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NEUROLOGICAL CLINIC _____

STUDY ON A NERVOUS AFFECTION CHARACTERIZED BY UNCOORDINATED MOVEMENT ACCOMPANIED BY ECHOLALIA AND COPROLALIA (J UMPING, L ATAH , M YRIACHIT );

By GILLES DE LA TOURETTE.

Bouteille, who, at eighty years of age, benefited from experience acquired from long years of practice, wrote his Treaty of Chorea. He exclaimed in its foreword: "Everything about this disease is extraordinary: its name is ridiculous, its symptoms peculiar, its character ambiguous, its cause unknown, its treatment problematic. Serious authors doubted its existence, others believed it simulated, some considered it supernatural". It is certain, that at the time Bouteille published his justly popular book, the nervous affections in which uncoordinated movement was the prevalent symptom, very wrongly differentiated the ones from the others and for the majority included under the generic term of Chrorea (dance), which, to be strictly accurate, hardly prejudges the nature of the disease. But, since 1818, great progress was accomplished and chorea saw evermore decreasing its pathological territory while attending the improvement of the nervous nosography. It is a remnant the old French author had a sense of, when he said : "I give the name of pseudo-choreas or false choreas for various nervous affections, spasmodic, convulsives, hysterical, etc., which do not present the characteristic symptoms of true chorea, and have resemblance to it only by involuntary agitations of the various parts of the body and by convulsive grimaces of the face". If, following the work of Bouteille, one had agreed to hold account of the last part of this old definition, it is extremely probable that the breakdown of the group of choreas would be still much more complete than it is it today. It is this embrace/encircling? which the disease would have left that we will study.

Its history is short, that much of it that exists, because we let us not know any description with true sense of the word; it can be divided into two periods, both of quite minor importance.

In 1825, Itard published an observation which was completely reported by Roth in 1850, and by Sandras in 1851. This observation which one will find at the head of those that we collected is extremely conclusive and all the more interesting as the patient who is the object of it lived until 1884, and was seen by Mr. professor Charcot, who controlled the retrospective diagnosis. Not one of three preceding authors did not think of bringing it closer similar cases, not more than to differentiate it from the other affections choreiformes. And even, Sandras, the last of the three, who regards this observation as a case of chorea, muddle still the question by bringing it closer a case of right and hemichorea aphasia with hemiplegia, since he adds: "I observed myself an extremely remarkable fact under more than one report of which it appears very useful to to me to consign the details here".

It is necessary to arrive at Trousseau, to find for the first time a mention, rather even as a very brief description, affection which we will describe; and still in places his chapter entitled: Various species of chorea. That he is allowed to us as of now quoting this passage of the medical Private clinic: "These tics are in some cases accompanied by a cry, of a very characteristic shout more or less noisy. And to this matter, I will recall the fact that I have well times told of one my former comrades of college which I had recognized, to twenty years of interval, while it went behind me, with the species of barking that I had intended to him to push into the past then that we made unit our studies. This cry this yap, this shout, true chorea laryngées or diaphragmatic can constitute all the tic. It is, not only one shout, a strange cry, it is still a peculiar tendency to always repeat the same word, same exclamation and even the individual aloud utters words which he would like to retain. These tics are very often hereditary".

Choreas laryngées, choreas diaphragmatic, misinterpretation comes too close a description which, we will see it, is exact on many points.

The observations of Trousseau passed unperceived; they however were reproduced without comments, in 1879, by Handfield Jones ; but they did not inspire any research.

The second period is of date very recent and more fertile than the first, although the sanction of an overall work is missing with the scattered observations that it includes/understands. The authors that one meets there, either that they had in their possession only insufficient documents, or which they did not even have quality - not being doctors - to conclude, gave facts, but did not constitute a disease.

The first of them, Beard (of New York), at neurological Association, in 1880, a report entitled presented: "jumpers of Maine", in which he brought back observations concerning of the individuals who obviously were reached affection that we will describe. The author announced moreover, in his communication, that there were similar cases among the Malayan one.

