Kitabı oku: «Tics and Their Treatment», sayfa 10

As a general rule it is only one part or segment of the body that is immobilised by a tonic tic, but in regard to the possibility of a general involvement, the following instance⁵⁰ may be cited, although we do not think it can be considered decisive:

A man thirty-two years old, who had recovered from a first attack of mental torticollis, underwent a relapse in quite a different form. If when walking with his head perfectly straight he were asked to go round to the right, he instantly appeared to become rooted to the spot and could not turn even his head in the required direction; at the same time he felt a compression of his throat as if he were being strangled, and for a few seconds he experienced acute anguish. A moment later he was all right again, and his action unimpeded.

Without going so far as to classify this incident as a tic, and without venturing to assert the existence of a tic of immobility, one cannot but be struck with its analogy to the attitude tics of which we have been speaking, and to catatonic conditions met with in the insane, of which too the pathogeny presents more than one point of similarity with that of this species of tic.

[In this connection reference may be made to certain conditions occasionally noted among those who tic – viz. a curious tendency to maintain abnormal positions of the limbs or trunks, and difficulty in or impossibility of relaxing various muscles (catatonic aptitudes). Patients are sometimes given to the exaggerated repetition of the ordinary movements of their members (echokinesis), as well as to imitation of the actions of others (echomimia). Such catatonic and echopraxic phenomena⁵¹ are not confined to sufferers from tic, for they are encountered among psychopathic subjects generally, and indicate defect of cortical control – what is called by Brissaud "passive activity." These catatonic aptitudes may be discovered by resort to clinical tests, such as letting the arm fall from the horizontal position.⁵²]

INTENSITY OF THE MOTOR REACTION

The muscular contraction varies considerably in intensity, in most cases exceeding that of the corresponding normal movement, and, especially in tonic tics, being often so powerful as to necessitate the exertion of great force to overcome it. Even though one's effort prove unavailing, however, it is only needful to distract the patient's attention to perform any and every passive movement with consummate ease.

In the case of S., any attempt to budge the head from its torticollic position on the left evokes strong muscular resistance; but engage him in conversation or otherwise divert his mind, and the difficulty soon vanishes. By similar means, the resistance awakened by sudden change of the direction of passive rotation will rapidly die down.

Occasionally the muscles brought into play surpass their fellows of the opposite side in size and power, this secondary hypertrophy being the natural sequel of repeated exercise. It was noted by Charcot that in rotatory tics the disused muscles atrophied, whereas the affected muscles hypertrophied, but they may do so only in appearance. The tonus of the muscles at the moment of examination may create differences inappreciable during relaxation. Sometimes one comes across such expressions as "paresis" or even "paralysis" of antagonistic muscles, and "contracture" of those in which the tic is localised. To draw a distinction between slight contracture of the latter and mild paresis of the former is a problem practically always insoluble. Opinion has been ever divided on this point; yet some, in their desire to harmonise the two, take up an eclectic position and do not hesitate to speak⁵³ of "paralytic contracture," or "mixed contracture, at once active and passive," a terminology by no means calculated to simplify the question, and one the discussion of which we do not care to pursue.

We should like, however, to allude to a matter of clinical observation that we frequently have had occasion to remark. What simulates muscular enfeeblement in the subject of tic is often nothing else than a want of accuracy and adresse in the performance of a given movement. For instance:

S. enjoys robust health; his only trouble is a lack of accurate control over his limbs. His execution of the most elementary movements is incorrect. There is no tremor, no jerkiness, simply a loss of the sense of position. He never knows whether he is holding himself straight, whether his arms are exactly horizontal or his shoulders symmetrical. Often he confuses right and left, and when requested to perform some act on one side, he declares he is tempted to perform it simultaneously on both. The order to fold his arms and rotate the upper part of his body to the right evokes an inconceivable display of contortions. In the attempt to bend his head and body backward, fear of losing his balance causes him to twist and turn about most strangely, and the remark that all this he might avoid by merely putting one foot further back seems to cause him infinite surprise.

