A. McLane Hamilton7has recently investigated the frequency of St. Vitus's dance among school-children in New York, and found that 20 per cent. of the children in the schools were affected with some variety of the disorder.
7American Psychological Journal, Feb., 1876.
Rheumatism is certainly a predisposing cause, in my experience, but I have not found it associated with chorea as frequently as some authors have. In 279 cases which I examined with regard to this question, there was a clear history of rheumatism in but 37. Cardiac complications were much more frequent. In 82 cases there was a cardiac murmur heard. In some of these the murmur was no doubt anæmic, but in the majority there had probably been a rheumatic endocarditis. Many cases in which there is said by the friends of the patient to have been a previous attack of acute rheumatism, on investigation are found not to have had articular rheumatism, but merely some muscular or joint pains which were not inflammatory. Quite recently Joffroy and Saric have expressed the opinion that the pains in the joints during an attack of chorea are to be regarded as choreal arthropathies of nervous origin.
English and French writers have observed the relation between rheumatism and chorea in a large proportion of cases. Hughes and Burton Brown8found that in 104 cases which they examined as to rheumatism and affections of the heart, there were but 15 in which some rheumatic condition had not preceded the attack or a cardiac murmur did not exist.
8Guy's Hospital Reports, 1856.
Sée and Roget consider the causal relation between the two diseases so close as to make their connection almost a pathological law.
On the other hand, Steiner made quite opposite observations in Prague. He saw among 252 cases of chorea only four which originated during the course of acute articular rheumatism.9This statement does not prove the absence of a relation between chorea and rheumatism, for Steiner does not say what proportion of cases occurred after an attack of rheumatism which had taken place some time previous. Hammond10believes “that the influence of rheumatism upon chorea is not greater than that of a depressing agent to the organism.”
9Ziemssen's Cyclopædia, loc. cit., p. 427.
10Dis. of the Nervous System, p. 715.
Octavius Sturges, physician to the Hospital for Sick Children, Ormond St., London, says that in two years 219 cases of chorea have been treated at that institution: 20 per cent. of them were connected with rheumatism, but he does not believe in the rheumatic origin of the disease, because 15 per cent. of all children have rheumatism.11
11Lancet, Sept. 20, 1884.
The following cases illustrate the direct sequence of chorea upon acute rheumatism:
Case II.—Kate S——, æt. 17 years, came under my care in May, 1877, with an attack of acute rheumatism which lasted for two weeks. She regained strength slowly, and about June 11th, not more than two weeks after the subsidence of the rheumatic symptoms, choreic movements were noticed in the right arm. The movements were constant, and were worse when she was conscious of being observed. She often dropped things. The right leg jerked often and gave away under her in walking. Under the use of liq. arsenici bromidi the patient was entirely well in one month. A sister had had chorea.
Case III.—Rudolph C——, æt. 5 years, was placed under my care May 12, 1883. He has had scarlatina, and in Dec., 1882, he had an attack of inflammatory rheumatism, accompanied with slight choreic movements, from which he recovered in a short time. Three or four weeks before coming under my charge he had a return of rheumatism from getting his feet wet, and at the same time choreic trouble began again. He rapidly became worse, and in a short time was utterly helpless. On examination he is found unable to sit up or make any voluntary movements. He is unable to move the legs on account of the intense pain in the knees and ankles from the rheumatism. These joints are swollen and red. The right arm is in constant and violent motion, swinging about or thrashing up and down. The left arm lies motionless, and, although painless, he seems unable to move it. The fingers are clenched, and they are continually and rhythmically being pressed against the palm. He cries if an attempt is made to open the fingers. The facial muscles are contorted all the time, and there is a peculiar vermicular movement of the upper lip. He cannot speak a word nor can he protrude the tongue. He sleeps but little, and during sleep the movements are occasionally seen. He is very fretful and irritable, but is perfectly intelligent. The appetite is poor. Bowels regular; urine phosphatic and contains no albumen. There is a loud but not harsh cardiac murmur heard at the apex, replacing the first sound of the heart. Temperature, May 12, evening, 100°; May 13, morning, 1002/5°. Pulse, 140. He was ordered sodii salicylat., gr. v. q. q. h., and inunctions of cod-liver oil.
On May 18 the rheumatism was so much better that the salicylate was stopped and Fowler's solution of arsenic was given instead. The chorea had also become better. The arsenic was continued in increasing doses, and the case convalesced steadily. On June 26 he was able to return to his home in Maryland entirely well.
He remained well until July, 1884, when he had a slight attack of chorea, which was preceded for about a week by rheumatism. The cardiac murmur, which was almost absent after recovery from the first attack, was now heard again, but faintly.
Scarlatina is sometimes the forerunner of an attack of chorea. Other diseases, whooping cough or measles, may also be the immediate cause of an attack of chorea, but it is generally in cases where there is a predisposition to the disease.
