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Tics and Their Treatment
Echolalia is amenable to treatment, and is even capable of cure. Noir gives an interesting example of the evolution of the process.
If I say to an echolalic, "Are you hungry?" he will instantly answer, "Are you hungry?" Under the influence of re-education the reply will eventually change to "Are you hungry? are you …? Yes, sir, I'm hungry," then to "Yes, sir, I'm hungry," and finally to "Yes, sir."
Echolalia, however, is not an exclusive appurtenance of those who tic. We can remember a case of general paralysis in the clinique of Brissaud at the Hôtel Dieu, who had the regular habit of repeating the question that was addressed to him; if it were a little long, only the last ten or fifteen words were echoed. A case is quoted by Cantilena of a woman with right hemiplegia and partial epilepsy who invariably reiterated the closing phrase of anything said to her. Several cerebral tumours were discovered at the autopsy.
It is conceivable of course that an actual lesion, as well as a congenital developmental defect, may interfere with the will's inhibitory powers, in which case auditory or visual stimuli are transmitted to motor centres unmodified, the result being the production of sounds or of other movements.
COPROLALIA
Coprolalia, the manie blasphématoire of Verga, is, according to Gilles de la Tourette, one of the most frequent affections of speech in the disease of convulsive tics.
There is no necessary connection, as a matter of fact, between tic and coprolalia, though of course they may co-exist, sometimes in association with other syndromes; they are in reality only episodic syndromes of hereditary insanity.
A distinction ought to be drawn between coprolalia and the use of trivial or inconvenient terms, words with which even some well-educated persons are wont to garnish their conversation. Guinon had a case of a man who in the presence of his mother resorted to language of a kind absolutely disallowed in polite society. In the etymological sense of the word, no doubt, he was a coprolalic, but it cannot be said that he was suffering from tic.
On the other hand, the abrupt and impetuous utterance of oaths or obscene expressions, to the ejaculation of which an irresistible impulse seems to drive the patient independently of time and place, amounts to a coprolalic tic of speech, and reveals a deplorable volitional debility on his part; for he is incapable of checking an act to the impropriety of which he is fully alive.
The victims of this disease (says Guinon) have an extraordinary propensity for choosing the foulest and most indecent words, however elevated their position and correct their breeding. Reference may be made to the classic instance of the Marquise de Dampierre, who all her long life was in the habit of repeating certain immodest sayings even on the most solemn occasions.
According to Guinon the reason of this bizarre preference for obscene remarks is absolutely unknown, although Charcot's view129 that coprolalia is frequently nothing more than echolalia is one of some plausibility. He refers to one of his patients who alternated her coprolalic utterances with a sort of barking noise that was an exact imitation of her favourite dog.
We ourselves have had for a long time under observation a youth in the service of Professor Brissaud whom some instinct seemed to prompt to repeat any lewd expression he happened to hear, or indeed any which might be so interpreted. It might then be said of him that his coprolalia varied with his surroundings and with his own ideas; it was accompanied by inconstant and irregular convulsive movements of the limbs.
After all, there is not so very much to choose between the coprolalic and the individual whom impatience or anger forces to blaspheme or at any rate to utter words that do not form part of his ordinary vocabulary. And though the ejaculation be not audible, the first degree of coprolalia consists in the mental presentation of the objectionable phrase. Among those who suffer from obsessions mental coprolalia is far from uncommon. A patient with folie du doute, mentioned by Séglas,130 was afraid to pronounce indelicate words because he felt himself articulating them mentally, and sometimes he used to ask whether they had not really escaped him. One step more, and these verbal hallucinations assume the characters of a genuine tic.
CHAPTER XI
THE EVOLUTION OF TIC
TIC is, from its nature, highly variable in its evolution; each tic has a development peculiar to itself. Mental differences among individuals have their counterpart in physical differences, in health as well as in disease, and a comprehensive sketch of the evolution of tic is therefore impracticable. We shall restrict ourselves accordingly to a few general remarks.
