sugar came also more thirst and discomfort. An examination of the chemical composition of glycerin would seem to confirm these results of experience. Glycerin is represented by C3H8O3, sugar by C6H12O3, and glycogen by C6H10O5; whence it is evident that a conversion of glycerin into sugar may take place in the liver. These facts seem to show conclusively that glycerin is no suitable substitute for sugar. I therefore do not use it.
56 On Diabetes, London, 1869, p. 259.
From what has been said it may be inferred that sugar of milk, mannite, and lævulose, or fruit-sugar, are admissible where sugar is demanded. They may be tried, but the urine should be carefully examined under their use, and if glycosuria occur or be increased they should be promptly omitted.
Almost every purpose of sugar in the cooking of acid vegetables is served by bicarbonate of sodium or potassium. As much bicarbonate of potassium to the pound as will lie upon a quarter of a dollar will neutralize the acidity of most fruits which require a large amount of sugar to mask this property. In this manner cranberries, plums, cherries, gooseberries, red currants, strawberries, apples, peaches, and indeed [p. 225]all fruits to which sugar is usually added in the cooking, become available to the diabetic.
In the matter of drinks, where the patient is not on a skim-milk diet, which usually affords as much liquid as is required by the economy, little restraint need be placed upon the consumption of water, which is demanded to replace that secreted with the sugar. Instead of water, Apollinaris water, Vichy, or the ordinary carbonated water may be used if preferred, and to many they are much more refreshing by reason of the carbonic acid they hold in suspension. Apollinaris water is particularly so, and one of my patients, who recovered completely under a suitable selected diet with which this mineral water was permitted, insists that it was that which cured her.
Where a simple selected diet is adopted, tea and coffee without sugar are usually permitted. The propriety of the substitutes for sugar already referred to must be determined by circumstances.
Of distilled and fermented liquors, moderate quantities of whiskey and brandy, dry sherry and madeira, the acid German and French wines—in fact, any non-saccharine wines—may be permitted. A medical friend who reports himself about cured of diabetes writes me that he has consumed eighty gallons of Rhine wine since he began to adhere closely to a diabetic diet. On the other hand, the free use of the stronger alcoholic drinks has been charged with causing diabetes, and I have known such use to produce a recurrence of sugar. No malt liquors, except those in which the sugar has been completely converted into carbonic acid and alcohol, should be used. Bass's ale may be allowed where no especial stringency is required.
HYGIENIC TREATMENT.—The patient should be surrounded by the most favorable hygienic influences. He should sleep in well-ventilated rooms; pass much time in the open air; bathe regularly, but not in water that is very cold, and especially the body should not be long submerged in cold water, as the liver must share the general internal hyperæmia incident to prolonged cooling of the skin, and increased glycosuria may result. I have known sugar to reappear after a prolonged drenching of the skin of patients overtaken by a rainstorm. Perhaps the most suitable time for the hot or tepid bath is on retiring in winter, but in summer it may be taken on rising. Thorough friction of the entire body should be practised after the bath or independently of it. An ounce or two of sodium carbonate may be added to it with advantage, as it softens the skin and facilitates the removal of the effete epithelium. The bowels should be kept regularly open, as the effect of their confinement is to produce torpor and congestion of the liver.
Certain natural mineral waters have always enjoyed a reputation for the cure of diabetes, and notably those of Vichy and Carlsbad. The former is an alkaline water with a slight laxative tendency, and the latter a decided aperient alkaline-saline water; and it is not unlikely that they owe a part of their good effects to an action upon the liver and upper bowel. This seems the more likely because Carlsbad, which enjoys the highest reputation, contains a far larger proportion of chlorides and sulphates, which are purgative. Vichy water contains 35 grains of carbonates to the pint, and Carlsbad 11, but the latter contains twice the proportion of chlorides, or 8 grains to the pint, and ten times as much sodium [p. 226]sulphate, or 19 grains to the pint. They may be used as adjuvants to the treatment, a pint of Vichy or half as much Carlsbad in the morning. Being imported waters, they are comparatively expensive, and I know of no American waters which closely approach them in composition.
Of American waters, the Saratoga Vichy contains twice as much chlorides as the Carlsbad, 17.7 grains to the pint, but no sulphates. It contains about the same amount of carbonates as Vichy. It is therefore a saline-alkaline water, and may be expected to serve the purposes of Vichy and some of those of Carlsbad, for which it may be substituted. Most of the American mineral waters vaunted as useful in diabetes will be found, on comparison with these waters, to be chemically indifferent, and therefore about as useful as so much ordinary spring-water. Of the Crab Orchard Springs in Kentucky, the Sowder's spring contains 25 grains of sulphate of sodium and magnesium and 7 grains of sodium chloride to the pint, therefore about the same proportion of the two substances combined as Carlsbad; yet I am not aware that these waters have any reputation in diabetes. The waters of Bedford Springs, Pennsylvania, also approximate them in the proportion of sulphates of sodium and magnesium.
Other Saratoga waters have an undoubted action on the liver through their chlorides, and may be used in lieu of the European waters above referred to, and of the Saratoga Vichy, when these cannot be obtained; such are the Geyser spring, which contains 70 grains of chlorides to the pint, and the Hathorn, containing 63 grains.
MEDICINAL TREATMENT.—While the dietetic treatment, and especially the skim-milk treatment, of diabetes mellitus is much to be preferred for its results over an exclusively medicinal treatment, and is of itself sufficient to control, if not to cure, a large number of cases, yet instances arise in which it is insufficient to complete the removal of sugar from the urine, and there are others in which it is impossible for various causes to carry out such treatment.
In my book on Bright's Disease and Diabetes, published three years ago, I gave the preference of drugs to ergot; but since then extended opportunities have convinced me that codeia is a far more efficient remedy. Repeated comparative trials of this drug in the wards of the Philadelphia Hospital and elsewhere have satisfied me of this. The trials have been made while the patients were upon a mixed diet, which I hold to be the only fair way of arriving at a knowledge of the true value of a drug in the disease. Codeia was first suggested by Pavy in lieu of opium and morphia, which had long been used, his reason being that it did not produce the same narcotic effect. Favorable reports upon its use have been made by Foster, Image, Brunton, R. Shingleton Smith, Cavafy, Austin Flint, Sr., Harvey L. Byrd, and others. It may be given in pill or solution. One should begin with ¼ of a grain three times a day, increasing ¼ of a grain daily until the sugar disappears or the remedy ceases to have any effect, or until drowsiness is produced. Thus gradually increasing, I have reached as high as 47 grains in a day. Cavafy has given 15 grains three times daily.
Opium—which is said to have been used by Aetius for this disease—or morphia might be used if codeia cannot be obtained, but they are less efficient, more dangerous, and more apt to produce the troublesome [p. 227]symptom of constipation. MacGregor57 gave in one case 60 grains of opium, and in another 90 grains, in the twenty-four hours.
57 London Medical Gazette, 1837.
While I have seen the most striking results upon the quantity both of sugar and urine during the administration of codeia, and at the same time have noted a gain in flesh and strength, I cannot say that I have ever seen a case totally recover under its use. Such cases are, however, reported by others. I have always used it in the very worst cases, where dietetic measures had also failed to remove the sugar. As to the mode of action of codeine, we can only speculate. It may be said that it quiets the irritation of the vaso-motor centre, whence result the glycosuria and other symptoms of diabetes.
Next to codeine in efficiency, of drugs, is ergot. The favorable results of its use are more easily explained by its physiological action—contraction upon the muscular walls of blood-vessels—than those of codeine, but it is not so efficient a remedy. It may be used by beginning with half a drachm, and increasing to a drachm, four times a day. Larger doses than this, as much as half an ounce four times a day, have been given, but the stomach rarely permits their continuation for any length of time.
Bromide of potassium, an old remedy for diabetes, has recently been revived and much lauded by the French physicians, but I have never found any results from its use. I can understand, however, how in certain cases of nervous origin it may be useful.
Comparatively recently, Clemens of Frankfort-on-the-Main has recommended the use of what he terms brom-arsen or bromide of arsenic. The dose is one-forty-eighth of a grain three times a day, gradually increased by this same amount until one-sixth or one-fifth of a grain is given daily.58 Clemens, however, unites with its use a dietetic treatment. I have used it in connection with an unselected diet, and have not found the results claimed by Clemens. It is, however, both tonic and sedative, and as such is to be recommended in conjunction with other measures.
58 The late Mr. R. F. Fairthorne, with Mr. James T. Shinn, apothecary, cor. Broad and Spruce streets, Philadelphia, prepared for me a solution of bromide of arsenic in the following manner: 77 grains of metallic arsenic in powder are added in small portions to 240 grains of bromine, the latter being placed in a long test-tube immersed in ice-water to control the otherwise violent reaction. One hundred grains of the tribromide thus obtained are dissolved in sufficient distilled water to make ten fluidounces. One minim will then contain one-forty-eighth of a grain.
Arsenic itself has some reputation in the treatment of diabetes, based upon the observation of Salkowsky that glycogen diminishes in the livers of animals poisoned with arsenic. It is at least a good adjuvant tonic. Leube gave it in diabetes in doses of one-third of a grain three times a day.
Strychnia is also very useful as a tonic, and may be used either alone or in the shape of the sulphate, or combined with arsenic and iron, or it may be given, perhaps preferably, in solution in combination with an acid. Given in combination with phosphoric acid, I believe it the most valuable tonic available in this disease.
To supply the phosphates, in which gluten bread is deficient, as well as for their tonic effect, the various preparations of phosphates are useful. [p. 228]The well-known compound syrup of the phosphates, or Parrish's chemical food, may be considered a type of these preparations. Every fluidrachm, which is a dose, contains 2½ grains of calcium phosphate, 1 grain of ferric phosphate, with fractions of a grain of sodium and potassium phosphate with free phosphoric acid. Similar is the solution of phosphates and phosphoric acid59 known in this country as solution of phosphoric acid with iron, or the latter may be omitted.
59 Rx. | Calcii phosphat. | gr. iij; |
Magnesii phosphat. | gr. ss; | |
Potassii phosphat. | gr. iv; | |
Ferri phosphat. | gr. ss; | |
Ac. phosphoric | minim vj; | |
Aquæ q. s. ad. | fluidrachm i, which is a dose. |
Iodide of potassium has been used in some cases with satisfactory results, and may be expected to be useful where syphilitic disease of the nervous system is suspected.
Seegen has seen sugar disappear from the urine under a dosage of 20 to 30 drops of tincture of iodine daily, but the sugar reappeared after the remedy was discontinued.
Lactic acid was recommended by Cantani on theoretical grounds as a substitute for sugar. He supposes that in health the sugar ingested is converted by the liver into lactic acid, and he would furnish the latter already formed, and thus spare the liver this function. Senator also favors the use of this acid for a similar purpose, but reasons that in health sugar is converted into lactic acid in the small intestine, while in diabetes this conversion is interfered with. Hence, too, it should be given fully formed. Patients under its use are said to gain in weight and to become stronger, while it is not claimed that it alone diminishes the glycosuria; this must be brought about by a selected diet. The lactic acid is simply an important force-producer not otherwise obtainable, because sugar fails to undergo its usual conversion. Cantani recommends that from 75 to 150 grains of the acid should be taken daily in from 8 to 10 fluidounces of water. Diarrhoea and pains in the joints are said to follow the use of large quantities of the drug, but these again disappear on its omission. My experience is limited to a single case, which recovered while taking 30 drops three times a day in conjunction with Carlsbad water and a pill of iron, quinia, and arsenic.
Senator suggested that the fatty acids—oleic, palmitic, stearic, and butyric—be used on the same principle that lactic acid is given, that their force-producing power may be availed of. To this end he prescribed, with partially satisfactory results, soap in pills containing 21/3 grains each, of which four were taken daily.
Cod-liver oil is especially suitable as a food where debility is to be combated. Even those who claim that fats are convertible into sugar in the liver admit that it is only in the most advanced stages of diabetes that such conversion takes place. Cod-liver oil, therefore, in common with other fats, may form part of a diabetic diet, and is especially indicated where phthisis is present, as it so often is, in the latter stages of the disease, or indeed whenever a good tonic is indicated.
In 1882, Moleschott60 suggested the use of iodoform in diabetes. He reported the effect of its use in five cases, giving .1 to .3 grm. (1.5 to [p. 229]4.5 grs.) in pill with extract of lactucarium and cumarin, the purpose of the latter being to disguise the odor. His formula was as follows: Iodoform, 1 gram (15 grs.); ext. lactuc. sat., .1 gram (15 grs.); cumarin, .1 gram (1.5 grs.), to be made into twenty pills. In one case the sugar disappeared in twelve days; in the second, at the end of six months; in the third case it had diminished from 14.4 to 1.6 grams in three months; in the fourth, from 28 grams to 1.6 in four months; and in the fifth case, from 9.2 to 6.1 grams.
60 Wiener Med. Wochenschr., Nos. 17, 18, 19.
The use of the remedy in Moleschott's hands produced no unpleasant results, but Drasch,61 who used the same treatment after Moleschott's method in three cases, with the effect of diminishing the thirst, the quantity of urine, and the proportion of sugar, found excessive itching of the skin, diminished appetite, and diarrhoea to result in such degree as to demand its disuse in the majority of cases. Iodoform has been used by the Italian physicians De Renzi,62 Bozzolo,63 and Silvestrini,64 and by Sara E. Post65 of New York, with varying but generally favorable results, except in Silvestrini's case. These results included diminution in thirst, quantity of sugar and urea, with increase in weight. The drug deserves a trial in doses of from 1 to 2 grams (15 to 30 grains) a day, but due regard should be had to possible toxic effects; and to this end the administration should be interrupted at the end of one or two weeks, and the interruption continued for a like period. It may be given in pill or in capsule, and in divided doses or in a single dose at bedtime. The latter course is recommended by Post, and is said to avoid eructations and anorexia. Theories of its action based upon experimental use of poisonous doses ascribe its effect to a primary stimulating and ultimately fatally degenerative effect upon the protoplasm of cells, and especially those of the liver and nervous system.
61 Wiener Med. Presse, 1882, xxiii. 1487.
62 "Tre Storie di Diabete." Gior. internaz. d. sc. med., Nap., 1882, N. S. iv. 913-917.
63 "Sur l'action du iodoforme dans la diabète sucre," Arch. ital. de biol., Turin, Feb., 1883, iii. 317-321.
64 "Iodoforme dans le diabète," La France Méd., October, 1883, ii. 567.
65 Archives of Medicine, April, 1884, p. 116.
Transfusion of blood has been recommended by Dieulafoy,66 and is approved of by Ralfe,67 especially to combat the symptoms of acetonæmia, which, if due to a toxic agent, as seems most likely, should be met by altering the percentage composition of the blood with relation to the toxic agent.
66 "Étude sur la Transfusion du Sang dans le diabète sucre," Bullétin et Mém. Soc. Méd. de Hôp. de Paris, 1884, 4, S. 1, 38, 41.
67 "Discussion before the Path. Soc. of London," Lancet, Apr. 7, 1883, p. 592.
Diabetic neuralgia yields generally to the treatment of the disease in general correspondingly to the reduction in the quantity of sugar, and at times to salicylate of sodium, while it does not respond to morphia or other remedies for ordinary neuralgia.
The alkalies, which attained some reputation after Mialhe claimed for them the power of destroying sugar in the blood and of neutralizing the fatty acids which were thought to accumulate there in consequence of the deficient action of the skin, are not often used at the present day. Potassium carbonate was the favorite preparation, and in the hands of Pavy its use seems to have been followed by good results. He gave it in 10, 15, and 20 grain doses in combination with aromatic spirit of [p. 230]ammonia. Sodium bicarbonate was less satisfactory, as were also potassium acetate, potassium citrate, and Rochelle salts. These were given in doses of from four drachms to an ounce daily. In Germany, too, the alkaline treatment has been used to some extent.
As is the case with so many diseases which are incurable by any special treatment, a large number of remedies have at different times been suggested for diabetes, mostly on a foundation which does not admit of close analysis. One of these was the nitrate of uranium, suggested by Dale of Lemont, Pennsylvania, who gave it in doses of 1 grain three times daily, increased to 3 if necessary, in pill, powder, or solution, by aid of a few drops of nitric acid. He appears, however, to have used it in connection with a selected diet. I have tried it both with and without a selected diet; in the latter case there was no effect, and in the former there was none which the diet alone would not have produced.
Sodium phosphate, salicylic acid, salicylate of sodium, have all been used, it is claimed, with good results, and the late Dr. Dougherty of Newark, New Jersey, used with apparent advantage a mixture into which all of these, together with sodium carbonate, entered, made up with glycerin, tincture of cardamom, and water, the doses being 2½, 2½, 4½, and 8½ grains respectively. Moleschott has also obtained good results with salicylic acid.
SYNONYMS.—Scrophula, Scrofulosis, Morbus scrophulosus, Struma, King's evil, The evil, Quince, Cruels and Crewels (Scotice).
DEFINITION.—A morbid condition of the system manifested by a peculiar liability to certain forms of nutritive disorders of the skin, mucous membranes, joints, bones, organs of special sense, and especially the lymphatic glands.
There is probably no disease of which it is more difficult to give an exact and satisfactory definition than scrofula. The general tendency of medical opinion within the last few decades has been to narrow the significance of the term, and even to restrict it to those slow and indolent inflammations and over-growths of lymphatic tissue which end in caseation and finally imperfect suppuration. Formerly almost every deviation from healthy functional activity in the young, as well as every disorder of nutrition which could not be assigned to any definite cause, was called struma; and thus, as Heule well remarks,1 "Scrofula became the receptacle into which one vaguely casts all the ailments which afflict children under fourteen years, and of which we do not know the cause."
