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

Chapter XXIX: Introduction: The term neurasthenia, signifying nervous weakness, and (7)

Text size

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,[30] who 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.

[Footnote 30: _Cyclopædia of the Practice of Medicine_, Von Ziemssen, vol. xiv. p. 450.]

Dickinson has contributed an excellent paper on the pathology of chorea.[31] He 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 middle {452} cerebral 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.”

[Footnote 31: _Medico-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,[32] gives 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.”

[Footnote 32: _Philada. 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.

{453} 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 be {454} asserted 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 by {455} hypodermic 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, DaCosta[33] has 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 given 1/100 gr. of hyoscyamine three times a day.

[Footnote 33: _Philada. Med. Times_, Jan. 23, 1886.]

Ziegler[34] has 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.

[Footnote 34: _Ibid._, 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 be {456} insisted 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.

{457}

ATHETOSIS.

BY WHARTON SINKLER, M.D.

This disease was first described by Hammond in his work on _Diseases of the Nervous System_ in 1871, and cases have since been reported by many observers, among them Clifford Allbutt, Claye Shaw, Eulenburg, Oulmont, and Gowers. The disease is named by Hammond from the word _ἀθετος_, without fixed position.[1] The principal features are an inability to retain the fingers and toes in any position in which they may be placed, and the continual movements which persist in the parts—a condition called by Gowers mobile spasm.

[Footnote 1: _Diseases of the Nervous System_, p. 722.]

Athetosis is often connected with impaired mental powers; many of Shaw's cases were in imbecile children.

The movements of athetosis are not confined to the hand in all cases, but they are sometimes met with in the foot, and even in the muscles of the face and back.

The following is Hammond's original case:[2] “J. P. R——, aged thirty-three, a native of Holland, consulted Hammond Sept. 13, 1869. His occupation was bookbinding, and he had the reputation, previous to his present illness, of being a first-class workman. He was of intemperate habits. In 1860 he had an epileptic paroxysm, and since that time, to the date of his first visit to me, had a fit about once in six weeks. In 1865 he had an attack of delirium tremens, and for six weeks thereafter was unconscious, being more or less delirious during the whole period. Soon after recovering his intelligence he noticed a slight sensation of numbness in the whole of the right upper extremity and in the toes of the same side. At the same time severe pain appeared in these parts, and complex involuntary movements ensued in the fingers and toes of the same side.

“At first the movements of the fingers were to some extent under the control of his will, especially when this was strongly exerted and assisted by his eyesight, and he could, by placing his hand behind him, restrain them to a still greater degree. He soon, however, found that his labor was very much impeded, and he had gradually been reduced from time to time to work requiring less care than the finishing, at which he had been very expert.

“The right forearm, from the continual action of the muscles, was much larger than the other, and the muscles were hard and developed like those of a gymnast. When told to close his hand he held it out at arm's length, clasped the wrist with the other hand, and then, exerting all his power, {458} succeeded, after at least half a minute, in flexing the fingers, but instantaneously they opened again and resumed their movements.

“In this patient there was impairment of intellect, his memory was enfeebled, and his ideas were dull. There was no paralysis of any part of the body, but there was slight tremor of both upper extremities. The involuntary movements were of the right arm, and continued during sleep. Sensation was normal. The spasm of the muscles causes severe pain in the arm, and keeps him from sleeping at night.” Hammond used various remedies without relief, and had the patient under his charge for many years. Finally, he showed the patient to the American Neurological Society at the annual meeting in 1883, with almost complete relief to the movements as a result of nerve-stretching.

[Footnote 2: _Ibid._]

Athetosis is found in two forms—the hemiplegic and the bilateral varieties. In the former there has usually been an attack of hemiplegia more or less marked, or there has been an epileptic fit or unconsciousness from alcohol, as in Case I. There is often hemianæsthesia or some disorder of sensation. In the bilateral type the movements exist in all of the limbs, and are unaccompanied by weakness or disorder of sensation. The degree of movement varies in different cases. In some it is very slight, and can be controlled by extreme effort on the part of the patient. In other cases the movements are violent and uncontrollable. The muscles of the affected limbs become hypertrophied from the constant exercise.