The connection between pregnancy and chorea is of great interest. Barnes12has collected 58 cases, and Bodo-Wenzel13has added 8 more, making 66 in all. Of 51 of these patients, 31 were primiparæ, and in the majority of cases the ages were between twenty and twenty-three years. Four cases which came under my own personal observation were all young primiparæ, and were between the second and fourth months of gestation when the disorder began. The affection usually appears in the first half of pregnancy. It may cease before the eighth month or may continue to the end of gestation. Sometimes it persists after delivery. It does not necessarily occur in patients who have had the disease in childhood, but from the above cases it would seem that an attack in one pregnancy would tend to its recurrence in another. Of the 66 cases, 14had had chorea in previous pregnancies. Sometimes the chorea appears only at the time of labor.
12Obstet. Transactions, vol. x. p. 147.
13Ziemssen's Cyclopædia, loc. cit., p. 428.
The immediate cause of chorea in pregnancy, when a cause can be found, is most often fright or rheumatism. In two of my own cases the patients were unmarried. Of the 66 cases of Barnes and Wenzel, fright is named as a cause in 7, and in 7 more rheumatism and endocarditis are stated to have preceded the attack.
Climate has been supposed to have a marked influence upon chorea. It was thought to be less frequent in warm than in cold regions; but Hirsch denies this, and Weir Mitchell14states that it does not appear to exist to any less extent in the southern than in the northern portion of this country.
14Loc. cit.
Season, according to Mitchell,15largely affects the production of chorea. He has gone to great pains to collect statistics on the subject. He shows that the majority of attacks in his cases occurred in spring. This agrees with Wicke,16who found that among 35 relapses, 13 were in spring, 12 in winter, 9 in autumn, and 1 in winter. On the contrary, Hammond17found 54 attacks in the six months from October to March, and but 28 in the remainder of the year. Gerhard18in a study of 80 cases found that of 68 attacks, 39 occurred in spring, 10 in summer, 7 in autumn, and 12 in winter. In Mitchell's cases a study was made of 170 attacks. In the spring and summer months there were 115 attacks against 55 in the winter and autumn months. This observer does not find that rain or dampness has any notable effect in causing the disease, but that the influence of storms is probably great.
15Loc. cit., p. 128.
16Ziemssen, loc. cit., p. 443.
17American Journ. Med. Sciences, July, 1876.
18Loc. cit., p. 715.
Mitchell has written upon the relation between race and chorea. He states that in answer to a circular bearing on this question, sent out by the Smithsonian Institution, he has received a large number of letters from physicians in our Southern States and the West Indies. The general testimony was that chorea is rare among negroes.
Among the exciting causes of chorea are fright or mental apprehension of some kind. Of my 279 cases, 44 were ascribed to fright.
Malaria has been pointed out as influencing the production and course of chorea. Kinnecut has reported some cases in which the movements were aggravated with a certain periodicity.
Chorea may also be brought on by reflex irritation from nerve-injury. In a case which I saw in the practice of John H. Packard several years ago there was an injury to one of the digital nerves of the thumb from a splinter, which was the apparent cause of an attack of chorea; a portion of the nerve was excised, and the chorea ceased in a short time.
I have lately seen a case in which an attack of chorea came on apparently in consequence of a severe mash of the finger. The finger had been crushed by the runner of a sled, and the choreic movements began before the wound was healed.
Hamilton19has found chorea associated with eczema. He saw a case in which eczema of the calves of the legs and of the scalp developed at the same time with an attack of chorea. Both got well about the same time.
19Nervous Diseases, p. 490.
SYMPTOMS.—An attack of chorea is usually preceded by more or less failure of the general health and evidences of some mental disturbance. It is quite common to be told by the parents of a child suffering from chorea that the little patient had seemed unwell for some time previous to the attack; that the appetite had failed, and that the child had looked pale; that he had been irritable or excitable, and at school the teacher had complained of restlessness or inattention in the pupil. In a little girl who was brought to me recently with her second attack of chorea her mother stated that for several days before the outbreak the child had been in excessive spirits, and that she had been singing loudly and in a peculiar manner. The same symptoms had preceded the first attack. Sometimes nothing is observed until it is found by the parents or teacher that there are abnormal twitchings and movements of the limbs.
At first there is a general restlessness and fidgetiness. The child may be punished at school for not keeping still or for dropping things. Soon irregular movements of groups of muscles are seen. The shoulder is shrugged or the fingers move spasmodically. At first the patient is aware of the movements and tries to control them, but before long the twitching and jerking are constant, and extend to most of the voluntary muscles of the body. He is then unable to control them for any length of time.
The sudden jerk of a limb followed by an odd grimace, the quick protrusion of the tongue, and the rolling of the eyes or snapping of the lids give a characteristic picture which can hardly be mistaken.