Or again:

The absence of precision in Mademoiselle R.'s movements, her habit of arresting the action before attaining the desired end, are not to be ascribed to any feeling of discomfort, but to her ignorance of the amplitude of her efforts, and of the position of her limbs. Her acts are always feeble, hesitating, and curtailed, a curious mixture of muscular languor and vigilance, "as if she were afraid of breaking herself." She appears to be constantly seeking some new position for herself, and to be as constantly oblivious of her actual attitude. With eyes closed, however, she indicates the relation of her limbs exactly.

Another example is furnished by the case of L., to which reference is made on p. 135.

There is no call to multiply instances. Enough has been said to demonstrate the frequent occurrence, if not of motor inco-ordination, at least of faulty orientation in space and of defective estimation in regard to the range and intensity of voluntary movements, among the subjects of tic. The topic is a very interesting and fruitful one, on which considerable light may be thrown by the application to it of the results of Pierre Bonnier's⁵⁴ remarkable studies on the sense of attitudes, a subject that we intend to develop on another occasion.

FREQUENCY AND RHYTHM – RHYTHMIC TIC

The frequency of the muscular contractions in tic is so very variable that it cannot be regarded as a distinctive feature, nor is there any evidence to show that it is rhythmical, as some would have us believe. Contrary to what obtains in tremor, there is no periodicity in the motor phenomena, even when the tic is based on derangement of a function whose manifestations are rhythmical, such as the function of respiration. Conditions described as rhythmic tics, or less well as rhythmic spasms, seem to form a group by themselves; probably they do not belong to the same family as the tics, indeed in some cases they are symptomatic of encephalic lesions, as in the spasmus nutans of infants, or the rhythmic tics of idiots and imbeciles. In this connection the remarks of Noir are very pertinent:

We shall be well advised to refrain from drawing too absolute conclusions in questions so difficult, where even the framing of an hypothesis demands prolonged observation, but we cannot withstand the temptation to note the co-existence of certain of these tics with certain definite lesions recognisable post-mortem. This has been done before us by our master Bourneville, who has on several occasions made the diagnosis of chronic meningo-encephalitis, cerebral sclerosis, etc., from this association of rocking, rotation, and krouomanic movements with a special symptom-complex, and verified it at the autopsy. Nevertheless, there is not always an absolute correspondence between them, wherefore Bourneville, with an altogether praiseworthy scientific reserve, has hesitated to consider these tics as actual symptoms of the affections alluded to, and we shall follow his prudent example.

To the combination of various rhythmical acts with hysteria we shall revert at a later stage. Under the title "rhythmic spasm" an interesting case has been reported at length by de Buck,⁵⁵ concerning a young woman, free of hysterical stigmata, in whom convulsive movements first appeared at the age of seven years.

When she had attained her nineteenth year she commenced to suffer from attacks of anguish of some hours' duration, but disappearing under the influence of sleep, in which she felt as though her breathing were going to stop and she herself were about to die. On the termination of these sensations some eighteen months later, their place was taken by convulsive movements of the tongue, lips, neck, trunk, left arm, diaphragm, pharynx, and muscles of respiration. These consisted of clonic rhythmical twitches, each preceded by an inspiration and succeeded by an expiratory ejaculation, repeated fifty or sixty times a minute. During the seizure the tongue was protruded and deviated to the left, the left arm was raised, the head and trunk bent down and forward. All day long the movements were continued with unflagging regularity. Rest in bed was without effect, but they were dispelled by sleep. Distraction and occupation exercised an inhibitory influence on them, whereas voluntary control was both feeble and fleeting. In the condition of the patient there was nothing else abnormal with the exception of slow, monotonous, and syllabic speech. Her mental development was perhaps a little immature, but signs of hysteria were lacking, and all attempts at treatment by suggestion and hypnotism failed of their object. Death ensued from pulmonary tuberculosis.

De Buck observes that while the action of some of the muscular groups involved in the rhythmic spasm was, so to speak, purposive, the whole did not constitute any known, conscious, and logical movement. It may have been a species of tic, but the rhythmical sequence of the convulsions imparts to it a quite peculiar character.

