BYWHARTON SINKLER, M.D.
BYWHARTON SINKLER, M.D.
Tremor is a prominent symptom of many diseases of the nervous system, and is met with as an effect of certain poisons which have been taken into the system; so it should not be considered as a disease in itself. It may, however, occur without being associated with any other abnormal condition which can be discovered. It is then called tremor simplex or tremor essentialis. The tremor of old age (tremor senilis) comes under this head.
Tremor is sometimes hereditary, and may exist from early life. I have a patient in whom there is a trembling of the hands which has lasted since childhood. This lady's mother and grandmother both had the same form of tremor, and one of her own daughters also has it. In this case the trembling is most marked when voluntary movements are attempted, but it does not materially interfere with writing, sewing, or any other act she wishes to accomplish. There is slight tremor when the hands are at rest.
Tremor simplex is seen in hysteria. In this disease it affects the hands and the facial muscles as well. It is not uncommon in these cases to find the tongue tremble excessively when protruded.
Tremor from chronic poisoning is usually from the absorption of lead, mercury, or some of the narcotic drugs or alcohol. Lead tremor is to be looked for among persons who are exposed to the action of lead, such as painters, printers, or manufacturers of white lead.1Such persons generally have had some other symptom of lead-poisoning, such as colic or paralysis. The tremor, however, may be the only symptom of saturnine poisoning. Mercurial tremor is not so often seen. It occurs in looking-glass makers or those who work in quicksilver, and may also be a result of the medicinal administration of mercury. The tremor from the excessive use of alcohol or opium is familiar to all. Tobacco, if used immoderately, also causes trembling in the hands. Tea or coffee may have the same effect. There are other drugs which, when taken for a length of time, are liable to cause tremor. Quinine is one of these.
1Lead in hair dyes or in cosmetic powders often gives rise to plumbism by its absorption by the skin.
Exhausting diseases, like the fevers, or any conditions which enfeeble the system, cause tremor which occurs in voluntary effect. I saw a lady some years ago who was greatly weakened by a malignant growth. She was extremely anxious to sign her name to a legal paper, but, although the hand was perfectly quiet when at rest, when she attempted to writethe first letter such intense tremor came on that it was impossible for her to make any mark which was legible.
Tremor follows violent bodily exertion or mental excitement. The action of cold or the chill of intermittent fever is accompanied with an extreme degree of trembling, which we all know. Tremor is also a result of neuritis, but in this case it is associated with other symptoms.
SYMPTOMS.—Tremor is met with as a fine or a coarse trembling. We may also find a fibrillar tremor, such as exists in progressive muscular atrophy. Tremor is divided by some (Van Swieten, Charcot, and others) into two classes: the first is where the tremor occurs while the part is at rest; the second is where it comes on during volitional muscular movements. The former has been termed by Van Swieten tremor coactus, because he believed that it arose from an irritation which affected the nervous centres in an intermittent way. The latter he conceived to depend upon a defect of stimulus, the result of an insufficient amount of nervous fluid, which causes contraction of the muscles under the influence of the will. This he called tremor a debilitate.2
2Charcot,Lectures on Diseases of the Nervous System.
In paralysis agitans we have an example of tremor coactus, and in disseminated sclerosis, where the tremor occurs only as muscular effect, it belongs to the variety of tremor a debilitate. Those divisions, however, are of but little importance.
When tremor first begins it is slight in degree and extent, and occurs generally only on voluntary effort. Later there may be a constant trembling even when the part is at rest. Beginning usually in the hands, it may extend to the head and legs. It is seen in the tongue and facial muscles after the disease has lasted for some time.
In some cases the trembling can be controlled to some extent by a strong effort of will. The tremor from alcohol or opium is most marked when the individual has been without the use of the stimulant for a short time, and the trembling may be temporarily checked by renewing the dose of alcohol or opium as the case may be.
