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.
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 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 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.
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.
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 excitements, and the same mechanism in the establishment of the paroxysms comes into play, as in the epileptic seizure itself.”
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 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.