A system of practical medicine. By American authors. Vol. 5

Chapter XXVIII: Introduction: The term neurasthenia, signifying nervous weakness, and (6)

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During this time all the characteristic movements are at their height. The moving of the thumb and finger, already referred to, is present, and seems like a partly co-ordinated movement. Charcot describes these movements as being in some cases like the rolling of a pencil or a paper ball between the thumb and finger, and in others the movements, he says, are more complicated, and are like what occur in crumbling a piece of bread.

The handwriting is almost illegible, and every letter shows the excessive trembling of the hand, most marked in the up strokes of the pen. All this time the head and neck are unaffected. There is no nodding or shaking of the head to be observed on the closest inspection. This is an important fact to bear in mind, for it is a distinguishing feature between the disease under consideration and disseminated sclerosis. There is no nystagmus, and the muscles of the jaw are unaffected by tremor. The tongue, however, while lying on the floor of the mouth undergoes tremor, and this is increased when the organ is protruded.

The speech is slow and jerky, and the patient usually speaks in a low tone of voice. He eats his food without difficulty, but in advanced cases the saliva sometimes escapes from the mouth during deglutition. A characteristic symptom of the disease is a rigidity of the muscles of the extremities, trunk, and of the neck. When the muscular stiffness first begins the patient complains of cramps followed by a sense of rigidity. The flexor muscles are first affected. This stiffness causes peculiarities in the patient's attitudes. The head and neck are usually bent forward, and seem fixed in that position. The body is inclined slightly forward in standing. The elbows are held somewhat away from the chest, the forearms are flexed on the arms, and the hands are flexed on the forearms. The thumb and forefinger are extended and brought together as if holding a pencil. The other fingers are also flexed. The attitude of the hand and the prominence of the knuckles make it closely resemble the hand of rheumatoid arthritis. In paralysis agitans, however, there are no bony deposits in the joints, and no cracking is heard on bending the knuckles.

In the lower extremities there is often intense rigidity. Contractions occur, and the legs are strongly flexed. The feet often are extended in the position of equinus. It is this rigidity which causes the difficulty in walking (Charcot).

The gait of the patient now becomes very striking. He gets up from his seat slowly and with difficulty; hesitates a moment before starting to {436} walk; then, once having made a few steps, goes at a rapid pace. The tendency is to fall forward; in order to preserve his equilibrium the patient hurries forward as if to catch up with his centre of gravity. This gait has been called paralysis festinans—festination or propulsion.

Although propulsion is the usual form the gait assumes, sometimes there is a tendency to fall or run backward. This tendency is not always apparent even when it exists. Charcot has a method of showing its existence which is very successful: when a patient is standing he pulls her slightly backward by the skirt, and this is sufficient to start a movement of retropulsion.

Propulsion and retropulsion are not necessarily always present. Many cases progress to the end without these symptoms.

As the disease advances the muscles become more rigid and the patient is confined to bed. He is, however, restless from a sense of prostration and fatigue. He is unable to turn himself, and often calls to his attendant to change his position. The sufferers from this disease, although not having actual pain, complain of disagreeable sensations. There is a constant sense of excessive heat whether the temperature of the room be high or low. In winter they cannot bear much bed-clothes at night, and prefer to wear very light clothing. Associated with this sensation of heat is often profuse perspiration (Charcot). Notwithstanding all these troublesome sensations the sensibility of the skin is not changed. Heat and cold are readily felt, and there is no anæsthesia or analgesia.

Terminal Period.—The duration of paralysis agitans is generally great. The disease may extend over many years—even as long as thirty years in some cases. As the tremors and rigidity increase in intensity the patient becomes obliged to sit all day in a chair or is confined to bed. Occasionally the tremor becomes less while the rigidity increases. The nutrition suffers, and the muscles especially become greatly wasted. Up to a certain point the intellect remains unaffected, but late in the disease the mind fails. General prostration of the whole system sets in, bed-sores occur, the urine and feces are passed unconsciously, and the patient dies of exhaustion. It is not often that the end comes in this way. It is much more frequent that some intercurrent disease, like pneumonia, ends the life of the patient. The disease is undoubtedly a most painful and trying one to both patient and physician. It lasts for years, and there is no prospect of relief. Charcot says that he has seen the terminal period last for three or four years. The following case is a fair example of the disease:

Case II.—Margaret Hays, aged fifty-four, single, applied for treatment at the Infirmary for Nervous Diseases, Oct. 9, 1882. She is housekeeper for her brother, who keeps a restaurant. She has had to be up late at night, and has had very much washing to do for many years. The kitchen in which she is most of the time is damp, and opens into a yard into which she has often to go. About two years ago she thought that she hurt herself in lifting something, and soon after this, on putting her hands from hot into cold water, suddenly felt a numbness in both forearms. One year ago she noticed tremor in both hands and both legs. The tremor interfered with her work, and has increased. She also felt as if there were loss of power.

Present Condition.—The face is expressionless and looks as if it were {437} a mask. She articulates without using her lips to any extent, and speaks in a low tone, scanning the syllables. The head is held stiffly, and the attitude of the whole body is peculiar.

The tremor is fine, and is constant while the hands are at rest. On voluntary effort the tremor ceases. On examination with the dynamometer, right hand 95°, left hand 80°. She feels weak generally, and says she cannot use her hands even to button her clothes or to dress or undress without aid. She performs all movements slowly and with great deliberation. The patellar reflex is not excessive.

Sensation.—She has lost the sense of numbness she used to have, but says she cannot feel a needle between the fingers when she attempts to sew. There is loss of sensation in the finger-ends; the compass points are not felt at less than three lines.

Her walk is slow and deliberate like all her movements, and there is no festination. Her eyes were examined, and there was no decided change in the fundus and vision was about normal. Pupillary reflexes good.

This patient was under observation for several months, and steadily grew worse. One peculiar feature was observed, however: it was that at one time the tremor almost ceased, although all of the other symptoms were worse.

ETIOLOGY.—As already mentioned, the causes which have been observed are fright or sudden grief and prolonged exposure to cold and dampness. A number of cases of the former are mentioned by Charcot, and a case which I have related above is a good illustration of paralysis agitans produced by fright. When caused in this way the disease does not present any peculiar features in its progress or termination. I have seen many cases in which the disease had been preceded by more or less exposure to dampness. One of my patients had worked in a basement room which was damp; another (Case II.) was a great deal of the time in a kitchen which opened on a wet yard, and she was constantly going in and out of doors, getting her feet wet frequently. Sometimes irritation of a peripheral nerve seems to have been the origin of the trouble. Charcot quotes several cases of this kind.

Sex does not appear to exert any special influence in the production of the disease. Some writers assert that it is more common in males than in females, but Charcot in his large experience at La Salpêtrière has not found this to be the case.

MORBID ANATOMY.—A number of autopsies have been made in cases of paralysis agitans without any constant lesion of the nervous system having been discovered. Charcot refers to three cases in which he made careful post-mortem examinations in which the results were negative. Parkinson and Oppolzer each report one case in which was found induration of the pons, medulla, and cervical portion of the cord. More recently, however, Charcot and Joffroy have examined cases in which microscopic examination revealed blocking up of the central canal of the cord by increase of the epithelium of the ependyma and pigmentations of the ganglion-cells.

Leyden has reported a case in which the disease was confined to the right arm, and on post-mortem examination a tumor of the left optic thalamus was found.[2]

[Footnote 2: Quoted by Hamilton, _Diseases of the Nervous System_, p. 500.]

{438} Dowse and Kesteven found degeneration of the nerve-cells of the anterior pyramids, changes in the olivary body, nucleus of the ninth nerve, laminæ and corpus dentatum of the cerebellum and of the anterior cornua of the spinal cord. Also cortical sclerosis of the right lateral column of the cord and miliary changes in the white matter of the corpus striatum and hemispheres.[3]

[Footnote 3: Ross, _Diseases of the Nervous System_, vol. ii. p. 797.]