The extent of the movements varies in different cases. In some they are slight and affect only certain muscles. Often the disorder is confined to one lateral half of the body. In other cases the movements involve all the limbs and the trunk, and are so violent and constant that the patient does not seem to have a moment's rest. The trunk may be suddenly drawn backward, then the arms are extended or thrown up, and the legs flexed and tossed about with great quickness. Sometimes the patient is thrown off the bed or from the chair on which he may be to the floor.
The speech is often affected. The patient speaks in a thick or jerky manner, as if the tongue were too large for the mouth, and saliva usually flows in great quantities. Sometimes in bad cases there are involuntary utterances made at frequent intervals.
The features undergo contortions continually, and when at rest relapse into a condition of vacancy which makes the patient look almost idiotic. The expression of a child with chorea is so peculiar that the disease may almost be diagnosticated by this.
During sleep the movements usually cease, but generally the patient is restless while asleep, and in some instances the irregular movements continue even at this time.
The mental condition commonly shows some change. The child is irritable and peevish, cries and laughs readily, or is sullen and morose. Sometimes he is violent to those about him, but this is rare. Intellectually the patient suffers somewhat. He is not able to study as before, and the memory may be impaired. Sometimes there is a mild form of dementia.
During the course of the disease there may be exacerbations, and sometimes after convalescence has seemed established there are relapses.
Recovery is gradual, and as the abnormal movements cease the mental condition improves, and the patient regains his health without any traces of the disease remaining.
We will now consider some of the symptoms separately. First, as to disturbances of motion. As before remarked, the disorderly movements occur soon after the general restlessness is seen. They most commonly begin in one upper extremity. The hand is thrown into various positions, the fingers are flexed and extended or separated, and all of the movements occur with great rapidity. In a day or two the whole arm is affected, and then the leg of the same side is involved in the jerkings and twitchings. In many cases the facial muscles are contorted, the mouth is pursed up or opened wide, and then quickly twisted into some other shape. If the patient is told to put out his tongue, it is protruded after a moment's hesitation, and then suddenly retracted, the jaws coming together with a snap. A smacking sound is made with the lips quite often, and words are uttered involuntarily. The movements may remain confined to one side of the body, constituting what is called hemichorea. This is quite common, and the right side is rather more frequently involved than the left. There is so great difference of opinion among authors on this point that it is probable that one side is affected about as often as the other. Of 252 cases which I have examined, 69 were right and 43 left hemichoreas. Gerhard20found in 80 cases of chorea that 32 were unilateral; of these 20 were right and 12 were left. Sée, however, found that in 97 of 154 cases the movements were either confined to the left side or were more marked on that side. He states that in his experience the proportion between left and right hemichorea is as 37 to 27. Pye-Smith in 33 cases of unilateral chorea found 15 on the right and 18 on the left side. Many cases which begin as hemichorea soon become general.
20American Journal of Med. Sci.
The disease reaches its greatest severity in about two weeks, and if the case is a bad one we find by this time all of the voluntary muscles are in constant movement. At this time the French name for chorea, folie musculaire, is most appropriate. Patients are often unable to walk or to sit up, and sometimes they may be thrown from the bed by violent spasmodic movements of the trunk. Strange as it seems, patients rarely complain of fatigue, notwithstanding the violent muscular exercise. This is probably because each set of movements is of short duration and is constantly changing its seat.
As a rule, the movements cease completely during sleep or under an anæsthetic. Sometimes occasional twitchings of muscles are seen in sleep, and in rare instances we are told by the parents of a child with chorea that the movements are as active in sleep as in the waking hours.
The movements of chorea occur either while the limbs are at rest or under the influence of voluntary effort. This fact has been pointed out by Mitchell and by Gowers. In some cases the movements are most marked when the patient is at rest. If a directed effort is made to use the member for a time, the choreic movements are suspended. For example, a patient may be able to carry a glass of water to the mouth withoutspilling a drop, while a moment before the hand may have been performing a continual dance. I have often observed that while the limb to which the whole attention has been directed in performing some movement has been steadied, the other limbs become violently agitated.
In another class of cases the movements are comparatively slight when the part is at rest, but when a motion is attempted the disorder of the muscles is so much increased that it is almost impossible for the act to be completed. The patient is told to pick up some small object: he throws the hand out toward it, and it is jerked away before he can grasp it. He again puts the hand forward, reaches the object, and the fingers open and shut and sprawl over the article before it is taken up. Sometimes it cannot be grasped at all. This has been called choreic ataxia, but it is only one type of the cases commonly seen.