The following case of athetosis has come under my care:

Case II.—W. A——, aged thirteen years, male. He had good health until 1877, when at the age of six years he had diphtheria. The attack was not severe, and he was up most of the time. About ten days after apparent convalescence he was suddenly seized with left hemiplegia. The paralysis was complete, involving the left arm and leg, the left side of the face, and the muscles of deglutition. There was also aphonia. In two weeks he began to talk, but indistinctly. In a month he could move the arm, but the movements were inco-ordinate. At about the same time he began to walk, but dragged the leg. The arm never regained power of voluntary motion, but instead there came on a spasmodic condition of the muscles which fixed the arm in various positions, and at the same time there were kept up constant but irregular movements of the hand and fingers. No spasmodic action of the leg-muscles occurred until a year later. He has never walked well since the attack of hemiplegia. The positions which the arm has assumed have varied at different times, but it usually retains one attitude for several months at a time. Sometimes the arm has been held in extension; at another time it has been flexed; indeed, the positions have been numerous. His general health improved and the speech became perfect.

Condition on examination Aug. 14, 1884: General health good, well grown for age. The nutrition of the affected side is good, but the left side of the face is markedly smaller than the right, although there is no paralysis. The position of the arm attracts immediate attention. The arm, hand, and fingers are in extreme extension, as shown in the cut, which is made from a photograph. The arm-muscles are tense and rigid. The fingers are continually in motion, sometimes flexed, sometimes extremely extended; then in a few moments they will be widely separated or distorted in some other way. The favorite {459} position, however, seems to be with the fingers extended until bent backward, separated from each other, and the thumb adducted slightly. The patient is unable to bring the arm down by a voluntary effort, but when asked to put it by his side he pulls it down with the right hand, and keeps it down by sitting upon the hand. The muscles of the arm are hypertrophied, especially those of the upper arm and shoulder. The circumference of the left arm around the biceps is almost an inch greater than that of the right. The leg is stiff and the foot is usually inverted. Here also the position changes at different times. When he walks the stiffness increases and the foot is dragged. The speech is clear and distinct, and the intellect perfect. He goes to school, and is fully equal to or more advanced than boys of his age. There is no evidence of cardiac disease.

The patient's condition in Oct., 1885, had changed somewhat. The arm is in a different attitude. It is still extended, but is held down by the side or away from the body. At times the forearm is strongly supinated. There are still constant but slowly-changing movements of the fingers. One plan which the patient has of keeping the arm flexed is to put the forearm behind the back with the right hand, and it becomes locked there by the action of the extensors. By a strong effort of will he can slowly and with great difficulty open and shut the fingers, and can flex the forearm. The leg is in the same condition as before.

The resemblance between athetosis and post-paralytic chorea is very close. Most of the cases of the former disorder which have been reported have been of the hemiplegic type. In Case I. the disease came on after an attack of delirium tremens, which was followed by six weeks of unconsciousness. The first symptom the patient was capable of noticing was numbness of the right arm and leg. The involuntary movements came on later. This would look as if there had been some lesion involving the left hemisphere of the brain. It is stated that no paralysis existed when the case was examined by Hammond, but there may have been a slight hemiplegia which had passed away. In another case reported by the same author the peculiar movements were preceded by hemiplegia and aphasia.

Oulmont has written a complete essay on athetosis, and has collected therein all the literature of the subject.[3] He sustains the view of the close connection between athetosis and post-paralytic chorea, but he {460} considers that, although nearly allied, a difference does exist between the two affections. He believes this is most marked in the bilateral form of athetosis, for here the disorder is usually not preceded by paralysis, and it is not accompanied by disturbances of sensation.

[Footnote 3: _Étude clinique sur l'Athétose_, Paris, 1878.]

Gowers[4] has also collected a number of cases, some of which came under his own observation, and has written a valuable treatise on the subject.

[Footnote 4: _Medico-Chirurgical Transactions_, 2d Series, vol. xli.]

