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Kitabı oku: «Tics and Their Treatment», sayfa 20

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TICS – INSANITY – IDIOCY

Insanity in any of its forms may be accompanied by clonic or tonic convulsive movements – movements that may be of the nature of tics or spasms or stereotyped acts, or that may belong to conditions which we distinguish by the names of myoclonus, polyclonus, myotonia, catatonia, etc. It is highly probable that many instances have been described as spasms which, according to our nomenclature, must be considered tics. Brodie, to take an example, quotes a case where a "spasm" of the spinal accessory was replaced by a mental affection. Alternation of hallucinatory mental confusion with "spasm" of the neck muscles has been observed by Oppenheim, as well as a case where the "spasm" originated in the course of an attack of alcoholic mania. In another, due to Gowers, "spasm" of the muscles of the neck was preceded, at a ten years' interval, by an attack of melancholia.

Most of the cases of this nature would be held to-day to be instances of mental torticollis.

That tics and mental disease accompany each other is notorious, but a discussion of the question would carry us beyond our limits. We must say a word, however, on the tics of idiots.

The study of tic as it occurs in idiots, imbeciles, and arriérés, has engrossed the attention of alienists since the days of Pinel and Esquirol. Cruchet says the mental state of the idiot and the imbecile is so characteristic that the diagnosis of convulsive tic in such cases is never attended with any difficulty. Yet the task is sometimes sufficiently delicate, for we maintain that upon our insight into the subject's mental condition depends our ability to analyse his tics.

Considerable light has been thrown on the question by the important information amassed by Bourneville, as well as by the fine psychological studies of Sollier and the meritorious thesis of Noir, from which we shall borrow largely in this place.

In the first instance, we meet with tics in every way comparable to those we have already described, and we may give one or two examples.

R. accidentally wounded his left eye at the age of eleven, and contracted a tic which consists in spasmodic blinking of the eyelids, though no sign of ocular lesion is left. A diminution in its intensity has been taking place, which has culminated recently in its spontaneous disappearance.

N. had an attack of ciliary blepharitis and keratitis which left an opaque patch on the upper and inner part of his left cornea, and he has blinked ever since. Yet there is no local irritation to justify the continuance of the movements.

The tics are occasionally as numerous and violent as in Gilles de la Tourette's disease, and are accompanied with cries and with coprolalia.

L. is afflicted with abrupt blinking of the eyelids, retraction of the head, and elevation of the lip. Once the tic is established, it persists on an average for from eight days to a month, and during this time no effort on his part will check it. Sometimes he makes peculiar growling noises; sometimes he cannot prevent himself from stooping down as if to pick up stones; sometimes he is unable to restrain himself from touching everything within reach.

From the age of five, C. exhibited frequent blinking movements, and gestures which seemed to indicate that his clothes were uncomfortable. No attempt at modification was attended with success. The tics steadily increased, till he found himself uttering cries and letting obscene words escape his lips. For a long time they remained in abeyance, then reappeared in his face and trunk, in the form of salutation movements. His propensity for clastomania, pyromania, and kleptomania necessitates his being kept under strict supervision, and though he is intelligent and has a good memory, he is also lazy and inattentive.

Other tics of still greater complexity and peculiarity are met with among those whose psychical imperfections are very pronounced. Some "co-ordinated tics" are remarkable for their intricacy; they consist of a series of movements which mimic some act of everyday life. In this group may be specified various rhythmical movements, such as those of balancing, head rotation, and striking or beating oneself – the krouomania of Roubinowitch; they may be compared to the mother's rocking of her infant, inasmuch as they have a soothing effect on their subject, however brutal the movement itself sometimes may be.

In most cases the patient is seated and rocks himself to and fro in an antero-posterior direction. Or it may be the head only that is rhythmically moved from side to side, and the performance may go on indefinitely. A mere touch or a word, on the other hand, is commonly sufficient to interrupt its sequence.

There remains a final class of co-ordinated tics, which Noir distinguishes by the epithet "large," tics which are confined to idiots of good physical development. They consist of a movement or series of movements of considerable amplitude, and constitute the predominant clinical feature of the patient's idiocy. Here we find subjects who jump, or climb, or turn round and round; in other cases they are reduced to the level of mere automata, and go through a long series of actions in a mechanical way.