In this disease, as in chorea, there must be two classes of cases—those in which there is no lesion to be discovered after death, and others in which there are changes throughout the brain and spinal cord more or less widespread. The cases in which the disease comes on suddenly from some moral shock probably belong to the former class; while in cases which have come on gradually during senility one would expect to find organic changes in the nervous system. Ross[4] suggests that the cause of the tremor is probably a diminution in the conductivity of the fibres of the pyramidal tract, which prevents impulses from the cortex reaching the muscles in sufficiently close proximity to produce a continuous contraction. This, however, does not explain the cause of the tremor in the cases where it began suddenly from fright.

[Footnote 4: _Op. cit._, p. 798.]

DIAGNOSIS.—The only diseases with which paralysis agitans may be confounded are disseminated sclerosis, senile tremor, and chorea in the aged. From senile tremor it may be distinguished from the fact that it begins before old age—that the tremor is more excessive and the gait and facial expression are distinctive. Chorea in old persons resembles paralysis agitans, but is not progressive, the tremor is not lessened as a rule by voluntary effort, and the peculiar gait and expression of the face are wanting.

There are many points of difference between paralysis agitans and disseminated sclerosis, as can be seen below:

TREATMENT.—The results of treatment are not encouraging. Cases have been reported in which cures were effected, but it is doubtful if they were true instances of paralysis agitans. Hyoscyamus and conium have been given with temporary relief to the tremor. Trousseau recommends strychnia, but Charcot declares that it aggravated the cases in which he gave it. Hammond advises the use of galvanism, at the same time giving strychnia or phosphorus. I have seen one case in which decided relief was obtained from arsenic hypodermically, and another in which the patient was benefited for a long time while taking small doses of strychnia combined with iron and quinine, and at the same time static electricity was applied.

{439}

CHOREA.

BY WHARTON SINKLER, M.D.

Chorea, or St. Vitus's dance, has been known for years, and the literature of the subject, especially among the older writers, is as extensive as that of any other disease.

It has been recognized by a variety of names, some of the most common being derived from some saint who enjoyed a popular reputation of power to cure the disease. For example, it has been called St. John's dance, St. Guy's dance, St. Modestus's dance, and St. Anthony's dance. Besides these names it has been termed ballismus, paralysis vacillans, epilepsia saltatoria, and orchestromania.

It will be observed that almost all of the names which have been applied to the disease relate to a dancing movement. This arises from the fact that the first notice of the affection dates back to the fourteenth century, when a kind of religious mania appeared in Southern Europe in the form of an epidemic. It was characterized by excessive dancing and gesticulatory movements, and affected large numbers of people at a time. In 1375 an epidemic which arose was spoken of as St. John's dance, and in 1418, in another outbreak of the disorder which occurred at Strasburg, by the order of the authorities those suffering were conducted in troops to the chapel of St. Vitus in Zabern, and there masses were said and other religious ceremonies performed for its cure.

We are informed that St. Vitus removed from Sicily when a boy, at the time of Diocletian's persecution of the Christians in the year 303, and suffered martyrdom in Florence in company with Crescentia and his tutor, Modestus.[1]

[Footnote 1: “Hecker's Epidemics of the Middle Ages,” _Sydenham Society's Transactions_, contains full details of these outbreaks.]

Von Ziemssen states that as a pandemic disease the dancing mania died out in the fifteenth century, but that traces have remained on the Rhine up to the present time.[2]

[Footnote 2: _Cyclopædia of the Practice of Medicine_, vol. xiv. p. 416.]

In our own country there have been many epidemics of the same disorder on a small scale, and we may regard the Shakers as representing a type of the dancing mania. Weir Mitchell reports[3] an outbreak which occurred quite recently In a children's asylum in Philadelphia. Prompt measures and separation of those affected from the well children checked the disorder, which at one time threatened to spread through the entire institution.

[Footnote 3: _Lectures on Nervous Diseases_, p. 69.]

{440} These epidemics were quite different from what we now call chorea, and the individuals suffering were clearly affected by a psychical disorder of an hysterical form. In time, however, the name has come to be applied to a systematic disease characterized by irregular spasmodic movements of the limbs and other parts of the body.

The disease has been divided by some writers into chorea magna and chorea minor. The former, however, as described, is only an aggravated variety of hysteria, and need not be considered in connection with this subject.

Although the name chorea does not correctly describe the disease under consideration, it has been used for so many years that it is more convenient to retain it.