ATTACKS

A further mark of the motor reaction is the circumstance that it ceases for a longer or shorter interval, independently of the tic's localisation, intensity, or form, the result being an alternating series of "attacks" and periods of respite. In different patients, and in the same patient, the number and the length of these attacks are as variable as are the spaces of rest that separate them. We remember a girl with a tic consisting in a toss of the head repeated perhaps fifteen times a minute, three or four occurring together at intervals of one or two seconds, and being succeeded by a relatively long pause. The effect of treatment was to modify the sequence entirely, and to reduce the tic to an isolated jerk reappearing not oftener than once in a quarter of an hour, and in itself constituting the attack. In another case the patient's head used to turn to the left, remain so for a moment, then resume its ordinary place. After a time of repose the tic began again, and even when the movements followed each other more rapidly, the intervening period was always appreciable. On the other hand, we have seen a youth afflicted with multiple tics which continued without intermission the whole day long; the attack lasted, strictly speaking, from morning to night, and any break in its continuity was altogether exceptional. It might then be more exact, perhaps, to use the epithet paroxysmal in reference to the external manifestations of tics, but it signifies little what word we employ provided we are familiar with the clinical facts.

The attacks vary with circumstances and environment. One of our patients remained quite free from them during a visit to the theatre. Tissié had a young patient who did not tic at all while on holiday, but the reopening of his classes was the signal for a fresh outbreak. Similarly, no rule whatever seems to govern the duration of the times of relief; they may never be longer than a few seconds, or they may run into months. In the face of these data we cannot supply further generalisations; it will be sufficient if we impress on ourselves the importance of one fundamental element in the constitution of tic – viz. its repetition.

LOCALISATION OF THE MOTOR REACTION – VARIABLE TICS – FIXED TICS

The localisation of the motor reaction in cases of tic is essentially physiological. In rare instances its sphere may be limited to a single muscle, if one muscle only be requisitioned for the performance of a functional act; but it is very much more usual to find several muscles contributing, whose synergic contractions fashion the movement of which the tic is a caricature. If the same effect is yielded by the action of either of two different muscles or groups of muscles, as in rotation of the head, and if one be hindered from fulfilling its function, the incidence of a tic originally located in it will promptly be transferred to the other. This is the explanation of the persistence of rotatory tics after exclusion of the sternomastoids by surgical means.

Two symmetrical muscles may be affected, as in tics of blinking and of affirmation, or a median muscle, such as the orbicularis oris. Much more frequently the tic is unilateral in its distribution, as, for instance, when it involves the face; in this respect its figuration as a functional disturbance is well exemplified, for expressional movements of the face are normally bilateral. A tic may settle itself on two mutually antagonistic muscles, and manifest its presence in the immobilisation of a limb or segment of a limb; or only a portion of a muscle may contract, as in the case of the deltoid or trapezius, which are composed of bundles anatomically associated but physiologically independent, and so capable of being functionally differentiated by voluntary education. Fibrillary contraction and tic have nothing in common.

Inasmuch as the muscles concerned are under voluntary control, and their contractions such as the will can effect, it follows that with adequate practice the movement of a tic can always be imitated, and in predisposed soil imitation tics may thus take root; it is not always feasible, on the other hand, to counterfeit a spasm.

Several functional muscular territories may be simultaneously affected, and several tics may follow one another in quick succession, the duration of any one tic on any one site being a more or less varying quantity.

We have already noted the occurrence of variable tics. They appear one day to disappear a few days later, and reappear again after another space. Weeks or months may elapse without any vestige of them, until they suddenly break forth again unheralded. As a general though not absolute rule, the younger the patient, the less stable his tics. Occasionally they are isolated, limited, and stationary, one of the most frequent of this kind being a tic of blinking, but the intimate alliance between the motor troubles and the mental level of the subject helps to explain why these tics of children are so changeable.