In the great proportion of cases of tic the onset is an insidious one. We have already made a sufficiently detailed examination into the pathogenic mechanism to obviate any repetition in this place, but we may note how unsettled the earliest manifestations are, how a tic may pass from one muscle or group of muscles to another, and even when its exciting cause is patent an apprentice stage always precedes its final establishment. Of the truth of this the history of J. provides an excellent instance. Another one is from Pitres:
A nine-year-old boy received a severe shock one day through being pounced on by some companions who were in hiding behind a wood pile, and though the emotion was of short duration, he commenced a few days later to exhibit involuntary muscular twitches of the upper part of his body, and to utter suppressed cries. The phenomena increased in violence and in frequency, and, in spite of treatment, a year later he was not freed of them entirely. For an unknown reason the tics renewed their activity when he was seventeen and continued so for the next three years, until a spell of Pitres' respiratory exercises effected a complete cure.
An evolution such as the above may be considered more or less typical of the great majority of tics.
We have seen that the tic may be localised indefinitely in one and the same muscle or muscular group, but its site may also vary from day to day. Two tics may co-exist and coincide, or a third may appear with the disappearance of the others. Unexpected resurrections may succeed periods of complete repose.
Tic always shows a tendency to invade; regarded as a functional act, it moves in the direction of greater complexity.
After involving the orbicularis, for instance, a tic will spread to neighbouring groups, in particular to those muscles whose synergic contractions form a special expression of countenance. That is why tics of the eyelids are associated with movements of the pyramidales, frontales, and corrugators. Tics of the lips or of the alae nasi very commonly extend to the corresponding elevators. It is not surprising that muscular groups accustomed to act in physiological unison should also be affected together (Noir).
Moreover, the fecund imagination of the victim to tic is calculated to facilitate the invention of all sorts of modifications, complications, parodies, and caricatures of the functional acts on which his tics are grafted.
Tics are constantly varying in amplitude, degree, and frequency; as O. remarked spontaneously, "We have our good days, and we have our mauvais quarts d'heure." The sedative effect of rest, solitude, silence, and obscurity may be contrasted with the detrimental results of fatigue, noise, fear of ridicule, etc.
However incapable S. is of rotating his head to the right when requested to do so, the movement is executed with the utmost readiness should his attention be drawn in that direction. But if he hesitates, even momentarily, before looking round, he cannot then do so without the preliminary performance of all sorts of contortions, ending in a twist of his body through a half circle to the right. Sometimes he actually turns round two or three times, after the fashion of a dog chasing its tail. Let him have a pleasant visit, on the other hand, let him engage in a discussion, or be engrossed in a play, let him administer a rebuke to some one, and immediately his trouble is forgotten, his speech is accompanied with animated gestures, the vicious position of his head vanishes – in short, he becomes normal.
An intercurrent affection may act either as a deterrent or as a stimulus; with convalescence, however, there is usually a re-establishment of the mischief. The most potent influence over the phenomena of tic is wielded by a sense of well-being, to employ Janet's discriminating expression. Well-being is a panacea for the tiqueur no less than for the hysteric. The tic of the worried financier disappears, as we have had occasion to note, under the magic of a rise in stocks or a knowledge of solvency. The child's happiness is bound up in his freedom, which explains the cessation, in Tissié's little patient, of all convulsive movements during the holidays.
Much evidence is forthcoming to support these points, but we must admit that the why and wherefore of a tic's amelioration or aggravation often escape us, nor must we forget that both in the child and the adult spontaneous cure is not unknown.
As has been remarked, the evolution of tic does not lend itself to systematic description, but there are cases that form an exception, their course being regularly progressive. Strictly speaking, they are instances of Gilles de la Tourette's disease.
GILLES DE LA TOURETTE'S DISEASE
Under the title, "Study of a nervous affection characterised by motor inco-ordination, and accompanied with echolalia and coprolalia," Tourette131 grouped together, in 1885, a certain number of cases presenting features in common and so enabling him to describe a morbid entity, specially remarkable for its progressive evolution. He was followed in the same line by Guinon, who supplied an account in nosographical form, and since then the disease has figured in all the text-books.