PATHOLOGY.—The symptoms point to a brain lesion, probably in the gray matter, and a perverted condition of the nerve-cells which leads to over-action, either spontaneously or under the influence of a motor impulse. In most of the cases reported by Claye Shaw there was imbecility. Charcot found in three post-mortem examinations lesions in each instance in the posterior portion of the optic thalamus, the most posterior part of the caudate nucleus, and the most posterior part of the corona radiata. Gowers has made an autopsy in one case in which there was post-hemiplegic inco-ordination affecting the arm only, without the spasmodic fixation of the limb. In the brain was found but one lesion, and that was a cicatricial induration of the optic thalamus, extending across its centre beneath its upper surface, and approaching at its outer part, but not involving, the ascending white fibres of the crus. No secondary degeneration was found in the cord.

PROGNOSIS.—This is almost always unfavorable. In some cases the inco-ordination decreases and the spasm becomes less violent, but it seldom disappears altogether. One of Gowers's cases was benefited by treatment, and two of those related by Hammond were relieved.

TREATMENT.—Many remedies have been given without marked benefit. In Gowers's case there is no doubt that the decided improvement which took place was due to galvanism. The treatment lasted three months, and a descending galvanic current was used. The positive pole was put upon the nucha, and the negative on the over-acting muscles and on the hand and foot. One of Hammond's cases, as related above, was apparently cured by stretching the median nerve. A prolonged course of galvanism and some alterative, like mercury or chloride of gold and sodium, would seem to offer the best prospect of benefit.

{461}

LOCAL CONVULSIVE DISORDERS.

BY ALLAN MCLANE HAMILTON, M.D.

Thomsen's Disease.

SYNONYMS.—Rigidité musculaire avec impuissance de la volonté; Rigidité et hypertrophie musculaire; Myotomé congenitale.

In the year 1876, Thomsen,[1] the medical officer of Kappeln, described a curious form of nervous disease which affected several members of his own family, himself included. It consisted of a tendency to cramp and limited spasm when a voluntary act was attempted, and a seeming and sudden loss of power. This condition disappeared after the performance of the act and its repetition. An attempt at walking would be attended by spastic rigidity of the flexor muscles of the lower extremities, by flexion of the thighs and legs at an angle of 120°, so that the patient often fell forward upon his knees. An attempt to arise from the chair was attended by the same difficulty, the person becoming utterly helpless. The arms and legs were most frequently affected, the trunk-muscles being usually exempt. In some cases the muscles supplied by cranial nerves were the seat of spasm, so that the patient could not close his mouth nor shut his eyes. Westphal, Erb, Peters, Schönfeld, Engel, and others have reported about eight cases in all, and in addition to the symptoms above referred to it was found that mental excitement and cold chiefly precipitated the seizure. The sphincters were never affected, and the general nervous functions were not impaired. The patients talked stiffly, and this was probably due to a spasmodic affection of the lips, tongue, and other articulating organs. The tendinous reflexes were normal, but irritation of the soles produced a peculiar cramp of the leg-flexors and bending of the knees. There is a species of muscular increase which resembles pseudo-hypertrophic paralysis.

[Footnote 1: _Berliner klin. Woch._, Mar. 12, 1883.]

The malady seems to be of an hereditary nature, if we are to judge by Thomsen's cases. It begins early in life, and does not materially shorten the same. I have seen one case, a young child, which presented the main symptoms of, and was first mistaken for, pseudo-hypertrophic paralysis.

Westphal regards the affection as an “anomaly of muscular tonus;” others have shed no light upon its pathology.

I believe the condition to be an occasional feature of certain organic disorders, notably posterior spinal sclerosis, and there is a variety of paralysis agitans where there is no tremor which presents all the symptoms. The very rare nature of the malady and its peculiar {462} expression render diagnosis easy. It possibly may be mistaken for pseudo-hypertrophic paralysis in young subjects, but in this latter disease we find electrical change in the muscles, and an absence of the tendon reflex. An examination of the enlarged muscles will not reveal fatty increase.

Painless Facial Spasm.

There is a form of spasm of the muscles supplied by the facial nerves which differs from ordinary tic douloureux by the fact that there is no pain in the former. The trouble may be one of a very limited nature, consisting of the involvement of a few fibres, or of limited groups of muscles, or of all the muscles of the side of the face. I never have seen a case of double spasm, though such undoubtedly exist.