It is what taught us in 1883, Mr. O Brien, which though not being a doctor, saw and well observed the facts well that he reports in the Journal of the Asian Society.

Lastly, in March 1884, Medicina contemporanea, a note of Mr. Hammond published (of New York), in whom this author reports, according to the testimony of officers of the American fleet, a conclusive though incomplete observation of this affection.

We have ourselves, on the councils of our Master, Mr. professor Charcot, analysed work of three preceding authors and shown, in July 1884, that Jumping of Maine, Latah of Malaysia, and Myriachit observed by the American officers in Siberia were one and the same affection. We gave at the same time the summary of a case-type which then observe we in Salpêtrière; to this one we will add of them today seven others, which, joined to those observed by the preceding authors, will provide us a solid base for the study of a disease which one could bring back observations, but that one did not think yet of describing.

O BSERVATION I.

First part: Itard: Memory on some involuntary functions of the apparatuses of the locomotion, gripping and the voice. (Arch. gen. of medicine 1925, T VIII, p. 403.) - Second new part. (Charcot).

Mrs. D..., currently twenty-six years old, was, at the age of seven taken with convulsives contractions in the muscles of the hands and the arms, which appeared especially in the moments when this child was engaged in writing, abruptly drew aside her hand of the characters that it traced. After this variation, the movements of her hand became regular again and subjected to the will, until another sudden start stopped again the work of the hand. One initially saw in that only small turns of high spirits or mischief, which, being repeated more and more, became subjects of reprimand and punishment. But soon, one acquired the certainty which these movements were involuntary and convulsions and one saw taking part in it the muscles of the shoulders, the neck and the face. It resulted from it from the distortions and the extraordinary grimaces. The disease still made progress, and the spasm being propagated with the bodies of the voice and the word, this young person made hear odd cries and words which did not have any direction, but all that without is delirious, without any disorder of mental faculties. Months and years were passed in this state of affairs to which one opposed only weak remedies in the hope of the favourable changes that puberty could bring. This hope was completely disappointed. Miss of... was then sent in Switzerland near a doctor who had especially devoted himself to the treatment of the nervous diseases that it especially fought by whey baths.

Either by the effect of these baths, or by the happy influence of the stay and the life of the mountains, the disease was dissipated almost completely; and when, at the end of one year, this young lady left Switzerland, it did not return calm, brilliant of freshness, and prone only to some visible, but not very frequent tugging of the muscles of the mouth and the neck. She was married at that time. But, the marriage, instead of consolidating and to complete its cure as one had hoped for, reproduced its disease rather quickly. It is true that Mrs. of D..., not having had children was deprived of the favourable chances which could have offered to him the physical and moral revolution usually produced by maternity. At all events, this convulsive affection which, if one has excluded of them eighteen or twenty months of respite hard for eighteen years, does not appear to have to worn down by time and seems on the contrary to make new progress. Here which is its current state: the spasmodic contractions continual, non-successive, and are separated by short intervals from a few minutes; sometimes the rest is longer, other times shorter and it occur about it even often two or three which follow one another without remission. They affect especially the muscles of the face and those which are used for the emission and the articulation of the sounds. Among the continual and disordered movements that these morbid contractions bring, those printed with the bodies of the voice and the word are the only worthy ones of all our attention like presenting a phenomenon of rarest and constituting an inconvenience of most unpleasant which deprives the person which is reached by it of all softnesses of the company, because the disorder that it door is there because of the pleasure that it takes there. Thus, in the medium of a conversation which interests her most highly, suddenly, without being able to prevent oneself some, she stops what she says or what she listens to by odd cries and words even more extraordinary, and which make a deplorable contrast with her appearance and her genteel manners; the words are for the majority of the coarse swearwords, the nasty names and, which is not less embarrassing for it and the listeners, the expression very strong of one swearing or an opinion unfavourable to some of the people present of the company. The explanation which she gives of the preference of her language, in its variations, appears to grant to these improper expressions is more plausible. It is that, more they appear to her revolting by their coarseness, more she is tormented fear to utter them and that this concern is precisely what as puts them to her at the end of the language she cannot any more control it. Finally, the general state of her health appears to feel form this long convulsive affection, as prove it an increasing slimming and the paleness of the complexion, although the digestive functions notably did not suffer.

