Part 7

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 or twenty-nine days 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.

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. 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. 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.

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, does occur, and lymph or pus is found, usually over the convexity of the brain, but sometimes at the base and down the cord. 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, 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. 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.

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.

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, 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, 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.

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, 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.

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; 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, 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.

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 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.

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 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.

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, there {43} are others which may be themselves rheumatic manifestations. Such are especially erythema marginatum, 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.

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. Jaccoud in 1871 mentioned the exceptional occurrence of subcutaneous nodosities in rheumatism, which he says Froriep first pointed out; but Homolle states that they had been previously mentioned by Sauvage and Chomel. 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: 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. 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.

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 was one of the first to study the natural history of the disease uninfluenced by active treatment, and he was followed in 1865, 1866, and 1869 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.

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." Yet an analysis of the 23 of the 41 cases contained in their first series 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.

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, but, unfortunately, his conclusions, as will hereafter appear, have not been confirmed by other observers.

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

Subacute Articular Rheumatism.