1 Handbuch der Rationellen Pathologie.
Before hereditary syphilis was understood all its manifestations were classed as scrofulous, and at least one eminent authority in the United States2 has expressed the opinion that scrofula is only a manifestation of the syphilitic poison in the second or third generation. Rickets, chronic hydrocephalus, favus, lice, and worms (Lugol), diabetes (Carmichael), and even scirrhus and cancer (R. Hamilton), have all been classed as scrofulous diseases. Then there is a large class of unhealthy persons whose morbid state can be no more definitely expressed than by saying that they are "delicate" or of "feeble health" or of "frail constitution," and by some all these are included under the term scrofulous. But as knowledge advances, and pathological knowledge as well as diagnostic acumen becomes larger and keener, many of these affections and morbid conditions can be eliminated from scrofula and assigned their true pathological and nosological position.
2 S. D. Gross, Transactions American Medical Association, 1878.
To many who have been educated in the more modern schools of medical thought, therefore, our definition will appear much too broad, while to others it may appear too narrow.
Scrofula is essentially and purely a diathetic, not a cachectic, disease. [p. 232]It is true that what may be called the manifestations or lesions of the disease are often excited by some preceding dyscrasia, and also that the long persistence of these lesions may excite a cachectic condition which we might call the scrofulous cachexia; still, as many children suffer from the lesions of scrofula who have never exhibited any evidence of a precedent dyscrasia, but on the contrary appear to be in perfect health, and many others, on the other hand, show unmistakable evidence of ill-health and are decidedly dyscrasic, yet are never attacked by scrofula, it is believed that every subject of scrofula becomes so not because of any pre-existing dyscrasia or cachexia, but because of some peculiar condition of the system—innate or acquired—which constitutes a diathesis.
"The hypothesis," says Niemeyer,3 "that scrofula depends upon a faulty composition of the blood (dyscrasia), and that the lesions found in scrofulous persons were due to a deposit in the tissues of a matter circulated by the blood and called a scrofulous material, is almost universally abandoned."
3 Textbook of Practical Medicine, vol. ii.
But while insisting upon the peculiar and, so to say, specific origin of the disease in some special condition of the system, without which it will never exist, it is admitted that the lesions of scrofula do not differ essentially from other similar lesions of the same tissues of a non-scrofulous origin. They are mostly of an inflammatory nature, and are only to be distinguished by the often trivial character of their exciting causes—often, indeed, by the total absence of any known exciting cause—and by their tediousness and intractability.
ETIOLOGY.—We believe, as already stated above, that the essential cause of scrofula is some peculiarity in the constitution of the tissues of the scrofulous subject; and we think it highly probable that H. F. Formad of Philadelphia has pointed out what constitutes this peculiarity. He declares—and the correctness of his observation has been abundantly verified—that microscopic examination of the tissues of certain animals characterized by their extreme aptness to be affected by scrofula and tuberculosis, as well as of children known to have been scrofulous or tuberculous, discloses the fact that the lymph-spaces in these subjects are always more numerous, larger, and more crowded with cells than in non-scrofulous subjects. The tissues of the scrofulous are therefore coarser, less compact and solid, and there is a greater tendency to undue cell-growth, than in the non-scrofulous. And these are precisely the characteristics which they present clinically, and such as we might have, a priori, expected to find.
This peculiarity of anatomical structure is in a large number of cases undoubtedly inherited from the parents, but while heredity plays, as is well known, an important part in the etiology of struma, it is not the essential factor. Bad hygienic surroundings, overcrowding, and consequent want of fresh air, improper food, consisting of a too great proportion of starch, during the early months or years of life, will cause the growing tissues to assume the peculiar anatomical arrangement alluded to above. "A coarse diet, containing but little nourishment in comparison with its bulk, is especially held in evil repute. The earlier this injudicious feeding of an infant commences, so much the greater danger that it will become scrofulous; hence the children fed on pap furnish a very important contingent to the army of scrofulous persons."4 The [p. 233]well-known fact that few children at the breast suffer from scrofulous lesions, but that a large number do so within the first two or three years after weaning, certainly tells in favor of the belief that too much starch and an insufficiency of animal food favor, if they do not actually produce, that faulty nutrition and construction of tissues which we believe lies at the foundation of the scrofulous diathesis. Independent, however, of improper food and the other predisposing causes mentioned, it is quite probable that faulty nutrition caused by accidental disease of the digestive or assimilative organs during infancy may create a predisposition. How else can we account for those not very rare cases in which from parents perfectly free from any scrofulous taint a large family of children may be reared, of which only one will suffer from any scrofulous lesions? Two such instances have been brought to my notice, and as the children in these cases lived upon a farm on the water-side, and enjoyed an abundance of pure air and salt-water bathing, and were certainly not stinted in food of proper quality, it is difficult to account for the acquired diathesis except upon the hypothesis above. Among the general predisposing causes of scrofula in addition to the special ones I have mentioned may be added—
4 Niemeyer, loc. cit.
1. Locality and Climate.—It has long been believed that scrofula is more common in the temperate zone than in the extreme north or in the tropics. While this is probably true, it must be stated that a sufficient amount of reliable statistics bearing upon this point have not yet been collected to prove the fact beyond cavil. That we should find that the disease prevails more extensively in cold and damp situations than in warmer and drier ones is to be expected, since the former conditions involve a greater confinement within dwellings, and consequently a diminished supply of fresh air, which, as we have seen, constitutes one of the predisposing causes of scrofula. Moreover, it is in these situations we would encounter a greater number of catarrhs, which, as we shall see, are known to be among the most active of the exciting causes of the glandular affections of scrofula.
2. Season.—For the same reason we find that a large number of cases of scrofula make their appearance in the early spring months, the results of catarrh contracted during the previous winter or of the sudden changes of temperature which accompany the transition of winter to summer.
3. Age.—Scrofula is essentially a disease of early life, but not exclusively so. As the diathesis can only be acquired directly from the parent, or fortuitously by malnutrition during the period of active growth, it follows that it becomes established, if at all, before the age of twenty years. And as the predisposition seems to be quite strong in most cases, and as the exciting causes are more apt to be applied during the earlier years of life, it is not surprising that a very large majority of the cases occur between the ages of three and fifteen years. A few, however, escape during childhood, and only suffer from it between twenty and thirty, while a small number only develop the disease in old age. Rindfleisch mentions the period between twenty and thirty as a common one for the development of hereditary scrofula; and senile scrofula was first pointed out by Sir James Paget.5 In all these cases of deferred [p. 234]manifestation of the scrofulous diathesis—and they are not very numerous—it is to be presumed that they have escaped the most active of the exciting causes of the disease. Indeed, it is natural that a person having inherited the predisposition should be more sedulously guarded—at first by his parents, and afterward by himself—against the exciting causes of scrofula during infancy and adolescence.
5 Clinical Lectures and Essays, London, 1875.
4. Sex.—There is no reason to believe that sex plays any part in the predisposition to this disease. Both sexes seem to be affected in about equal proportions, but from the statistics bearing upon this point it does seem to have some influence in determining the variety of its lesions. Thus, females seem to be more frequently affected with glandular disease, while males suffer from diseases of the joints in the form of coxalgia, white swelling of the knee, and Pott's disease.
5. Condition in Life: Social Position.—If what we have said about the predisposing influence of improper or insufficient food, overcrowding, etc. be true, it will naturally be inferred that a large proportion of the cases of scrofula will be found in the lower strata of society; and this is true. Especially in cities, where the disease prevails most extensively, we always find that the denizens of narrow streets, lanes, and alleys furnish the largest contingent to the deaths as well as the deformities from scrofula. It is here that the poor congregate to avail themselves of the cheaper rents, and here will be found combined all those predisposing causes which may be briefly summed up in one word—poverty. It is true that cases of scrofula are quite numerous in the country, and in a note to Sir Thomas Watson's Practice of Physic (1851) D. Francis Condie quotes from a work on The Nature and Causes of Scrofula, by Phillips, statistics which showed a greater preponderance of deaths from scrofula in a given number of the rural population than a nearly equal urban one. But at the time these statistics were gathered in England (and perhaps now) it is probable that there was a comparatively greater number of abjectly poor people among the rural population than in London, where was congregated such a large number of small tradesmen, artisans, and laborers, who, though not well-to-do, were better paid, and consequently lived better, than the agricultural laborers. Of course, a certain number of cases of scrofula are found in the United States, and perhaps in all other countries, among the children of the wealthy. These, however, are almost invariably caused either by direct transmission from parents or by some accidental injury to the digestive and assimilative organs in early childhood, as we have already pointed out. When it is remembered that in the constantly changing fortunes which are so frequently witnessed in this age of excessive activities, and that in the grand opportunities for obtaining wealth furnished by the liberal institutions and rapidly-growing industries of the United States the descendant of the pauper of the last generation may be the millionaire of the present, it is not surprising that so many who are now wealthy may possess the strumous diathesis as an inheritance from their parents or grandparents, and which they in turn transmit to their offspring.
6. Consanguineous Marriage.—It has long been a popular belief that the offspring of parents closely related by blood are more apt to be scrofulous than when no such relation has existed. Indeed, not only scrofula, but numerous other diseases, deformities, and imperfections have been [p. 235]ascribed to such unions. Idiocy or feeble-mindedness has also been especially accredited to the production of such marriages. But a thorough investigation of this point in England some years ago demonstrated positively that no more idiotic, feeble-minded, or insane children are born of such marriages than of an equal number of marriages contracted between persons not related by blood to each other. There is, however, this amount of truth in the popular belief: if persons closely related to each other possess the scrofulous diathesis, there will be a greater probability—almost certainty—that the diathesis will be transmitted to their offspring. If one parent only is tainted with scrofula, and the other is entirely free from it, there is a possibility—even a probability—that some or all of the children may escape.
7. Complexion and Temperament.—It has been stated by some observers that scrofula occurred principally in the fair-haired, and with equal positiveness by others that it was in the dark-haired that the disease found the most of its victims. Such statistics as have been furnished, however, upon this subject seem to show that there is no connection whatever between scrofula and complexion. It will generally be found that whenever in any country or locality more cases of scrofula occur in persons of one or the other of the complexions, it is only because that particular complexion is the predominant type among the inhabitants of that locality.
8. Race and Nationality.—While it would seem that no race or nation is entirely free from struma, yet there are certainly in the United States two peoples who furnish an enormously disproportionate number of scrofulous cases: these are the Irish and Jews. Among the first of these both scrofula and tuberculosis abound with exceeding frequency, while among the latter it is scrofula alone which seems to predominate. The last, however, are not exempt from tuberculosis, but only exhibit about an equal predisposition to it with their fellow-citizens. It is not difficult to explain the special predisposition of these peoples to scrofula when their past history is taken into account in connection with what has been said about the bad influence of food and surroundings in producing the scrofulous diathesis. The principal food of the Irish peasantry—oppressed and ground into poverty by their Anglo-Saxon conquerors for hundreds of years—have been bread and potatoes, often potatoes alone. It cannot be surprising, therefore, that Irish children fed upon this diet and reared in ill-ventilated hovels should develop the scrofulous diathesis in legions. The Jews, too, oppressed by all nations through ages, have been during many generations reared in poverty and squalor. Even those of them who in not very remote times had acquired by thrift the means of securing both the comforts and luxuries of life dared not live according to their means, lest a show of wealth should attract the unpleasant, often fatal, attention of their rapacious and unscrupulous Christian or Mohammedan neighbors. This condition, this mode of life, has existed among them for many hundreds of years, and has so intensified the strumous diathesis among them that almost the whole race may be said to be patently or latently scrofulous. The negro or African race, however, as observed by the writer in the Southern States of the American Union, do not seem to have developed any special predisposition to struma, notwithstanding their servile condition. This, at first sight, would seem to [p. 236]be contrary to our expectation based on what has been said about Jews and Irishmen. But as my remark has been predicated only on observation of the African in the Southern States, where the climate is not favorable for the development of scrofula, the fact is not so surprising. Besides, the food of these people consisted largely of bacon or pork, fish, milk, and the succulent fruits and vegetables, with a moderate quantity of corn bread, and very rarely potatoes. As the rude cabins in which they dwelt were usually constructed of unhewn logs and covered with rough boards, and cost almost nothing except labor, overcrowding was unknown and ventilation always perfect. The waiter practised medicine fourteen years in Wilcox county (S. W.), Alabama, containing a population in 1870 of 28,377, of whom 21,610 were colored, and during this time saw only two cases of genuine scrofula and one of tuberculosis among the colored population.
Pork as an article of food has often been accused of producing a tendency to scrofula, but evidently with great injustice, for we have seen that the Jews, who never eat it, are almost universally scrofulous, while the Southern negroes, whose staple animal food it was, were conspicuously free from it.
9. Acquired Scrofula.—Although in perhaps a majority of all scrofulous cases the diathesis has been inherited from the parents, the fact cannot be too strongly emphasized that in a large number of cases the disease may be developed de novo, independent of such heredity. To scrofula developed from the influence of bad ventilation and overcrowding, absence of sunlight, insufficient, bad, or unsuitable food, cold and damp, imperfect clothing—in short, all those conditions associated with poverty, squalor, and ignorance—Grancher has well applied the term la scrofula a miseria. And it is only by a clear comprehension that scrofula may be, and often is, developed under these conditions that the medical profession in general, and municipal health authorities in particular, may be induced to teach and enforce upon the poor both the knowledge and the practice which may prevent it. Even in the open country, where there is at least no lack of pure air and light, the lesson can be enforced with equal profit; for the children of the farm-laborer are likely to be imperfectly and improperly fed, and lodged in apartments at night that in the matter of foul air and filth could not be well surpassed in the purlieus of the dirtiest and most overcrowded city.
EXCITING CAUSES.—The actual exciting causes of scrofula when the diathesis already exists are too numerous to be mentioned in detail. Indeed, almost any trivial injury or inflammation, any disease which has produced a temporary cachexia, may rouse into activity the perhaps hitherto latent tendency. How often do we see a slight blow upon the knee-joint produce a white swelling which lames for life the heretofore healthy and active boy or girl! A fall upon the hip which was almost unnoticed at the time excites a coxalgia which either destroys life or renders the child for life a cripple; or a slight jar of the spine induces a disease of the vertebræ which, if recovered from at all, produces a terrible deformity. A slight eczema of the face or scalp or a catarrh of the mouth or throat will excite that slow and generally painless enlargement and induration of a neighboring lymphatic gland which always ends in its caseation and final destruction by suppuration. A slight injury to the [p. 237] periosteum may excite a destructive caries or necrosis of the underlying bone, and a temporary catarrh of the intestines a tabes mesenterica with all its fatal consequences.
Speaking generally, it may be said that anything that produces a local disorder of nutrition or impairs the health generally of a person predisposed to scrofula is sufficient to bring about some manifestations of the disease. They are especially apt to follow the eruptive fevers. Measles and scarlatina are very commonly arousers of the scrofulous process, not only by the temporary impairment of health which follows them, but also through the catarrhs which are usually present in both diseases. Vaccination has often been accused of imparting scrofula; and, although this is untrue, since scrofula cannot be imparted in the sense of transference from one person to another, there can be no doubt that the predisposition may be roused into activity by the slight impairment of health associated with vaccination or by the slight injury inflicted at the point of introduction of the vaccinal virus. In some cases the disease has manifested itself for the first time during pregnancy or lactation, and there is no doubt that in cases where the disease has existed in childhood these conditions often cause it to reappear. In conclusion, it must be said that many cases apparently occur spontaneously—"the disease came on of itself"—or if there are any exciting causes they were so trivial as to have escaped notice altogether.
Finally, it must be remembered that the eczemas, catarrhs, ophthalmias, otitis, chilblains, erysipelas, and numerous other local disorders of nutrition which are often the causes of graver manifestations of the disease, are themselves very prone to run a peculiar course characterized by chronicity and intractableness; and many regard these disorders as themselves manifestations of scrofula. Indeed, Virchow, basing his argument upon the fact "that scrofulous enlargement of the lymphatic glands of the neck often follows upon certain diseases affecting the throat, such as mumps, diphtheria, and scarlet fever, maintains that scrofulous proliferation of these glands, like ordinary inflammatory hyperplasia of the same organs, is always secondary to some peculiar process going on at the mucous surface or other part which is in direct relation with them by means of the lymphatic vessels; that scrofulous disease of the glands of the neck is traceable to some inflammatory condition of the throat, fauces, or contiguous parts; of the bronchial and mediastinal glands, to pulmonary or bronchial inflammation; and of the mesenteric and retro-peritoneal glands, to similar conditions of the alimentary canal. But he considers that there may be some special element or quality in the primary inflammation, and a tendency in its products to undergo rapid decay similar to that which characterizes the morbid products of the diseased lymphatic glands, but that generally they are not recognizable, from the fact that in this case the cells are mostly developed at a free surface, and are speedily shed from it." He admits, however, "that there may be some special aptitude or weakness, congenital or acquired, in the lymphatic glands of certain persons, or of certain parts of them, which makes their inflammations, induced by indifferent causes, assume the scrofulous character."6 From the last of these propositions no one will be likely to dissent, but that there is "some specific quality or [p. 238]element in the primary inflammation," etc. few will be inclined to agree. There is nothing peculiar in the eczemas, ophthalmias, catarrhs, etc. spoken of, except that they occur in persons possessing the scrofulous diathesis; and it is this alone that gives them their special characters, if there are any. In other words, we cannot say that these disorders occur in certain children because they are scrofulous, but that they are specially intractable on account of the scrofulous diathesis upon which they are engrafted. Millions of children have catarrh and ophthalmia (indeed, few escape these disorders throughout the first ten or twelve years of life) who never show any other evidence of the scrofulous taint; and nearly all have measles and scarlatina, but it is only the scrofulous who usually suffer severely from the secondary effects of these diseases. But they do not have measles or scarlatina because they are scrofulous, and we can with no more justice say that they have catarrhs or other inflammations because they are so. We do not believe, therefore, that strumous children have cutaneous and catarrhal inflammations simply because they are strumous; and if we speak of scrofulous catarrh or ophthalmia or eczema, we use the terms in the same sense as when we would speak of a scrofulous measle, scarlatina, or whooping cough.
