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TIC AND PARAMYOCLONUS MULTIPLEX – TIC AND MYOCLONUS
It is not our intention here to seek to provide a differential diagnosis between tic and the various conditions usually classed as myoclonus, and that for two reasons: in the first place, we cannot admit that the latter form a distinct clinical or nosographical entity, since the term myoclonus seems simply to be an abbreviation for clonic muscular convulsion, and is a symptom rather than a clinical syndrome; secondly, the fact that the tics themselves have been incorporated with myoclonus involves the investigation of all the published cases with a view to their critical sifting. This task we have pursued for our own edification, but to enter on it here would serve no useful purpose, and we shall rest content with examining succinctly several recent cases described as myoclonus, in the hope that the prosecution of further research will introduce order into what is at present chaos.
Among the various forms of myoclonus there is one which presents a certain individuality, and which was described originally by Friedreich under the name of paramyoclonus multiplex.
This disturbance of motility supervenes, in patients with a neuropathic heredity, after some psychical accident such as a sudden fright or emotion, and consists in clonic muscular convulsions affecting the body generally, with the exception of the face. The contractions appear without obvious cause in one or in several muscles, are instantaneous, involuntary, and usually bilateral, but their most important feature is their inequality and irregularity. They may or may not effect displacement of the limbs; in any case they compose neither gesture nor gesticulation. Volition occasionally seems to have some transient inhibitory influence over them; they are exaggerated by cold and by emotion, and usually disappear in sleep.
It is obvious that this account of a typical case precludes the possibility of any confusion with tic, but the published cases are not always in conformity with it.
In 1892 Lemoine181 reported a case where the movements of paramyoclonus multiplex were accompanied with echolalia and psychical changes. Raymond quotes an instance of the disease being preceded by facial tic, and another associated with tremor and choreic movements.
D'Allocco182 has recorded twenty-four cases of differing forms of myoclonus, of which nineteen occurred as a family disease, in conjunction with stigmata of degeneration, epilepsy, and hysteria.
In a patient, aged twenty-six, suffering from general paralysis, Hermann183 noted the presence of abrupt, irregular, myoclonic twitches in the sternomastoids, recti abdominis, adductors, and in some of the toes and fingers, first on one side and then on the other, also in both legs, and subsequently in both arms, the face being unaffected.
Jancowicz considers diagnosis possible only in typical cases, and expresses the opinion that paramyoclonus is a syndrome common to many affections. Further, Schupfer makes the perfectly justifiable remark that under this denomination have been included cases of chorea, tic, hysteria, and rhythmic spasm; others have been secondary to organic disease of the cerebro-spinal axis, such as rolandic lesions, spinal muscular atrophy, chronic poliomyelitis, syringomyelia. Others, again, depend on one or other of the psychoses, others on infective conditions such as malaria, diphtheria, typhoid, or on intoxications such as uræmia, mercurialism, or lead poisoning. Only a few recorded cases cannot be attributed to any of the conditions enumerated above, hence Schupfer's objection to the promiscuous classification of them all as paramyoclonus multiplex is quite warranted, in the absence of a uniform etiology and symptomatology.
Schultze184 has suggested the term monoclonus for the tics, and he distinguishes monoclonus, polyclonus, and paraclonus. Embraced in the last of these is the paramyoclonus of Friedreich, which, according to Schultze, is usually unilateral, voluntary action diminishing the intensity of the involuntary movements, whereas the converse is the case in tic or monoclonus. Mixed forms are met with, however, and Schultze himself mentions one in which the movements were bilateral and increased with voluntary activity.
Heldenberg185 applies the term intermittent functional myoclonus to twitches occurring from time to time in antagonistic muscles during voluntary movement, twitches exaggerated by excitement and diminished with rest. They occur in combination with well-marked vasomotor phenomena.
The myokymia of Kny and Schultze is characterised by fibrillation, pain, hyperidrosis, and changes in electrical excitability.
A case which seemed to be a combination of paramyoclonus with Thomsen's disease has been reported by Hajos186 under the title myospasmia spinalis.
