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.
From youth she had at intervals been stricken with pains in the limbs, and with recurrent bilious attacks. Two years ago the death of her husband was the occasion of great mental strain and distress. Sixteen months ago she noticed a curious sensation in the right eye, not painful, accompanied from time to time by blinking of the lids. Very gradually the convulsive movements spread over the whole of the right face, and for the last month their frequency and intensity have been such that rest is an impossibility.
When she came under observation what impressed the mind first was the remarkable asymmetry of her figure: the right side of the face was smaller than the left, the right eye appeared to be at a lower level than the other, while the mouth was strongly deviated to the right and the chin twisted in the same direction. For a minute or two the facial contortion held sway, disappearing only to reappear quickly.
Not solely to the old torticollis was the facial asymmetry attributable, but also to the convulsive movements of the right half of the face. The effect of these was to close the right eye, deflect the nose to the same side, drag the mouth in a similar fashion, and wrinkle the skin of the chin and neck. Hence was evolved a unilateral grimace quite unlike any ordinary expression, resembling rather the facies in contracture secondary to facial paralysis.
During the next few months there was a gradual change from this tonic to a clonic stage, in which the movements were of less frequent occurrence, but more rapid. In repose there was no further indication of the old facial palsy than the flattening of the facial lines on the right. Under the influence of any emotion, or any passing contrariety, or in the course of an animated conversation, or if circumstances call for their repression, the spasms increase in number and degree, whereas solitude and tranquillity favour their subsidence.
A recent development has been the discovery of a means of checking the spasm—viz. by compressing the larynx with the fingers of the two hands. Madame L. admits the illogical nature of the manœuvre, but extols its efficacy. As a matter of fact, it sometimes fails of its object.
How, then, is this localised convulsive movement to be designated? Is it a tic or is it a spasm?
The march of the disease, its painlessness, the absence of any reaction in sleep, the success of the little laryngeal trick, the inhibitory effect of the will, the definite influence of attention, distraction, in short of the psychical condition of the moment—all plead in favour of its classification in the former category. On the other hand, we cannot shut our eyes to the fact of the pre-existence of specific organic disease, and, moreover, the spasm is strictly confined to the anatomical distribution of the facial nerve. Even in periods of repose there is a certain amount of fibrillation on that side. On these counts are we to hazard the diagnosis of facial trophoneurosis?
A subsequent opportunity of examining the same patient served to confirm the diagnosis of spasm secondary to facial dystrophy, and treatment failed to make any impression on the condition.
Our object in giving these cases has been to point out the difficulties in the way of diagnosis, especially where spasm is superadded to a mental state that itself predisposes to tic. The wisest plan in many instances is to confine oneself to a description of the symptoms and to tabulate the arguments for and against a particular view, without perpetrating the error of committing oneself.
Many cases labelled convulsive tic might be quoted where the expression of so definite an opinion ought to have been reserved, as in one reported by Mayer[171] under the title of convulsive tic consecutive to infraorbital neuralgia:
A man, thirty-two years of age, had suffered from a severe infraorbital neuralgia of some weeks' duration, apparently attributable to a chill. The pains recurred at intervals till their substitution five years later for slight spasmodic twitches of the left eyelid, which gradually developed into violent convulsions of the whole of the left half of the face. These spasms were preceded by a sensation of numbness in the left ear, while during repose no modification of facial expression was to be remarked.
Further, there was a history of exactly similar neuralgia and spasm in the mother of the patient, although in her case the latter had been the first to appear, and had been replaced after a six years' interval by left facial neuralgia, which resection of the nerve failed to relieve.
In these cases the condition is undoubtedly one of painful facial spasm, inaccurately and unfortunately styled "tic douloureux."
Bruandet[172] has recorded a typical example of right facial hemispasm consequent on facial neuralgia, in which, however, no certain macroscopical or microscopical lesion was detected, in either cortex or bulb. But the mere fact that no structural alteration was discovered post-mortem cannot invalidate the diagnosis; the imperfection of our methods of investigation suffices to explain the negative results of such researches.