The nature of the paroxysm is cumulative, and, as a rule, the attack increases until it reaches an acme of intensity, then quite suddenly ceases. It often begins by a slight drawing of the corner of the mouth, the levator anguli oris and other muscles in the neighborhood being the seat of chronic spasms. This limited spasmodic action is followed by further facial contractions. The orbicularis palpebrarum is often the sole seat of the convulsive movement, and this is common in excessive smokers.

As a rule, the trouble is peripheral and due to some reflex cause, such as cold, injury, bad teeth, which produces reflected irritation through the branches of the fifth nerve, or in rare cases it may be central or due to some bony or other pressure upon the nerve in its passage through the skull. In a recent case the trouble was of evident central origin, and I obtained a history of lightning pains in the lower extremities, some inco-ordination of muscles in both upper and lower extremities, and some paresis of the facial muscles. In this case the spasms involved all the muscles of the right side of the face, and recurred every few minutes. They had first appeared five or six years before I saw the patient, and had gained in frequency from two or three daily until within the past four months they occurred, as I have said, every few minutes. The other symptoms were of insignificant character compared to the spasms.

Of the large number of cases I have seen, many impressed me as being the result of a simple bad habit, yet moral or other measures did not avail much.

It is important to diagnose certain unusual forms of facial spasm from the petit mal of epilepsy, and careful observation will detect a transitory loss of consciousness in the latter. It is equally important to find a cause if any exists, and a careful examination of the state of the teeth, the integrity of vision, and the possible existence of aural disease should be made in all cases. In some cases over-use of the eyes, which may be defective in their power of accommodation, may precipitate blepharospasm. In other cases the act of masticating hard substances or taking very hot or cold fluids into the mouth may give rise to the spasm.

Hyoscyamine in repeated doses of from 1/200 to 1/25 grain of Merck's crystals does more in the way of relieving the spasms than any other drug of which I know. If this does no good, gelsemium cautiously used is often of great service. Local galvanic applications with currents of great intensity will form a valuable adjuvant. So far, I have never tried {463} nerve-section or stretching in these cases, but recommend them as a dernier ressort.

Torticollis.

SYNONYMS.—Wry neck, Rheumatismus colli, Obstipite, Cephaloxia.

Wry neck or torticollis consists essentially of a spasm of the sterno-cleido-mastoideus, though other muscles are nearly always involved: the result is a peculiar and striking distortion which is quite familiar. It consists in the drawing downward and backward of the head on the affected side, while the chin is pointed forward and upward to the other. The disease is presented in several forms. It may be a temporary disorder as the result of a rheumatic condition, disappearing rapidly, or occurs as an hysterical affection, or it may be a chronic and intractable nervous disease. We must also consider it from the standpoint of the form of distorted motility. In some cases there is simple tonic contraction, which may eventually result in tense contracture, shrinking, and tendinous hardness; and in others the attention of the physician is attracted by a species of tremor and agitation. Unlike the tremor of sclerosis, this is uninfluenced by the attempts of control upon the part of the patient, but is aggravated by fatigue and excitement. It rarely happens that both muscles are affected so that the head is drawn backward. Most of the cases are single, chronic, and progressive, and, though very slow in the onward march, are usually beyond the reach of remedies. The patient becomes greatly annoyed by his infirmity, and seeks every measure to overcome his unfortunate deformity. He commonly tries to hold his head or chin, pressing the latter downward, or, holding his cane against his head upon the dependent side, strives to keep it up. As a result, there is a sagging or drooping of the affected side of the body, so that one shoulder is lower than the other.

The disease, as a rule, begins in adult life, yet there are many young cases.[2] Wilks calls attention to the fact that in these latter there is apt to be some facial asymmetry on the contracted side. One side of the head is smaller than the other, and one eye seems to be lower.

[Footnote 2: _Diseases of the Nervous System_, p. 454.]