The influence of the disease on the state of moral is even more appreciably marked and one observes here as in all the excessively prolonged neuroses of this kind, a great mobility in the ideas and a lightness of spirit and character which belong only to the extreme youth and which resist the revolutions of the age.

Second part. - Mr. professor Charcot saw on several occasions this patient who, until a advanced age, had preserved his muscular uncoordination and pronounced in spite of it, even in the public places, of the words obscenes like Mr. Charcot in was witness.

In last analysis, the political newspapers announced her death which has occurred about July or August 1884, and some offered to their readers a treat of obscene words which she pronounced and which were in particular "shit and fucking pig".

O BSERVATION II (Personal).

(the first part was collected by Mr. P. Marie, senior registrar of Mr. Charcot.)

S. J..., born on July 1, 1864 in Le Havre; accounting employee in an office of the Highways Departments. Father alive; the mother has tuberculous antecedents. She lost a girl of pulmonary tuberculosis; four other children died young; three are still alive. It is impossible to take note of nervous, syphilitic or alcoholic antecedents in the parents who are small shopkeepers steady and appearing to enjoy a modest affluence.

S... was never sick during his childhood; he was very intelligent and gained all the prizes of his class. The last year he had the prize of honour; at this time (July 1880) his teacher noticed that the right shoulder and arm were from time to time raised by small abrupt and involuntary movements. Little time afterwards, he entered an office and could write in spite of these movements until January-February 1881, time to which he had to stop any work. The movements tended to spread; they had invaded the right leg and it was only about June (1881) that the left side was taken in its turn. About January of this same year another order of phenomena had appeared: involuntarily and jointly with these movement, S... let out a light initially inarticulate cry, kind of hem! and of ouah! emitted to be heard perfectly rather high by the surrounding people.

It was to consult Mr. Dr. Gilbert (Le Havre), who subjected him to a tonic regime and hydrotherapy, regime that the patient followed very irregularly, of its which occurred even, and whose it did not withdraw any improvement. Throughout all year 1881 and until October 1882 time to which the patient entered to the hospice of Salpètrière (Bourvier room, service of Mr. professor Charcot), the movements nothing but did increase just as particular phonetic phenomena took shape more and more.

At that time he presented the following state: seventeen years, large, rather thin, enjoying an excellent general health, eating well, sleeping well, not suffering; soft and timid character, pulse 82: light basic feeble breath, without lesions, the patient not having a remainder never have rheumatisms. Without incentive appreciable, S. carries out a series of very particular, located and generalized movements, sometimes occurring only one side of the body, sometimes on the two sides with time. These movements are fast: with the head they occupy the muscles of the face, epicranium, of the outer ear, corner of the mouth which is quickly drawn in top and outwards; the patient carries out a series of grimaces to which neither the eyes nor the language take any share. With these grimaces generally very fast movements of swinging and rise in the arms join, just as simultaneously the legs, especially his right, are bent and are rectified alternatively, the right foot coming to strike the ground with force. At the time of the peak of this whole of odd movements S... lets out a raucous and inarticulate cry. These phenomena reproduce sometimes very frequently; an emotion brings back them: the sleep, which is very good, the fact of ceasing completely. It never occurs a day nor even half an hour without them not being shown: they bother the food only in what, glass or the fork, if they are seized at the time of ?unesecousse?, is sometimes projected abruptly apart from their primitive destination.

