by Oliver Sacks
Three years later, in 1974, I found that my fantasy had become a reality: that there had indeed come into being a Tourette's Syndrome Association. It had fifty members then: now, seven years
( A very similar situation happened with muscular dystrophy, which was never seen until Duchenne described it in the 1850s. By 1860, after his original description, many hundreds of cases had been recognised and described, so much so that Charcot said: 'How come that a disease so common, so widespread, and so recognisable at a glance- a disease which has doubtless always existed-how come that it is only recognised now? Why did we need M. Duchenne to open our eyes?'
later, it has a few thousand. This astounding increase must be ascribed to the efforts of the TSA itself, even though it consists only of patients, their relatives and physicians. The association has been endlessly resourceful in its attempts to make known (or, in the best sense, 'publicise') the Touretter's plight. It has aroused responsible interest and concern in place of the repugnance, or dismissal, which had so often been the Touretter's lot, and it has encouraged research of all kinds, from the physiological to the sociological: research into the biochemistry of the Tourettic brain; on genetic and other factors which may co-determine Tourette's; on the abnormally rapid and indiscriminate associations and reactions which characterise it. Instinctual and behavioural structures, of a developmentally and even phylogenetically primitive kind, have been revealed. There has been research on the body-language and grammar and linguistic structure of tics; there have been unexpected insights into the nature of cursing and joking (which are also characteristic of some other neurological disorders); and, not least, there have been studies of the interaction of Touretters with their family and others, and of the strange mishaps which may attend these relationships. The TSA's remarkably successful endeavours are an integral part of the history of Tourette's, and, as such, unprecedented: never before have patients led the way to understanding, become the active and enterprising agents of their own comprehension and cure.
What has emerged in these last ten years-largely under the aegis and stimulus of the TSA-is a clear confirmation of Gilles de la Tourette's intuition that this syndrome indeed has an organic neurological basis. The 'It' in Tourette's, like the 'It' in Parkinsonism and chorea, reflects what Pavlov called 'the blind force of the subcortex', a disturbance of those primitive parts of the brain which govern 'go' and 'drive'. In Parkinsonism, which affects motion but not action as such, the disturbance lies in the midbrain and its connections. In chorea-which is a chaos of fragmentary quasi-actions-the disorder lies in higher levels of the basal ganglia. In Tourette's, where there is excitement of the emotions and the passions, a disorder of the primal, instinctual bases of behaviour, the disturbance seems to lie in the very highest parts of the
'old brain': the thalamus, hypothalamus, limbic system and amygdala, where the basic affective and instinctual determinants of personality are lodged. Thus Tourette's-pathologically no less than clinically-constitutes a sort of 'missing link' between body and mind, and lies, so to speak, between chorea and mania. As in the rare, hyperkinetic forms of encephalitis lethargica, and in all post-encephalitic patients over-excited by L-Dopa, patients with Tourette's syndrome, or Tourettism' from any other cause (strokes, cerebral tumours, intoxications or infections), seem to have an excess of excitor transmitters in the brain, especially the transmitter dopamine. And as lethargic Parkinsonian patients need more dopamine to arouse them, as my post-encephalitic patients were 'awakened' by the dopamine-precursor L-Dopa, so frenetic and Tourettic patients must have had their dopamine lowered by a dopamine antagonist, such as the drug haloperidol (Haldol).
On the other hand, there is not just a surfeit of dopamine in the Touretter's brain, as there is not just a deficiency of it in the Parkinsonian brain. There are also much subtler and more widespread changes, as one would expect in a disorder which may alter personality: there are countless subtle paths of abnormality which differ from patient to patient, and from day to day in any one patient. Haldol can be an answer to Tourette's, but neither it nor any other drug can be the answer, any more than L-Dopa is the answer to Parkinsonism. Complementary to any purely medicinal, or medical, approach there must also be an 'existential' approach: in particular, a sensitive understanding of action, art and play as being in essence healthy and free, and thus antagonistic to crude drives and impulsions, to 'the blind force of the subcortex' from which these patients suffer. The motionless Parkinsonian can sing and dance, and when he does so is completely free from his Parkinsonism; and when the galvanised Touretter sings, plays or acts, he in turn is completely liberated from his Tourette's. Here the T vanquishes and reigns over the 'It'.