This brings us to the influence of the will on the movements in chorea. There are some cases, as mentioned above, in which the movements may be controlled by the will for a brief period, but they will sooner or later return. In other cases it is quite impossible for the patient to check the movements at all, and one frequently sees in a case of hemichorea the sound hand used to grasp the other, so as to control the movements. We have referred to this because of Niemeyer's opinion that corporeal punishment would shorten an attack of chorea.
Chorea is sometimes confined to a single muscle or group of muscles. When limited in this way it is generally in the head, face, or perhaps in the shoulder. These cases of localized chorea have been spoken of by Mitchell as habit chorea.21They are often very obstinate in resisting treatment, and sometimes last during life.
21Lectures on Nervous Diseases, p. 146.
PARALYSIS.—Not infrequently in chorea there is paralysis to a greater or less extent. It is generally one-sided, and most often involves the upper extremity. The limb affected is the one in which the movements were most violent. The arm may hang entirely powerless or it may be only enfeebled, and feeling to the patient like a dead weight. The paralysis always recovers with the chorea or soon after.
POST-PARALYTICCHOREA.—Under this term Mitchell and Charcot have described a variety of chorea which is seen in patients after an attack of hemiplegia. The movements are chiefly on voluntary effort, and are those of inco-ordination. They come on from one to several months after an attack of unilateral paralysis, and are sometimes seen in cases in which almost complete recovery has taken place. Mitchell has reported22a case which was under my care for several years, and which he saw in consultation with me. This patient had two attacks of left hemiplegia, the last being fatal. After the first attack there was great gain of power to use the arm and leg, but the movements were performed awkwardly and with an irregular jerking movement. A post-mortem examination revealed a spot of softening the size of a filbert in the left corpus striatum, which was apparently recent, and a point of red degeneration in the right crus cerebri. The vessels at the base of the brain were extensively atheromatous.
22American Journal of the Med. Sci.
Of the electrical condition of the muscles in chorea but little is known. Rosenthal23found increase of faradic contractility in three cases of hemichorea, and the galvanic test showed a high degree of excitability,demonstrated by the fact that weak currents gave contractions at cathodic closure, or even tetanic contractions, and also contractions were produced at cathodic opening.
23Ziemssen's Cyclopædia, loc. cit., p. 434.
The affection of speech which is so common in chorea is due to disordered action of the laryngeal muscles, or it may be from choreic action of the abdominal muscles. Sometimes it is chiefly from the awkwardness of the tongue. The usual form of trouble is that the patient speaks in a staccato manner and the syllables seem as if they were driven out. When the chorea is in the laryngeal muscles, the tone and pitch of the voice are altered.
Chorea of the heart is sometimes spoken of, but it has never been satisfactorily demonstrated that there is any real disorder of cardiac rhythm in chorea. It is not unusual in chorea to meet with over-action or palpitation of the heart, but these conditions do not necessarily depend on the disease.
Valvular murmurs are often met with from the beginning of an attack. In some instances they are the result of an endocarditis, but frequently they are functional or anæmic. They are usually heard at the apex. Sometimes there is a reduplication of the first sound, giving the idea of a want of synchronism in action of the two sides of the heart; but this is probably not the result of chorea of the heart. I recall one patient, a child of seven or eight years, in whom the reduplication of the first sound was very distinct during an attack of St. Vitus's dance. She was brought to me at the beginning of a second attack a year later, and the reduplication of the cardiac sounds was heard again, so it is likely that it had continued during the interval, and was probably a congenital condition.
The pupils are commonly dilated in chorea and respond sluggishly to light.
REFLEXES.—I have examined the condition of the patellar reflex in 50 cases. In 26 of these it was present in normal degree, in 15 it was diminished, and in 9 it could not be excited. In one patient it was absent during the height of the choreic movements, but could be readily produced after the patient had recovered. The condition of the reflexes has also been examined by Joffroy and Saric,24and they found that of 16 cases of chorea the reflexes were abolished or diminished in 12.
24L'Union médicale, Sept. 22, 1885.
SENSIBILITY.—Authors state that disorders of sensation are met with in chorea, such as localized anæsthesia or a general hyperæsthesia: I have never met with any such instances. Patients often complain of pain in the joints or in the limbs, and this may be unaccompanied with swelling or tenderness on pressure. Tenderness on pressure over the vertebræ is rare in my experience, although others speak of its being of frequent occurrence. Mental disorders are generally present, but only to a slight extent. There is almost always irritability of temper and peevishness. The most sweet-tempered children become cross and perverse, laugh immoderately at trifling things, or cry as readily if they are annoyed. There is generally failure of memory and incapacity for study or thought. In most cases, however, this exists to so slight an extent as not to be noticed except on very close observation. Sometimes there is marked mental disorder amounting almost to imbecility, and occasionally the mental weakness remains for some time after the motor disorders have recovered.
The condition of the pulse is generally unchanged, but sometimes it is abnormally frequent. The temperature, according to Von Ziemssen, is unchanged.