Their memory for recent occurrences is very poor, but in their minds are stowed away vague souvenirs of events long past, which they translate into action, and which they are incapable of modifying, even as they are unable to add to their mental store or to alter their mental routine.

A classic instance of this variety of tic is Ros., long known at Bicêtre as "the waltzer."

CHAPTER XV
THE DISTINCTIVE FEATURES OF TIC

WE are scarcely inclined to believe in the possibility of condensing into an adequately concise and adequately precise formula our conception of tic, or at least all the notions which contribute to it. Because most authors feel it incumbent on them to fall in with this nosographical custom, definitions have been proposed whose brevity only serves to confuse the issue. Opinion on the interpretation of certain words which concern our subject is far from being unanimous, and, as we remarked at the outset, accuracy in our terminology is urgently called for. This has been our reason for preceding our definitions by the results of clinical observation and pathogenic analysis.

Our idea of tic, however, may be couched in the following terms:

A tic is a co-ordinated purposive act, provoked in the first instance by some external cause or by an idea; repetition leads to its becoming habitual, and finally to its involuntary reproduction without cause and for no purpose, at the same time as its form, intensity, and frequency are exaggerated; it thus assumes the characters of a convulsive movement, inopportune and excessive; its execution is often preceded by an irresistible impulse, its suppression associated with malaise. The effect of distraction or of volitional effort is to diminish its activity; in sleep it disappears. It occurs in predisposed individuals, who usually show other indications of mental instability. 167

We are in a position, now, to elaborate the details of this definition. Tic is a psychomotor affection, and there are two inseparable elements in its constitution, a mental defect and a motor defect.

The prevailing mental defect is impairment of volition, which takes the form either of debility or of versatility of the will. This being characteristic of the mind of the child, its continuance in spite of years argues a partial arrest of psychical development. Hence the epithet infantile may be employed to qualify the patient's mental state.

Other psychical troubles, which similarly are anomalies of volition, may be superadded, in particular impulsions and obsessions.

Speaking generally, a certain degree of mental instability is a distinguishing feature of the patient with tic.

The defect of motility consists at first in the provocation of a motor reaction by some external cause, or by an idea.

In the former case, the reaction is the cortical response to a peripheral stimulus, and its logical execution becomes by dint of repetition habitual and automatic. With the disappearance of the stimulus it continues to manifest itself, without cause and for no purpose, in which circumstances the feebleness of the inhibitory power of the will is revealed.

In the latter case, the motor reaction is called into being under the influence of an idea, normal or pathological, which eventually ceases to operate, and by virtue of the same pathogenic mechanism the act remains, inopportune and exaggerated.

The objective manifestation of tic is a clonic or tonic convulsive movement, an anomaly by excess of muscular contraction.

In the clonic variety there are undue rapidity and increased frequency of the movements.

In the tonic variety, the duration of the contraction is prolonged.

The intensity of the movements, likewise, is abnormal in degree.

In spite of these disfigurations, so to speak, of the original movement, it is practically always possible to detect in them co-ordination and purpose, the cause and the significance of which ought to become the object of our search.

The motor disorder can never be reduced to mere fibrillation, nor indeed to fascicular contraction unless in some one muscle different bundles have different physiological attributes. It is usual for several muscles to be concerned, and their anatomical nerve supply may be from separate sources.

Like ordinary functional motor acts, tics are distinguished by co-ordination of muscular contraction and repetition; they are preceded by a desire for their execution, and succeeded by a feeling of satisfaction.

These features, however, are carried to excess.

In addition, the functional act is inapposite, sometimes even harmful; it may be described as a parasite function.

The muscular contractions follow each other at irregular intervals; they come in attacks, which, it is true, are highly variable in frequency, duration, and degree.

Volition and attention exercise a restraining influence on the motor phenomena, but repression is accompanied by malaise, sometimes by actual anguish.

Distraction suspends the activity of tic; physical fatigue and emotion are calculated to arouse it.