6 The Theory and Practice of Medicine, by Jno. Syer Bristowe, M.D., 1879, pp. 80, 81.
Although we have few reliable statistics bearing upon the question, it can scarcely be doubted, judged by the results of casual observation, that scrofula is much less frequent in America than in Europe, and that in the latter there is less of it than formerly. The cheapness of land in America has prevented that excessive overcrowding that exists in the older and more densely populated countries, and the abundance and cheapness of animal food has prevented that excessive feeding on bread and potatoes which constitutes such an important factor in the production of the scrofulous diathesis in some other countries.
PATHOLOGY AND MORBID ANATOMY.—The most important and central anatomical and pathological facts both in the causation and progressive development of struma, according to the writer's views, are—
1st. That faulty anatomical—or rather histological—construction of the tissues of the scrofulous individual already alluded to as having been first brought to the notice of the profession by Formad of Philadelphia, which consist of an unusually large number of lymph-spaces (which are also unusually large), and consequently an excessive number of lymph-vessels and lymph-glands.
2d. Excessive production of rudimentary lymphoid cells, and probably also of lymphatic tissue.
3d. Diminished and insufficient number of the capillary blood-vessels; and, as a necessary consequence of these,
4th. Diminished nutritive activity of all those processes, both physiological and pathological, which depend upon a full supply of nutritive blood.
The most striking feature in all scrofulous inflammation is excessive cell-growth, but these cells show little tendency to differentiation and organization, probably for two reasons: 1st, because they are derived from the blood-vessels principally, and not from proliferation of the proper connective-tissue cells of the part; and 2d, because they are insufficiently supplied with nutrition from the scanty blood-vascular [p. 239]network, and this supply is too rapidly absorbed into the lymph-spaces, and is carried off by the too numerous lymph-vessels. The cells, therefore, speedily perish, undergo partial or imperfect fatty degeneration, and finally caseation, unless the process is going on at a free surface, in which case, of course, they are shed and thus gotten rid of.
Virchow some time ago called attention to the predominant cellular character of the scrofulous exudation and the low vitality of the cells which compose it. Rindfleisch declares that the fresh scrofulous exudations contain relatively large cells with glistening protoplasm, and that the white blood-corpuscles have a tendency in scrofulous persons to grow larger on their way through the connective tissue. He adds that they swell up by the imbibition of albuminous substances, and by this very swelling die and slowly degenerate.
It seems to the writer, however, that it is probable that herein lies the reason why swelling and apparent hyperplasia of the lymphatic glands in the neighborhood of a local inflammation occurring in a scrofulous person always takes place. The swollen cells become arrested at the first gland they reach, and block the channels through the gland. Successive additions of cells continue to block these channels, and finally the passage of lymph through the gland becomes impossible, and then begins that secondary increase of the lymph-cells in the gland resulting from their inflammatory proliferation.
"In scrofulous inflammation," say Cornil and Ranvier,7 "there is a remarkable tendency to permanent infiltration of the affected tissue. In simple inflammation (i.e. inflammation in non-scrofulous persons) the infiltration is a temporary condition which terminates in suppuration, in organization, or in resolution." Now, the several steps in this process of resolution are—contraction of the distended blood-vessels, thus cutting off the excessive supply of blood which has caused the exudation and cell-proliferation; fatty degeneration of the new cell-formation; liquefaction of this fat by union with the alkaline blood-plasma, converting it into a dialyzable (saponaceous) liquid which can now be readily absorbed by the veins. In scrofulous infiltration the cells are speedily attacked by fatty degeneration (which seems to be strictly a physiological process), but instead of becoming liquefied, it (the fat) remains, slowly dries and hardens, and finally becomes converted into the so-called cheesy mass or cheesy infiltration. It does not liquefy, because it does not receive a sufficiently abundant supply of the alkaline blood-plasma from the scanty blood-vessels, and that which is supplied too rapidly flows into the numerous large lymph-spaces and is carried off by the lymph-vessels. In the case of the infiltrated gland the supply of this plasma is cut off in both directions. The passage of lymph through the gland is blocked, when, of course, none can then reach it through the lymph-vessel leading to it, while the swelling of the gland itself from accumulated cells compresses the neighboring nutrient vessels and cuts off the supply from this direction also. Hence the speedy death, fatty degeneration, and caseation (not liquefaction) of the cells.
7 Ed. 1880, p. 114.
"The newly-formed material not only interrupts the lymph-passages of the gland, but also compresses the blood-capillaries in such manner that the circulation completely stagnates. It is impossible by any [p. 240]method of injection to penetrate into the most swollen parts of the gland. With the supply of blood the nutrition also self-evidently ceases; the gland falls into caseous degeneration. Where this enters in the gray mass first becomes opaque, then whitish-yellow, non-transparent, dry, friable. If the whole gland has passed into the caseous condition, it appears upon a section as a fresh potato, only not quite so moist, but just as homogenously yellowish-white."8
8 Rindfleisch, Textbook of Pathological Histology, 1870, p. 202.
The subsequent fate of these glands seems to depend somewhat upon their situation. In the mesenteric and bronchial glands almost always the caseous mass is attacked by calcareous infiltration, and finally dries into a solid chalky concretion. The writer counted seven of these chalky masses around the primary bronchi of a boy about fifteen years old whose body was brought into a dissecting-room in Baltimore City. But the more common result of the caseous process in the glands of the neck is softening. "The caseous dépôt melts from within outward into a whitish-yellow, whey-like fluid, which holds a fatty granular detritus suspended in smaller or larger fragments. If all the caseous material has softened, the neighborhood of the gland is wont to inflame; this inflammation facilitates the way for the scrofulous pus outward. This is evacuated, and we have the scrofulous ulcer, with its overhanging, bluish, hyperæmic, flabby edges. At length this opening also closes, and a drawn-in, radiated cicatrix marks the place where the evacuation took place."9
9 Ibid., loc. cit.
But it must be borne in mind that all so-called scrofulous hyperplasias of lymphatic glands do not run this destructive course. Undoubtedly, in a few cases there remains a sufficient nutritive supply to carry on the liquefactive process which normally follows fatty degeneration, and thus resolution of the affected gland takes place. We are, however, of the opinion that Virchow was mistaken when he asserted that complete resolution of the cheesy material could take place; and from what we know of the dangerous and usually fatal consequences of the absorption of this cheesy detritus, Rindfleisch is certainly in error in describing this as the most desirable possibility of decomposition.
We have heretofore purposely avoided any mention of tubercle or tuberculosis as a part of the scrofulous process. In the views of many physicians the relations between the two processes are so close that to them tuberculosis and scrofulosis mean one and the same thing. While medical opinion as to the true meaning of the word tubercle was so discordant and unsettled, while so many products of diverse pathological processes were included in that term, and while many, following the view of Burdon-Sanderson of England, believed that tubercle always takes its origin in small, even microscopic, collections of lymphatic tissue, such a belief in the identity of the two processes was not only possible, but reasonable. But since, by very many good authorities, the term tubercle is now limited to the miliary or submiliary tubercle, since numerous inoculation experiments have shown that tuberculosis can be induced in non-scrofulous animals, and Koch of Germany has proved that there exists in decaying tubercle a peculiar and distinctive bacillus which even when cultivated out of the body of a tuberculous person will excite tuberculosis also if inoculated upon a non-scrofulous animal,—a belief in the identity of the two diseases seems to be no longer tenable. Certainly, it [p. 241] would seem that to Sanderson's view that tubercle always takes its origin in lymphatic tissue it is only necessary to reply that the subjects of miliary tuberculosis do not more frequently than those suffering from other non-scrofulous diseases present those larger glandular hyperplasiæ which are so distinctly characteristic of scrofula, and to which many persons limit the term scrofulosis. It may be said, perhaps, that the converse of this is not true, and that scrofulous persons are more frequently attacked by miliary tuberculosis than an equal number of non-scrofulous persons. But the extreme susceptibility or liability of the scrofulous to be attacked by numerous and even diverse morbid processes, and the profound cachexias and dyscrasias which the scrofulous processes engender, amply account for the apparent susceptibility of the scrofulous to be attacked by miliary tuberculosis. The strumous are more susceptible to the exciting causes of tuberculosis undoubtedly, but perhaps the same may be said in regard to measles, scarlatina, and the various other exanthemata. It is undoubtedly true also that among the lower animals (and probably also in the higher ones) the introduction into the circulation of the semi-purulent fluid resulting from the breaking down of a cheesy scrofulous gland will produce that peculiar (perhaps specific) dyscrasia which results in miliary tuberculosis. But as it has also been abundantly proved that a similar fluid derived from a cheesy pneumonia, or from the inflammatory products of any other disease which have undergone the cheesy degeneration, will also excite tuberculosis, the fact does not seem to tell in favor of the identity of, or even of any close relationship between, the two processes. Still, as the scrofulous more frequently than other people are the subjects of this cheesy process, it is not surprising that they should more frequently be poisoned by the entrance into their blood of the cheesy detritus.
We do not deem it necessary to adduce all the evidence or to state authorities upon this subject, but we think we are justified in stating the following doctrine in regard to the relation of scrofula to tuberculosis as best supported by facts and by the consensus of medical opinion:
Scrofula is a purely diathetic disease inherent in the individual.
Tuberculosis is a cachectic (possibly a purely dyscratic) one, not inherent in the individual, but always caused by some morbid influence from without. Tuberculosis may therefore occur in the non-scrofulous as well as in the scrofulous.
But the scrofulous are more likely than others to have tuberculosis—1st, because of their greater susceptibility to all morbid influences; 2d, because the scrofulous processes are apt to produce some cachectic condition which is always a condition precedent to tuberculosis; and, lastly, because the products of decay resulting from scrofulous processes may enter the circulation and directly produce the tuberculous dyscrasia. These remarks of course apply only to primary tuberculosis.
But while we thus deny anything else than a purely incidental relation of scrofula to tuberculosis, we believe that there exists the very strongest possible relationship of scrofula to pulmonary consumption. We think we are justified in stating that fully 95 per cent. of all cases of pulmonary consumption are of inflammatory origin, and of that variety miscalled catarrhal pneumonia. Broncho-pneumonia or catarrho-pneumonia more exactly describes the process. It begins as a catarrh, [p. 242]sometimes in the nasal passages or post-nasal fossæ, sometimes in the pharynx, but most frequently in the trachea and large bronchial tubes, and sometimes rapidly, but oftener more slowly, travels downward and invades the lining membrane of the air-sacs, which soon become packed with cells derived partly from emigration of leucocytes, partly from proliferation of the epithelium lining the sacs. These cells soon undergo the cheesy degeneration, and, finally breaking up, as in the case of the scrofulous gland, cause the formation of vomicæ attended with the familiar signs of pulmonary consumption. Every step in this process is attended with that abundant cell-production, and the process itself is marked by that inveteracy and intractableness, which always characterize scrofulous inflammations, or rather inflammations in the scrofulous. Occurring as they most frequently do in young adults, these cases are often mistaken for pulmonary tuberculosis; and as post-mortem examination generally reveals a more or less abundant secondary tubercular eruption caused by absorption of infective material from the centres of cheesy degeneration and softening, the diagnosis is claimed to be confirmed. But they are for the most part, nevertheless, cases of genuine scrofulous inflammation of the bronchial membrane and lining membrane of the alveoli, and should be called scrofulous pneumonia. It is true that Mr. Phillips, Mr. Kiener, Villemin, Grancher, Mr. Treves, and others have collected numerous statistics which would show that comparatively few of those who had died of pulmonary phthisis bore any evidence of previous scrofulous disorder. But as the principal evidence relied upon to prove this fact was an absence of scars resulting from suppurating glands, their statistics are inconclusive. Besides, it is a well-known fact that there is a decided antagonism between scrofulous diseases of all kinds, and a patient who has one severe or well-marked manifestation of scrofula is not likely to develop another strumous disease at the same time. The records of the Margate Infirmary for Scrofula show this fact very strongly, and numerous writers—among whom may be mentioned Holmes, Birch-Hirschfeld, Walsh, Mr. Treves, and others—strongly express the same opinion. Indeed, some of them go so far as to maintain that one form of the scrofulous manifestation confers protection against others. The question may perhaps be more clearly stated by saying that the scrofulous, like the non-scrofulous, have their special predispositions and indispositions to certain morbid affections, and while one scrofulous child may be specially predisposed to affections of the bones, joints, skin, or other tissues, it may have no predisposition whatever to affections of the lungs or lymphatic glands, etc. This difference in vulnerability or invulnerability of certain tissues or organs in individuals, whether scrofulous or not, is so distinctly recognized as a controlling factor in determining the special form of disease resulting from a given irritant that its discussion is entirely unnecessary. It is argued against the identity of scrofula and pulmonary consumption that the commoner manifestations of the former occur in childhood for the most part, while consumption is a disease of adult life. But this is readily accounted for by the different morbid tendencies and exposures in the two periods of life. "Scrofula tends to appear in early life on account of the unusual activity of the lymphatic system at that period, and phthisis somewhat later—at a time, indeed, when the lungs are in more active use, when [p. 243]sedentary and perhaps unhealthy pursuits are assumed in exchange for the liberty of childhood, when the modifying influences of puberty are active, and the structural responsibilities of adult life press heavily on an organization never other perhaps than frail.... I would, on the contrary, assert that scrofula and phthisis are as much manifestations of the same morbid change as acute bubo, acute orchitis, and acute pneumonia are outcomes of one single process—acute inflammation."10
10 Scrofula and its Gland Diseases, by Frederick Treves, F.R.C.S., Eng.; New York, 1882, p. 62.
It is entirely unnecessary—and indeed it would be too tedious—to describe the anatomical appearances of the almost innumerable lesions met with in the scrofulous. Holding as we do that scrofula is not a disease per se, but merely a condition resulting from malnutrition and consequent faulty construction of the tissues during the early years of childhood, no peculiar or distinct anatomical lesion can be ascribed to it; and yet every lesion of nutrition as well as of function may have certain specific characteristics impressed upon it by the scrofulous diathesis. These may be briefly summed up as great slowness in evolution, intractableness, incurability, and chronicity of all pathological processes, and in all inflammatory processes abundant cell-production and tendency to caseation.
SYMPTOMS, COURSE, DURATION, AND TERMINATIONS.—A great deal of fine writing has been expended in describing the physiognomy of scrofula, and for ages writers exercised their descriptive powers upon the type of face and form supposed to be indicative of the disease. It is almost needless to say that much of this has been evolved from the imaginations of the writers, while many of these descriptions are not pictures of those liable to suffer from scrofulous processes, but of those who are already the subjects of these, and are simply types, not of the scrofulous diathesis, but only of the scrofulous cachexia. Many of these pictures, too, were drawn not from the scrofulous, but the tuberculous patient, because they were considered identical. Scrofula is not confined to the dark or the fair, the dull or vivacious, nor even to the weak and puny or the strong and robust; but all these may have this faulty and often fatal construction. Nor do we believe that scrofulous children are either more brilliant or more stupid than other children. At most we can only say that the scrofulous habit is marked by a deficiency of blood and a bad nutritive state of the more important and more highly organized tissues. In some an abundance of fat is found, giving to the individual a certain amount of plumpness, which might be thought to be inconsistent with a state of bad health; in others there is an imperfect development not only of the subcutaneous fat, but of the skin and muscles also, so that they appear tender and delicate. In the first of these conditions there is supposed to be an indolent state of the processes of constructive and destructive assimilation; in the second, an unnatural activity of these processes. These differences have led to a classification of scrofula into the phlegmatic or torpid and the sanguine or erethistic forms, which Canstatt has thus described: "An unusually large head, coarse features, a thick chin, a swollen abdomen, enlarged cervical glands, and flabby, spongy flesh." The erethistic form is said to possess "a skin of remarkable whiteness, with a tendency to redden easily, and through which the [p. 244]rose-pink or bluish subcutaneous veins are visible, a deep redness of the cheeks and lips, blueness of the thin and transparent sclerotica, which imparts a swimming and languishing look to the eyes. The muscles of such persons are thin and soft, and their weight is light in proportion to their stature, indicating a slightness of their bones. The teeth are handsome and of a bluish lustre, though long and narrow; the hair is soft."11 Although this description may be characterized as diagrammatic, since it describes rather the extremes and not the mean of the general appearance of the scrofulous, and numerous cases will be met with that cannot be assigned to either of the above categories, yet as quite a large number of cases will be seen that obviously belong to one or the other of these types, and as, moreover, we shall see that by this classification we shall obtain valuable data for therapeutic indications, it may be well to preserve this division of the scrofulous into the lymphatic and sanguine types.
