Chapter II: Front Matter (2)

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.

[Footnote 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 {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's[44] cases 34.3 per cent.; in Peacock's,[45] 32.7 per cent.; in Sibson's[46] (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."

[Footnote 44: _On Rheumatism, Rheumatic Gout, etc._, 3d ed., p. 280.]

[Footnote 45: _St. Thomas's Hospital Reports_, vol. x. p. 19.]

[Footnote 46: Reynolds's _Syst. of Med._, Eng. ed., vol. iv. 186.]

[Footnote 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.]

[Footnote 48: _Lib. cit._, vol. xiv. 6.]

[Footnote 49: _Lib. cit._, 264.]

[Footnote 50: See Senator in _Ziemssen's Cyclopæd. Pract. of Med._, xvi. 49.]

[Footnote 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 table[52] 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 {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 experience[54] 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]

[Footnote 52: _Lib. cit._]

[Footnote 53: Raynaud, _Nouveau Dict. de Méd. et de Chir._, t. viii. 367.]

[Footnote 54: _Lib. cit._, pp. 77-278.]

[Footnote 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's[57] 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's[61] case appear to have been examples of ulcerative {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. Goodhardt[62] has lately insisted upon the tendency of ulcerative endocarditis to appear in groups or epidemics, but the evidence is not conclusive.

[Footnote 56: _Archives Médecine_, vol. v., 1881; _Trans. International Med. Cong._, vol. i. 341.]

[Footnote 57: _Clin. Soc. Trans._, xiii. 227.]

[Footnote 58: _Brit. Med. Jour._, i., 1880, 798.]

[Footnote 59: _Medical Record N.Y._, 24th Sept., 1881, 361.]

[Footnote 60: _Canada Med. and Surg. Journ._, vol. xi., 1882, 1, and _ib._, vol. ix., 1881, 673.]

[Footnote 61: _Lancet_, ii., 1882, 976.]

[Footnote 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. Maclagan[63] 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.

[Footnote 63: _Rheumatism: its Nature, Pathology, and Successful Treatment_, 1881.]

[Footnote 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] {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's[66] celebrated case, produce disturbances of the cerebral system rather than of the circulatory.

[Footnote 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.)]

[Footnote 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 case[67] 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.

[Footnote 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. West[68] 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. Sibson[70] 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 {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.

[Footnote 68: _Barth. Hosp. Repts._, xiv. 228.]

[Footnote 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.]

[Footnote 70: Reynolds's _System. Med._, Eng. ed., vol. iv. 463.]

[Footnote 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]

[Footnote 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's[78] 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. {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]

[Footnote 73: Latham's _Works_, Syd. Soc., i. 98 _et seq._]

[Footnote 74: _Lib. cit._, 317.]

[Footnote 75: _Bartholomew Hospital Reports_, xv. 14.]

[Footnote 76: _Guy's Hosp. Reports_, 3d Series, xi. 434.]

[Footnote 77: _Ib._ xix. 324.]

[Footnote 78: _St. Thomas's Hospital Reports_, x. 12-17.]

[Footnote 79: Reynolds's _System Med._, iv. 233.]

[Footnote 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 minutes[81] and in an hour and a half[82] 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.

[Footnote 81: _Thèse d'Aigue pleur._, 1866, par B. Ball.]

[Footnote 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 {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 sixteen[83] or twenty-nine days[84] 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.

[Footnote 83: Southey's case, _Clin. Soc. Trans._, xiii. p. 25. Sleeplessness preceded it for four days, and there was no hyperpyrexia.]

[Footnote 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]

[Footnote 85: _Lancet_, ii., 1870, 467.]

[Footnote 86: _Clin. Soc. Trans._, xiii. p. 29.]

[Footnote 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 {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.

[Footnote 88: Senator, in _Ziemssen_, xvi. 50.]

[Footnote 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.]

[Footnote 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.]

[Footnote 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 {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]

[Footnote 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.

[Footnote 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.]

[Footnote 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 {41} with disturbances of the nerve-centres, we may inquire what are the circumstances in the disease capable of developing into activity the predisposition.

[Footnote 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]

[Footnote 96: "Abstract Report upon Hyperpyrexia in Ac. Rheum.," _Brit. Med. Jour._, 1882, p. 807.]

[Footnote 97: _Lib. cit._, p. 264.]

[Footnote 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 argument[99] 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 {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.

[Footnote 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 instances[100] 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.

[Footnote 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 {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.

[Footnote 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.]

[Footnote 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 1871[104] 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 {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]

[Footnote 103: _Phlebite Rheumatismale Aigue_, Paris, 1869, par M. Lelong. In _Revue de Méd._, t. i. 492-499, 1881, a case by Dr. Launois.]

[Footnote 104: _Pathologie Interne_, ii. 546, 1871.]

[Footnote 105: _Die Rheumatische Schwiele_, Weimar, 1843.]

[Footnote 106: _Lib. cit._, p. 628.]

[Footnote 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.]

[Footnote 108: _Brit. Med. Journ._, i., 1883, 868.]

[Footnote 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 {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 Flint[110] 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 1869[113] 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.

[Footnote 110: _American Journal of Med. Sciences_, July, 1863.]

[Footnote 111: _Ib._, vol. xii.]

[Footnote 112: _Medico-Chirurgical Transactions_, vol. lii.]

[Footnote 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 series[115] 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.

[Footnote 114: _Med.-Chir. Trans._, lii. 82.]

[Footnote 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 {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.

[Footnote 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. Roussel[117] found that the time during convalescence occupied in regaining the weight previously lost was inversely proportional to the duration of the attack.

[Footnote 117: _Essai sur la Convalescence du Rheumatisme Artic. Aigue_, Paris, 1881, 66.]

Subacute Articular Rheumatism.

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.