The nutrition generally suffers. The patient rapidly loses flesh, and becomes anæmic; the skin grows dry, and the hair gets harsh. The digestion is apt to be disordered. The tongue is large, pallid, and coated thickly, and there is sometimes nausea or vomiting. The appetite is not good. The bowels are often constipated. The urine has been examined by several observers. Bence Jones found an excess of urea at the height of the disease. Albumen is not present except accidentally, but there is usually an excess of phosphates. In several cases in which we have examined the urine at the Infirmary for Nervous Diseases we found that the specific gravity was high while the chorea was at its height, but fell to normal as the patient recovered.
Chorea is spoken of as acute and chronic, but all cases are more or less chronic. Those cases which last eight or ten weeks may be considered acute, while those running on for months or years are properly called chronic.
DURATION.—Considerable difference of opinion exists as to the duration of chorea. Some writers speak of three or four weeks as an average attack. Gray and Tuckwell, in a series of cases treated by the expectant plan,25found an average duration of ten weeks. Occasionally a patient is seen with an attack of chorea which lasts only a few days. The parents of a little patient whom I saw a few days ago assured me that her second attack lasted only a week. They are educated and intelligent persons whose statement can be relied upon.
25Lancet, Nov. 28, 1876.
The course is not always regular. In some cases the disease gradually reaches a crisis, remains stationary for a few days, and then by degrees declines; in others there are exacerbations. The patient will seem to be almost well, and then become very much worse for a time. Relapses are not infrequent, and are generally caused by fright or excitement.
The recurrence of attacks of chorea is well known. A child who has had the disease one year may have it a second or third year. It is most likely to recur in the spring. Some cases have as many as five attacks, but as puberty approaches the attacks are lighter, and finally cease. Of 282 cases to which I have referred, 198 were first attacks, 47 had had chorea twice; 23 were in their third attack, 8 in the fourth, and 3 in the fifth attack.
TERMINATION.—The disease in most instances terminates in complete cure, but sometimes there is nervousness or want of co-ordination remaining for a time. Rarely the inco-ordination or a certain quickness in movement becomes permanent.
Death is a rare termination of chorea except in pregnancy. If it does occur, it is usually from some complication. In pregnancy the mortality is great. Of 64 cases collected by Wenzel, 18 died. In Philadelphia, in seventy-four years from 1807 to 1881, there have been but 64 deaths from chorea; of these, 38 were under twenty years, and 26 over that age.
Hutchinson reports a fatal case in a boy of twelve years.26After complaining of headache and rheumatic pains for several days, choreicmovements began. They soon became general and very violent. At the end of two weeks he was admitted to the Pennsylvania Hospital. At this time the patient was so extremely convulsed that it was impossible to keep him in bed without tying him. The movements continued but little abated, and the child died in two days.
26Philadelphia Med. Times, vol. vi. p. 535.
Another case of unusual interest is reported by Hunt.27The patient, a man of twenty-nine years, had suffered from chorea of the face and arms for years. In consequence of a fall on the pavement he fractured the left humerus. The movements were immediately exaggerated, and in spite of a carefully adjusted splint it was impossible to keep the arm at rest. The fragments were in a state of constant movement, and the points of bone threatened to penetrate the skin. The skin was so much excoriated that it was determined to dispense with the splint and attempt to keep the limb at rest by the administration of morphia hypodermically in half-grain doses three times daily. This failed to keep the arm quiet, and the seat of fracture became greatly inflamed. No form of appliance or medication succeeded in keeping the arm at rest, and the patient finally sank and died from exhaustion on the tenth day after admission to the hospital. The post-mortem examination revealed no gross lesion of the brain or cord. No microscopic examination was made of the brain.
27Pennsylvania Hospital Reports, vol. ii.
MORBIDANATOMY ANDPATHOLOGY.—In a disease so seldom fatal as chorea it is not surprising that there have been but few post-mortem examinations made. In the earlier autopsies, before the microscope was extensively used, but little of value was recorded. Sée, who collected 84 cases in which post-mortem examinations were made, reported that in 16 no changes were found in the nervous system. In 32 there were lesions in the brain and nervous centres, usually softening and tuberculosis, and in the remainder inflammatory changes in the serous membranes. In 29 there were evidences of heart disease. Sée considered that but few cases of death in chorea were caused by inflammatory diseases of the heart, but that the majority should be referred to nervous excitement and anæmia.
Ogle28in a report of 96 cases of chorea mentions 16 which were fatal. Post-mortem examinations were made in all of these. Cardiac lesions were found in 13. In 10 of these deposits were found upon the valves, and in 3 there was some change in the pericardium. He speaks of having noted congestion of the nervous centres six times, and softening of the cord once.
28Brit. and For. Med.-Chir. Review, Jan., 1868.