Little time after its entry at the hospital and thanks to a followed examination and more deepened, one realized soon completely characteristic phenomenon. The cry which S pushed... took in certain circumstances a very special character; indeed, although the ouah! ouah! always existed, the patient now had the faithful echo of the words and even of the short sentences which it intended to pronounce: "Here is Mr. Charcot" - "Charcot" repeated it immediately, by exaggerating its usual movements. And it added: "Ah! Here Is Mr. Charcot, Mr. Charcot, Mr. Charcot "; the whole accompanied by grimaces and distorsions. Apart from these kinds of nominal suggestions so to speak, translated aloud and without the patient being able to prevent himself some, there were idéatives suggestions which he translated in the same way. One day S... intended the director of the hospital to say to a caretaker that she did not take care sufficiently of her duties: at once, while making distorsions, it repeated high: "Ah, the cow, does not do her duties, her duties..."

We insist on this lewd naming, because at S... this character lewd of word or the sentence which accompany the gesture is constant when the patient was not struck by a word, by a fact that he can translate by the language, he often accompanies his distorsions by the word shit; and that, in front of any audience. In the same way he expresses an ordinary idea in a way lewd: Mr. X returns in the room: "Ah! here it is this old C... of father X., this old C...!"- the whole pronounced quickly and in front of a person for whom it must have and it has the largest respect. An injury returns in the room: "Ah! the cow: I ... must have, etc.": two or three short sentences, and one cannot any more lewd, known as with an exaggerated accompaniment of tics and distorsions, stirring up the arms, being raised and dropping on several occasions, raising the shoulders, reversing the head behind and on side. These words are so involuntary that he is able to him to pronounce them in front of his mother that he likes much and that he was one day out obliged from to go away from a restaurant where he scandalized the cusomers by his marked lustful words with force. Let us add that the gestures did not present anything lewd. S... was still forced to imitate by his gestures, just as he imitated by repeating the words as he intended to say, but in circumstances more limited perhaps. S... was in the court of the infirmary of Salpêtrière: X came towards him: "Ah! ah! X.., X., shit, shit ", said S.. while raising the arms in the air and lowering them alternatively and raising at the same time rather high the right leg. Then the movements stopped or even if they had not been shown, X... and well of others which were made a play of he repeated the ordinary gestures and of the words, then the force of imitation was such at this one that while raising the arms and the right leg he stumbled and fell by ground; however without hurting himself.

About May-June, S... made with the hospital's knowledge of a person with whom it entered in regular correspondence. From this moment, the state that we described worsened in front of the obstacles which one put at this liason. Previously, sometimes he had enough empire on himself not to pronounce his lewd usual words: consequently, gestures and words were made and pronounced with an unaccustomed showiness and frequency.

At that moment and in front of the inefficiency of a treatment very irregularly followed, the patient was returned to his family on July 1, 1883. He returned in Le Havre in this state, testing a sorrow such as on several occasions he thought of leaving the paternal house to return to Paris. Until the end of 1883, no improvement was shown: little by little however about January 1884 a certain sedation occurred; imperceptibly, these phenomena carried to their peak were amended and here the state in which we find S.. on July 15, 1884, in Le Havre in his family, state of which we supplement the talk by means of the particular documents that his father and his mother provide us in his absence.

S... imperceptibly lost the practice to pronounce lewd words, but he is still ecolalic; if one calls him in the street, he fails only seldom to repeat his own name. The great disordered movements also disappeared: there are nothing any more but limited movements of the upper limb right as a whole: the orbicular ones of the two eyes also return in fast action, finally, which had not been noted during his stay at the hospital, the language is from time to time projected with the outside and. All the emotions act very highly on him: he jumps, says his mother, as he is called, and, although the improvement is considerable he could not still take again his employment. The general state is excellent; the clear intelligence and lives: S... contracted some practices of idleness and strolls while waiting for his final cure.

Since he is in Le Havre he made any treatment and allots his improvement to the great sorrow which he tested during several months: he speaks yet about Salpêtrière only in very good terms and very affectionate for the people who gave him care and for which, says us him with sincerity, he will preserve the largest recognition. there exists at his place no disorder of sensitivity: the heart is healthy; the visual field is normal.