Tics always disappear in sleep.

They are unaccompanied by any alteration in sensation, in the reflexes, or in the trophic functions.

They are not associated with pain.

In this general way we have indicated the distinctive features of tic, and we may take the opportunity to remind ourselves of their extreme variability.

In discussing the question of diagnosis, we shall have occasion to emphasise the importance of fruste, atypical, and transitional cases, not because we think they can be systematised as yet, but because they may be capable of new pathogenic interpretations which we cannot afford a priori to set aside.

We venture to believe that tic has a clinical individuality of its own which we have tried to portray, and we go so far as to say that an appreciation of the points we have touched on will prove of service in matters of diagnosis.

CHAPTER XVI
DIAGNOSIS

TICS AND STEREOTYPED ACTS

WE have already, on more than one occasion, drawn attention to the phenomena known as stereotyped acts, demonstrating their intimate kinship with the tics and the frequent difficulty of establishing a differential diagnosis. To ensure precision of ideas and of terminology, we must restrict the expression to motor disturbances in which the characters of the muscular contraction are identical with those of normal acts. On this view many motor reactions are really classifiable as stereotyped acts, and among them are those denominated by Letulle "habit tics."

Stereotyped acts occur in normal individuals, and it may fairly be said there is no one but has his habitual gesture, his movement of predilection. As a matter of fact, a certain number of what Letulle calls co-ordinated tics belong to the group under consideration; others, no doubt, are genuine tics, and between the two may be found innumerable intermediate varieties.

From the diagnostic standpoint the stereotyped acts that occur in the course of mental disease, of which a conscientious study has recently been made by Cahen,168 are highly instructive. He defines them as non-convulsive, co-ordinated attitudes or movements, resembling intentional or professional acts, repeated at frequent intervals and always in the same fashion, till their conscious and voluntary performance is replaced by a degree of subconscious automatism. In the case of the insane they are secondary to some delusion, and persist though the latter may disappear. Hence the patient may be incapable of explaining his movements and attitudes, however much he may persevere in their automatic execution – an evolutionary process akin to that of the tics.

A typical instance may be quoted from Séglas:

B. passed under observation in 1891, suffering from delusions of persecution, and not long afterwards it was noticed that from time to time he used to come to a halt in the courtyard, gaze at the sun, and rotate his hands round an imaginary axis. The reply he vouchsafed to interrogation on this point was that he was effecting the sun's revolution. At present, however, he has sunk into a state of dementia, and while the gesture continues he is unable to furnish any explanation of it.

Of course it is inadmissible to apply the term to co-ordinated acts that are neither conscious nor voluntary, such as the teeth grinding of the general paralytic, or the body oscillation of the idiot. Similarly one must differentiate them from impulsive seizures, abrupt irresistible motor explosions neither frequent nor prolonged.

A distinction has been drawn between akinetic (attitude) stereotyped acts and parakinetic (movement) stereotyped acts. As instances of the former we may give the following:

A woman reclines continuously in bed because she believes she has an infernal machine in her abdomen.

Another patient sits on the ground all day long, buttoning and unbuttoning his clothes.

An old gymnast maintains while he stands a professional attitude in which his head is raised, his right fist closed on his hip, his right leg crossed in front of the left, and his right foot elevated vertically.

Conditions such as these present the most intimate analogies to our attitude tics, though in the case of the latter there is always a more or less pronounced exaggeration of muscular contraction, a certain degree of tonic convulsion.

Parakinetic stereotyped acts are of common occurrence, and embrace every variety of movement or gesture.

A former acrobat leaps staircases, climbs railings, exercises his arms rhythmically and regularly, etc.

A patient promenades untiringly in the same corner and at the same pace.

An old engraver, now a dement, passes the day in reproducing in a more or less modified form certain actions associated with his former profession.

Alike in tics and in stereotyped acts, a time comes when the motor habit establishes itself, for no apparent reason or purpose; hence the co-existence of the two classes in chronic delusional insanity, in dementia precox, in catatonic states, in systematised mental disease of other forms, and in general paralysis.