To obtain a schematic picture of the condition we shall borrow from Tourette's132 last communication on the subject:
About the age of seven or eight a little boy or girl – for the sexes are affected equally – commonly with a wretched family history, begins to exhibit a series of tics. The attention of the parents is soon drawn to the fact, but they seldom give much heed at first, since the twitches are limited preferably to the facial musculature. At this stage, too, expiratory laryngeal noises are occasionally superadded.
The movements may be confined for a long time to the face, but under the influence of causes very difficult to determine they gradually invade the shoulders and the arms. First one shoulder is shrugged and then the other, then the trunk is inclined en masse to right or left; then the patient waves his hands or his arms, or bends backwards and forwards, or jumps up and down, flexing the knees alternately and tapping with his feet. The muscles of the larynx sometimes participate in the abnormal functioning, whence it is that many sufferers from tic give vent to quick expiratory "hems" and "ahs," which coincide often with the twitches of trunk and limbs.
The disease may be limited to this stage, but it is not uncommon to find, a few months or years after the beginning of the facial movements, that the inarticulate laryngeal sound becomes organised and develops in a particular direction, thus, in a sense, showing a pathognomonic value. Under the influence of causes whose action we are, in the majority of cases, powerless to appreciate, the patient gives vent one day to a word or short phrase of a quite special character, inasmuch as its meaning is always obscene. These words and phrases are exclaimed in a loud voice, without any attempt at restraint. There must be a complete absence of the moral sense where there is coprolalia such as this; at the moment of the ejaculation some irresistible psychical impulse must drive the patient to utter filthy words unreservedly and with no consideration for other people.
Another psychical stigma – echolalia – is occasionally, though less frequently, observed in these cases.
Such, then, is Gilles de la Tourette's disease, a clinical type of which many examples have been recorded. We do not think, however, that all tics can be brought under the same category; we lose sight of its distinguishing features if we make the attempt. Of course fruste and atypical cases are encountered, but even in them it is rare not to find a certain degree of mental instability in dependence on which echolalia and coprolalia rest, so completing the morbid syndrome, and it is important to recognise the successive development of these various constituents.
It is, indeed, this evolution of symptoms which is so characteristic of Gilles de la Tourette's disease. A careful scrutiny of recorded cases of tic, however, makes it abundantly clear that they do not all belong to the disease of convulsive tics; their localisation, form, and progress are so different that the effort to assimilate them to Tourette's disease would abolish the nosographical value of the latter. One patient may have an ocular tic all his life, and nothing else; the affection of another may be limited to a tic of the shoulder and arm; a third blinks and makes a facial grimace; a fourth is a coprolalic who has never suffered from tic. Are they all to be considered incomplete cases of the disease of convulsive tics? To answer in the affirmative is equivalent to a failure to appreciate the distinctive characters of a judiciously isolated syndrome, and a refusal to describe tics as they are met with in everyday life. One questions, in fact, whether some of the cases allotted to Tourette's disease really conform to it. Take an instance from Chabbert133:
A woman, aged forty-two, had had an injury to the left side of her face at the age of nine, as a result of which appeared a convulsive facial tic, accompanied at times by hysterical attacks which continued for eight years. The tic itself, an abrupt contraction of the inferior portion of the left orbicularis palpebrarum, underwent no subsequent change, in degree or extent. At a later stage a fairly definite tendency to coprolalia became manifest.
An unvarying post-traumatic palpebral tic in an hysterical subject cannot be said to constitute the syndrome of Gilles de la Tourette, in spite of the coprolalia.
In another of his cases the diagnosis is no less open to doubt:
The son of the previous patient was a youth of nineteen, with a bad heredity on the father's side. In boyhood he had been a somnambulist. Some months previously to his coming under observation he developed a convulsive tic limited to the frontalis. Stigmata of hysteria were present in dyschromatopsia, restriction of the visual fields, and left hemihyperæsthesia.