The muscular trembling from plumbism and mercurial poisoning is more violent than the other forms of simple tremor, and often resembles the tremor of paralysis agitans. In toxic tremors there are often secondary paretic symptoms and indications of other disturbances of the brain and nervous system.
In simple tremor there is no loss of muscular power, and the electrical reactions of the affected muscles are not abnormal. The duration of simple tremor is almost always great. Usually it persists throughout life, becoming more general and more intense as the subject of it grows older. The tremor of hysteria is shorter in duration. Occasionally there are seen cases of simple tremor, which are apparently the result of some trivial cause in a nervous person, which last but a short time.
I have seen a case of tremor of the head in a woman of about forty years, in which the trembling ceased entirely after it had lasted several weeks. Hammond3describes what he calls convulsive tremor. Under this name he includes cases of non-rhythmical tremor or clonic convulsions, which are unaccompanied by loss of consciousness, but are paroxysmal in character. Pritchard in 1822 presented an account of this affection andrelated two cases; Hammond mentions six cases. The affection is characterized by paroxysms of violent and rapid convulsive movements, which are more or less general and occur many times a day. The seizures last from a few minutes to several hours.
3Diseases of the Nervous System, p. 696.
ThePROGNOSISin convulsive tremor seems to be favorable.
Tremor may be regarded as a form of clonic spasm. It consists of slight intermittent contractions of individual muscles or groups of muscles. Fibrillar tremor, such as is seen in progressive muscular atrophy, depends on contractions and relaxations of the muscular fibrillæ, and can be seen under the skin, but does not cause any movements of the limb.
There are no pathological data for explaining what portions of the nervous system are the seat of disease in simple tremor. In experiments upon the lower animals it has been found that trembling occurs in muscles which have been separated from the nerve-centres by division of the nerve. So too in man: when there has been a wound or section of a nerve accidentally, there is likely to be tremor in the muscles which it supplies.
The tremor does not begin at once on section of the nerve, but comes on after a few days. As the peripheral end of the nerve undergoes degeneration the tremor increases. It may last months or even years.
In some of the conditions where tremor occurs the influence of the will is weakened or is entirely absent. This is seen in hysterical trembling and in the tremor of old age as well as in those cases where there is general enfeeblement of the body, as in the fevers.
Trembling is connected with disease of the pyramidal tracts, because in this way the influence of the cerebral centres is withheld from the muscles. When a muscle is in a condition of tonic spasm, it is the result of the running together of very rapidly-repeated muscular contractions. It is like the contraction in a muscle from an interrupted electrical current. If the interruptions are slow, the muscular contractions are seen at intervals like a tremor; but if the interruptions are rapid from frequent vibrations of the hammer of the instrument, then the contractions in the muscle are fused together, as it were, and the muscle is in a state of tonic spasm.
It is held by some writers that tremor is caused by the want of balance between the cerebrum and cerebellum. When, for example, the control of the cerebrum is enfeebled the action of the cerebellum is so great as to bring about tremor by its uncontrolled power.
If we accept the first view, we must consider the tremor as a preliminary stage of paralysis; for the lesion, which at first is slight and causes only an interruption of the conduction of impulses from the brain to the muscles, as it becomes more extensive totally prevents conduction, and paralysis ensues.
Hughlings-Jackson's view, that general convulsions are the result of discharges from the cortex of the brain, and that the tonic contractions of tetanus are caused by discharges from the cortex of the cerebellum, may be applied to the pathology of tremor as well. When, for instance, in a disease like disseminated sclerosis a voluntary effort instead of causing a steady muscular contraction results in irregular spasmodic contractions and relaxations of the muscle, we may imagine that a series of discharges were taking place from the cortex of the cerebellum as long as the voluntary efforts were persisted in. On the other hand, in paralysis agitansit is more probable that a lack of conducting power in the pyramidal tracts prevents the influence of the centres being continuously exerted upon the muscles through their motor nerves.