DEFINITION.—Chorea, as we now understand it, is a spasmodic neurosis, characterized by constant involuntary and irregular jerkings and twitchings of muscles or groups of muscles, which, in the majority of cases, cease during sleep, and are accompanied by more or less psychical disturbance in most instances.

ETIOLOGY.—Hereditary influence in the production of chorea is more or less marked. In some cases the connection seems to be remote, but in many instances it will be found that one of the parents has suffered from some form of nervous disease or has inherited a nervous diathesis.

George Huntington of Pomeroy, Ohio,[4] has recorded some remarkable instances of hereditary chorea. The affection, as he describes it, differs in many features from chorea as ordinarily met with, but it is apparently the same disease. It is found in the eastern end of Long Island, and has been studied in several generations by Huntington, his father, and grandfather. This part of Long Island is remarkably free from the usual type of chorea, none of these physicians having ever met with an example of it. The hereditary chorea is confined to a few families. It occurs more frequently in males than in females, and never attacks the patient until after middle life. It comes on gradually and takes years to develop, but when once established it yields to no form of treatment. In most cases there is a marked tendency to insanity and suicide in the later stages of the disease.

[Footnote 4: _Medical and Surgical Reporter_, April 13, 1872.]

Some additional cases of hereditary chorea have been lately recorded by Peretti in No. 52 of the _Berliner klin. Wochenschrift_, 1885, and others by Clarence King in the _New York Medical Journal_, vol. i., 1885. The history of all these cases is strikingly like those of Huntington, and establishes without question a distinct form of chorea.

Mrs. N., one of Peretti's cases, had a mental affection with choreic movements, and there was a history of a similar condition in her parents and grandparents. Two of Mrs. N.'s four children, Mrs. A. and Anton N., had chorea in adult life; some of these became insane. Mrs. A. had five children; three of these became choreic; one had tremors and one became insane. Anton N. had ten children; of these six had chorea. In all of Peretti's cases the disease came on after the age of forty years, and persisted. In several members of the family insanity was associated with the chorea.

In the families where it occurs the nervous temperament predominates. It sometimes will be found that neither of the parents of the patient has {441} had chorea or any other nervous disease, but that an uncle or an aunt has had St. Vitus's dance in childhood.

Chorea may occur at any period of life, from infancy to extreme old age. I have reported two cases in patients over eighty years of age[5]—one at eighty-two and the other at eighty-six—who had characteristic attacks of St. Vitus's dance. Robert Saundby has collected twelve cases of chorea in the aged. The two cases just referred to are included in the number. He considers the affection very rare in old persons.[6] The following case is an example of congenital chorea, and I believe this to be very unusual. The movements of all infants are choreic, so that it is difficult to say when the chorea begins; still, it seems fair to infer that when a child has never had any but choreic movements it is a case of congenital chorea:

Case. I.—Jennie W——, aged nineteen years. Family history is good as regards nervous diseases. Her mother was frightened by seeing a case of chorea some time before the child was born. The movements were observed at birth, and have continued always. The patient was brought to my clinic at the Infirmary for Nervous Diseases, and her condition noted as follows: The movements are general and continuous; the arms and legs are in constant motion, and the mouth is perpetually grimacing; there is tremor of the tongue when it is protruded; volitional efforts increase the movements; during sleep they cease entirely; tendon reflexes are normal; there is no paralysis; heart-sounds are normal and the general health is good; menstruation is regular, and the choreic movements were not influenced in any way at the time of its first appearance.

[Footnote 5: _Journal of Nervous and Mental Diseases_, July, 1881.]

[Footnote 6: _Lancet_, Nov. 24, 1884.]

Chorea occurs most frequently during the period of approaching puberty. Sée in an examination of 531 cases found 453 between the ages of six and fifteen years.

I have examined the notes of 282 cases of chorea, most of which are in the case-books of the Infirmary for Nervous Diseases; the rest are from my own note-books. Of the 282 cases, 217 were between six and fifteen years. They were distributed as follows:

Under 1 year 2 | At 7 years 23 | At 16 years 6
At 2 years 6 | 8 " 24 | 17 " 10
3 " 4 | 9 " 31 | 18 " 5
4 " 7 | 10 " 23 | 19 " 5
5 " 9 | 11 " 18 | 20 " 1
6 " 24 | 12 " 25 | 21 " 3
At 22, 25, 27, 28, 35, 38, 82, and 86 years, each 1 case.