Between 1973 and his death in 1977, I enjoyed the privilege of corresponding with the great neuropsychologist A.R. Luria, and often sent him observations, and tapes, on Tourette's. In one of his last letters, he wrote to me: 'This is truly of a tremendous
importance. Any understanding of such a syndrome must vastly broaden our understanding of human nature in general … I know of no other syndrome of comparable interest.'
When I first saw Ray he was 24 years old, and almost incapacitated by multiple tics of extreme violence coming in volleys every few seconds. He had been subject to these since the age of four and severely stigmatised by the attention they aroused, though his high intelligence, his wit, his strength of character and sense of reality enabled him to pass successfully through school and college, and to be valued and loved by a few friends and his wife. Since leaving college, however, he had been fired from a dozen jobs-always because of tics, never for incompetence-was continually in crises of one sort and another, usually caused by his impatience, his pugnacity, and his coarse, brilliant 'chutzpah', and had found his marriage threatened by involuntary cries of 'Fuck!' 'Shit!', and so on, which would burst from him at times of sexual excitement. He was (like many Touretters) remarkably musical, and could scarcely have survived-emotionally or economically- had he not been a weekend jazz drummer of real virtuosity, famous for his sudden and wild extemporisations, which would arise from a tic or a compulsive hitting of a drum and would instantly be made the nucleus of a wild and wonderful improvisation, so that the 'sudden intruder' would be turned to brilliant advantage. His Tourette's was also of advantage in various games, especially ping-pong, at which he excelled, partly in consequence of his abnormal quickness of reflex and reaction, but especially, again, because of 'improvisations', 'very sudden, nervous, frivolous shots' (in his own words), which were so unexpected and startling as to be virtually unanswerable. The only time he was free from tics was in post-coital quiescence or in sleep; or when he swam or sang or worked, evenly and rhythmically, and found 'a kinetic melody', a play, which was tension-free, tic-free and free.
Under an ebullient, eruptive, clownish surface, he was a deeply serious man-and a man in despair. He had never heard of the TSA (which, indeed, scarcely existed at the time), nor had he heard of Haldol. He had diagnosed himself as having Tourette's after reading the article on 'Tics' in the Washington Post. When
I confirmed the diagnosis, and spoke of using Haldol, he was excited but cautious. I made a test of Haldol by injection, and he proved extraordinarily sensitive to it, becoming virtually tic-free for a period of two hours after I had administered no more than one-eighth of a milligram. After this auspicious trial, I started him on Haldol, prescribing a dose of a quarter of a milligram three times a day.
He came back, the following week, with a black eye and a broken nose and said: 'So much for your fucking Haldol.' Even this minute dose, he said, had thrown him off balance, interfered with his speed, his timing, his preternaturally quick reflexes. Like many Touretters, he was attracted to spinning things, and to revolving doors in particular, which he would dodge in and out of like lightning: he had lost this knack on the Haldol, had mistimed his movements, and had been bashed on the nose. Further, many of his tics, far from disappearing, had simply become slow, and enor
mously extended: he might get 'transfixed in mid-tic', as he put it, and find himself in almost catatonic postures (Ferenczi once called catatonia the opposite of tics-and suggested these be called 'cataclonia'). He presented a picture, even on this minute dose, of marked Parkinsonism, dystonia, catatonia and psychomotor 'block': in reaction which seemed inauspicious in the extreme, suggesting, not insensitivity, but such over-sensitivity, such pathological sensitivity, that perhaps he could only be thrown from one extreme to another-from acceleration and Tourettism to catatonia and Parkinsonism, with no possibility of any happy medium.