A third case reported by the same author does probably belong to the disease of convulsive tics:
A woman aged forty-four, of a strumous diathesis, exhibited tics of face and limbs, occurring in the form of attacks sufficiently violent to cause bruises, attacks which were invariably associated with coprolalia. In addition, she suffered from echolalia, echokinesis, and folie du doute.
We can only repeat, of course, that each type of tic passes by insensible gradations into others that precede it or succeed it in the hierarchy of tics; but we must, provisionally at least, neglect the links that unite neighbouring groups if we are to avoid losing sight of admittedly distinctive characters in too comprehensive summaries. It is desirable to retain the term "disease of convulsive tics" for those cases whose progressive evolution ends in the generalisation of the convulsive movements, to the accompaniment of coprolalia and sometimes of echolalia. This clinical form represents the most advanced degree attained by the disease; it might be called the tic's apogee. From its psychical aspect, moreover, the development it undergoes may culminate in actual insanity.
According to the teaching of Magnan, the disease of convulsive tics does not constitute an entity, since each and all of its symptoms may occur separately as episodic syndromes of degeneration. The general considerations with which we introduced our study are applicable in this connection, and we shall be content to say with Noir:
We cannot deny the validity of the objections raised by Magnan and his school; but the fact that these various symptoms may and do most frequently occur singly is no reason for expunging the disease of Gilles de la Tourette from the text-books. The combination of these symptoms constitutes a clinical entity which has a specific evolution, and while its subjects are degenerates in the sense of Magnan and of Charcot, they may be ranged by themselves in a very definite group.
In some cases which apparently come under this category, psychical disturbance has not been a prominent feature.
Sciamanna134 is the reporter of a case where a young man with neuropathic antecedents was afflicted with tics involving various muscular groups; his intellect, however, was normal, and the only psychical change was an insignificant disorder of affectivity.
In such a case it would be instructive to know the mental condition after the lapse of some years.
Two typical examples of Tourette's disease have been described by Köster135 as "disease of impulsive tics"; a third case – in which widespread muscular twitches, the muscles of respiration and the cremasters included, were coupled with sometimes a monotonous intonation and sometimes a jerky speech, though psychical functions were unimpaired – is considered by Kopczynski136 to be a case of convulsive tic, which he distinguishes from the "disease of convulsive tics."
A last instance, published by Innfeld137 as a case of "chronic progressive muscular spasm," is an unmistakable example of tic, in spite of the author's declaration that it does not correspond to any known morbid type and his attempt to liken it to chronic chorea. A boy of fifteen exhibited convulsive movements which had begun in the facial musculature and thence spread to the head, shoulders, and hands, and were accompanied with respiratory noises and involuntary exclamations. There was no alteration in sensation or in reflectivity, or in electrical excitability. Sleep banished while emotion aggravated the movements.
VARIABLE CHOREA OF BRISSAUD
If the disease of Gilles de la Tourette, by reason of the uniformity of its symptomatology and the regularity of its evolution, justifies its differentiation as a separate entity among the tics, a comparison of it with another type, of polymorphic manifestation, irregular evolution, and uncertain duration, may prove instructive. We refer to the affection described by Brissaud as variable chorea.
The form of the motor reactions in this condition warrants the application to it of the term chorea, but the analogies the disease presents to tic are very close, nevertheless, and sometimes the two occur in the same individual. Patients suffering from variable chorea reveal the same mental abnormalities as are found among those who tic, while the troubles of motility are sometimes so similar to what we meet with in the latter that Gilles de la Tourette regarded the condition simply as one form of convulsive tic, the more so that it is occasionally accompanied by explosive utterance and even coprolalia.