Sex exerts considerable influence on the disease. Sée states that three-fourths of all the cases observed in the Children's Hospital in Paris occurred in girls. Of 328 cases which I have examined in reference to this point, 232 were females and 96 males.

Social condition has little or no effect on the production of chorea, but it is more common in cities than in the country. Indeed, everything which increases the excitability of the nervous system during development adds to the tendency to the disease.

West and Hamilton point to over-study as a cause, and I have frequently verified their observations. The annual examinations at our public schools give a number of cases of chorea.

{442} A. McLane Hamilton[7] has 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.

[Footnote 7: _American 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 Brown[8] found 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.

[Footnote 8: _Guy'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.[9] This 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. Hammond[10] believes “that the influence of rheumatism upon chorea is not greater than that of a depressing agent to the organism.”

[Footnote 9: _Ziemssen's Cyclopædia, loc. cit._, p. 427.]

[Footnote 10: _Dis. 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]

[Footnote 11: _Lancet_, 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.

{443} 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, 100-2/5°. 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. Barnes[12] has collected 58 cases, and Bodo-Wenzel[13] has 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, 14 {444} had had chorea in previous pregnancies. Sometimes the chorea appears only at the time of labor.

[Footnote 12: _Obstet. Transactions_, vol. x. p. 147.]

[Footnote 13: _Ziemssen'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 Mitchell[14] states that it does not appear to exist to any less extent in the southern than in the northern portion of this country.

[Footnote 14: _Loc. cit._]

Season, according to Mitchell,[15] largely 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,[16] who found that among 35 relapses, 13 were in spring, 12 in winter, 9 in autumn, and 1 in winter. On the contrary, Hammond[17] found 54 attacks in the six months from October to March, and but 28 in the remainder of the year. Gerhard[18] in 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.

[Footnote 15: _Loc. cit._, p. 128.]

[Footnote 16: _Ziemssen, loc. cit._, p. 443.]

[Footnote 17: _American Journ. Med. Sciences_, July, 1876.]

[Footnote 18: _Loc. 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.

Hamilton[19] has 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.

[Footnote 19: _Nervous Diseases_, p. 490.]

{445} 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.

{446} 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. Gerhard[20] found 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.

[Footnote 20: _American 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 without {447} spilling 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.[21] They are often very obstinate in resisting treatment, and sometimes last during life.

[Footnote 21: _Lectures 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-PARALYTIC CHOREA.—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 reported[22] a 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.

[Footnote 22: _American Journal of the Med. Sci._]

Of the electrical condition of the muscles in chorea but little is known. Rosenthal[23] found increase of faradic contractility in three cases of hemichorea, and the galvanic test showed a high degree of excitability, {448} demonstrated by the fact that weak currents gave contractions at cathodic closure, or even tetanic contractions, and also contractions were produced at cathodic opening.

[Footnote 23: _Ziemssen'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,[24] and they found that of 16 cases of chorea the reflexes were abolished or diminished in 12.

[Footnote 24: _L'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.

{449} 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,[25] found 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.

[Footnote 25: _Lancet_, 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.[26] After complaining of headache and rheumatic pains for several days, choreic {450} movements 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.

[Footnote 26: _Philadelphia Med. Times_, vol. vi. p. 535.]

Another case of unusual interest is reported by Hunt.[27] The 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.

[Footnote 27: _Pennsylvania Hospital Reports_, vol. ii.]

MORBID ANATOMY AND PATHOLOGY.—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.

Ogle[28] in 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.

[Footnote 28: _Brit. and For. Med.-Chir. Review_, Jan., 1868.]

In all of 11 autopsies reported by Pye-Smith[29] there 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.

[Footnote 29: _Guy'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 swollen {451} and 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.

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A system of practical medicine. By American authors. Vol. 5Chapter XXVIII: Introduction: The term neurasthenia, signifying nervous weakness, and (6)

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