B. Tic or Spasm of the Neck—Torticollis Tic and Torticollis Spasm
To make a diagnosis of torticollis, it is essential to satisfy oneself of the integrity of the bones, muscles, and articulations of the cervico-scapular region, previous to directing attention to the psychical state of the patient. In regard to the latter point, the question of heredity must not be neglected. If personal and hereditary defects are prominent, the presumption is in favour of mental torticollis; and if the convulsive movements present the characters of tic, the diagnosis is practically certain.
In three cases under the observation of Fornaca,[173] for instance, there is no room for doubt. Not merely was there no sign of irritation from peripheral sources, but also no one of the three was psychically normal.
Nevertheless we frequently find ourselves confronted by the question: is the movement a tic, or is it a spasm? For, strictly speaking, there are both a torticollis tic and a torticollis spasm, and their separation one from the other is often a matter of the greatest perplexity.
We must refer the reader to the chapter devoted to mental torticollis for a consideration of the features of that condition, and we need not dwell on those cases of spasmodic torticollis that are obviously occasioned by irritative lesions of nervous centres or conductors. In this latter category may be placed the case put on record by Oppenheim, where torticollic spasms were produced by pressure of a cerebellar tumour on the cranial nerves.
But in the affection known as hyperkinesis of the accessory of Willis we have little doubt both tics and spasms have been included. Apart from the cases of spasmodic torticollis, so called, which Babinski has published and to which reference has already been made, we may be allowed to cite one or two more, in order to exemplify the differences of interpretation to which they are liable.
At the Congress of Toulouse two patients were shown by Desterac,[174] both of whom had suffered since the age of eight from a disease akin either to Friedreich's disease or to hereditary cerebellar ataxia.
They presented the spastic gait of the former with the involuntary movements of the latter, in addition to spasm of the hand in writing, spasmodic movements of the trunk, and spasmodic torticollis. Both had club foot and scoliosis, and one was afflicted with spasm of the face and left arm. In his case, further, there was nystagmus, together with loss of reflexes and difficulty in articulation, while fibrillary contractions were to be observed in his muscles. The other patient's reflexes were exaggerated, and he showed a double extensor response.
In Desterac's opinion their spasmodic torticollis was dependent on this congenital constitutional affection, which might be regarded as a fruste form of one of the diseases above mentioned.
Through the kindness of M. Desterac the opportunity has been granted one of us of examining the two patients, and we should like to point out why we think his interpretation of their symptoms must be considered with reserve.
Speaking generally, we thought the cases closely resembled those in which a long-standing mental torticollis is accompanied with convulsive movements of the limbs. The scoliosis was not permanent, the deformation of the foot could be overcome, and at the same time we failed to convince ourselves of the presence of nystagmus and the absence of the knee-jerks. Moreover, we happened to observe one of the patients in the street unawares, and remarked how between two phases of bizarre contortions his vicious attitudes and convulsive gestures almost entirely vanished. In fact, the clinical picture seemed to us to be quite other than that associated with organic disease such as Friedreich's disease or hereditary cerebellar ataxia.
Another case recently brought before the Neurological Society of Paris by Marie and Guillain[175] serves even better to illustrate the intricacies of diagnosis.
The patient was a man of fifty-eight, who for years had exhibited certain movements apparently of an athetoid nature. His head was extended and rotated to the right synchronously with elevation and eversion of the left shoulder, then it passed into flexion. Except for a few odd movements of the tongue, the face conserved immobility. In the arms the localisation of the contractions was mostly proximal, though there were alternating flexion and extension movements of the fingers which suggested athetosis. Flexion, inversion, and adduction of the thighs also occurred. The recti abdominis were similarly involved.