11 Niemeyer's Text-book of Practical Med., vol. ii. p. 741.
The leading points in the physiognomies of each of these types were admirably shown in the composite photographs exhibited by Dr. Mohamed at the last International Congress in England. By some special process a composite photograph of many faces was, as it were, condensed into a single picture, in which all that is common remains, all that is individual disappears. And although Mohamed's pictures were all of phthisical patients, it must be admitted that the two types of coarse struma and sanguine struma were strikingly illustrated, and were very suggestive of Canstatt's descriptions as given above. But it must be borne in mind that a large number of the strumous belong strictly to neither of these types, but rather to a medium between the two. "Such a type would include what is known as pretty struma. The general features of the individuals so termed belong to the so-called phlegmatic type, but the coarseness of the features is toned down; the lips would be called full, not tumid; and a coarse flabbiness would subside into a pretty, plump condition of the body. The limbs, if not actually graceful, are at least prettily rounded. The skin may not be thin and fine, but it is soft, white, and clear. The general expression is not absolutely apathetic, but would be termed gentle and eminently feminine. Excellent representations of this type of pretty struma were also shown in the photographic series above mentioned."12
12 Treves, Scrofula and its Gland Diseases, p. 84.
This matter of physiognomy of the scrofulous has this much at least of practical importance—viz. that to the sanguine or erethistic type belong those cases that show distinct heredity, while the phlegmatic or torpid is usually the type assumed in the acquired forms. While there are doubtless numerous exceptions, it will generally be found that scrofula in the rich assumes the first, and in the poor the second, of these forms. It has been asserted that the erethistic form is more apt to develop tuberculosis or phthisis; and to a certain extent this is doubtless true, but the torpid are by no means exempt from this grave accident. The first are undeniably more liable to the more severe and fatal forms of the disease, which run a more rapid course and are less amenable to treatment, while in the second phthisis is more apt to be chronic and incomplete recoveries are by no means rare. The first form is said to [p. 245]be more frequent in women, while the second is more frequent in males; and this accords with my own observation and experience.
There are certain features more or less peculiar to scrofula, besides those appertaining to the general physiognomy already discussed, which it may be well to call attention to, since these may aid us in detecting the scrofulous diathesis even before the grosser manifestations have declared themselves.
Allusion has already been made to the defective blood-vascular capillary network in the scrofulous as a necessary consequence of the excessive predominance of lymph-spaces and lymphatic vessels. Indeed, there can scarcely be a doubt that the slowness of evolution of various pathological processes, their chronicity, and the absence of tendency to resolution and cure of inflammatory lesions, so prominent a feature in all scrofulous manifestations, is due to this very condition. It is especially in the coarser type of struma that these defects in the circulation are most conspicuous. In these the pulse is often below the average, soft, and wanting in vigor. The cheeks and limbs often assume a bluish and mottled aspect, due perhaps to a tendency to stagnation of the blood in exposed parts. The extremities appear swollen as if from cold, and in the winter generally appear chapped. They are particularly liable to chilblains, which persist far into the summer and often take on a very unhealthy action. This last feature is so common as to constitute an important symptom in scrofula. These defects in the circulation also probably explain the frequent catarrhs and eczemas with which such persons are affected, and account also for their intractableness as well as the unwholesome character of their wounds.
For the same reason (deficient circulation) the temperature is generally found to be a little lower in the coarsely strumous than in healthy children, and even in their fevers a very high temperature is rarely met with. Acute sthenic inflammations are rarely seen, and hence these persons seldom have acute croupous pneumonias; it is rather the catarrhal variety, and of this the subacute and chronic forms, which they suffer from.
Opinions are completely at variance as to the influence of the scrofulous habit in delaying or hastening menstruation. Lugol referred to the frequency of dysmenorrhoea among the strumous, and there is no doubt that the scrofulous as a rule often suffer from suppressed or scanty menstruation. But it is improbable that the diathesis exerts any influence whatever in determining the period of puberty in either sex.
We have already stated our belief that the strumous are neither more intelligent nor stupid mentally than other people. An exception ought perhaps to be made to this in the case of the exaggerated type of the coarsely strumous. In these extreme cases we must confess that we have generally found associated great slowness and dulness of the mental faculties. If great intelligence and precocity are sometimes met with, it is only in the erethistic or pretty struma, who, because it is the delicate one of the family, is petted, has more notice taken of it, and afforded every facility for the development of the points that make up the precocious infant. The prettiness of these children, moreover, attracts more attention to them than to other children or than the bulk of the sickly would receive.
In young scrofulous children we often observe a considerable amount [p. 246]of close-lying downy hair upon the forehead, more abundant upon the sides of the forehead. Upon the arms and back from the occiput to below the shoulders also a like condition is often seen. Later the eyelashes appear thicker and longer, and the eyebrows more abundant, coarser, and longer, than in the non-scrofulous. The color of these is also apt to be darker than the rest of the hair.
Constantine Paul, as quoted by Treves, has drawn attention to certain changes in the ears, after they have been pierced for earrings, that he considers to be diagnostic of scrofula. The mere weight of the earring seems to cause the puncture to slowly ulcerate, and the ring thus cuts its way out, either leaving behind it a linear scar or a slit in the lobule. If the lobule be repaired the ring may cut its way out again, and this may occur three or four times. These changes seem not so frequently to be observed in England and America, and may be due in part to the fact that earrings of greater weight, and more frequently of base metal, are worn in France than in the countries named. But still, from what has been said concerning the histology and minute anatomy of the scrofulous, and the consequent less resistance of the tissues, this cutting-out process by earrings is just what we would be led to expect in strumous persons.
The thick upper lip is never absent from the older descriptions of the physiognomy of the strumous. This is almost invariably present in the coarse type of struma, and seldom absent even in the erethistic. It is not always due to irritation from acrid discharges from the nose, as is maintained by Treves, though doubtless the eczematous and herpetic eruptions are often caused and maintained by these discharges, and these may in time cause and increase this thickening.
The teeth in scrofula show nothing that is distinctive, though there is undoubtedly a tendency to early decay. As this tendency to decay is, however, so common in many persons who have at least shown no other evidences of the scrofulous diathesis, no positive conclusions can be drawn from this fact.
Clubbed fingers, too, so common in persons who have become cachectic from the long persistence of scrofulous disorders, are not characteristic. Clubbed fingers and incurvated nails will generally be found in persons suffering from any disease characterized by slow wasting. They are seen in phthisis of all varieties, as well as in cancer, heart disease, aneurism, Bright's disease, empyema. They therefore have no significance as far as struma is concerned.
GENERAL MANIFESTATIONS OF SCROFULA.—As, according to our view, there is no such disease per se as scrofula, but simply a diathesis which impresses its own malign influence upon every other disease with which the strumous individual may happen to be afflicted, increasing perhaps the general predisposition to be injuriously affected by all morbific influences, or impairing the powers of resistance to these, and especially intensifying any special predisposition which age, sex, personal peculiarities, occupation, habits, mode of life, or heredity may have created, we cannot describe any morbid processes as specifically scrofulous. At most, we can only say that struma is more apt to impress its malign influence upon certain diseases or upon inflammations and injuries of certain tissues, that some diseases in the scrofulous are more apt to be [p. 247]attended by certain complications and followed by certain sequelæ, and that all of these are characterized by chronicity and incurability, by slowness of evolution of pathological processes, and, in the case of inflammations, by a tendency to profuse cell-production and to rapid caseation. Thus, measles is apt to be complicated with or followed by otorrhoea, chronic bronchitis, caseation of bronchial glands, phthisis, and even tuberculosis; scarlatina by otitis, hyperplasia of the tonsils, caseation or suppuration of the submaxillary and other lymphatic glands about the neck, and by chronic catarrh of the renal mucous membranes, causing dropsy and finally death; eczemas about the face or catarrhs of the mouth and throat by hyperplasiæ and caseation of lymphatic glands in the neighborhood. Boils and other subcutaneous inflammations of the areolar tissue, so common in childhood and adolescence, do not run their usual rapid course, ending in suppurations and cicatrization, but become in the one case the scrofulous gumma, degenerating into the scrofulous ulcer, or if more deeply seated become a cold abscess. A single injury of a joint, whether mechanical or rheumatic, will "sometimes take the form of a simple hydrarthrosis, sometimes that of a so-called tumor albus, while at others it assumes the nature of a malignant arthrocace, accompanied by suppuration, caries of the articular surfaces, burrowing of pus, and the establishment of fistulæ."13 A slight injury inflicted in the sports of childhood and soon forgotten—the prick of a pin perhaps—is followed by a disease sometimes beginning in the periosteum, sometimes in the bone itself, and presenting at one time the character of periostitis and ostitis, and at another that of caries or necrosis, or of the two combined.
13 Niemeyer, loc. cit.
"As long as the existence of cheesy masses," says Niemeyer, "was regarded as characteristic of the tuberculous nature of a disease, it was of course necessary to ascribe many of the inflammations of the joints and bones of scrofulous persons to a complication of scrofulosis with tuberculosis."14
14 It is a well-established fact, however, that true miliary tubercles are often found in the neighborhood of bone and joint affections in the scrofulous, as well as in lupus, in cold abscess, and in softening caseous glands, which last are considered by many as specifically scrofulous diseases. It is suggested that an explanation of this may be found in the probable fact that caseous pus may be capable not only of producing a general tuberculosis when carried by veins or lymphatics into the blood, but that it may also set up a local tuberculosis by a morbid influence exerted upon the neighboring lymphatics and blood-vessels with which it may come in contact. We are aware that Wilson Fox (according to the Medical Times and Gazette), captivated by the theory of Koch, has recently recanted his belief in the inoculability of tuberculosis with anything except tubercle. But we are afraid that Dr. Fox (who we believe was one among the first to confirm Ferdinand Cohn's experiments in producing tuberculosis in rabbits and guinea-pigs by inoculating them with caseous pus) is suffering from that most active and virulent of all contagions, the contagion of popular belief. Just now a belief in specific bacilli and micrococci may be said to be riding upon the crest of a very high wave of popularity, and we are afraid that many of those who are rushing forward to mount this wave also will ultimately find themselves stranded upon that shore which has been strewn with so many wrecks in the past.
A simple bronchitis, possessing nothing specific in its origin at least, will persist and extend to the lobuli of the lung and excite a catarrho-pneumonia which ends in consumption and death; a simple intestinal catarrh will result in inflammation and caseation of the mesenteric glands—a tabes mesenterica; or a simple dysentery, persisting in spite of the most [p. 248]approved treatment, causes proliferation and caseation of the endothelial cells of the follicles, terminating in that obstinate and intractable follicular ulceration which wastes the strength and wears out the life of the little patient.
A simple conjunctivitis of the globe often will be followed by ulceration of the cornea, giving rise to intense photophobia, and leave behind it opacities of that organ which remain a perpetual evidence of the scrofulous diathesis, if they do not shut out for ever the light from the eye. Or if it is the palpebral conjunctiva that is affected, the meibomian glands and follicles of the ciliæ become involved, destroying the lashes and leaving the lids raw and everted or inverted—a perpetual deformity. In short, there is no conceivable disease or injury occurring in what we may call the intensely scrofulous that does not have impressed upon it some one or more of the malign characteristics which we have spoken of as indicative of the scrofulous diathesis. But it is not probable that there is ever any special disorder or lesion which can be said to be caused exclusively by scrofula; or, in other words, there is no such disease as a specifically scrofulous one. Lupus, cold abscess, and particularly caseous glands, are especially attributed to struma, because they are often thought to make their appearance independent of any assignable cause; but as boils, eczema, impetigo, and numerous other affections of the skin and areolar tissue affect children who are not scrofulous, and equally independent of any known causes, the argument is not conclusive. Besides, all these affections occur sometimes in the non-scrofulous; and even caseation of a single inflamed gland quite often occurs in children who are weak or in ill-health, but who show no other evidences at that or at any other time of the scrofulous habit.
DIAGNOSIS.—The only affection likely to be mistaken for scrofula is congenital or acquired syphilis in its later manifestations. In this disease we see the same tendency to increased cell-production, the same tedious, slow, and intractable inflammations and ulcerations, which are characteristic of scrofula. And this apparent similarity has induced many persons to believe that scrofula is nothing else than syphilis in the second or third generations. But in congenital syphilis the lesions usually make their appearance soon after birth or are present at birth, and long before even hereditary scrofula begins to show its malign influence. In most cases, too, a history of syphilis can be obtained, and even when this is not obtainable a few inunctions or fumigations with mercury, in connection with a few large doses of iodide of potassium, will very quickly decide the question of diagnosis for us.
In the case of lupus, in which Erichsen admits there is no means of positively distinguishing the syphilitic from the so-called scrofulous varieties, the diagnosis is more difficult. But as this disease appears later in life than the more ordinary scrofulous manifestations—when, therefore, a history of syphilis can generally be obtained if there is one, and when there would almost certainly be also a history of scrofula if it existed—it would seem that the diagnosis even in this case cannot be so difficult. Diagnosis here, however, is of little consequence, since the treatment recommended for both forms is the same.
PROGNOSIS.—This of course depends upon the nature of the special lesion. The simpler lesions incident to childhood, such as glandular [p. 249]hyperplasiæ, catarrhs, eczemas, impetigoes, etc., usually do well under appropriate treatment and proper hygienic conditions. Diseases of joints, bones, mesenteric glands, etc. often terminate fatally or result in serious deformities and permanent impairment of function. Not infrequently diseases of the bones and articulations, attended with profuse and protracted suppuration, cause amyloid degeneration of the liver, kidneys, spleen, or other glandular organs, and, as a consequence, death. Catarrho-pneumonia in a scrofulous subject almost invariably causes phthisis sooner or later. Occasionally the caseated cellular exudation in the air-sacs remains quiescent for months, and even years, the patient remaining quite well except for a harassing cough during the winter months; but sooner or later the caseous mass will soften, the symptoms of active consumption ensue, with fever and wasting, and death closes the scene. Far more frequently, however, softening and suppuration follow swiftly upon the caseous degeneration, and the whole process occupies a period of only a few months. Tuberculosis especially runs a rapid course in these subjects, and while a few perhaps only develop tuberculosis of the lungs—in which case the duration of the disease may be a little longer—in by far the larger number there is a generalization of the tubercular process which puts a speedy end to their existence.
TREATMENT.—This may be most profitably discussed under two heads—prophylactic and therapeutic.
Prophylactic.—Scrofulous persons who are closely related by blood should be earnestly advised not to intermarry. We have so often seen the deplorable results upon offspring of such marriages that we cannot too strongly urge this upon the profession. Such persons should be frankly and clearly told what are most likely to be the consequences of such marriage, and all possible moral influences should be exerted to prevent them. The canons of the Church wisely interdict such marriages, but, unfortunately, its ministers seldom attempt to enforce them, or if they do their efforts are made ineffectual by the facility with which the marriage-rite can be obtained from civil officers in most of the States of the American Union. The medical profession can do more than any other class to diffuse knowledge and create a correct public opinion upon this subject, but, unfortunately, it too often neglects this important mission.
The children of scrofulous parents should be nursed (at the breast) longer than other children, so as to ensure an abundance of animal food during the first two years of life. Some advise scrofulous mothers not to nurse their children, lest they should imbibe the scrofulous taint through the milk. This fear is entirely groundless. We know of no reason why such a mother should not nurse her offspring, unless it be that it injures her. The child receives its scrofulous inheritance not through the mother's milk, but from the ovarian or spermatic cell. Milk can convey no disease or diathesis except on account of its deficiency in nutritive properties. If, therefore, there is any special reason why the mother should not nurse her infant on her own account, it may be well to turn it over to a healthy wet-nurse; but the temptation to give an infant raised on the bottle starchy foods prematurely is too strong generally to be resisted. The numerous infant foods advertised consist principally of starch, and young infants would infallibly starve on any or all of them [p. 250]if their venders did not always direct that they should be taken with a large quantity of cow's milk. If the circumstances of the parents do not enable them to obtain a wet-nurse, then good cow's milk constitutes the best food for infants until they have cut their canine and anterior molar teeth. The custom of weaning infants at a certain age in every case is a pernicious one. Some infants are as well developed as to their digestive organs at fifteen months as others are at thirty, and the eruption of the teeth may generally be taken as a safe guide as to that question. A moderate amount of food containing starch after the period indicated may be allowed, but always with a preponderance of animal food. It is not so much the starch that acts injuriously upon the nutrition of children as the excess of that substance; and if the food contains but little nutrition in proportion to its bulk, it is so much the worse. Even milk containing too little casein and fat in proportion to the watery elements may be perhaps quite as injurious as potatoes. And hence if the mother's milk should be poor in these elements, it ought to be supplemented with cod-liver oil or other animal fat in small doses.
A practice existed among the Southern slaves (and to some extent also among the whites) before emancipation which at first I was inclined to condemn until I saw the excellent effects resulting from it. Within an hour or so after birth a piece of fat salt pork or bacon was placed between the child's lips, and it was permitted to suck this at all times when not nursing. Tied to its wrist by a short string, so as to prevent swallowing it, this piece of pork furnished both nutrition and amusement to the infant for many hours while the mother was at work in field or garden. The children throve well on it, and thus treated we found them to be as well developed at twelve months as most other children were at twenty. It was doubtless due in part to this practice that there was so little scrofula among them.
An abundance of pure air is also a valuable factor in preventing the establishment of the strumous diathesis. Strict regard, therefore, should be had to ventilation, and overcrowding should if possible be avoided. Children over twelve months of age should not even be permitted to sleep with their parents, but should have in cold weather a crib, cradle, or other bed to themselves; and in warm weather they should be put to sleep in a net hammock, which is now so cheap as to be within the means of almost everybody. This will not only secure to them a better supply of air, but it will also prevent them from suffering so much from the heat, which is so potent a factor in the production of cholera infantum.
Bathing in proper season is also useful as a prophylactic. Sea-bathing especially has long enjoyed great credit as a remedy for scrofula, but we think this is often resorted to too soon and practised at improper times. In warm countries a bath of cold water may be taken every day in the year, but it should be given at the warmest hour of the day, not early in the morning. In all climates due regard should be had to the powers of resistance to cold and the promptness of reaction after the bath. If children remain cold and pale for a long time after the cold bath, the practice should be discontinued and tepid water substituted. In colder climates tepid bathing should be practised once or twice a day during the winter, and in summer a little lower temperature may be used. Bathing children under three or four years in the sea at any time is pernicious, [p. 251]both because the temperature is too low and on account of the fright which it always causes in these young children. After four years a child will take to the water almost as instinctively as a young duck.