There cannot possibly be any hesitation in arriving at a diagnosis between tonic tic and Thomsen's disease, a condition consisting in slowness of relaxation of a strongly contracted muscle, and conceivably due to defective metabolism or organic change in muscular tissue.187
Examples such as the above, culled at random from an abundant medical literature, and variously entitled, will serve to demonstrate the protean nature of what the medical world is content to call myoclonus, and if from this collection of motor disorders we may hope to extricate the tics, there will remain still no inconsiderable labour of differentiation for the student.
TIC AND ATHETOSIS
The athetotic movements that may accompany hemiplegia are scarcely likely to be confused with those of tic, but difficulties may arise where the athetosis is double.
It has been universally remarked that athetotic movements of the face reproduce the expression of emotions, such as admiration, astonishment, sorrow, gaiety, etc. Of course the same may be said of the grimaces of chorea; the latter, however, are usually more abrupt and pass less readily one into the other. The gesticulations of athetosis are undulatory, so to speak, and their excess leads to deformities principally in the direction of forced extension. The musculature is often rigid, and the reflexes are increased in activity. Sometimes there is a considerable degree of mental disturbance.
Now, it is precisely in cases where mental deterioration is a prominent feature that "nervous movements" have been described resembling those of athetosis, for which the term pseudo-athetosis has been coined. Two examples may be quoted from Noir.
E. is a girl of eleven years. Her expression is grimacing; her tongue is often protruded, but never bitten; her head is regularly flexed or extended, or rotated rhythmically to left or right. The arms are moved spasmodically at shoulder and elbow, while the hands are the seat of athetotic movements. She walks curiously, throwing her feet out in advance without bending her knees. She has a silly smile, and her mouth almost invariably hangs open. On request she can keep her hands quite steady, but one observes at once the effort this entails in the sudden seriousness of her expression. The ordinary acts of every-day life are performed satisfactorily enough: she can dress and undress, use a knife and fork, thread a needle, sew, etc.
J. is eleven years old also. She puckers her lips, contracts her eyebrows, elevates her alæ nasi; at the same time she exhibits pseudo-athetotic movements of her fingers which are entirely under voluntary control.
The question may indeed be asked whether pseudo-athetosis and variable chorea are not really identical. Further, all sorts of combinations of athetosis and myotonia have been noted,188 but more light must be shed on the subject before any further classification can be attempted.
The following case has recently been published by Marina189:
A blacksmith, aged seventeen years, already treated three times for recurrent chorea, suffered from slow contractions of the shoulder muscles, involving the elevators and internal and external rotators successively, and accompanied by movements of the head and arm, and by twitches of the quadriceps. Nothing seemed to have any influence over these movements except sleep. The faradic excitability of the shoulder muscles was augmented, the galvanic excitability diminished. Application of the constant current to the head and back sufficed to effect a cure in three weeks.
Marina proposes the term athetotic myospasm for these incessant slow alternating contractions, impulsive myospasm being employed to signify convulsive movements of more than one muscular group, purposive yet irresistible, as in tic and chorea major. Simple myospasm consists of single twitches in individual muscles, recalling those produced by electrical excitation. If several muscles are implicated, the condition is one of multiple myospasm or myoclonus.
TICS AND TREMORS
All tremors, whether they occur during muscular repose or muscular activity, are distinguished by the relative restriction of their range and the regularity of their time. The tremors of paralysis agitans, disseminated sclerosis, senility, toxæmia, hysteria, ex-ophthalmic goitre, etc., are not liable to be mistaken for tic.
It is true, of course, that tremor is sometimes combined with choreiform or athetotic movements in patients with psychical stigmata.190 A proposal, too, has been made to unite hereditary and functional tremor and to describe them as a tremor neurosis.191
However simple be the diagnosis between tremor and tic, it is worth while to note in passing the etiology they may have in common. In a case recorded by van Gehuchten an intention tremor of the right arm co-existed with a tic of the right sternomastoid.
A sudden twitch of the whole body Letulle particularises as a "tic of starting," and Noir too thinks that a start of this nature may constitute a tic, but we are inclined to consider it a generalised reflex.