In all of 11 autopsies reported by Pye-Smith29there were cardiac lesions found. In every case old or recent deposits were observed upon the valves. In two instances the heart was hypertrophied, and in one there was pericarditis. Changes in the nervous system were less often found by this writer. In 1 case there was hyperæmia of the cord, and in 3 cerebral hyperæmia.
29Guy's Hospital Reports, 1874.
Dickinson found in 22 fatal cases of chorea 17 in which the heart was diseased. “In every instance making up the large tale of cardiac disease there were recent vegetations on the mitral valve, and often also elsewhere.”
In the fatal case of Hutchinson referred to above the heart was found diseased, the aortic valves were incompetent, the leaflets being swollenand softened, and the aorta was atheromatous above the sinus of Valsalva.
Of late years a number of careful autopsies have been made in cases of chorea. The brain and spinal cord have been closely examined, and in almost every instance some lesion has been found in both of these organs.
Steiner reported in 1868 the results of post-mortem examinations in 3 fatal cases of chorea. In 1 case he found cerebro-spinal anæmia, serous effusion into the spinal canal, and proliferation of the connective tissue in the upper part of the cord; and in another hyperæmia of the brain and cord.
Elischer,30who reports a fatal case in a parturient woman who had an attack of chorea in her eighth year, two in her sixteenth year, and another in a previous pregnancy, found at the autopsy hyperæmia and œdema of the brain and gray substance of the cord. Microscopically, the brain showed fatty, amyloid, and pigmentary changes in the nerve-elements and vessels of the large central ganglia, small secondary extravasations of blood in the connective tissue, and numerous emboli in the smallest vessels, especially in the cortex. In the spinal cord there was seen abundant proliferation of nuclei in the adventitia of the vessels. In the central canal serum was found, and the surrounding connective tissue was harder than usual.
30Cyclopædia of the Practice of Medicine, Von Ziemssen, vol. xiv. p. 450.
Dickinson has contributed an excellent paper on the pathology of chorea.31He relates the particulars of the autopsies in 7 fatal cases in which he personally made microscopical examination of the brain and spinal cord. He also adds the results of post-mortem examinations in 17 other cases at St. George's Hospital and at the Hospital for Sick Children. In all of the 7 cases in which microscopic examinations of the brain and cord were made there were found hyperæmia of both of these structures, in many instances hemorrhages into the substance of the nervous tissues, dilatation of the smaller vessels, and in chronic cases sclerotic changes in the course of the vessels. “The first visible change,” he remarks, “would seem to be the injection or distension of the arteries, succeeded by extrusion of their contents, to the irritation and injury of the surrounding tissue.” The changes seemed to affect both brain and cord in all cases. The parts of the brain most constantly affected lay between the base and the floor of the lateral ventricles in the track of the middle cerebral arteries, the substantia perforata, the corpora striata, and the beginning of the Sylvian fissures. “Of the cord no region was exempt, but perhaps the cervical and dorsal regions were usually more affected than the lumbar. With regard to the vertical or physiological divisions of the cord, these all, whether white or gray, shared in the vascular destruction; this condition, however, was usually most marked in the vessels belonging to or in connection with the lateral part of the gray matter about the root of each posterior horn. And it is to be observed that this was also the chosen situation of the more definite and special changes, whether hemorrhagic (as in two instances), sclerose, or exudatory. Speaking generally, the chosen seats of the choreic changes are the parts of the brain which lie between the beginning of the middlecerebral arteries and the corpora striata—the parta perforata; and in the cord the central portion of each lateral mass of gray matter comprising the root of each posterior horn.”
31Medico-Chirurgical Transactions, vol. xli. p. 1, 1876.
The embolic theory of chorea has been held by several investigators, among them Hughlings-Jackson. It is undoubtedly an attractive and reasonable view, especially when we consider the large proportion of cases in which there is valvular disease of the heart. Dickinson, however, does not consider this hypothesis tenable. In none of the cases in which he made post-mortem examinations did he find evidences of embolism. “In none of the instances described were decolorized fibrin, detached clots, or signs of impaction detected, and the erraticism of embolic accident was wanting: the constancy indeed with which the changes repeated themselves in certain positions, and the equality with which they affected both sides of the body, are conclusive objections to this hypothesis. The corpora striata, for example, were affected with almost absolute symmetry, notwithstanding that these bodies receive their blood respectively from the right and left carotids and different parts of the aortic arch.”
Rheumatism is associated with or precedes chorea in a large proportion of cases, and this was pointed out by Kirkes in 1850 and again in 1863. This connection between rheumatism and chorea, and the frequent occurrence of endocarditis in chorea, has led some authors to believe that the endocarditis is always rheumatic, and that the chorea is the result of the endocarditis. Dickinson, however, points out that in cases in which there is a distinct history of the chorea beginning suddenly from fright there are often well-marked cardiac murmurs heard. He believes that in all cases of chorea in which there are cardiac murmurs they are due to endocarditis, and suggests that in these cases from fright the endocarditis is due to irregularity of cardiac action. This, of course, is mere hypothesis, and we must bear in mind that in all cases of chorea there is anæmia, and that the murmur may be purely functional.