Therapeutic.—Almost all of the so-called scrofulous manifestations belong to the surgeon, dermatologist, or oculist and aurist, and we shall therefore say nothing about the special and local treatment of these manifestations, but refer the reader to works upon these several departments of medicine. But as little success will be had in the treatment of these special disorders unless due regard is had to the general condition, and unless the local treatment is supplemented by constitutional measures, we shall briefly give some directions for this constitutional treatment of the scrofulous individual.
It is important in determining upon the proper treatment in any given case to bear in mind the division of the scrofulous into the two types of torpid or lymphatic and sanguine or erethistic already described. It is true that in many cases it is not easy to determine to which class a patient belongs, and many possessing some of the characteristics of both certainly cannot be referred to either. Still, in many cases the discrimination is easy, and then furnishes very clear and valuable indications as to treatment. Iodine (and its preparations) has since the time of Lugol, who first brought it into prominent notice, been regarded as a useful remedy in scrofula. But burnt sponge (spongia usta), which contained the iodides of sodium and potassium, had been used to dissipate goitrous and scrofulous swellings many hundreds of years before the time of Lugol. It is a valuable remedy in certain cases, and if it is falling into disuse it is probably for the want of proper discrimination in the selection of cases. In all cases in which there seems to be an abundant production of fat, and therefore in nearly all the cases of coarse struma where there is an indolent process of assimilation and disassimilation, iodine and its preparations will be found useful. Indeed, in the form of syrup of iodide of iron we have rarely failed with it to cause strumous enlargements of glands to disappear when the remedy was used soon after their first appearance. Of course, neither iodine nor any other medicine can have any effect in removing these enlargements after the glands have become caseous. While good results may be obtained with the syrup in all forms of scrofula, it is unquestionably in the sanguine and neutral types that it is most useful. It should be given in doses of 10 to 30 drops to children under five years of age, and to older ones ½ to 1 fluidrachm three or four times a day may be administered. We have given the latter dose to children four or five years of age for a long time, with the best effect upon their scrofulous manifestations, and without any injury whatever to their digestive organs.
In the torpid types preparations stronger in iodine should be used. Here Lugol's solution or iodide of potassium or sodium will be found very useful, either alone or in connection with the iron preparation above mentioned. Indeed, as in these cases it seems to be disassimilation that appears to be specially faulty, even very small doses of mercury in the form of bichloride or biniodide will be found useful. Donovan's solution may be prescribed in these cases along with the active preparations of iodine with good effect, or if the arsenic in that preparation is objectionable, one-fiftieth of a grain of bichloride or biniodide of [p. 252]mercury may be substituted. The mercurial should not, however, be continued longer than one or at most two weeks at a time, after which it should be suspended and the iodine continued.
Cod-liver oil, which is too indiscriminately prescribed in all cases, will be found to be of little use in the lymphatic types, if indeed it is not actually injurious; but in those cases with pale, thin skin, with deficient development of fat, and with small muscles—in short, those in which emaciation or delicacy is prominent—it is a most valuable remedy. It is almost surprising to see how rapidly ulcerations, caries, eczemas, catarrhs, etc. occurring in this class of subjects will disappear under the use of this medicine alone.
The hypophosphites and lactophosphates are also useful in this class of cases, especially where there is disease of bone or joints, in connection with the cod-liver oil. We have long been in the habit of using the following formula, which we have found very useful:
Rx. | Pulv. Acaciæ, | drachm ij; |
Ol. Amygdal. amar., | gtt. vj; | |
Syr. Calcii hypophosphit., vel Syr. Calcii lactophos., |
fluidounce iv; | |
Ol. Morrhuæ, Ft. mist. |
fluidounce iv; |
S. Teaspoonful to tablespoonful three times a day according to age. Syrup of iodide of iron may be added if desirable, though we prefer to give this by itself.
Gentle exercise, passive or active, pure air, well-ventilated sleeping apartments, a generous diet—in which wholesome animal food should predominate—and bathing are of course as necessary and as useful in the treatment as in the prevention of the scrofulous diathesis.
Alkalies should be given in all cases in which we are trying to dissipate enlarged lymphatic glands, for the reason that caseation of these glands occurs because of insufficient alkalinity of the blood to effect reduction of fat, and because also the strumous almost always suffer from excessive acidity of the gastric and other secretions. When the iodides of potash or soda or the hypophosphites of lime and soda are given, the additional administration of alkalies may not be necessary; but if not, bicarbonate of sodium or potassium (which have long enjoyed a good reputation in the treatment of struma) should be added to the other remedies.
Since the appearance of Niemeyer's Handbook of Clinical Medicine the proper treatment of scrofulous glands that have undergone the caseous degeneration has been a moot question. Some recommend the ablation of these glands by the knife, some advise spooning out the caseous matter through a small opening, while others prefer to await the natural process of softening and the discharge of the caseous matter by suppuration. There can be no question that the removal of these glands by the knife, when this can be done without serious risk, will leave behind a less unsightly scar, and will be attended with less fever and consequent deterioration of the general health, than usually attends suppuration. Spooning out the caseous matter will perhaps leave no extensive cicatrix, but we can never be sure that by this operation we have removed all the caseous matter, and it must certainly be more painful than the knife. Mothers will generally object to either of these [p. 253]operations, and as the risks of infection by absorption of the caseous pus during the suppurating process do not seem to be very great, it is perhaps best to leave these glands to nature, unless the vitality of the patient is so low as to give reasonable ground for fear that the child may succumb to the effects of the natural process. If any surgical interference is deemed necessary, we are decidedly in favor of removing the caseous gland entire by the knife.
About a half century ago Mr. Abraham Colles, who had just resigned the professorship of the Theory and Practice of Surgery in the Royal College of Surgeons in Ireland, the duties of which, in the opinion of the college, he had discharged for thirty-two years in an "exemplary and efficient manner," wrote the following introductory paragraph to his remarkable chapter on "Syphilis in Infants:" "Perhaps there is not in the entire range of surgical diseases any one the contemplation of which is more calculated to arrest our attention or to excite our interest than syphilis infantum."
Although it was not then, and is not at the present day, strictly relegated to the domain of surgery, hereditary syphilis, like its parent disease, was generally treated of by the practitioner of that branch of medicine. And yet in the great majority of instances the management of such cases, especially as regards their family relations, the relations of husband and wife, the management of the latter during pregnancy, the delivery and subsequent care of the child, the necessary attention to the safety of other members of the family—in fact, all of the most weighty responsibility—falls upon the ordinary medical attendant. It is therefore in every way proper that the condition should receive some notice in a system of general medicine.
A proper presentation of the subject of hereditary syphilis involves a consideration of the vexed question as to the mode by which the disease is conveyed from parent to offspring. That it may be so transmitted has been generally believed since the doctrine was first announced by Torella at the end of the fifteenth century; and the facts in its support are so numerous and convincing that, in spite of a few distinguished opponents—among whom John Hunter was the most conspicuous1—it has been unhesitatingly accepted by the profession down to the present day. As regards the manner of transmission, however, controversy has been and still is rife. Opposing theories have been constructed and ardently supported, differing radically as to essential points, often resting upon exceptional or anomalous, and still oftener upon imperfectly observed, cases.2
1 Works of John Hunter, vol. ii. p. 383.
2 Parrot, in a clinical lecture on syphilitic abortion (Le Progrès Médicale, Nov. 3, 1877, p. 798), says: "The infection of children was known, but its true origin was not suspected. The belief of Gaspard Torella (1498) and Matthioli (1536) that it came from the nurses through the milk was generally accepted."
According to Diday, Paracelsus (1529) was the first to plainly state the heredity of syphilis: "Fit morbus hereditarius et transit a patre ad filium."
Others attribute the original announcement to Augier Ferrier (1553), and it seems certain that he was first to specify the three modes of infection of the product of conception: "La semence du père, celle de la mère, et la contamination de la mère durant la grossesse."
Fallopius in a posthumous treatise on the Mal Français (1566) adds the authority of his name to this view: "Præterea videbitis puerulos nascentes ex foemina infecta, ut ferant peccata parentum, qui vedentur semi cocti."
Ambroise Paré also acquiesced in the theory, saying, "Souvent on voir sortir les petits enfants hors le ventré de leur mère, ayant ceste maladie, et tost après avoir plusieurs pustules sur leur corps; lesquels étant ainsi infectés, baillent la vérolle à autant de nourrices qui les allaictent."
Subsequently, Mauriceau, Boerhaave, and Astruc sustained the same view, which, with the single exception of Hunter, had no prominent antagonist.
It was not, however, until the eighteenth century that it was described with any attempt at detail or exactness by Rosenstein, and his essay is loaded with errors. It was in the foundling hospitals of Paris at the end of the last century, in the wards of Salpétrière and Bicêtre, and in the hospitals of Vaugirard and in the Capucin convents of the Rue Saint Jacques, where pregnant women and nurses attacked with syphilis were admitted, that methodical and trustworthy observations were made (1780-1810) by Colombier, Despenières, Doublet, Mahon, Cullerier, and Bertin. Since then the history of the disease has been the history of syphilis itself.
A full consideration of these, or even a recapitulation of the respective arguments pro and con, would far exceed the limits allotted to the present article, and we will confine ourselves simply to stating the questions which most nearly concern the practical physician, and the conclusions which the accumulated observation and experience of the profession seem to justify. The points bearing upon the general subject of hereditary syphilis which exercise an important influence upon advice or opinions of the utmost gravity as regards the happiness and well-being of the individual or family may be enumerated as follows:
1. Is syphilis in all its stages transmissible (a) to the wife or husband, (b) to the offspring? Or, in other words, is it ever proper to consent to the marriage of a person who has had syphilis? If so, under what circumstances?
2. By what means or through what channels can the disease of the parents reach the child?
3. What are the pathology and symptoms of hereditary syphilis?
4. What is the treatment—(a) prophylactic, applied to the parents, and (b) curative? We may now take these up seriatim.
No more important questions can be submitted to a medical man than those pertaining to the marriage of syphilitics. Involving as it does the welfare of many individuals, modifying or fixing the conditions or circumstances of one or more lives, his opinion should be exceptionally definite and well grounded. The responsibility of advising or consenting to the marriage of a person who has once had syphilis is undoubtedly great; the responsibility of prohibiting it is, however, no less so. Matrimony is the natural condition for the majority of people. Enforced celibacy, especially in males, brings with it not infrequently a long train of attendant evils, moral and physical. It will not do to assume that professional duty is properly discharged by telling all patients to be on the safe side and to remain single for fear of inoculating wife or offspring, unless it can be clearly shown that there is a definite and unavoidable risk in every case, which continues throughout life.3
3 "The surgeon who, on account of past syphilis, forbids marriage to an otherwise [p. 256] eligible man must remember that he forbids it at the same time to some woman, who, possibly, if well informed as to her risks, would willingly encounter them.... Respecting a malady so common as syphilis, while it is often our duty to warn, it is also not unfrequently our duty to encourage" (Mr. Hutchinson, preface to the English translation of Fournier's Syphilis and Marriage, p. vii.)
There are two distinct methods of arriving at an answer to the question under discussion: first, by considering the probabilities in regard to the essential nature of syphilis; and, second, by carefully weighing the clinical evidence in the matter. It seems evident that belief in any particular theory of syphilis assigning it to this or that class of disease must have an important influence in determining the opinion which is held as to its curability, or at least as to its indefinite transmissibility.
If the late symptoms, the so-called tertiary outbreaks of the disease, are held to be evidences of the presence in the system of the specific virus, which has simply remained for a long period, perhaps for many years, latent or quiescent, and which is thus again manifesting its power; if syphilis is believed, accordingly, to be a practically unlimited disease, conforming to no known law as regards its duration, corresponding to no other infectious or contagious malady in having a period of termination—more or less delayed perhaps, or more or less indefinite, but still invariably present—at which time either the particular poison or the susceptibility of the system to its influence has become exhausted;—if tertiary syphilis, in other words, is regarded as simply a continuance or recurrence of the disease, differing in no essential respect, except as to the particular tissues involved, from the same disease in its early stages, it is difficult to see how marriage can ever be conscientiously recommended to a person who has once contracted it.
It is unfortunately true that in no given instance is it safe to assure a patient of further complete immunity from the disease. In any case, however mild in its course or under whatever treatment, there is always an element of doubt as to the development of subsequent symptoms. The probability of their appearance may be reduced to a minimum, the character of the case and the thoroughness of the treatment may both seem to give assurance that a cure has been completed, and yet both patient and physician may be mortified and annoyed by an outbreak of tertiary lesions. This, I think, would be admitted by every one of large experience with the disease, and indeed furnishes the chief argument to those who deny or are sceptical as to its curability. If, then, it were impossible to predict with any sort of certainty that the contagious and highly transmissible stage of syphilis would terminate during the life of the individual, it would manifestly be unwise to permit marriage, with its risk of inoculating the innocent partner and the consequent double risk to the offspring.
If, however, syphilis is, as taught by Mr. Hutchinson,4 one of the exanthemata, having, like them, a period of outbreak, a period of efflorescence or eruption, and a period of subsidence, and followed, like them, by certain non-contagious sequelæ, which we call the tertiary symptoms, but which are merely relapses or degenerations of parts affected during the secondary stage, it becomes evident that the risk of transmission to wife or husband or children after the lapse of a certain interval becomes greatly reduced or almost nil. No one thinks of forbidding marriage on [p. 257]account of a previous attack of small-pox or scarlet fever or typhoid fever, even although there are unpleasant consequences which sometimes follow these diseases.
4 The London Lancet, Feb. 5, 1876; Reynolds's System of Medicine, Am. ed., p. 423.
Or if another and still more plausible theory of syphilis is adopted, and we look on the tertiary period as one of contraction or obliteration of lymphatics due to long-continued irritation by the new cell-growths of the secondary stage, which from the very onset affect those vessels, our views will be materially modified. During the primary period, then, when the new cells, which either constitute the poison of syphilis or are its carriers, are accumulating at the site of original inoculation, constituting the induration of the chancre, or are slowly finding their way into the general system through the lymphatic vessels, proliferating in their walls and thickening and hardening them, or during the secondary period, when they are rapidly multiplying in all the tissues of the body, the risk of inoculation or transmission would be manifestly great. When, however, by destructive metamorphosis and degeneration, either with or without the aid of drugs, they have been eliminated from the body, the contagious element disappears with them; and although here and there throughout the body some important lymphatic trunk may have undergone irreparable injury, and may have been contracted or obliterated, permitting of the accumulation of waste products until the node or gumma or tubercle which we call a tertiary symptom makes its appearance, yet the disease has lost much of its terror, and has become dangerous only to the patient himself.5
5 Clinical Lectures on the Physiological Pathology and Treatment of Syphilis, by F. N. Otis, M.D.; Syphilis, by V. Cornil, Am. ed., 1882, pp. 17-27.
These theories are only alluded to by way of elucidation of the statement that belief in one or the other of them has an important bearing on the relation of syphilis to marriage, and because, whichever is thought to be the most plausible, they equally lessen or altogether do away with a certain proportion of the danger formerly thought to surround the marriage of a syphilitic even after a most protracted interval. It is not necessary to accept either the one or the other implicitly. The essential point is the recognition of the fact that modern syphilographers, as a rule, regard the tertiary or late symptoms as indicative of damage done during the active period—as relapses or sequelæ, and not as fresh outbreaks, of a highly contagious and transmissible disease. Their time of appearance, their entire want of symmetry, their non-contagiousness, their non-inoculability, all favor this view, and we may now see what evidence corroborative of it may be obtained from clinical facts.
It will be necessary, in the first place, to admit that there seems to be but little doubt in the minds of most syphilographers that in rare instances syphilitic children have been born to parents who had long passed the limits of the secondary period. At least the great majority of writers upon this subject speak confidently of the exceptional occurrence of such cases, and assert that syphilis may be transmitted during any of its stages.6 If, however, we come to look for positive evidence in this respect, we will find very little that is entirely satisfactory. Cases are reported, to be sure, in which eight, ten, twelve, or even fifteen or twenty, years after the primary sore, syphilitic patients have become the parents [p. 258]of children who showed unmistakable indications of the disease. When we examine the history of the cases, we find usually that many important points are omitted without which it is impossible to be certain of its true character. Were both parents originally infected? If not, has a recent case of syphilis occurred in the one who at first escaped? If they were both diseased originally, has either been subsequently re-infected?—a much more frequent accident than has been commonly supposed.7 On applying these tests to the cases in question it will be found that few if any of them are thoroughly convincing. Kassowitz's observations,8 made, it must be remembered, upon persons with whom no mercurial treatment had been employed, seemed to show that the average limit of transmissibility was about ten years, after which time healthy children began to be born. His observations were, however, incomplete in many respects, and, like all such investigations, are of course open to the suspicion of intentional deception on the part of the patients.9 Even these cases, however, show unequivocally, as do all which have ever been recorded, the steady diminution of the transmissive power under the influence of time alone.
6 Belhomme et Martin, Traité de la Syphilis, p. 413.
7 Cornil, op. cit., p. 20.
8 Die Vererbung der Syphilis, Wien, 1876.
9 See also Hutchinson, British and Foreign Med.-Chir. Rev., Oct., 1877.
I have said that the majority of writers seem to have no doubt of the long continuance of this transmissive power in rare cases. There are, however, a few notable exceptions. Fournier, whose immense experience and acuteness of observation entitle his opinion to the utmost consideration, says that in cases of paternal heredity the duration of the force of transmission never exceeds at the maximum three or four years.10 In no case of the many hundreds he has observed has he known a syphilitic father to infect a child—the mother being healthy—at a later period than the one mentioned. And he is equally positive that the gradual diminution and final extinction of the syphilitic reaction of the parents upon the children is a veritable pathological law, "absolutely demonstrated."11
10 Syphilis and Marriage, Am. ed., p. 87.
11 Op. cit., p. 88. Of course when both parents are diseased a somewhat longer period of activity is to be expected for the poison.