TICS AND PROFESSIONAL CRAMPS
We have already had occasion to enlarge on the distinguishing features of professional or occupation cramps, spasms, or neuroses. Writers, pianists, violinists, flutists, dressmakers, telegraphists, watchmakers, milkers, knackers, blacksmiths, shoemakers, tailors, dancers, embroiderers, barbers, etc., etc., are all liable to suffer from occupation cramps. In every case the condition is one of inability to perform the professional movement, and that alone.
Grasset proposes to separate intra-professional from post-professional spasm, the former consisting in the impossibility of making the necessary professional movements, the latter in the involuntary over-reproduction of the familiar act. Properly speaking, the post-professional spasm is a tic.
We need not do more than remind the reader of the close affinities we have already seen to exist between tics and professional cramps, and of the mental instability which both classes of patient present.
L. supplies an instance of variable hemichorea followed by writers' cramp and later by mental torticollis.
When L. was eight years old choreiform movements of the right arm began to appear, and soon rendered writing an impossibility. The disease continued for so long a time that one might not unreasonably expect to find considerable actual impairment of her caligraphy. As a matter of fact, it is scarcely affected: the patient can make her letters correctly, but after each letter she lifts her pen to allow her fingers to perform an abrupt movement, then she proceeds.
It cannot therefore be considered a true writers' cramp, but when she had learnt to write with the other hand it was not long ere that became the seat of a genuine cramp. The moment she attempted to make the pen move over the paper her grasp of it tightened and her fingers stiffened; her wrist would no longer answer her. To obviate the trouble she used a pencil, at first with complete success; but the cramp occurred afresh, and she gave up writing altogether. Prolonged holidaying, however, and respite from the exercise, had a salutary effect, and to-day there is no trace of former mischief.
CHAPTER XVII
PROGNOSIS
THE prognosis in a case of tic depends solely on the mental state of the patient. After what has been said of the rôle played by psychical disorders in the genesis of tic, we can readily comprehend the reason for this. The intensity and tenacity of any tic are determined by the degree of volitional imperfection to which its subject has sunk. He who can will can effect a cure; be it a simple tic, or be it a case of Gilles de la Tourette's disease, if he can struggle long and energetically, the tic's doom is sealed. Permanent cures have undoubtedly been obtained, but they are the exception. Left to himself, the victim to tic can seldom escape from it.
As far as life is concerned, tics are harmless, yet, according to Gilles de la Tourette, the prognosis is by no means always unchanging.
The establishment of a tic is never followed by its ultimate disappearance; it may be modified in all sorts of ways, yet the expert observer will not fail to mark its presence. A complete cure is not to be expected, for however much paroxysms may be alleviated and their frequency reduced, the morbid condition has become a sort of function, a product of the patient's mental constitution.
The statement may be taken to imply that no tic abandoned to itself ever vanishes completely, but the generalisation is inaccurate. Systematic treatment may lead not only to amelioration, but also to cure. Certain tics of children are by nature ephemeral, and disappear spontaneously, never to return. It is easy to understand how that may be. Psychical evolution and physical evolution alike are liable to singular variations. Hence the development of a tic in early life is no reason for despair, seeing that we are not justified in the assumption that the volitional debility which it proclaims is to persist. We must believe that volition may be reinforced, and we must further the attainment of this end by every means at our disposal. Negligence on our part is highly culpable.
Tics of childhood are curable: we draw attention to the fact afresh. Their spontaneous dissolution is not unknown, but parents must not consider the question merely one of time. They must impress on their children the sobering effect of good behaviour and decorum. Discipline of this kind may be a long and delicate task, but to condone indulgence in untimely movements, on the pretext that they are merely quaint, is a mistake fraught with the gravest consequences.
When a child holds its knife or fork incorrectly, or puts its elbows on the table, or its finger in its nose, we feel that the habit is displeasing; but how much more serious the outlook if the trick consists in biting the lips, or tossing the head, or blinking the eyes! The former is an offence against good taste; the latter is a tic in embryo.