Most of the cases I have seen have been men, though I have met with many hysterical examples in young girls. The double torticollis (Newnham's salaam convulsion) usually affects children, and it is the rule to find associated strabismus and intellectual disturbance. In the adult cases there has usually been a history of hereditary neurotic influence and overwork.

Electrically, we find a susceptibility to both currents, and the reaction of degeneration may be detected in the affected muscles in old cases. The opposing muscles are usually the seat of diminished electrical excitability.

Torticollis may be due to peripheral or deep causes. Cold, reflex irritation, forced and uncomfortable positions, are to be mentioned among the former, and intracranial or vertebral disease as examples of the latter. The pathological explanation lies in a disorder of the motor fibres of the spinal accessory nerve. Any affection of the external branch will result in the condition above described.

{464} In young cases the PROGNOSIS is good as a rule, though this is by no means invariably so, for sometimes the special symptoms are but forerunners of others of a more grave character. Hysterical torticollis is often instantly, or at least very readily, cured by electro-therapeutics and cauterization. A well-established adult case is almost hopeless and resists all ordinary treatment.

Many forms of TREATMENT have been recommended from time to time, and such drugs as the bromides, chloroform, conium, and hyoscyamine are suggested. The latter I believe to be the most serviceable remedy. Electricity has done good in either form, and vigorous faradization with the electric brush is earnestly recommended in hysterical, rheumatic, or functional cases. Some years ago I devised a method which in a number of cases has been of great service. I allude to the combined and simultaneous use of the galvanic and induced currents. A double electrode is applied to the back of the neck. This contains the anodal pole of the galvanic current and the cathodal of the faradic. The two other poles are placed—one, the galvanic cathode, over the origin of the affected muscle, the induced anodal pole over the weakened muscle which is not in spasm. I suppose in old cases the most valuable treatment is that of a surgical nature. I have twice seen the spinal accessory exsected with the result of a complete cure; and I think this is the only sure measure. H. B. Sands of this city has performed the operation quite successfully in other instances. Care should be taken not to operate in cases presenting other symptoms which suggest the remotest suspicion of organic cerebral disease, and the existence of unilaterally increased tendinous reflexes or tremor should be carefully looked for as contraindications.

Braces and apparatus are often worse than useless, increasing not only the patient's discomfort, but aggravating the malady.

Eclampsia.

(_Εχλαμψις_ and _λαμβω_, “I shine,” brilliancy, flashes of light from the eyes.—Dunglison.)

DEFINITION.—A term used to express certain irregular convulsive attacks, as a rule due to eccentric irritation, uræmic poisoning, or like causes, and chiefly used in connection with the convulsions of infants.

This term has gradually been dropped in scientific medical literature, and many authors think it useless, believing the condition to be usually epileptoid. Nothnagel,[3] who is more liberal than many other clinicians, says: “What is there now remaining of what was formerly eclampsia? Are we altogether justified in still retaining the name? We believe so, and are of the opinion that the title eclampsia should be reserved as the name of an independent affection, which, it is true, can at present be defined only by its clinical symptoms. We propose that the designation eclampsia should be made use of for those cases of epileptiform spasms which, independent of positive organic diseases, present themselves as an independent acute malady, and in which, so far as our present knowledge allows us to judge, the same processes arise generally in the way of reflex {465} excitements, and the same mechanism in the establishment of the paroxysms comes into play, as in the epileptic seizure itself.”

[Footnote 3: _Cyclopædia of the Practice of Med._, Von Ziemssen, Am. ed., vol. xiv. pp. 301, 302.]

If any distinction at all be made, it should be one founded upon the fact that epilepsy itself is usually an organic disease, or, more precisely, a disease which when established is dependent upon some disorganization, while eclampsia is used to express those seizures of a purely functional nature.

By far the greater number of eclamptic seizures are found among young children. There is no uniformity in their expression or return. Any eccentric irritation is apt to precipitate one or more attacks, and those forms of irritation dependent upon sensory disturbances of the mucous membrane are commonly present. The convulsions of teething belong to this class, as well as those in which gastric disorder plays a part; and in the first instance the cutting of the large teeth, and in the latter the presence of indigestion with diarrhœa, are quite commonly associated with the convulsion.