M. Mireur, a careful and accurate observer, records12 a striking instance in which in the history of a couple, both syphilitic and untreated, eight pregnancies occurred. The first resulted in abortion at fifth month; the second, in abortion at seventh month; the third, in a stillbirth; the fourth, a syphilitic child dying in one month; the fifth, in a syphilitic child dying in forty-five days; the sixth, seventh, and eighth, in living, healthy children. To me the most interesting fact in the whole relation is that during a portion of the time, and immediately after the last three pregnancies, which resulted in the birth of healthy children, both husband and wife manifested grave tertiary syphilitic symptoms—gummata, tubercles, ulcers, etc. This is direct evidence of the strongest kind in favor of the view that syphilis ceases to be transmissible by heredity at the end of a certain period, as we know that it ceases to be contagious or inoculable.
12 Essai sur l'Hérédité de la Syphilis, Thèse de Paris, 1867, p. 91.
M. Ricord long ago,13 and even before him Astruc and Doublet,14 had [p. 259]promulgated the same theory, Ricord asserting that in the tertiary stages the only effect of the disease upon the children was so to modify their organization and temperament as to expose them to developments of a scrofulous character—a view of the relation between syphilis and struma which has been so ably supported in our own day by Professor Gross.
13 Traité pratique des Maladies vénériennes Paris, 1838, p. 644.
14 Legendre, Nouvelles Recherches sur les Syphilides, 1841 (quoted by Belhomme et Martin).
M. Bazin also15 denies absolutely that tertiary syphilis is any more transmissible than it is communicable in other ways, although he fails to give his reasons for this belief.
15 Leçons sur les Syphilides, 1859, p. 35.
Hill and Cooper state16 that the transmissive power continues as long as the secondary eruptions are present, but usually ceases when the tertiary stage is reached.
16 Syphilis and Local Contagious Disorders, London, 1881, p. 62.
Van Buren and Keyes17 believe that fathers with tertiary syphilis certainly, as a rule, procreate non-syphilitic children; and in speaking of the fact that when the mother has syphilis the child is generally infected, they except the later tertiary stages.
17 Genito-urinary Disease and Syphilis, 1874, p. 521.
Bumstead18 and Taylor say that without mercurial treatment the danger of transmitting the disease to offspring usually persists up to the fourth year of syphilitic contagion.
18 Venereal Diseases, 1879, p. 739.
Mr. Lane says:19 "It is certainly the rule that when the parents have fully reached the tertiary stage the children born to them are free from all signs of syphilis."
19 Lectures on Syphilis, London, 1881, p. 65.
Mr. Hutchinson says:20 "It is almost an acknowledged law that parents in the late tertiary stages do not transmit taint."
20 The Med. Press and Circular, Aug. 2, 1882, p. 85.
It will be seen from the foregoing extracts, which might be greatly multiplied,21 that there is a strong tendency on the part of many authors to limit more or less strictly the period of transmissibility of syphilis even when the disease is allowed to progress without treatment. As to the facts that it becomes milder with time, both in parents and offspring, that it ceases to be conveyed from husband to wife or vice versâ, that with each succeeding year after the termination of the secondary period the chances of escape of the product of conception increase in a rapidly augmenting ratio,—there is no difference of opinion whatever. Neither is it seriously disputed that the length of time during which the disease remains active, as well as the degree of its activity, may be markedly and beneficially influenced by the administration of mercurial treatment. Under proper medication patients who have rashly or disobediently married in the height of the secondary period have been enabled to escape the danger of transmission either to spouse or offspring—have, in fact, had children born healthy and who never subsequently manifested any symptoms of the disease.
21 M. Diday, Traité de la Syphilis des Nouveau-nés, Paris, 1854, p. 183; M. Bertin, Traité de la Maladie vénérienne chez les Nouveau-nés, Paris, 1870, p. 142; M. Bazin, op. cit., p. 164; M. Roger, L'Union Médicale, 1865, t. i. p. 147 (quoted by Fournier).
I may add that my own experience seems to confirm the views which have thus been set forth. I have notes of all my cases occurring in private practice in a large city—some of them, I regret to say, among personal friends or acquaintances, some of them in our own profession—and have repeatedly [p. 260]given permission to men to marry or to resume marital relations after three years or three years and a half of mild mercurial treatment, to which during the last six months or a year had been added iodide of potassium. In many instances healthy children have been born: in none, so far as I know, has the wife or mother been directly infected. There have been a few doubtful cases in which premature deliveries or stillbirths have occurred, but in nearly every such instance there seemed to be other and entirely competent causes for the accident; and in none of them, as I learned from the father or from the obstetrician in attendance, were the children the subjects of unmistakable syphilitic symptoms.
As to the exact time at which it is safe to permit marriage, and as to the proper treatment before and after that event, it is hardly possible in an essay like this to enter into many details. Yet so much is involved in the answer to our first question that it may not be altogether out of place here to indicate briefly the views of the writer as to general methods of treatment. This is the more proper because in every case of suspected syphilis in a new-born child, in every case of threatened or actual abortion or miscarriage in the wife of a man who has at some time in his life had syphilis, these questions will present themselves, and the answers to them will greatly influence not only the diagnosis and prognosis, but even the treatment, of such cases.
1. In the first place, then, the diagnosis of syphilis should have been assured. No venereal sore can with certainty be pronounced to be syphilitic before the occurrence of general constitutional symptoms, either the early cutaneous eruptions or at least the general glandular involvement. Treatment begun prior to these developments leaves the whole case open to the suspicion of mistaken diagnosis.22
22 "It is unsafe to predict confidently that any venereal ulcer, even a soft sore attended with suppurating bubo, will entail no further consequences. There is a strong probability that an indurated sore will prove infecting; and there is a probability, though not nearly so strong, that a soft suppurating sore will not; but exceptions to both these general rules will be met with, and there is really no absolute proof of the infecting nature of any given sore but the fact of infection itself" (Lectures on Syphilis, James S. Lane, London, 1881, p. 23).
2. The drug which should at once be begun when the character of the case is fully recognized is mercury in one of its various forms. It may be given by the mouth, by inunction, by vaporization, by hypodermic injection, according to the preferences of the physician or patient; but, however administered, it should be given in sufficient quantity—i.e. in each case the full physiological dose of that particular patient should be employed. To ascertain this the amount used should have been gradually increased until commencing symptoms of salivation are produced, when it should be diminished about one-half.
3. The quantity which has been thus determined should be given continuously, or stopping only for the management of intercurrent complications, for at least eighteen months. If during this time new syphilitic symptoms make their appearance, the dose should be temporarily raised until they have vanished, when it should be brought down again to the original amount.
4. At the end of eighteen months or two years small doses of iodide of potassium should be added to the mercurial, and this mixed treatment should be persevered in for six months or a year longer, or should be [p. 261]still further prolonged if during that time any evidence of syphilis is seen.
5. At the end, then, of from two to three years, if no symptom has been seen for six months or a year, treatment may be stopped and the patient kept under observation for a year; and if during that time no symptom develops he may consider himself as in all probability cured. Any course of treatment less thorough than this should be set down as insufficient to afford any reasonable presumption of further immunity from the disease.
There is evidence to prove, on the other hand, that this plan of treatment, rigidly carried out, results in the majority of cases in curing the disease, or at any rate in putting the patient in such condition that he may with safety marry and may expect to have healthy children.
To recapitulate: Syphilis after a certain period, not extending much over four years where the disease is allowed to run its own course, and probably much reduced by treatment, ceases to be a contagious disease; and at about the same time or some time after loses, in the majority of cases, its capability of being transmitted.
As there are probably exceptions to the rule that this power of transmission disappears spontaneously within any specified time, it is never safe to trust altogether to the unaided efforts of nature, but a vigorous and sufficient specific treatment must be employed.
Given, however, the lapse of a sufficient time—say from three to four years as a minimum—the history of a proper and continuous plan of treatment, and the absence for a year or more of any specific symptoms whatever, and the risks of marriage are so reduced as probably to warrant a careful physician in permitting it.23 And conversely, of course—and this constitutes the reason for introducing the foregoing matter into a paper on hereditary syphilis—in any doubtful case where such a history can be elicited, and where all these precautions have been observed, it is improbable that any taint of syphilis has been transmitted.
23 This refers, of course, to an ordinary case of syphilis. If the symptoms have been unusually grave, if the deeper tissues or the viscera have been seriously involved, if cerebral or spinal complications have occurred, the situation is of course much more grave, and no step should be taken without the most thoughtful deliberation. The work of Fournier already alluded to (Syphilis and Marriage) furnishes an admirable guide under these circumstances.
Mr. Frederick Lowndes, surgeon to the Liverpool Dock Hospital (Lancet, July 8, 1882), says: "Each case must be judged on its own merits. When the constitution is good, and there has been sufficient specific treatment, marriage may be permitted within a much shorter period than M. Fournier suggests, and with safety. Syphilis alone and syphilis combined with scrofula are two very different foes to contend with, and if our patient be of a scrofulous temperament a delay even longer than M. Fournier's may be desirable."
He quotes Dr. Thomas Edward Beatty in an address at the annual meeting of the British Medical Association at Leeds in 1869: "Mercury given to the man when first diseased would, I firmly believe, have prevented this terrible calamity—i.e. the syphilitic infection of the wife; and I would now humbly suggest to all who undertake the treatment of venereal disease that if they have a certainty that their patients will remain celibate all their lives, they may heal up their sores and dispel their eruptions and sore throats in any manner they like, but that they have no right to expose the pure, innocent, high-minded females of society to contamination by marrying men treated without mercury."
Beyond this in positiveness of assertion it is not safe to go. There may be exceptions to these as to most other hygienic or therapeutic rules, but they will surely be of excessive rarity.
[p. 262]Before considering the methods by which syphilis can reach the child from one or the other of its parents, it may be well to mention the modes in which they can infect each other.
The father can derive syphilis from the mother only in the usual way—i.e. by contagion through a breach of surface permitting of the direct absorption of the poison, the development of the disease being attended by the usual phenomena—chancre, lymphatic enlargement, skin eruptions, etc. The woman may—and in the vast majority of cases does—acquire the disease from the husband in a similar manner. But there seems to be good reason for believing that she may also become infected through the medium of the child, who receives its syphilis directly from the father, the mother up to the time of conception having escaped contagion. More than this, it appears to be highly probable24 that no woman ever bears a syphilitic child and remains herself absolutely free from the disease.
24 The strongest argument against the theory that every woman who has had a syphilitic child has herself been infected lies in the existence of cases like the following, several of which have been observed: The wife of a man having active but untreated syphilis gives birth to one or two syphilitic children, she herself developing no symptoms. Later, the husband is placed on mercurial treatment. She then conceives and gives birth to a healthy child. He stops treatment, and she again bears a syphilitic child, which on his resuming his mercurial course is followed by another healthy infant. Such cases certainly indicate that the syphilis of the mother, if any exists, is incapable of transmission to the child, as the effect of the treatment of the father is too direct and unvarying to admit of doubt.
The existence of this form of infection—syphilis by conception—has been vehemently denied by many authors25—by some on the theoretical grounds that as the essential elements or carriers of the syphilitic virus have been shown to be cells or protoplasmic particles, and as, after conception, the embryo is supplied with serum, but not with cells of any kind, it is impossible that syphilis can be conveyed either to or from it;26 by others on reported observations of numerous cases in which mothers who have been delivered of syphilitic children have shown no evidences of the disease.
25 Kassowitz, Von Baerensprung, Bidenkap, and others.
26 Bumstead and Taylor, op. cit., p. 742.
It would appear, however, that, setting aside arguments based on theoretical considerations, the weight of clinical evidence is altogether in favor of the frequent, if not the invariable, contamination of the mother through the medium of the foetus. No physician of large experience in this class of cases can fail to have seen some in which the husband, having had syphilis and having married after an insufficient interval or an imperfect course of treatment, has infected his wife with the disease, although at the time no discoverable symptom is to be found upon his body—no abrasion, sore, mucous patch, no lesion of continuity or suspicious point of any description. An equally careful inspection of the woman will also in such cases be attended by negative results—no initial lesion, no spot of induration, no adenopathy being at all discoverable—and yet she will be found with unmistakable evidences of constitutional syphilis.27 There is a clue to all such cases which will immediately resolve the difficulty. In every instance, providing that no mistake has [p. 263]been made and that both husband and wife are really free—the one from any contagious lesion, the other from any evidence of a present or previous primary sore—it will be found that pregnancy has occurred; that the woman has either been delivered of a syphilitic child or has had an abortion or miscarriage at some time before the outbreak of the symptoms of syphilis. Although I am firmly convinced that this is a statement of facts based upon careful clinical observation, and although this view has received the unqualified endorsement of no less an authority than Fournier,28 it is yet strongly combated by many excellent authorities. They say in reply to the above arguments that the reported cases are open to just criticism, that trifling and unnoticed lesions of the father—chafes or abrasions almost microscopic—suffice to transmit it on his part; while as an explanation of the supposed absence of the primary lesion in the mother they plead the well-known difficulty of discovering it in women under any circumstances.
27 For a typical case see Colles's Works, New Sydenham Society, London, 1881, p. 253. From that date to this hundreds of such cases have been observed, and it would be idle to refer to them.
28 Op. cit., pp. 26-30. He confesses to complete ignorance as to the precise method by which this contagion takes place, whether by poisoning of the fecundated ovule at the moment of conception (the theory of Von Baerensprung) or by exchanges of the utero-placental circulation, and regards the various hypotheses upon these points as without practical value.
Another argument, however, which seems to me to be unanswerable lies in the application to the case in question of the well-known "law of Colles," which from the date of its first enunciation by its distinguished expounder in 1837 down to the present day has been found to be absolutely without exception. I know of no other statement in reference to disease which is at once so sweeping and comprehensive in its bearings and so completely substantiated by clinical experience. It may be given in his own words: "One fact well deserving our attention is this: that a child born of a mother who is without obvious venereal symptoms, and which, without being exposed to any infection subsequent to its birth, shows this disease when a few weeks old,—this child will infect the most healthy nurse, whether she suckle it or merely handle and dress it; and yet this child is never known to infect its own mother, even though she suckle it while it has venereal ulcers of the lips and tongue."29
29 The Works of Abraham Colles, edited by Robert McDonnell, the New Sydenham Society, London, 1881, chap. xiii. p. 287.
As to the absolute and unvarying truth of this law there is no excuse for a shadow of doubt. To quote Mr. Hutchinson: "It has received the assent of every authority who has written on the subject since it was announced. It has attracted attention both at home and abroad, and I am not aware that a single exception to it has been recorded.30 We have all of us seen chancres on the nipples of wet-nurses. They are, indeed, not very infrequent. We have, however, none of us seen such on those of the mothers of infected children. Let us remember that it is very unusual to put a syphilitic infant out to wet-nurse—a thing which no prudent physician would ever permit—and that, probably, for one so nursed a hundred are suckled by their mothers, and we can appreciate the weight which this entire absence of proof that mothers ever suffer [p. 264]bears. It amounts, I think, to all but proof that they are absolutely insusceptible."31
30 The cases of Cazenave (1847), Cocchi (1858), Müller (1861), Ranke (1878), Guibout (1879), Scarenzio (1880), and Zingalès (1882), are defective in important particulars, the first two so much so as to render them valueless. Ranke's case is by far the strongest yet recorded, but lacks fulness and rests upon his unsupported testimony. (See Hill and Cooper, op. cit., p. 55, and Nouveau Dict. de Méd. et de Chir., vol. xxxiv. p. 687.)
31 Dr. J. N. Hyde suggests (Archives of Dermatology, April, 1878, p. 103) that "the full weight of Colles's law is to be estimated in connection with the question whether the child whose hereditary syphilis is derived from the mother exclusively is capable of infecting its healthy father; and if no instance of this latter can be adduced a higher law becomes defined—viz. that the child whose hereditary syphilis is transmitted by one parent only is incapable of infecting either." He explains this hypothetical immunity, in which he seems to believe, by saying that "it is probably due to the fact that the syphilis-bearing cell-element cannot readily be implanted upon the soil from which it sprang—a fact illustrated by the infecundity of consanguineous marriages and the non-auto-inoculability in general of the primary lesion of syphilis." When, however, we exclude the large number of cases in which the father is already syphilitic, and remember that in the others the contact between him and the child is slight, infrequent, and fleeting—being usually limited to an occasional kiss or caress—it does not seem strange that no instances of such infection have been reported; nor does the fact seem at all worthy of being made the foundation of a general law.
There can be but one rational explanation of these facts—viz. that the mothers who have thus acquired an immunity have done so by first acquiring the disease. It may, in those cases where no secondary symptoms appear, be in a modified form, due, as suggested by Mr. Hutchinson, to some heteromorphism or alternation of generations on the part of a specific fungus, or to the excessively small quantity of the poison which finds its way from child to mother, or to some other obscure cause with which we need not now concern ourselves. It is, at any rate, no more mysterious than the protective influence of vaccine in small-pox, and when one is rationally explained doubtless the other will be found to be closely allied in its mode of action. The fact which interests us at present is that it is in the highest degree improbable that anything but some form of syphilis itself could afford this entire protection, and that it is not in the least unreasonable, but, on the contrary, logical and consistent with all the known facts, to suppose that while in some cases no observable symptoms might be produced, in others where the cause was the same, but more active, or the powers of resistance less, the usual constitutional phenomena would be developed.
We may conclude, then, that the husband may infect his wife—(1) In the usual manner or by direct contagion; (2) through the medium of the child, or at any rate by the production of conception.