It may be said, as a general rule, that the chances of spontaneous cure are in inverse proportion to the age of the patient and the duration of his tic.
Tics of adult life may also be cured, less often, it is true, than in the case of children. Oppenheim gives the history of a woman with a rebellious facial tic of twelve years' duration, which ceased on the occasion of a certain happy event in the family life. Of course one wants to know whether it ever returned, for many so-called cures are simply remissions.
T. had suffered from torticollis for a whole year, but on the eve of her son's marriage it stopped entirely for three days, and she deemed the cure permanent; it was not long, unfortunately, ere she underwent a relapse.
Brissaud192 quotes an instructive case of temporary cessation of tic. A patient afflicted with mental torticollis of three years' standing learned that his son had been injured and had been removed to hospital to undergo an operation. In an instant his torticollis disappeared, but a reassuring report from the surgeon a few days later was followed by a recrudescence of the condition.
It is true a hardened tiqueur may be relieved of his tic, but the potentiality remains. He is still at the mercy of the impulse to tic, should it arise. Cruchet gives the history of a young man who suffered in succession from convulsive movements of negation, facial tic, blinking of the eyes, abrupt yawning, and twitches of the shoulder – all in the space of two years. Each disappeared in its turn, independently of treatment, without leaving any trace behind. In cases of this description a new tic is ever imminent. The facility with which one tic replaces another is a matter of common observation. We have often had occasion to observe relapses, or partial relapses, in which an altogether new tic suddenly makes its appearance on the top of one which has either been improving or has actually been checked.
Apart, however, from obdurate forms of long standing, especially such as are accompanied by signs of grave mental defect, we maintain that the subjection of patients to appropriate treatment for an adequate period has a favourable influence on prognosis. The curability of tic was denied by Oddo, but he has recently seen fit to change his opinion, and to confine his pessimistic views to Gilles de la Tourette's disease.
The prognosis of the mental state of victims to tic is outwith our province: it is a topic long since handled by psychiatrists. We may ask, however, whether any particular prognostic import is to be attached to the tics themselves.
In cases of Gilles de la Tourette's disease the progressive unfolding of motor disorders suggests a corresponding evolution of psychical derangements which may end in dementia. Brissaud warns us that in cases of mental torticollis we must be on our guard against the apparition of some much more redoubtable affection than the torticollis, for that, sometimes, is an incident in the prodromal stage of general paralysis of the insane. Séglas has had a case of ærophagic tic which eventually became one of general paralysis, and a similar instance occurred in the practice of one of us.
Not long ago Dufour193 advanced the opinion that the occurrence of a motor syndrome consisting of the automatic movements of tic, in a case of delusional insanity, heightens the gravity of the prognosis as regards chronicity. It had been already remarked by Morel that such of the insane as contract tics usually degenerate into dements. Most of the contributors to the study of idiocy have noted the relation between the degree of intellectual debility and the extent of the automatic and rhythmical movements.
In this connection Joffroy has made some interesting statements.
Sometimes there is not merely co-existence, but an actual parallelism between the motor and the psychical disturbance. I have under observation at present a young woman suffering from attacks of agitation, with delusions and hallucinations, who has developed a facial tic in the course of her psychosis, and increase in the violence of the tic is associated with abrupt utterance of imperfectly formed syllables. During the last two months she has been having attacks in the evening, when the psychical troubles have become more intense, and simultaneously there has been aggravation of the tic and incessant emission of laryngeal sounds and syllables. Here then is a parallelism between the two groups of symptoms.
I am disposed, however, to believe that the usual prognosis given where motor and mental defects co-exist is too guarded. I have seen the catatonia of dementia præcox disappear spontaneously, in spite of its intensity and the unfavourable outlook prophesied by all who had seen the case.
In distinction, then, from the value of a knowledge of the patient's mental condition, we consider the motor reactions of tic of little prognostic significance.