H. C. Wood, in a communication read before the College of Physicians of Philadelphia,32gives his views of the pathology of chorea, based upon the results of post-mortem examinations made in a number of dogs who had the disease. He believes the history of chorea to be this: “Owing to emotional disturbance, sometimes stopping of various vessels of the brain, or sometimes the presence of organic disease, there is an altered condition of the ganglionic cells throughout the nerve-centres. If the cause is removed and the altered condition of the nerve-cells goes only so far, it remains what we call a functional disease. If it goes so far that the cells show alteration, we have an organic disease of the nervous system.”
32Philada. Med. News, May 30, 1885.
In two dogs which were choreic the movements continued after section of the cord. This shows that in dogs, at any rate, the movements originate in the cord. In four instances of canine chorea in which Wool made autopsies there were found in the cords of three mild grades of infiltration of leucocytes in the gray matter. In the fourth, in which the dog had died of the disease, the ganglion-cells were degenerated, and in some places had disappeared. He concludes, therefore, that choreic movements may depend upon a diseased condition of the motor cells of the cord.
Although there are several recorded cases of human chorea in which lesions of the spinal ganglionic cells have been found, we cannot believe that this can be a constant lesion in chorea. The disease is too transient in many cases, and presents too many variations and anomalies, for the cord to be always the seat of the diseased condition.
In an interesting paper read by Angel Money before the London Medical and Chirurgical Society in 1885 he detailed some experiments in which, by injecting a fluid containing arrowroot, starch-granules, or carmine into the carotids of animals, he produced movements closely resembling chorea; and this was found to be associated always with embolism of the capillaries of the cord. In the discussion which followed Broadbent and Sturges expressed their disbelief in the embolic origin of chorea in man. Hughlings-Jackson said that he held the view of the cerebral origin of chorea, one of his reasons being the frequency with which the face-muscles are affected in this disease.
The probabilities are that in chorea there is a disordered condition of the brain and cord more or less general. The lesions are no doubt slight in mild cases of short duration, but in severe cases of long standing there occur well-marked changes in portions of both brain and cord. We cannot do better than to sum up the pathology of chorea in the words of Dickinson: “A widely-distributed hyperæmia of the nervous centres, not due to any mechanical mischance, but produced by causes mainly of two kinds—one a morbid, probably a humoral, influence which may affect the nervous centres as it affects other organs and tissues; the other, irritation in some mode, usually mental, but sometimes what is called reflex, which especially belongs to and disturbs the nervous system, and affects persons differently according to the inherent mobility of their nature.”
DIAGNOSIS.—The only diseases for which chorea may be mistaken are paralysis agitans and disseminated sclerosis. The former occurs only in adult life, and the tremor is of a regular rhythmical character. In the latter the tremor occurs only on voluntary effort, and is also more regular than the movements of chorea. There are forms of congenital sclerosis seen in children which closely resemble chorea. Here the duration of the disease and the association of contractures with it distinguish it from chorea.
Hysterical subjects have a form of chorea which can only be differentiated from the true disorder by noting the general hysterical character of the case and the result of treatment, which strongly influences the will-power of the patient.
PROGNOSIS.—In the great majority of cases this is favorable. If the disease occurs in childhood and is without complications, recovery generally takes place spontaneously after a few weeks. Should the movements be violent and continuous, so as to interfere with sleep and the taking of food, or should there be any complication, such as acute rheumatism or cardiac disorder, then the prospect of recovery is not so good.
The prognosis as to relapses should be given with caution. If in a child, it is possible that there will be a return of the disease after a longer or shorter interval. It is not likely to recur until after several months, usually at about the same season the following year. As the child grows older the intervals become longer, and it may safely beasserted that after puberty is passed and bodily development completed there will be no more returns of the affection.
The cure is usually complete. It must be remembered, however, that for some time after an apparently complete cure there may be slight inco-ordination of movements, particularly in the arms and the face. These are shown in the unnecessary haste in making uncertain motions or in slight grimaces, or if excited an awkwardness in the use of the fingers.
Death is a rare termination in uncomplicated cases, especially in children. The fatal cases are generally when acute rheumatism has been associated with the chorea or when there has been a fracture or an injury as a complication.
In Sée's statistics there is a mortality of 5.7 per cent. in 158 cases in the Children's Hospital. In adults, and more particularly in pregnant women, death is more common. Wenzel's cases referred to above gave a mortality of 27.3 per cent.