It is probable that in simple tremor the lesion is situated in the spinal cord; for in this disease we seldom see any evidences of cerebral disturbance. There are no paralytic or psychical symptoms, and no vertigo. In toxic tremors the disease is no doubt located in the brain, for accompanying the trembling resulting from alcohol, opium, mercury, and other drugs are mental changes and more or less muscular enfeeblement.
TREATMENT.—Should the tremor depend upon some cause which can be discovered, of course the obvious course is to attempt to remove the source of trouble. The effort is of greater or less success in different conditions. The tremor from mercurial poisoning sometimes yields to treatment which is directed to the elimination of the mercury. The free administration of the iodide of potassium is the best means to be used, and is often successful. The same means are available in lead tremor. Of course the patient must be removed during treatment from the risk of further absorption of the poisonous substances.
In simple tremor many remedies have been recommended, but the results of treatment are not encouraging. Baths of various kinds and galvanism have been used, and many drugs are advised. Hyoscyamus and its alkaloid, hyoscyamine, have enjoyed a high reputation, and good results have been reported from their use. I have seen relief, but not cure, from their administration. Arsenic is a more reliable remedy and it may be used hypodermically. Eulenburg4has used this method with good results. I have given arsenic per orem with beneficial effects in cases of simple tremor. In a case to which I have referred above the tremor was relieved while the patient was taking Fowler's solution, and on changing to hyoscyamus the trembling got worse. On returning to the arsenic the symptoms improved, and finally the tremor ceased after the remedy had been taken for some weeks. Hysterical tremor requires that the hysteria should be relieved. Franklinic electricity sometimes controls the tremor in these cases.
4Ziemssen's Cyclopædia, vol. xiv. p. 392.
BYWHARTON SINKLER, M.D.
BYWHARTON SINKLER, M.D.
SYNONYMS.—Parkinson's disease; Shaking palsy; Trembling palsy; Senile chorea; Chorea festinans. The first name is due to the fact that the disease was first fully described by Parkinson in a book published in England in 1817.
Paralysis agitans is a neurosis, chronic in its forms and characterized by a tremor which gradually increases in extent and severity. The tremor is not increased by voluntary muscular movements. A peculiar manner of walking, known as festination, comes on later in the disease, and there are also alterations in the attitude of the head and trunk.
It is a disease which belongs to middle age, being rarely seen before forty years, although cases are quoted by Charcot as early as twelve and sixteen years. Constant and prolonged exposure to dampness and cold seems to bring on the disease, and it is sometimes caused by sudden emotion, like fear or distress. The following case is an instance of the latter:
Case I.—Mr. A. M——, æt. fifty-two years, consulted me Oct. 1, 1883. He is a bookbinder by occupation. His habits have been good. He had a chancre in 1861, but had no secondary troubles. He was in the army from 1861 until 1866. In 1866 he went into business for himself, and, although his business was large, he had no great anxiety or worry. His general health has been good, and he has had no illness except an attack of malarial fever about six years ago. In May, 1883, he was standing by an elevator door on the fourth floor of his place of business, and, seeing that the elevator was caught by something, released it. It immediately fell with a crash to the second floor, and as there were two or three persons on it, Mr. M—— thought they must have been killed or severely injured. He was greatly excited and alarmed, and soon after he had assured himself that none of the occupants of the elevator had been hurt, discovered a trembling of the right hand. The tremor has continued ever since, and has extended to the arm and leg.
On examination there is seen a coarse tremor of the right arm and leg while the limbs are at rest. A voluntary muscular movement stops the tremor, and it also ceases during sleep. When he makes an effort with the right hand, as, for instance, in squeezing the dynamometer, the tremor ceases in the arm, but becomes greatly exaggerated in the right leg. While occupied in doing anything he does not notice the tremor, and it stops when he is lying down.
The dynamometer shows, right hand 150°, left hand 120°. After two years have elapsed the disease has gradually progressed in severity.
SYMPTOMS.—The course of the disease has been divided into three stages—the period of invasion, the stationary period, and the terminal period.1
1Lectures on Diseases of the Nervous System, by J. M. Charcot.