CHAPTER XVIII
THE TREATMENT OF TICS
THE CURABILITY OF TICS
TICS are commonly held to be trivial affections of but passing medical interest, while in addition they have gained the notoriety of being peculiarly rebellious to treatment. Such undeserved criticism is at once too superficial and too severe. As far as life is concerned, the prognosis is favourable, but they often contrive, quite as forcibly as many graver diseases, to render existence intolerable. To neglect them or to consider them a priori incurable is entirely unwarranted. Some degree of amelioration is practically always attainable, and even complete cures may be effected.
It is an old doctrine this of the incurability of tic, but the sufferers have not always been left to their fate. Forecasts of methods of treatment likely to ensure success were made long ago. In the "Dictionary in Sixty Volumes" of the year 1821 will be found a definition of tic, a little out of date perhaps, but affording a glimpse of therapeutic possibilities: "The word tic is ordinarily employed to designate certain unnatural habits, bizarre attitudes, peculiar gestures, etc., whose correction demands a painstaking perseverance that is not always sufficient to procure the desired result."
Trousseau later introduced an element of precision into current therapeutic measures by the application of a sort of gymnastic exercise to the muscles involved. He declared his opinion, however, that the arrest of one tic would soon be followed by the development of a second, which would in turn give place to a third, and so on; for the disease was essentially chronic, and in a sense formed part of the constitution of its subject. Subsequent observation has frequently borne witness to the truth of this remark, though the expression is too absolute.
For the majority of the older writers, nevertheless, the incurability of tic was axiomatic.
Pujol held non-dolorous facial tic to be most intractable. In the hands of Duchenne of Boulogne faradisation of the muscles was followed by only transient improvement. Axenfeld considered idiopathic facial convulsions hopeless from the point of view of treatment.
It has been remarked already that many of the earlier observers failed to discriminate between tic and spasm. In the article "Face" in the Encyclopædic Dictionary, for instance, Troisier includes every sort of facial movement under the term "convulsive tic," among them reflex spasms from dental caries or buccal ulceration, and muscular contractions occasioned by peripheral or nuclear irritation. His opinions as to the curability or otherwise of these movements are sufficiently dogmatic: "Convulsive tic is not a serious condition, yet it is in a majority of cases incurable and as a consequence most distressing. One can hope for success only if the tic is of reflex origin, where extraction of a tooth, or local treatment of an ulcer, or resection of part of the trigeminal nerve may be indicated."
Here the confusion is obvious.
Gilles de la Tourette's description of the disease known as convulsive tic accompanied with echolalia and coprolalia is couched in equally pessimistic terms.
"It is no menace to existence, and the patient may well attain a ripe old age, but in revenge he stands very little chance of escaping from it. A radical cure is yet to be found. Isolation, hydrotherapeutics, electricity, and constitutional treatment cannot do much more than retard its evolution."
In Guinon's article on convulsive tic in the Encyclopædic Dictionary of the Medical Sciences of 1887 thirty pages were devoted to description and the following few lines to treatment:
"This chapter will of necessity be brief… In presence of this affection the physician is unfortunately helpless. During exacerbations any nerve sedative may be tried. In severe cases or if the symptoms become aggravated, the sole treatment likely to be accompanied by improvement, scarcely by success, is a combination of hydrotherapeutics with isolation."
Nor is Charcot much more encouraging194:
We cannot say that cure is certain, but we may count on longer or shorter intervals of arrest, either spontaneous or as a sequel to the employment of serviceable measures such as hydrotherapy or rational gymnastics.
It should be said that the cases which Charcot, Tourette, and Guinon had more especially in mind were of a graver nature, such as the disease of generalised convulsive tics with echolalia and coprolalia, and peculiarly resistant to treatment. Patients suffering from these forms of tic present in the most advanced degree psychical instability and volitional fickleness, and betray an irresistible tendency to impulsion and obsession, calculated to render the institution of any methodical treatment futile. In their case patience and perseverance may be rewarded, but they never consent to undergo for a sufficiently long period the discipline indispensable for their cure.
Fortunately, these severer varieties are exceptional. The vast majority of cases are certainly more amenable to modern therapeutic measures, and the results obtained so far place the disease in a much more favourable light. Letulle had already remarked, in 1883, that the most tenacious of co-ordinated tics might be amended, mitigated, and even wholly inhibited.