The cause of death in chorea may be from the intensity of the disease, and in this case the symptoms are generally violent from the outset, increase to an extreme extent, and then collapse and coma come on. The movements may cease when the collapse occurs, but they may continue to the last, growing gradually less until death.
TREATMENT.—A vast number of remedies have been popular in this disease from all ages. The medicine which is most generally depended upon at the present day is arsenic. It is advised by most writers, and in my own experience is decidedly the most reliable remedy for chorea which we know. The best way to administer it is in the form of Fowler's solution, and it should be given in large doses. I have given the bromide of arsenic, but did not find it superior to Fowler's solution. The amount of arsenic which can be safely borne by children with chorea is surprising to those who have not had experience in its administration. The medicine should be given in gradually increasing doses until the toxic effects are well marked or until the patient is convalescing. In a child of six years three drops may be given to begin with, three times a day. One drop additional should be added to the dose each day, and the child soon acquires a remarkable tolerance of the drug. As much as twelve or fifteen drops at a dose is borne by a child of eight years. If vomiting or much œdema of the face occurs, the medicine should be stopped for a day or two, and then the original dose should be taken, to be again increased as before.
Seguin recommends that the patient should begin again with the dose at which tolerance ceased. For instance, if vomiting occurred after a dose of nine drops, he stops the medicine for a day, and begins again with eight drops. I have found that sometimes this causes vomiting again, and I think it preferable to resume the medicine with a small dose.
It is often seen that a patient becomes worse during the first few days that the arsenic is taken, but improvement generally begins after a week of the arsenical treatment, and is well marked after two weeks.
In obstinate cases it is of marked advantage to give the arsenic hypodermically. Cases which do not yield to the drug when given by the mouth often improve at once when it is given hypodermically. Chronic cases which have resisted all forms of medication sometimes are cured byhypodermic injections of arsenic. For giving arsenic in this way it is best to use Fowler's solution, made without the compound spirit of lavender. It is less likely to cause abscess to form at the point of puncture.
Other remedies enjoy a reputation in the treatment of chorea. Sulphate of zinc is relied upon by many, and it is the means which Ross recommends. It should be given in increasing doses like arsenic, and very large doses may often be taken without disturbing the stomach. Trousseau, Hammond, and Hamilton favor strychnia, but I have had no experience in its use.
Cimicifuga and conium are both often beneficial in their effects. I have seen the former do good when arsenic had failed. Conium to be efficacious must be given in large doses. Eserine and hyoscyamine have both been successfully employed, the former by Bouchut, and the latter by Oulmont and Laurent. Recently, DaCosta33has reported, in a clinical lecture at the Pennsylvania Hospital, a case of very severe chorea successfully treated with hyoscyamine. The patient was a boy of eleven years, and the disorder had followed an attack of acute rheumatism. He was given1/100gr. of hyoscyamine three times a day.
33Philada. Med. Times, Jan. 23, 1886.
Ziegler34has recorded several cases which recovered under the use of nitrite of amyl. The bromides and chloral are useful adjuncts to treatment in case of sleeplessness or mental irritability. Cases of cure by the use of chloral alone have been reported. Bouchut gave a girl of fourteen and a half years, with chorea and dementia, 45 grains of chloral a day for twenty-seven days. She slept most of the time, but improvement was seen on the fifth day, and cure was completed on the twenty-eighth day of the use of the chloral. Electricity has been efficient in the hands of many writers. I have found galvanization of the spine to produce a quieting effect in some cases.
34Ibid., vol. vi. p. 486.
Iron is always of use in chorea; it may be given during the course of the disease, and is generally necessary in convalescence. Cod-liver oil or malt extract should be given in feeble persons.
It is scarcely necessary to mention the other remedies which have been recommended. DaCosta has used the bromide of iron. H. C. Wood has used a preparation of skunk cabbage, and there are a great number of other remedies which have been found of value.
Next to the internal means come external applications. Baths and frictions are useful in their effect on the general health. The ether spray to the spine or the application of an ice-bag for ten minutes once or twice daily is sometimes found to assist the other means. Cold douches have been advised by some, but they may do harm. The care of the general health of the patient is of first importance, and his surroundings should be as quiet as possible.
It is of the greatest value in bad cases to place the patient in bed and keep him there until the symptoms improve. John Van Bibber of Baltimore has treated a number of cases of chorea successfully by keeping them secluded in a darkened room. Such an extreme degree of isolation is not often necessary, and it might make a child more nervous.
In children the patient should always be taken from school and kept from exciting play. Plenty of fresh air and wholesome food should beinsisted upon. Change of air to the mountains or to the seashore often effects a cure in a short time.
Some cases do not appear to be benefited by any treatment. These are the hereditary form of chorea and some of the localized choreas. The latter are often helped or cured by the hypodermic use of arsenic even in long-standing cases.