Under the influence of emotion the movements were increased, but they could not be inhibited by an effort of attention. Their rate was too slow for chorea. Ordinary voluntary movements were performed without apparent trouble; the patient was able to dress himself, and to drink without spilling the liquid. Diminution of the knee-jerks was noticed, with what seemed to be an extensor response. Slight scoliosis of the vertebral column and a misshapen right foot recalled Friedreich's ataxia. There was nothing to justify a diagnosis of hysteria.
This curious condition dated from the year 1874, when the patient had a febrile attack, in the course of which pain and tingling appeared in the toes of the right foot, followed by involuntary movements of the same member. Analogous symptoms were not long in appearing in the left arm. Two months later the condition had become general, but from that time no special modification took place.
In the subsequent discussion it was remarked by Souques that the case resembled one recorded by Chauffard[176] as Friedreich's disease with athetotic attitudes, where the patient was a child with club foot, diminution of the knee-jerks, and generalised athetotic movements.
Notwithstanding our inability to assign a definite nosographical position to examples of this kind, we think it desirable to make some reference to them, in the hope that further observations will aid in their diagnosis. They at least remind us that convulsions occurring in the course of organic disease may be simulated by the manifestations of certain motor neuroses.
TICS AND CHOREAS
A. Sydenham's Chorea
It would be difficult to find a better description of chorea minor than that given originally by Sydenham himself:
The dance of Saint Guy, chorea Sancti Viti in Latin, is a sort of convulsion whose incidence is greatest, in both sexes, between the age of ten and puberty. Its onset is characterised by weakness of one limb, which the patient drags behind him, and soon the arm of the same side is affected in the same way. He finds it impossible to maintain the same position of the arm for two consecutive moments, however great be his efforts to attain this object. Before he can bring a full glass to his lips he makes innumerable gestures and antics, as the convulsive moments of the limb deviate it from one side to the other, until at length he has piloted the glass opposite his mouth, when he empties it at a gulp.
If we were to confine ourselves to this description by Sydenham, which so far as typical cases of the disease are concerned is perfectly accurate, differentiation between tic and chorea would not be a matter of any complexity. Unfortunately, however, the varieties of this form of chorea are legion, and in practice one constantly meets with conditions suggesting alike the gesticulations of chorea and the convulsive reactions of tic. Moreover, it has been pointed out by Oddo[177] that the fact of the habitual exaggeration of tic during the very years when chorea is liable to appear is calculated to confuse the issue.
He has attempted, however, to specify certain factors in the differential diagnosis. In the first instance, the form of the movements is of significance: there is no co-ordination in the muscular play of the choreic; it is amorphous, indefinable, and erratic, whereas the gestures of tic are purposive, and may be said to have a shape. One never sees in chorea a succession of similar movements, but though a patient be suffering from several tics, each of them is reproduced always in the same fashion. Unilaterality of distribution is more common in chorea than in tic; in other words, chorea, more or less, follows anatomical lines in the regions it affects, whereas the incidence of tic is physiological.
Both are arhythmic in their manifestation; nevertheless the repetition of tic is noteworthy for its regularity as compared with the changing mode and rate of the other. Noir emphasises the diagnostic value of its frequency, abruptness, and reiteration of identical movements. In a majority of cases the interference of the will is futile as far as chorea is concerned, while the victim to tic is usually capable of restraining his muscular activity at least for a space. The choreic exhibits his movements in public, but the tiqueur seeks the seclusion of his own room. The association of tic with obsessional ideas is frequently encountered, but there is no similar connection between obsessions and chorea. In addition, the myasthenia, pains, and alterations in the reflexes that often characterise chorea are awanting in the other affection.
It cannot be gainsaid, however, that the frequency with which atypical varieties of chorea occur is inimical to a ready diagnosis, and the onerous nature of the task is not lessened by the circumstance that many choreics are the offspring of neuropathic parents and reveal psychical anomalies comparable to those of the subjects of tic.
In a disease such as variable chorea, which has features in common both with tic and with chorea properly so called, the problem of diagnosis is still more complicated, though excellent hints for its solution have been furnished by Brissaud.[178]