This view, however, is calculated to obliterate the distinctive characters of the two affections, and ought not to be entertained. We cannot do better than repeat Brissaud's original description:
The use of the word chorea need occasion no ambiguity: the chorea consists in the appearance of meaningless and apparently idiopathic involuntary movements, whose repetition during rest and action alike is proof of their irrationality and incongruity; the duration of the symptoms may be limited as in chorea minor or Sydenham's chorea, or unlimited as in chorea major or Huntington's chorea. "Variable" is the epithet we apply to the chorea because of the lack of uniformity in its exteriorisation, the irregularity of its development, and the inconstancy of its duration. It comes and goes, waxes and wanes, vanishes abruptly to reappear unexpectedly; it is a neurosis without a characteristic march.
Notwithstanding the fact that we are dealing with a chorea – that is to say, with a disease which is almost as readily recognisable by the public as by any professional – the difficulty of fixing its onset is paralleled by the difficulty of knowing when it has ceased. This uncertainty is explained by the facile and changeable nature of the patient; until the condition is revealed by unmistakable signs it passes for an insignificant muscular caprice of no pathological importance, while its disappearance is not associated with any particular modification of the patient's ways.
There is a natural tendency to identify all "nervous movements" with myoclonus, but the conception is a remarkably nebulous one, and means nothing more than "muscular twitch." On the other hand, it is well understood that "nervous movements" are more or less sudden movements of limbs, shoulders, face, always involuntary and generally increasing in force and frequency with the nervous state of the patient.
Parents say, for instance, that their child has become more restless and irascible, and at the same time that he has had "more movements of the nerves." The coincidence is unfailing. Is the expression "nervous movement" lacking in precision? Yet it signifies what it is intended to signify. We are concerned neither with tonic convulsions nor with clonic spasms, nor yet with tics of habit; what the term stands for is a complex contraction, brisk but not violent, closely allied to the simplest of automatic acts, such as a step in advance, a shrug of the shoulders, a frown, a sigh, a moan, a crack of the fingers, an exclamation – in any case usually a gesture of impatience. The whole thing, however, is so variable and fugitive, that it cannot be said to constitute a definite convulsive phenomenon. The contractions, further, in spite of their complexity, escape the notice of their originator, who is quite surprised at being asked the meaning of the movement he has just made, as he is almost entirely ignorant of it.
Briefly, the "nervous movements" of which we have been speaking do not belong either to myoclonus or to tic, but owe their distinctiveness to their multiplicity and inconstancy. At the same time they are always grafted on a certain neuropathic diathesis akin to that of chorea; in fact, they are nought else than a form of chorea themselves.
The psychical peculiarities of the patient with variable chorea may be summed up in instability of thought and action, combined with mental infantilism. Hence the terms "polymorphous chorea" and "chorea of degenerates" are used synonymously for variable chorea.138 Sometimes the disorders of the mind include hallucinations, and various forms of phobia or mania.
One or two examples may be given:
A microcephalic youth of sixteen, a monorchid, developed what appeared at first to be an ordinary chorea subsequently to an orchidopexy. The movements, however, varied from day to day and from hour to hour. Sometimes they disappeared for days at a time, to reappear suddenly just when the neurosis seemed cured. The influence exerted on them by the will was both mild and transient. They constituted, in short, a particular kind of chorea, changing and changeable, and differing from intermittent chorea in that neither remissions nor relapses were ever wholly complete. Further, the condition was implanted on a basis of mental and physical degeneration, and seemed likely to become established as a permanent functional stigma.
In another case a peculiar chorea gradually supervened, for no obvious reason, in an adult female of tardy and imperfect physical and intellectual development. It was difficult to decide whether the psychical or the somatic phenomena were preponderant; but to the material, tangible, and visible signs of constitutional inferiority was superadded a choreiform instability of the whole voluntary muscular system, consisting in agitation, gesticulation, and incorrigible motor restlessness, coupled with a conspicuous incapacity for rational action.
The steps in the evolution of this functional defect were very slow, and coincided with final confirmation of the intellectual insufficiency. As for the chorea, its localisation and its intensity, its increase and its decrease, its extension and its limitation, seemed to vary, in a way that could not be foreseen, at the call of certain undetermined circumstances.