Stereotyped acts may be the embodiment of ideas of persecution and of grandeur, or the outcome of mystical, hypochondriacal, and other states. A patient with delusions of persecution writhes because he is being "electrified." A hypochondriac rests motionless because he believes himself made of glass. A mystic maintains an attitude of genuflexion for hours at a time.

Obsessions also play a part in the genesis of the acts we have under consideration, but of all delusional ideas those of defence are the most fertile in this respect.

A patient under the care of A. Marie used to carry a fragment of glass between his teeth and other pieces beneath the soles of his feet, the idea being that they formed insulating cushions whereby to protect himself from the electricity of his enemies.

The suggestion was thrown out by Bresler that the movements of tic are often of a defensive character – that the disease, in fact, is a sort of "defence neurosis" linked to hyperexcitability of psychomotor centres. This theory is not unlike the view of hysteria taken by Brener and Freud, and as the movements themselves are usually of the nature of mimicry, Bresler has proposed the term mimische Krampfneurose.

In some cases of mental torticollis, the attitude assumed may be considered as a stereotyped act. Martin has recorded an example of torticollis in relation to melancholia. Another of his patients suffered from rotation of the head to the left, a position which could easily be rectified by asking the man to make the sign of the cross. The moment he put his finger on his forehead the displacement of the head was corrected. If, however, he were requested to look straight in front of him, he remained incapable of altering the vicious attitude, the reason he advanced being that he could no longer see the sun.

One cannot but be struck with the remarkable analogies to the cases given by Cohen. And it is worth remembering further, that sometimes mental torticollis degenerates into actual dementia.

TICS AND SPASMS

Nothing is more arduous, at first sight, than the differentiation of a tic from a spasm, the similarity of their external forms being a fertile source of confusion. Yet the establishment of a correct diagnosis is of prime importance, since in their case prognosis and treatment alike are diametrically opposed.

Tic is a psychical affection capable of being cured, if one can will to cure it: at the worst we may fail, but there is no idea that it is indicative of a grave organic lesion prejudicial to life. A spasm, on the contrary, though it appear in almost identical garb, is excited by a material lesion on which depends the degree of its gravity. The focus of disease may disappear, no doubt, but it is only too likely to persist and to occasion other disorders. Hence the desirability of making sure of one's diagnosis – a proceeding not necessarily of insuperable difficulty. If we apply the principles of diagnosis enunciated by Brissaud, to which our attention has already been directed, we shall not find the task beyond our powers.

Let us take a concrete instance.

Here is a cabman, forty-nine years of age, the left half of whose face is the seat of convulsive twitches. These commenced eighteen months ago by brief insignificant contractions of the left orbicularis palpebrarum, which have gradually spread to the whole of the muscular domain supplied by the left facial nerve. Their momentariness and rapidity, their apparent independence of extraneous stimuli, their indifference to treatment and resemblance to the twitches produced by electrical excitation, their occurrence in sleep, the fact of voluntary effort, of attention or distraction, serving so little to modify their range and intensity – all make clear the spasmodic nature of the condition.

The motor manifestation is the consequence of irritation at some point on a bulbo-spinal reflex arc; its abruptness and instantaneousness negative the possibility of recognising in it any sign of functional systematisation. It is not a co-ordinated act of a purposive nature, but a simple, unvarying, constant motor reaction to a particular stimulus. That its intensity should be in direct proportion to the intensity of the latter, changing from feeble contractions to a state of transient tetanus, is further proof of its spasmodic origin. When the excitation is at its maximum, there is sometimes involvement of the opposite side of the face, by virtue of the law of the generalisation of reflexes.

It is true there is no association of pain with his attacks, as in so-called tic douloureux, but the spasm is heralded by a tingling sensation below and to the inner side of the outer corner of the eye. This sensation, "like an electric battery," persists during the spasm and disappears in the intervals. Its occurrence suggests that the ascending branch of the infraorbital nerve, springing from the trigeminal, is affected, and indeed pressure over its point of emergence evokes a certain amount of pain. Moreover, there is occasionally a flow of tears when the spasm is at its height. It may be difficult to decide whether this is the result of mechanical compression of the lachrymal gland or an exaggerated secretion of tears under the influence of stimulation of the lachrymo-palpebral twig of the orbital nerve. In any case the pathogeny of this facial spasm is entirely comparable to that of tic douloureux of the face, and it is quite within the bounds of possibility that a minute hæmorrhage – for the patient is of a very florid type – somewhere on the centrifugal path of the trigemino-facial reflex arc, may be giving rise to the phenomena.