In the case of young J., for instance, it was shortly after attaining his tenth year and entering school that first he began to tic, and thenceforward, at unequal intervals, trunk, arms, shoulders, legs, became in turn the seat of "movements of the nerves," while other more definite tics were not slow in developing.

When only six years old B. exhibited a respiratory tic, which changed a year later to one of the tongue, and after another year to one of the leg; at the age of twelve he used to nod his head in affirmation, and this was eventually succeeded by movements of negation, etc. He has since started a salaam tic, and finally a torticollis with deviation of the eyes.

We may cite an analogous case from Grasset:

A young girl, who had had eye and mouth tics in childhood, commenced at the age of fifteen to advance her right leg involuntarily – a sort of tic which lasted several months and gave place to paralysis of the same limb; for this affection was next substituted a whistling tic, and then for a whole year she used from time to time to give vent to a loud "Ah!" When she came under observation she was suffering from a tic of salutation, with retrocollic jerking of the head and shrugging of the right shoulder.

One of our own patients furnished us with the following story:

The disease made its debut by a blinking tic of both eyes, whose origin in the absence of any visual defect remained undetermined; grimacing and distortion of the mouth were the next to appear, as well as wrinkling of the nose and forehead, twitching of the eyebrows and contraction of one platysma, sometimes even of the ear muscles and the entire scalp. Then ensued up-and-down tossing of the head, or rotation of it from right to left, and, later, elevation and advancement of the shoulders, with restless agitation of hands and arms. A former trick of his of biting his nails is quite in abeyance at present; instead, he catches hold of his under lip every moment and abrades its mucous membrane with his nails, so much so that the lip is swollen and cracked like those of children with nibbling tics. Some months ago he acquired the habit of giving utterance to a soft little cry not unlike the sound made by a guinea-pig.

One tic has succeeded another in an unbroken series. The facial tics were more of the nature of grimaces, which the child amused itself in repeating; no doubt the scratching of the lip was a sequel to the desire of experiencing a new sensation, while the movements of hands, arms, and shoulders were very variable and different enough from the accompanying phenomena. No one of the tics was at all of protracted duration; on the contrary, each was fugitive and changeable, and therein presented a resemblance to the child's mental status. In sleep they completely disappeared; in the presence of strangers or if his interest was in any way aroused, they quieted down, while they increased on holidays, during games, or with physical fatigue.

It is clear that determination of the tic's localisation and mode can come only with the mental evolution of the patient, and that the transformation from the psychical inconsistency of childhood to the stability of the adult is paralleled by the change in the tic's manifestations as the scale of age is ascended. Any individual, whatever his years, who is in the stage of mental infantilism, will tic after the manner of a child, for the characters of a tic are dependent on the state of mind of its subject.

CHAPTER VIII
ACCESSORY SYMPTOMS

REFLEXES

THE question whether in cases of tic there is any alteration in superficial or deep reflexes can be decisively answered only by an appeal to statistics, the information afforded by which has hitherto been too scanty and too incomplete. Judging from our own observations in about thirty cases, we feel compelled to admit that disorders of this kind are altogether exceptional. Careful and repeated examination has convinced us that in patients suffering from tic the knee, ankle, wrist, elbow, and other jerks, the plantar and fascia lata reflexes, as well as those of the pharynx, eyes, etc., are all but universally normal, and any trifling exaggeration or diminution not only varies from day to day, but also in no wise exceeds the differences met with in health, and is therefore symptomatologically negligible. In the manifold varieties of tic represented by R., S., P., N., M., B., etc., whose cases are quoted here in part, our inquiries have always been negative. Noir's research on the state of the reflexes in idiocy complicated with tics failed to expose any abnormality, and even where the knee jerks were increased no departure from the usual manifestations of the tic was discoverable. It is of course permissible to suppose that a combination of the latter with organic disease of the nervous system might explain the modification of the reflexes. In this connection it may be remembered that on one occasion we found the customary diminution of O.'s knee jerks had passed into actual loss, and although on the next day they were present again, the occurrence was suspicious enough to justify one in entertaining the idea of incipient tabes. Even if the existence of other signs had corroborated this diagnosis, the incontestable genuineness of O.'s tics would have remained, so that the attempt to correlate the derangement of the reflexes with the existence of tics is somewhat questionable.