The high degree of irritability of the nervous system of children renders them peculiarly susceptible to causes which in after years would effect little or no disturbance; and this is especially true before the fifth year. West and Reynolds are of the opinion that convulsions occur in children as delirium in adults; in other words, they are the most common expression of neurotic instability.

Infantile convulsions are usually general, and neuro-spasms are not common, except when they are dependent upon neoplasm, ventricular dilatation, or local meningitis. It evidently takes very limited peripheral excitement to precipitate a general convulsion in the child, and a familiar example is the disturbance which may involve the peripheral branches of the fifth nerve in difficult dentition.

Eclamptic seizures occur at any time, and may be very slight: a trifling twitching may be all, or, on the other hand, the attack may consist of violent opisthotonos. There is great difficulty, of course, in getting anything from a young child as to its feelings before an attack, and I hardly think we are authorized in saying that there is any aura or ascertainable precursor. The history of a previous nervous state is, however, usually ascertainable, which is expressed by crying fits, peevishness, and great restlessness. Sometimes there is a disposition to sleep which almost amounts to stupor. The behavior of a child is often likened to that which marks the commencement of acute tubercular meningitis—night-terrors, grinding of the teeth, and flushing of the face. The first convulsions may be only partial, but in a short time their character becomes general, and they become exceedingly violent, and are sometimes fatal. The clinical features of an eclamptic seizure may in every respect resemble one of epilepsy, making allowance for the age of our patient.

The stage of pallor is perhaps more extended in the child.

Handfield Jones speaks of a form of eclampsia of hyperæmic causation in association with certain exanthemata. In children convalescing from scarlatina he observed the development of attacks which were relieved by carotid pressure and bleeding in subjects who presented anasarca and other indications of renal disease.

We are familiar enough with the convulsions of puerperal women, which, as a rule, though not always, are presented by subjects whose {466} urine is albuminous. It can sometimes distinctly be traced to what must be regarded as uterine excitement. Just as chorea is often a feature of the pregnant state, so may convulsive seizures arise. The peculiarities of the epilepsy of pregnancy will, however, be elsewhere considered.

I shall purposely refrain from the consideration of those forms of symptomatic infantile convulsions which mark the occurrence of cerebral accidents or grave disease.

{467}

EPILEPSY.

BY ALLAN MCLANE HAMILTON, M.D.

SYNONYMS.—Epilepsia; l'Épilepsie (_Fr._); Fallsücht (_Ger._); Epilessia (_Ital._); Epilepsin fallendsot (_Scand._); Falling sickness; Fainting sickness.

DEFINITION.—According to the most recent authorities, an epileptic attack is nothing more nor less than a discharge of nervous energy from an overexcited, or what may be called a dynamo-pregnant nerve-centre, or collection of centres, and the predominance of motor or sensory phenomena determines the extent and order of the parts involved. As a rule, an epileptic paroxysm is but a symptomatic expression of a complex derangement, and it is best to formulate our nomenclature with the idea, in the first place, of location; in the second, with reference to the prominence of motor or sensory expressions; and, finally, with regard to etiology.

HISTORY.—There is probably no nervous disease which has been more extensively written upon (even in ancient times) than that under consideration. We find references to it as early as the tenth century, when it figured in the text of Avicennes. Hippocrates called it _ιερόν νοσεμα_, or sacred malady, and _πάθοϛ παιδήιον_, or malady of children, believing that the attacks had their origin always in early infancy and never later. Plato and Aretæus advanced the theory that the disease sprang from a thirst for gold, and equally absurd and unreasonable explanations are found in the writings of the fathers of medicine. The older French writers were diligent investigators, but with them prevailed the tendency to explain the origin of the disease by mysticism, and among many it was supposed to bear some connection with the coming of St. John. The popular humoral theory of the malady originated by Mercurialis was afterward opposed by Averrhoes and Fernel, but even to this day it has devoted adherents. Bouchet and Causauvieilh maintained that the disease was inflammatory—a view that was vigorously combated by Bouillaud and Delasiauve. The theory of Broussais—which was and is the basis of the conclusions of modern investigators—is that it is dependent upon cerebral irritation. It is hardly necessary to refer to the many untenable and curious attempts that have been made to explain the pathology of the malady: suffice it to say that many of them were as extravagant as that of Vepfer, who considered the pineal gland to be the locus morbi of the affection—a conclusion in which Descartes coincided. Marshall Hall was the first writer to advance the theory of reflex irritation, {468} believing that gastro-enteric or uterine irritation acted upon the brain. In later years Schroeder Van der Kolk, Reynolds, and others have written quite fully upon the part played by the medulla, while recently numerous French and German writers—among them Bourneville, Meynert, Sommers, and Tagges—have attached much importance to the discovery of a sclerosis of parts lying at the floor of the lateral ventricles.