The theory upheld by Von Baerensprung, that the syphilis of the mother is imparted to her at the moment of impregnation, the disease being impressed upon the fecundated ovule, does not materially conflict with the above views, conception being in either case the essential factor, but in the latter instance the intervention of the foetus itself not being necessary. It has to support it the clinical fact that in those cases where syphilis appears during pregnancy the outbreak of symptoms occurs at about the ninth or tenth week after the date of conception, or a period which closely corresponds to that of the appearance of general symptoms after exposure to ordinary contagion—allowing about three weeks for the so-called incubation of the chancre and six weeks for the secondary incubation.32
32 This has been shown not only by Von Baerensprung (Die Hereditäre Syphilis), but also by Diday, whose observations were intended to prove the possibility of syphilis being derived from the child by the mother—"choc en retour." In 24 cases the period at which the first eruption appeared in the mother averaged sixty-five days after conception; only once did the first signs appear after the fourth month of pregnancy.
[p. 265]It may also be said to be rendered probable by the following line of argument:
Colles's law, which is without exception, demonstrates that every woman who has had a syphilitic child has been herself infected, even if she has had no observable symptoms;33
Cases are recorded, however, in which a woman having given birth to one or more syphilitic children, and therefore herself syphilitic, bears healthy ones in consequence of specific treatment administered to the father before and during the period of conception, she remaining untreated;34
The determining cause, therefore, of the syphilis of the child is not the syphilis of the mother, but the condition of the fecundating germ of the father; and, as a corollary,
The determining cause of the syphilis of mothers in whom the disease follows conception is not by infection from the foetus through the utero-placental circulation35 or otherwise, but is the diseased male procreative cell which becomes blended with the female ovule.36
33 It is obviously no explanation of the law of Colles to say that "it would seem to indicate that the escape of the mother is due to some occult, undiscernible change in her system" (Bumstead and Taylor, op. cit., p. 745).
34 See foot-note, p. 262.
35 On account of the absence of cellular elements in the fluid interchanged.
36 An elaborate paper by Fraenkel (Archiv für Gynaekologie, 1873, vol. v. p. 1), based on twenty-one cases of childbirth, was written to prove by the condition of the placenta that direct infection of the child by the father was possible without the participation of the mother, and that when the latter became infected it was through the medium of the child (choc en retour). In fourteen of his cases the specific change in the placenta began in the foetal portion or affected it exclusively. These cases, however, as analyzed by Hill and Cooper (op. cit., p. 57), are altogether defective in important particulars. Of course to sustain his theory there should be indubitable evidence that the fathers were syphilitic, and that the mothers were not so. So far from this being the case, but one of the fathers was known to be syphilitic, while two of the mothers had evidences of constitutional syphilis at the date of childbirth, and nine others had markedly suspicious histories. The lesions of the placenta which are thought to be syphilitic consist in the development of papular or gummatous growths which give rise secondarily to inflammatory troubles affecting either the placenta itself or the placenta and the uterine mucous membrane.
If the premises are admitted the conclusions seem irresistibly to follow.
There is no proof whatever that the semen of a syphilitic man is contagious or can transmit the disease in any but the way above discussed. On the contrary, it has been shown experimentally37 that it is entirely innocuous and non-inoculable.
37 Mireur, Annales de Derm. et Syph., 1876, p. 77.
All other theories as to methods of contagion are so entirely hypothetical and unsupported by trustworthy evidence that we can afford to disregard them.
We may now consider the ways by which syphilis reaches the child, and they may be broadly classified into—
1. By descent from the father.
2. By descent from the mother.
3. By direct infection.
As a matter of course, the influence of the father upon the child, so far as regards heredity, ceases at the moment of conception; or, to be more exact, no subsequent condition of the male parent, no development or acquirement of disease, can exert any further effect. That the existence of active syphilis in the father may result in the transmission of the malady to the child can hardly be doubted. To be sure, there are numerous [p. 266]examples of cases where both wife and child have escaped though the husband showed at the time of conception very active secondary lesions. These, however, are exceptions, and do not in the least invalidate the rule that it is possible for a father to hand the disease directly to his child. There is no other possible interpretation of the cases already alluded to in which successive pregnancies in the same woman alternately resulted in healthy or in syphilitic children according as the father was or was not kept under specific treatment. Other arguments might be advanced, but that seems to me conclusive.
The relative effect of paternal as compared with maternal influence may be considered after we have described the latter.
Descent from the mother may occur theoretically in consequence of—
1. Infection of the mother previous to conception.
2. Infection of the mother at the moment of conception.
3. Infection of the mother during the period of utero-gestation.
As to the first of these methods of transmitting the disease there is little if any difference of opinion. Even those who claim the most for paternal influence38 include among the conditions which may give rise to syphilis in the child disease of the ovule, and it may be stated as incontrovertible that recent or active syphilis in the mother at the time of conception will almost certainly be followed by syphilis in the child. As a rule, women who have borne syphilitic children, even when they do not give unmistakable evidence of the disease, fail in health, become anæmic, and often develop glandular or osseous swellings which, according to Zeissl,39 are only relieved by antisyphilitic treatment. The bearing of Colles's law upon the alleged immunity in many instances of the mothers of syphilitic children has already been shown (p. 264); and there is other evidence, not perhaps so conclusive, but strongly corroborative, of the same view—viz. that their escape is only apparent, and that syphilis, either latent or active, always affects such mothers.40 There can be no manner of doubt that in every instance at or about the time of childbirth there are not to be found pathognomonic lesions of syphilis, nor do such lesions always make their appearance in cases where the period of observation is a short one; but it is claimed with much show of truth that prolonged and patient inspection of such patients will in time result in the discovery of some symptom which betrays the presence of the disease.
38 Bumstead and Taylor, op. cit., p. 745. Hutchinson in Reynolds's System of Medicine, Am. ed., vol. i. p. 431: "In a large proportion of the cases met with in practice the taint is derived from the father only." (On this point see foot-note to p. 270.) If there were any doubt as to the fact that syphilis in the mother only may be transmitted to the child, it would be removed by the cases of Bardicet and others mentioned in Nouv. Dict. de Méd. et Chir., vol. xxxiv. p. 688. In these cases nurses who had contracted the disease from their sucklings subsequently became pregnant (without having infected their husbands) and gave birth to syphilitic children.
39 Jahrbuch, vol. ii. p. 303, 1872.
40 Zeissl, Sigmund, Oewre, Flindt, Woodman, and others are quoted by Hill and Cooper to this effect. Woodman, for other purposes, gave the histories of 200 cases of infantile syphilis. In all of these the mothers had suffered from typical secondary lesions.
The cases in which treatment of the father has resulted in healthy children, whereas without treatment he procreated only syphilitic children, the mother being without either symptom or treatment, have been urged as evidence of the direct descent of syphilis from the father to the child without the intervention or participation of the mother. Doubt [p. 267]has been thrown upon them by those who uphold the contrary view;41 but after looking into them carefully I am constrained to admit that some of them, notably those of Kassowitz42 and R. W. Taylor,43 are convincing of the facts—(1) that treatment of the father controlled the condition of the child, and (2) that there was no evidence that the mother had syphilis. But we have seen that the only proof of the universal infection of mothers of syphilitic children is the law of Colles, and that in many cases the disease is for a long time latent or unrecognizable. Let us admit that this was the condition in the cases in question; it does not at all follow, necessarily, that because the mother has latent or hidden syphilis she must infect her child. Every case even of active syphilis in the parents is not handed down to the children, a certain proportion of whom escape even when both parents are in the height of the secondary stage at the time of conception.44 But the activity of the disease in the children, and even more the likelihood of its reaching them, are in direct proportion to its activity in the parents.45 A mother, therefore, who under the influence of active syphilis in her husband has given birth to two or three syphilitic children, and has herself shown no symptoms of the disease, may nevertheless have it in the latent form and have no strong tendency to transmit it. Consequently, treatment of the father will result in the procreation of healthy children, because it removes the active and efficient cause of their infection. To take any other view of these cases is to assume that every syphilitic parent must hand down the disease to the children—an assumption which is not in consonance with numerous well-attested clinical facts.
41 Hill and Cooper, op. cit., pp. 52, 53.
42 "Die Vererbung der Syphilis," Stricker's Med. Jahrb., 1875, p. 391.
43 Archives of Clinical Surgery, New York, Sept., 1876.
44 Fournier, op. cit., pp. 35-37.
45 Hutchinson, op. cit., p. 431.
Leaving this interesting question, however, we may consider the other methods by which syphilis descends from the mother to the child, having seen already that it is beyond doubt that it may be handed down by disease of the ovule due to syphilis acquired previous to conception, and having seen that there is a high degree of probability that the mother herself rarely, if ever, escapes the disease. The second method, or that in which the mother becomes syphilitic at the moment of conception, has already been sufficiently discussed. It is really, strictly speaking, an example of paternal heredity, as the resulting germ is syphilitic—not because the ovule of the mother was infected, but on account of the disease of the spermatozoid of the father.
There remains for consideration the influence upon the child of a syphilis acquired by the mother during some period of utero-gestation. That under these circumstances the child can become infected has been and is still absolutely denied by some very respectable authorities.46 All that is [p. 268]necessary for proof of its occurrence is, however, (1) freedom of both parents from syphilis at the time of conception, or, in other words, syphilis must have been acquired by both—not alone by the mother—after the beginning of pregnancy; (2) that the syphilis of the child be unmistakably pre-natal—that is, not acquired by some accident during or after birth.
46 Bumstead and Taylor, op. cit., pp. 742, 744. They base their denial, first, on the physiological fact (?) that no interchange of cellular elements between mother and father is possible, and next on the absence of satisfactory evidence of the occurrence of infection during pregnancy. Zeissl's case seems sufficient answer to the last assertion, and there is strong evidence that the first is without good foundation. "The placenta is penetrated by the virus, and does not play the part of a filter for the elementary particles of matter which, so far as we know, represent the true active contagion of the disease. In the absence of direct experiments, which it would be almost impossible to institute, we may argue from the facts known to exist in certain acute infectious diseases in which there are very interesting points of resemblance.
"It has been known for a long time that small-pox occurring in the mother may be transmitted to the product of conception enclosed in the uterus, and it is supposed that the virulent particles traverse the walls of the maternal vessels in order to penetrate the circulating apparatus of the foetus. But what is only a supposition in the case of small-pox seems to have been actually demonstrated in symptomatic charbon (Arloing, Cornevin, and Thomas), bacteridian charbon, and recurrent fever, in which the poisonous element is easily recognized. The recent experiences of Strauss and Chamberland (1882) have shown that the foetus participates in the 'infection charbonneux' of the mother. Albrecht has shown (1880) the presence of numerous spirochoetæ in the blood of the heart of a child born at seven months of a woman with a second attack of relapsing fever. It may be supposed, therefore, though not demonstrated, that the transmission of syphilis takes place by the same method as that of relapsing typhus or of charbon" (Nouveau Dict. de Méd. et de Chir., pp. 682, 683).
The following case,47 reported by a most accurate observer, seems to combine both these requisites. Zeissl the younger reports that O. X., thirty-six years old, never having had syphilis, left his wife, to whom he had been married two years, to go a journey on July 15, 1877. The wife was then in the second month of her first pregnancy. On July 24th O. X. had extra-marital intercourse. About twenty-one days after this coitus he observed a small lump on the inner surface of the foreskin, and on Aug. 22d he consulted Zeissl the elder. On Sept. 23d a maculo-papular eruption of the skin with erythema faucium appeared. Under treatment these symptoms completely disappeared. On Oct. 29th he went home to fetch his wife to Vienna for her lying-in, and had intercourse with her soon after his return, notwithstanding Zeissl's strict prohibition. At the beginning of December a hard sore developed on the left nympha of the wife, who was then in the seventh calendar month of her pregnancy. At the end of December a maculo-papular eruption spread over the body and was treated with mercury. On Feb. 14, 1878, a well-grown and apparently healthy female child was born at full term. When eleven days old48 a pustulo-scaly eruption came out on the child's soles and toes, and soon afterward a maculo-papular eruption over the body generally. A few days later the child died. No post-mortem examination was permitted. In July, 1878, the wife had iritis, and after that gummata on the leg. She miscarried in July, 1878, at the third month, and again in February, 1879, at the second month.
47 Quoted by Hill and Cooper, op. cit., p. 60.
48 Of course much too early for constitutional symptoms if the disease had been acquired during or after birth.
There seems to be no reasonable escape, after reading this carefully, from the conclusion that in some manner the poison of syphilis found its way from the mother to the child. The old idea that the latter was directly infected in utero from the semen of the father is altogether without foundation. Other cases equally satisfactory and complete have been reported, and, unless the intelligence or the truthfulness of the observers be impugned, establish without doubt the possibility of infection during utero-gestation.
In the above case the contagion of the mother occurred in the seventh [p. 269]month of pregnancy; and this, I believe, is as late as it has ever been known to be communicated to the child. The exact date at which it becomes impossible so to transmit it is unknown, but as a general rule it may be said that the earlier a mother is infected during gestation the less likely is it that the child will escape. Treatment of the mother—as of the father in cases where he is at fault—very greatly modifies the whole problem and adds immensely to the chances that the child will not be infected.
Direct infection of the child during birth could not properly come under the head of hereditary syphilis. There is no possible reason why, when the mother has contagious lesions of the genitals, acquired too late to infect the child in utero, this should not occur, but as a matter of fact no such case has ever been recorded. One explanation of this circumstance may be found in the protective covering of vernix and mucus which coats the infant's body and lessens greatly the risk of absorption. This hardly accounts satisfactorily, however, for the entire absence of such cases from medical literature, and it is fair to suppose that in all but those cases in which the primary sore is acquired during the last month of gestation—which for obvious reasons are excessively rare—the infant acquires some immunity which protects it from its mother, and is similar to that which, under Colles's law, operates in her favor. In other words, even though apparently free from syphilis at birth—a not uncommon event, as we shall see—it has a latent or modified syphilis which protects it from contagion.
We may now briefly restate the conclusions at which we have thus far arrived:
1. After a certain interval, not less than four years, and after thorough specific treatment, a person who has contracted a syphilis not especially severe or malignant in its type may be permitted to marry. The assent to marriage will then be based on a belief in the curability of syphilis or the cessation of its contagiousness, its inoculability, and, in the vast majority of cases, its transmissive power at the end of the secondary stage.
2. It may be inherited from either parent or from both, and the probability that this will occur increases in a direct ratio with the nearness of the time of conception to the date of their infection with the disease. The severity of the inherited disease in the child increases in the same proportion.
3. It is undoubted that, the father being healthy and the mother syphilitic, the child may, and in all probability will, have the disease.49
49 Dr. Sturgis, who disbelieves altogether in the possibility of paternal heredity, concludes, after examining the subject carefully, that (1) a mother begets non-syphilitic children as long as she is not infected, even though the father is syphilitic; and (2) the moment she is diseased the children are inevitably so (Paper on "The Etiology of Hereditary Syphilis," New York Medical Journal, July, 1871). This doctrine was previously supported by M. Cullerier, whose views gave rise to the remarks of M. Voillemier (quoted by Fournier) that if they were accepted "the father would be only the accidental occasion of a child; one would be, in reality, the child of his mother only." Cullerier's cases are invalidated by the fact that the syphilitic fathers who had healthy children had been subjected to mercurial treatment (Mém. de la Société de Chirurgie, Paris, 1854, quoted by Taylor in Archives of Clin. Surg., vol. i. p. 83). The theory is a very old one. Vassal has sustained this idea as long ago as the end of the last century. Kostum (1804), and after him Hufeland, were of the same opinion. Cullerier (1857) wrote: "In order that a child acquire syphilis hereditarily it is necessary that the mother is or has been [p. 270] syphilitic." Notta, Follin, Charrier, Mireur (1867), and Langlebert (1873) support this theory more or less earnestly. Oewre wrote (1873): "Paternal influence is nil as regards hereditary syphilis." Isseff (1879) wrote: "Where a man suffers or has suffered from syphilis he cannot transmit the disease to his descendants without infecting his wife; that is to say, in fewer words, there is no infection from the father." Sigmund says: "The heredity of syphilis is derived in its last analysis from the mother" (Nouveau Dict. de Méd. et Chir., vol. xxxiv. p. 689).
4. It is probable, but less so,50 that, the mother being healthy and the father syphilitic, the child will be infected.
50 This refers simply to the comparative probability of infection, and does not conflict with the statistical fact expressed by Hutchinson (Reynolds's System of Medicine, vol. i. p. 431) in his words: "In the large proportion of cases met with in practice the taint is derived from the father only." This numerical predominance of paternal influence is very readily explained. There are many more syphilitic men than syphilitic women, and especially among the couples who contract fertile marriages the number of women who are infected before becoming mothers is inconsiderable. On the other hand, it frequently happens that men who have had syphilis, but have been without symptoms for a longer or shorter interval, marry and transmit to a series of children a disease which has ceased to be directly contagious to their wives, the transmissive power continuing after the possibility of ordinary contagion has disappeared. As in the majority of such women the disease is latent, and may be only displayed in their immunity from infection, it becomes evident that, history and symptoms both being wanting on their part, the conditions justify the assertion of Mr. Hutchinson. (See Nouveau Dict. de Médecine et Chirurgie, p. 684.)
That assertion (quoted above) has, however, been thought by several writers to indicate his belief in the escape of the mother. That I have not misinterpreted him is evident from the following extract from an article on "The Transmission of Syphilis," written by him (Brit. and For. Med.-Chir. Rev., Oct., 1877): "I take it for granted (although I know that there are still some who doubt) that it is possible for a father to transmit the taint, the mother being at the time of conception wholly free. I believe, indeed, that in practice this is by far the most common way in which syphilis is transmitted. Whether in these cases it is correct to speak of the inheritance being paternal only is, as we have just seen, another matter, since it is possible that in every instance the mother derives an infection from the father, and may thus in turn influence it."