What we wish to insist on, however, is the dissimilarity between this facial spasm and tic. In the movements we have been describing we fail to distinguish any purposive element, any co-ordination for the fulfilment of a particular function: they are not imitative in character, nor do they express any sentiment; no impulse precedes their execution, no satisfaction follows.

The patient's mental state presents no peculiarities, as far as we have been able to discover. There is no volitional debility or instability; if he cannot control the convulsions, it is to be remarked that he cannot control them even for a moment, whereas all sufferers from tic are capable of inhibiting it for a longer or shorter period by an effort of the will, by concentrating their attention on it.169

The following remarks on this case are due to Professor Joffroy:

If the patient be asked to open his mouth, the spasm of the left cheek remains in abeyance at long as it is open, but the platysma of the same side then begins to twitch spasmodically. Or if he be requested to shut his eyes, so long as they continue closed the cheek is quiescent; but, on the other hand, both orbiculares palpebrarum, as well as the pyramidal muscles and the adjacent fibres of the frontalis, are seen to contract irregularly. There is a sort of transference of spasm, and this is of peculiar interest, inasmuch at it affords evidence that the lesion is not so restricted as one might suppose.

The explanation no doubt is to be sought in the law of the diffusion of reflexes, confirming the diagnosis of an irritative lesion at some point on the trigemino-facial reflex arc.

In the differential diagnosis of spasm assistance may be obtained by a consideration of the following points:

The extreme abruptness of the movement recalls the contractions produced by electrical stimulation.

There is no purposive or co-ordinated feature in the spasm, which is confined to some nerve area anatomically limited.

Volition, attention, distraction, emotion, all fail to effect any modification of the phenomena.

No irresistible impulse precedes their manifestation, nor is it succeeded by a feeling of satisfaction. Sometimes the spasm is accompanied by severe pain.

As a general rule the patient's mental state does not present the anomalies met with so frequently among those who tic.

Important information may be gleaned from a scrutiny of the condition during sleep. Should the convulsive movement persist, it may be said with confidence to be a spasm; whereas if it completely disappear, it is probably a tic. Whether a spasm may vanish in sleep, however, is another question, which clinical observation has not yet satisfactorily answered, and if no other indication of organic disease be forthcoming, the problem must in the present state of our knowledge be left unsolved.

A. Tic or Spasm of the Face

In cases where the face is the seat of the convulsive movements this problem of diagnosis becomes one of the utmost nicety. That a distinction may be drawn, however, is universally admitted. Hallion,170 for instance, specifically separates clonic spasms due to structural changes from the "nervous movements" of neuroses such as chorea or tic. Facial spasm is rigorously limited to the distribution of the nerve, and is commonly the result of some alteration in it effected by causes similar to those that occasion facial paralysis.

Clonic spasms of the face are occasionally a sequel to local traumatism – that is to say, they are the result not of direct but of reflex excitation of the facial nerve. Tic douloureux belongs to this class. Tic non-douloureux also is sometimes merely a simple reflex spasm.

One of the most pregnant of Brissaud's lessons is devoted to the elucidation of this part of our subject, and we have already made several quotations from it. In many cases he is forced to say, "I decline to hazard a diagnosis when etiology is silent." We too have been face to face with this diagnostic difficulty on several occasions, and it may be instructive to give the details of one or two cases where no definite conclusion could be arrived at.

A man thirty-seven years of age had been suddenly seized with facial paralysis on the left side thirteen years before, accompanied after an interval of eight days by bilateral fronto-temporal cephalalgia, nausea, vomiting, and disturbances of vision. These attacks recurred irregularly during the next four years, since when they have ceased, although the palsy persists. Recently the patient woke up abruptly in the middle of the night to find that the left side of the face was in a state of spasmodic contraction, a condition which has continued absolutely without intermission. There is no pain in relation to the spasm, merely a peculiar sensation at the site of the muscular twitches. Of what nature are they?