Period of Invasion.—There are several modes of invasion, but the most frequent by far is slow in its onset. The disease comes on gradually, first showing itself as a slight tremor in the hand or fingers while the part is at rest. It is not constant, and ceases as soon as the patient's attention is called to it. There is sometimes preceding the tremor rheumatic or muscular pain in the affected arm. The tremor may first occur in the foot. Should the disease begin in the hand—and this is most common—the movements are peculiar. They may consist of a fine rhythmical tremor, or the fingers move in a methodical way over each other. Charcot speaks of the thumb and forefinger being rubbed together as if the patient were spinning wool. While this movement of the thumb and finger is going on the wrist is being flexed by jerks. During the early stages of the disease the tremor is observed only at intervals. It comes on intermittently when the patient is not thinking of it and while the limb is at rest, and ceases as soon as any voluntary muscular effort is attempted. The act of grasping the hand or taking up an object is enough to check it for the time. In a patient now under my care I have often noticed during the early stages a well-marked tremor of the right hand while it was lying in her lap, but it would at once cease when I called attention to it. As soon as the mind of the patient was diverted to some other subject the tremor would begin again.
As the disease progresses voluntary effort no longer controls the tremor, or if it does at all it is only for a few seconds, when it begins again. As the tremor increases in violence it extends to other parts of the body. At first it may have been confined to the hand; now it extends to the arm, a little later to the foot and leg on the same side. Then the other arm will be affected, and finally all of the limbs will succumb to the tremor.
Charcot speaks of decussated invasion—that is, the disease begins in the right upper extremity, for example, and next passes to the left lower extremity. This is a rare form; it is much more frequent to see the hemiplegic type, which may persist for some time, or the paraplegic type, when both legs are affected.
There is a progressive form of invasion when the tremor is not the first symptom. The patient has neuralgic or rheumatic pains in the limbs, which are afterward affected with tremor. Sometimes there is some mechanical injury of the limb, which subsequently is the seat of pain and tremor. The general health of the patient is at the same time more or less impaired. There is a sense of general weakness and lassitude; the temper is irritable, and there may be some vertigo. The features and countenance are characteristic even at the earliest periods of the disease. There is an absolute absence of expression, and the features are fixed. The face looks like a mask, and although the patient may smile or laugh, immediately after the features return to the original blank expression. Amidon showed two cases of paralysis agitans to the American Neurological Society in 1883, in which there was no tremor whatever, but all the other features of the disease were present.
After a great mental or moral shock the trembling begins suddenly, abrupt invasion, as in Case I., or the case described by Charcot, where the wife of a gendarme, seeing her husband's horse return riderless to the barracks, received a shock of great severity, which was followed on the same day by tremor. The tremor is at first confined to one limb. It may even disappear for a time, but gradually and slowly extends to the other limbs, and takes the same progressive course.
Period of Stationary Intensity.—After the disease has become fully developed the tremor is incessant. The intensity is not the same all the time. It may be augmented by cold, over-excitement, or voluntary effort, and is lessened by repose and sleep. The trembling ceases during anæsthesia.
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 towalk; 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 werea 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.
MORBIDANATOMY.—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
2Quoted by Hamilton,Diseases of the Nervous System, p. 500.
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
3Ross,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. Ross4suggests 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.
4Op. 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.
BYWHARTON SINKLER, M.D.
BYWHARTON 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
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
2Cyclopædia 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 reports3an 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.
3Lectures on Nervous Diseases, p. 69.
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,4has 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.
4Medical and Surgical Reporter, April 13, 1872.
Some additional cases of hereditary chorea have been lately recorded by Peretti in No. 52 of theBerliner klin. Wochenschrift, 1885, and others by Clarence King in theNew 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 hashad 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 age5—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.6The 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.
5Journal of Nervous and Mental Diseases, July, 1881.
6Lancet, 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:
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.