5. It is highly probable, though it can hardly be considered as proven, that in all cases where a child becomes syphilitic through paternal influence the mother is also the subject of syphilis, which may, however, assume a latent form, the only evidence of its presence in a few cases being the protection which it affords against contagion through the medium of the child.
6. Syphilis may be transmitted from mother to child even when it is acquired by the former as late as the seventh month of utero-gestation.
Since writing the above the thirty-fourth volume of the Nouveau Dictionnaire de Médecine et de Chirurgie has been published. In the article on syphilis seventeen pages are devoted to the question of heredity, which is reviewed in a most thorough manner and finally summed up as follows (p. 698):
"The most definite views which we possess on the subject of the hereditary transmission of syphilis may be thus expressed:
"Children may be infected by heredity, not only when the two parents are syphilitic, but also when only one, either the father or mother, is diseased at the time of conception.
"When both parents are diseased at that time there is more certainty that the child will be infected, and infected gravely, than if only one of them has the pox.
"The hereditary disease is not always fatal, even when both progenitors have actual specific symptoms. The more recent the disease of the parents the greater the chances of their transmitting the disease and of its assuming [p. 271]a serious form. There is no proof that inherited syphilis is more grave when derived from the father than when coming from the mother.
"It is altogether exceptional for the mother, healthy at the moment of conception, not to participate in the disease transmitted by the father to the child. If she escapes direct contagion—which is rare when the disease of the father is active—she undergoes a species of infection from contact with the contaminated foetus.
"Syphilis by conception, which is thus transmitted from the foetus to the mother, may present the usual characters and evolutions of the acquired disease; frequently, also, it is latent, and is betrayed only by the existence of immunity from further contagion on the part of the mother. It may finally manifest itself by tertiary symptoms or by systemic troubles without specific characters.51
51 "This form of syphilis shows itself in the mother in three ways:
"1st. By the usual signs of syphilis by contagion, with the exception of the primary sore, appearing about the sixty-fifth day after conception.
"2d. Appearing at a later date as secondary or even as tertiary symptoms, and preceded merely by a little disturbance of the general health, unhealthy appearance of skin, falling of hair, etc., but nothing truly specific.
"3d. Showing itself some years afterward in a tertiary form, having in the mean while given no indication of its existence save only in the protection it afforded against contagion from the child" (Nouveau Dict. de Méd. et Chir., vol. xxxiv. p. 696).
"The power of transmitting syphilis hereditarily decreases spontaneously as the disease of the parent becomes older. The influence of treatment is no less certain than that of time. When the two progenitors are at the moment of conception free from syphilis, the foetus may still be infected if the mother acquire the disease during her pregnancy."
Syphilis of the placenta is of especial interest in its relation to the abortions and stillbirths so frequent in syphilis. Until the elaborate paper of Fraenkel upon this subject (see foot-note, p. 265) almost nothing was known about it. He describes52 the macroscopic changes as consisting of increased size and weight of the placenta, closer and firmer texture of the placental tissue, the presence of old and recent extravasations of blood in all stages, opacity and thickening of the decidual covering and of the amnion and chorion, which are in places adherent to each other. Microscopically, it was found that the placental villi were filled with small nucleated cells, which were especially abundant in the centre of the villous spaces along the axis where the vessels usually take their course. The ends of the villi were enlarged with knob-like processes.
52 I condense here from the translation of Fraenkel's paper, which constitutes the bulk of chap. xxvii. of Bumstead and Taylor's excellent work.
Fraenkel's explanation of these changes is as follows: Under the influence of syphilis53 cell-proliferation begins in the villi, which are, normally, only sparingly supplied with cells. These new cells excite proliferation of the cells of the connective-tissue stroma and of the epithelium. This proceeds to such an extent that it leads to compression of the vessels,54 interfering with the circulation, and finally obliterates them. The [p. 272]vascular spaces into which the villi dip become filled up and narrowed, and often disappear. In this way, and also by reason of the thickening of the epithelium, the interchange between the maternal and foetal blood is interfered with, and at last is prevented.
53 Fraenkel took as his criterion of syphilis the presence of the osteo-chondritis described by Wegner (see p. 286).
54 "Hennig was the first who called attention to the intimate relation of the cell-growths to the vessels. The obliteration or compression of numerous vessels of the villi interferes with the mutual interchange of gases between the maternal and foetal blood, causing fatty degeneration of the villi and, if the process is extensive, the death of the foetus" (article "Syphilis," Ziemssen's Cyclopædia, vol. iii. p. 237).
If this process is spread over the whole placenta, the foetus perishes before it is complete. If it is limited to circumscribed areas, it may continue to live for a shorter or longer period.
These observations require to be repeated and confirmed, but they have served to open up a most interesting branch of syphilitic pathology.
Syphilis in the parents will manifest itself in the children in one of several ways, which are determined chiefly by two factors—viz. first, the length of the interval between the infection of the parent and the date of conception; and, second, the thoroughness of the treatment of the parents during that interval. To these may be added as subsidiary, but still of definite importance, a third, the type of disease which has affected the father or mother, whether mild or severe, benign or malignant.
From what has already been said in reference to the question of marriage, it will be at once understood that the danger to the offspring in untreated cases, and in those where conception has occurred during the early secondary period of the disease, is of the most extreme gravity.55 In such cases the usual result of pregnancy is abortion at from the first to the fifth or sixth month,56 the foetus sometimes exhibiting the evidences of the disease in the shape of large bullæ upon the palms and soles, or in the presence of characteristic visceral lesions, but quite as often showing nothing distinctive. It has generally undergone more or less maceration, and the skin, which is readily detachable, is of a congested, purplish color.
55 According to Kassowitz, in women who are not treated all pregnancies occurring within the first three years of their infection terminate either in abortion or in the birth of children who survive for only a few weeks or months.
Weber (quoted by Parrot, Le Progrès Médicale, Nov. 24, 1877, p. 882) treated thirty-five pregnant women by mercurial inunction, and they all went to full term under normal conditions. Among those treated with mercury and iodide of potassium, but who, by reason of intolerance of the former drug, took chiefly the latter, 20 per cent. aborted; when the mixed treatment was carried out regularly 15 per cent. aborted; and when only iodide of potassium was given 36 per cent. aborted. His studies were made on 109 syphilitic pregnant women. Parrot himself says (ibid.) that "abortion occurs in about one-third of all syphilitic women. Syphilis should always be suspected when this accident occurs several times successively." "The date of abortion depends on the age of the syphilis of the woman. It is most to be feared during the evolution of secondary symptoms, particularly when they are grave. There is more probability that it will occur when infection has preceded pregnancy than when it occurs during its course. Nothing is so much to be feared as contamination of the ovule. The nearer the date of infection of the mother approaches to full term, the fewer the chances of abortion. It is not likely to occur even when the mother is infected at five months."
56 Many observers think that abortion results directly from the death of the foetus. Babington (notes to Hunter's Treatise on Venereal), Trousseau, and Von Baerensprung were of this opinion.
Dr. Cory thinks that in many cases it is possible that the effect of syphilis may be to effect so early an abortion that the case is simply regarded as one of delayed menstruation or of menorrhagia. Such a conception would, however, be competent to infect the mother, and might seem to explain cases otherwise involved in obscurity.57 At least one-third [p. 273]of all syphilitic children are dead born.58 As time goes on, however, and the intensity of the parental disease is lessened, or in cases where appropriate treatment has been applied, either the abortion occurs at a later period of pregnancy or the children are brought alive into the world. Even then, however, and although at birth they may show no evidences of the disease, their chance of escape is but small. One-fourth of them die within the first six months. If they survive that period, the chances for life are slightly in their favor, but those for health or freedom from deformity and disease are still overwhelmingly against them.
57 As, for instance, when a woman married to a syphilitic man, but without issue, remarries a man with no history of syphilis, and yet gives birth to a syphilitic child. In such a case there would be no history of direct infection and none of pregnancy, the only two modes by which she could have contracted the disease, and the father—the second husband—might be unjustly suspected.
58 Kassowitz, op. cit.
The course of inherited syphilis differs strikingly from that of the acquired disease. It will hardly be necessary to do more than remind the reader of the ordinary stages of the latter affection—the primary, which includes the period of the chancre and of lymphatic engorgement, lasting about sixty to seventy days; the secondary, or exanthematic, characterized by copious cutaneous eruptions and extensive involvement of mucous surfaces, lasting from one to three years; the intermediate,59 or the stage of latency and relapses, lasting for a very variable period, from three to ten years, but under proper treatment very much reduced or altogether abolished; and finally, the tertiary period, beginning four or five years after contagion, extending indefinitely throughout life, but often in cases properly treated absent altogether.
59 So designated by Mr. Hutchinson, and for clinical purposes a very valuable addition to the periods of syphilis. He describes it as follows: The patient may be either wholly free from symptoms and in good health, or he may remain pale and rather feeble, and liable from time to time to slight returns of eruption on the skin, sores on the mucous membranes, condylomata, etc. He is protected as regards fresh contagion, and should he beget children they are almost certain to suffer. The relapses during this stage are usually easy to be distinguished from true secondary symptoms. There is little or no febrile disturbance, the rash is not copious, and often not symmetrical. Acute iritis, retinitis, etc. never occur for the first time, though they may do so in the form of relapses.
For purposes of description and of contrast we may similarly divide the whole period of evolution of a case of inherited syphilis,60 omitting the primary stage, which has never been found to exist in true cases of hereditary syphilis. Of course in congenital or infantile syphilis, in which by direct contagion, either from the mother or from any one else, the disease was acquired by the child, the course would not differ materially from that observed in the adult. But as this stage in all probability corresponds to the period during which the poison is already finding its way into the system through the lymphatics, of course it is not found in the child who is infected from the moment of conception or who receives the poison from the mother directly into the circulation.61 For from one to three weeks the infants often show no symptoms of the disease. In 158 cases collected by Diday, 86 manifested symptoms of the disease before the expiration of the first month, and 60 of the remainder before the end of the third month.62 When to these are added the [p. 274]statistics of Roger, we find that of a total of 172 cases, 159 showed syphilitic symptoms before the end of the third month.63 When the symptoms are present at birth, they consist largely in a general withered, atrophied, weazened appearance of the child; a hoarse cry, due to swelling, with subacute inflammation, or even ulceration, of the laryngeal mucous membrane; a coryza,64 due to a similar condition of the Schneiderian membrane; and certain cutaneous eruptions, the most common of which at this early date is the large vesicular or bullous eruption known as syphilitic pemphigus.
60 The idea that the character of the symptoms which first appeared depended upon and corresponded with the stage of the disease in the parent has now, I believe, no supporters. It was once thought, at least by some syphilographers, that if the parents were in the tertiary stage at the time of conception the child would develop tertiary symptoms, omitting the other stages.
61 If chancre were the first symptom of constitutional syphilis, why should it not appear in cases of hereditary syphilis?
62 Infantile Syphilis, p. 101
63 About 1823, from 16,000 to 17,000 children were admitted annually to the wards devoted to foundlings at Lyons. Clièt, recording his experience with this disease, says that syphilis is one of the most common of their maladies, but that "it exceedingly seldom shows itself at birth by evident signs" (Compte-Rendu Méd. Chir. des Observations à l'Hôpital général de la Charité de Lyon, 1823). Cristoferi, physician to the foundling hospital at Bologna, says that syphilis generally manifests itself between the ages of one and three months. Never once, he states, was a newly-born infant admitted with the disease unquestionably developed (Gazette Medica di Milano, 1844). Trousseau says that it "rarely appears before the second week, and very exceptionally after the eighth month" (Lectures on Clin. Med., vol. iv. p. 331, London, 1871, ed. of New Sydenham Society).
64 Usually appears later, but exceptionally at birth.
Pemphigus.—With regard to the specific or non-specific character of this eruption there has been much difference of opinion, and, as it is often the earliest distinctive expression of syphilis, a diagnosis of which could hardly be founded on the general appearance of the child, or even on the hoarse cry and the coryza, it becomes important to have definite ideas upon the subject. Nearly a century ago (in 1794) it was denied65 that this eruption was a manifestation of venereal disease; and this view has been supported by many able and accurate observers down to the present day. In 1851 a discussion upon the subject took place in the French Academy of Medicine, which elicited the opinions of the majority of those members who were entitled to speak with authority in the matter, and which has since been referred to by most writers. Cazeaux upheld the non-syphilitic hypothesis on the ground (1st) that the so-called syphilitic pemphigus of children does not differ from the simple pemphigus of adults, presenting none of the physical characters which distinguish the specific cutaneous eruptions; (2d) that it appears at birth or immediately after, while the symptoms of hereditary syphilis generally show themselves later;66 and (3d) that at that time there had been seen no cases of pemphigus at the Lourcine Hospital, where so many syphilitic children were born.67 In this view he was supported by Trousseau, Lasègue, Gibert, Bazin, and other obstetricians and syphilographers of note.68
65 Ariander, Mémoires de Méd. et d'Accouch., quoted by Diday.
66 This is now known to be an unreliable distinction, expressing perhaps a general rule, but one with so many exceptions as to render it void of diagnostic significance.
67 This may have been true at that time, but has certainly not continued to be so. Cornil says: "We often see at the Lourcine children born prematurely or at the full term with pemphigus, either fully developed at the moment of birth or appearing a few days afterward, and who commonly die with syphilitic cachexia, the sad heritage derived from their maternal parents."
68 Pemphigus may indeed be a specific affection, but no characteristic sign has been discovered sufficient to distinguish it from the ordinary form of pemphigus. "On the other hand, there is no inconsistency in admitting that syphilis, which so deeply impairs the constitution of the parent, may act like any other common cause and excite non-specific pemphigus; for an infant is badly lodged and poorly nourished in the womb of an enfeebled mother, apart from the influence of the virus" (Ricord, note to John Hunter's Works, 1853).
On the other hand, Dubois claimed a specific character for the affection [p. 275]on the ground (1st) of the fact that in the majority of cases there was a syphilitic history in the parents; and (2d) that the eruption often coexists with well-known syphilitic lesions. This was supported by Cazenave, Danyan, Bouchut, Vidal, Ollivier, and others.69 Diday, who devotes several pages of his interesting work on Infantile Syphilis to this subject, regards the eruption as simply a manifestation of a cachexia produced by syphilis,70 founding this opinion on (1st) the absence of specific characters in the eruption; and (2d) that syphilitic pemphigus is a rare affection in the adult, if it occurs at all, so that to recognize it in the child would be to make a single exception to the general rule that "all the syphilitic eruptions of new-born children have their equivalents in those of adults." He explained the two cases which were then (1858) recorded of cures of pemphigus by mercury71 by saying that it was the treatment of the diathesis, not of the disease, which caused the improvement. He acknowledges, however, the very frequent association of pemphigus in the child with syphilis in the parent, and says that it springs from the latter affection, "specially, but not specifically"—a rather wire-drawn distinction.72
69 Jullien (op. cit., p. 1005), after considering the opposing views as to the character of this eruption, says: "We have no hesitation in declaring ourselves in accord with Roger, Ollivier, Ranvier, Parrot, and others, and in distinctly separating from the specific affection the rare eruption known as simple pemphigus, sometimes epidemic, occasionally febrile, and appearing most frequently about three months after birth. We consider likewise that an evidence of congenital syphilis which is by no means doubtful is found in the bullous eruption seen at birth or within the first two weeks, comparatively frequent, and involving by preference the palms and soles. This opinion is based upon (1st) its appearance in children whose parents are known to be syphilitic; (2d) its association with syphilitic lesions of the lungs, liver, kidneys, thymus gland, etc.; (3d) its partial disappearance under mercurial treatment, and its reappearance when that treatment is discontinued."
70 Op. cit., pp. 70-77.
71 Depaul, Gaz. Méd. de Paris, 1851, p. 472, and Galligo, Gaz. Med. Toscana, 1852, p. 123.
72 Trousseau (Clinical Lecture on Syphilis in Infants), after detailing a case in which there was some doubt as to the existence of hereditary syphilis in a child born alive, and in which case the previous pregnancy had resulted in a stillborn child at seven months, the body of the latter having been preserved in alcohol and exhibiting numerous traces of pemphigus, says: "So far as I was concerned, this demonstration did not amount to more than the establishing of a probability, and several physicians who participated in this indecision finally accepted a compromise. They considered that maternal syphilis had determined a sort of cachexia in the foetus which had led to an eruption of bullæ which was not specific. By accepting this too-facile hypothesis you will imprudently open a door which you will with difficulty be able to close."
As these differences of opinion have been perpetuated to the present day, it has seemed to me proper to make this reference to their history, although I am strongly convinced that the progress of clinical and pathological knowledge enables us now to assert that although, as an exception, bullæ may be due to a profound cachexia not dependent on syphilis, yet that in the large majority of cases they are specific in their character.
The argument which always seemed to me the strongest, the fact that a similar eruption is almost—or quite—unknown in the adult, has been removed by the observations of Cornil, who has shown that it belongs properly with the papular rather than with the bullous eruptions, and should be classed with the roseola and papules of early syphilis—just where, from its clinical history, we should expect to find it. The raising of the epidermic layers is due chiefly to their delicacy, their slight resistance, and their previous immersion in the amniotic fluid—i.e. to [p. 276]conditions which are peculiar to the skin shortly after birth.73 He founds these very important opinions upon the autopsy of a child stillborn a little before full term, the mother being in the height of secondary syphilis. The child presented characteristic bullæ on the soles and palms. After hardening these were found to consist of the two layers of epidermis placed one above the other. Fig. 6 represents a bulla about one centimeter in diameter which was situated on the plantar surface of the great toe.
73 Cornil, op. cit., p. 203.
FIG. 6. |