If we analyse the muscular play somewhat more closely, we observe that with the exception of the frontalis all the muscles of the left face, including the platysma, contribute. On a background of more or less permanent contraction are outlined short, incomplete, greatly varying twitches, affecting one muscle after another, and sometimes only a few fibres, in a highly erratic way. The march of the movements obeys no law, either of space or time, nor is there any co-ordination in their activity. That the condition is one of tic, therefore, is scarcely conceivable. No purposive element is discoverable in the phenomena, no systematisation, no expression of emotional excess. All is disorder, confusion, contradiction.

We should, accordingly, be content to make a diagnosis of spasm, but an examination of the patient's mental condition must not be neglected, and in this particular case it is very instructive.

It appears that his imagination has always been singularly fertile, amounting indeed to eccentricity. The picturesque description he furnished of the unusual sensations in face and neck lent support to the view that his muscular activity was intended, consciously or unconsciously, to free himself from their insistence, so that his grimacing may have been but a gesture of defence.

But however much his lack of psychical equilibrium may favour the relegation of his affection to the category of tic, certain considerations make one question the validity of the hypothesis.

In the first place, it is rather an uncommon functional adaptation of the facial muscles to utilise them in an attempt to disembarrass oneself of disagreeable sensations; and in the second it is no less uncommon for the sufferer from tic to be unable to restrain his muscles even momentarily, as our patient appears to be. The actual time of onset of the movements is significant enough, but of supreme importance is the fact of their supervention in an area previously the seat of paralysis. To our mind this is more than a coincidence; from the history supplied by the patient it is plain that the paralysis was peripheral and that the lesion involved the facial trunk somewhere in its intracranial course after its emergence from the side of the pons. Thirteen years later, convulsive movements appear in the same domain. Taking all the circumstances into consideration, we think the hypothesis tenable that the trigeminal is implicated in the pathogeny of the spasm, although the condition is not strictly comparable to the classic tic douloureux.

The exact nature of the lesion is more difficult to determine. A review of the details of the facial palsy suggests its vascular origin, to which theory the headache, nausea, and photophobia of succeeding days and months – indicating, as they do, a circulatory disturbance in the basilar region – lend support. With the gradual restoration of vascular equilibrium the migrainous attacks lessened in frequency and severity, though the facial trunk remained compressed, till the spasm appeared, no less suddenly than had the paralysis. It is feasible that the former, too, is the derivative of a minute hæmorrhage irritating either the centrifugal or the centripetal arm of the facial reflex arc, probably the latter, which would explain the paræsthesiæ.

The possibility of this explanation being accurate is confirmed by a case reported by Schültz, where facial spasm of ten years' duration was shown at the autopsy to have been caused by an aneurism of the left vertebral artery impinging on the facial nerve in the neighbourhood of the basilar trunk.

The arguments, therefore, which plead in favour of the spasmodic nature of the condition seem to us so cogent that the hypothesis of tic must be rejected. We ought not to forget, on the other hand, that a spasm, of whatsoever origin, may be transformed into a tic by the perpetuation of a morbid habit.

Let us take a second case, no less instructive than the preceding.

Madame L. was sent to one of us by Professor Pierre Marie. She had always been nervous, impressionable, and high-spirited, but had never suffered from fits. At the age of eight years, during convalescence from one of the exanthemata, she got a chill, and the very next day developed an acutely painful torticollis, the head resting on the right shoulder and the chin touching the left clavicle. A complete cure ensued, but from that time a certain degree of facial asymmetry was remarked. At the age of eight and a half menstruation commenced, and it still continues, at the age of fifty-nine.

167.MEIGE, Les tics, July, 1905 (Masson).
168.CAHEN, "Contribution à l'étude des stéréotypies," Archives de neurologie, 1901, p. 474.
169.MEIGE, "Spasme facial franc," Soc. de neur. de Paris, April 17, 1902.
170.HALLION, "Convulsions localisées," Traité de médecine, vol. vi. p. 897.

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