Perhaps our knowledge of epilepsy has received its greatest impetus from the elaborate and exact researches of Hughlings-Jackson; and his observations, taken in connection with the recent work of the numerous students of localization, open up a new field of research, and, as matters stand, the future study of epilepsy must be fruitful in the extreme.

DIVISION.—It has been the custom to divide the epilepsies into epilepsia gravior and epilepsia mitior, the haut mal and petit mal of the French. These terms are in one way misleading, and only define differences in degree. The terms general and limited would much more properly express the forms of attack, and I shall use them as far as possible in the present article. A general epilepsy is one that corresponds with haut mal, in which there is an extensive convulsion, absolute loss of consciousness, and perversion of a widespread character. Limited epilepsy includes those forms in which there is a convulsion confined to a small group of muscles, and in which loss of consciousness plays an unimportant part. Under this latter head belongs the form known as petit mal and those monospasms which depend upon a cortical irritative lesion. There are other divisions which partake of the nature of one or the other, and are unilateral and dependent upon the destructive discharge of a motor centre. Under this head may be placed the epilepsie partielle of the French or the hemi-epilepsy or Jacksonian epilepsy of the English. There are also irregular or aborted attacks—the so-called masked epilepsy—and lastly the sensory varieties.

ETIOLOGY.—It cannot be denied that heredity plays the most important part in the genesis of epilepsy. Nearly one-half of my own cases when carefully investigated were clearly traceable to some inherited predisposition. Insanity, epilepsy, and phthisis in ancestral history stamp their constitutional imprint upon the unfortunate descendant, and the history of ten cases from my notebook (see Table) will show the extent of saturation that may exist in paternal or maternal branches and the evolution of the disease in male and female subjects. The statistics of other writers, though not showing quite so large a proportion of cases with hereditary history as my own, are quite significant. Gowers found that in “1218 cases, 429, or 35 per cent., presented evidence of neurotic inheritance.” Echeverria estimated the proportion of hereditary cases at 28 per cent., while Reynolds fixes it at 31 per cent. Of 980 cases, the notes of which I have examined, many of whom have been under my personal care, 435 presented a family history of insanity, phthisis, epilepsy, cerebral apoplexy, tumor, or some lesser neurosis. So far as the history of hereditary influence is known, it appears that females are more apt to present this form of epilepsy than males, and, according to Gowers's as well as my own investigations, the transmission comes from the mother's side more frequently than the other. So far as my own inquiries have gone, I find insanity more often among the progenitors of the epileptic than any other nervous disease, and in many cases phthisis. I am {469} {470} inclined, therefore, to give greater weight to this relationship than Nothnagel and others. Anstie, Bastian, Savage, and other careful clinicians have pointed out not only the close connection between phthisis and epilepsy, but between the former disease and migraine; and no one who has seen much of epilepsy can fail to be impressed with the frequent association of migraine with the more serious convulsive affection of which it is quite often the precursor. Perhaps the fact that phthisis was found so often—in 230 of my 980 cases—may be due to the fact that many of the patients were of the lower classes and among the Irish, who in this country are so frequently phthisical.

_Heredity in Ten Cases of Idiopathic Epilepsy, with Existing Disease
or Cause of Death_.

Previous Next

Comments

A system of practical medicine. By American authors. Vol. 5Chapter XXIX: Introduction: The term neurasthenia, signifying nervous weakness, and (7)

0%36 min left in chapter