We have enjoyed the co-operation of M. Babinski in the examination of one of our patients, L., in whom we were able to determine a definite and symmetrical exaggeration of the patellar reflexes, a slight increase in the right triceps jerk, and, in making the subject rise from a prone to a sitting position with the arms folded, a very minor degree of flexion of the thigh on the trunk.

The first of these symptoms is of no pathognomonic value, and while the others no doubt are characteristic of organic disease, their development in this instance is too imperfect to warrant the deduction of pyramidal involvement. For the last ten years L.'s motor control has been very defective. The muscular activity of the right half of his body takes the form of irregular contractions, badly timed and inaccurate in range; in spite of the absence of pain, the timidity with which they are executed hinders their ever attaining a normal amplitude; and at the same time his inability to appreciate the direction and intensity of the motor reaction, the outcome of excessive muscular vigilance, illustrates a certain loss of the sense of position of his limbs.

The existence of an actual irritative lesion is therefore problematical, and it is scarcely conceivable that organic mischief of ten years' duration could have produced these clinical symptoms without creating graver disturbance of the reflexes, or effecting changes of a trophic nature in muscular and other tissues.

From the pathogenic and diagnostic point of view, the detection of conspicuous and persistent alterations in the reflexes is of deep significance. It is an important factor in the differentiation between tic and spasm.

Sometimes the task is unusually arduous, as when the unilateral distribution of the motor troubles recalls the clinical picture of lesions of the pyramidal paths. In L., for instance, the hemichorea and the torticollis were on the right side, and in a case published by Desterac a similar condition obtained, the writers' cramp, hip spasm, and head rotation being all confined to the right. Notwithstanding the fact that this patient had exaggerated knee jerks, ankle clonus, and a double extensor response, an opportunity for examination given to one of us made it clear that the untimely movements and bizarre attitudes were similar to what has been noted in certain cases of tic.

At the Neurological Society of Paris a case was shown by Babinski⁵⁶ of left spasmodic torticollis, with marked spasms of the left arm and left leg, and a homolateral extensor response, and it was contended that if one and the same cause underlay these phenomena – nor did there appear any adequate reason to doubt it – and if the reversal of the plantar reflex was, conformably to received opinion, to be interpreted as indicating a derangement in the function of the pyramidal system, then it was allowable to attribute the muscular spasms to the same derangement, in which circumstances the natural conclusion was that spasmodic torticollis itself might sometimes at least be dependent on pyramidal irritation of an as yet undetermined kind.

More recently still, another patient was exhibited by the same observer,⁵⁷ in whom the association of head rotation and convulsions of the arm on the left, with increase of the triceps reflex, was conceivably the outcome of pathological stimulation of the pyramidal tract. Yet the symptoms in each of these cases were curiously analogous to what we find in mental torticollis, in which abnormalities of the reflexes are conspicuous by their absence. We ought not on that account to reject the hypothesis of concurrent organic disease, inasmuch as a structural modification may be no longer the cause but the consequence of inordinate repetition of a motor reaction. Muscular hypertrophy or atrophy may be the sequel to tics born of ideas that find motor expression, and circulatory and even cellular changes may ensue on gesticulatory excess. The objective signs that reveal the existence of a point of irritation, on the presence of which the diagnosis of spasm depends, are commonly so trivial as to be wellnigh valueless, and should they be awanting, the motor disturbance appears to be purely functional, and may be considered a tic. At the same time we must admit the possibility of mixed forms, where the functional element is linked with primary or secondary organic disease, and perhaps their occurrence is more general than is ordinarily imagined. We repeat, however, that rigorous and lengthy investigation alike of the psychical and the somatic phases of the condition, embracing the state of the reflexes, will usually furnish sufficient information to facilitate the question of diagnosis and justify a positive statement.