He was understandably discouraged by this experience-and this thought-and also by another thought which he now expressed. 'Suppose you could take away the tics,' he said. 'What would be left? I consist of tics-there is nothing else.' He seemed, at least jokingly, to have little sense of his identity except as a ticqueur: he called himself 'the ticcer of President's Broadway', and spoke of himself, in the third person, as 'witty ticcy Ray', adding that he was so prone to 'ticcy witticisms and witty ticcicisms' that he scarcely knew whether it was a gift or a curse. He said he could not imagine life without Tourette's, nor was he sure he would care for it.
I was strongly reminded, at this point, of what I had encountered in some of my post-encephalitic patients, who were inordinately sensitive to L-Dopa. I had nevertheless observed in their case that such extreme physiological sensitivities and instabilities might be transcended if it were possible for the patient to lead a rich and full life: that the 'existential' balance, or poise, of such a life might overcome a severe physiological imbalance. Feeling that Ray also had such possibilities in him, that, despite his own words, he was not incorrigibly centred on his own disease, in an exhibi-tionistic or narcissistic way, I suggested that we meet weekly for a period of three months. During this time we would try to imagine life without Tourette's; we would explore (if only in thought and feeling) how much life could offer, could offer him, without the perverse attractions and attentions of Tourette's; we would examine the role and economic importance of Tourette's to him, and how he might get on without these. We would explore all this for three months-and then make another trial of Haldol.
There followed three months of deep and patient exploration, in which (often against much resistance and spite and lack of faith in self and life) all sorts of healthy and human potentials came to light: potentials which had somehow survived twenty years of severe Tourette's and 'Touretty' life, hidden in the deepest and strongest core of the personality. This deep exploration was exciting and encouraging in itself and gave us, at least, a limited hope. What in fact happened exceeded all our expectations and showed itself to be no mere flash in the pan, but an enduring and permanent transformation of reactivity. For when I again tried Ray on Haldol, in the same minute dose as before, he now found himself tic-free, but without significant ill-effects-and he has remained this way for the past nine years.
The effects of Haldol, here, were 'miraculous'-but only became so when a miracle was allowed. Its initial effects were close to catastrophic: partly, no doubt, on a physiological basis; but also because any 'cure', or relinquishing of Tourette's, at this time would have been premature and economically impossible. Having had Tourette's since the age of four, Ray had no experience of any normal life: he was heavily dependent on his exotic disease
and, not unnaturally, employed and exploited it in various ways. He had not been ready to give up his Tourette's and (I cannot help thinking) might never have been ready without those three months of intense preparation, of tremendously hard and concentrated deep analysis and thought.
The past nine years, on the whole, have been happy ones for Ray-a liberation beyond any possible expectation. After twenty years of being confined by Tourette's, and compelled to this and that by its crude physiology, he enjoys a spaciousness and freedom he would never have thought possible (or, at most, during our analysis, only theoretically possible). His marriage is tender and stable-and he is now a father as well; he has many good friends, who love and value him as a person-and not simply as an accomplished Tourettic clown; he plays an important part in his local community; and he holds a responsible position at work. Yet problems remain: problems perhaps inseparable from having Tourette's-and Haldol.
During his working hours, and working week, Ray remains 'sober, solid, square' on Haldol-this is how he describes his 'Haldol self. He is slow and deliberate in his movements and judgments, with none of the impatience, the impetuosity, he showed before Haldol, but equally, none of the wild improvisations and inspirations. Even his dreams are different in quality: 'straight wish-fulfilment,' he says, 'with none of the elaborations, the extravaganzas, of Tourette's'. He is less sharp, less quick in repartee, no longer bubbling with witty tics or ticcy wit. He no longer enjoys or excels at ping-pong or other games; he no longer feels 'that urgent killer instinct, the instinct to win, to beat the other man'; he is less competitive, then, and also less playful; and he has lost the impulse, or the knack, of sudden 'frivolous' moves which take everyone by surprise. He has lost his obscenities, his coarse chutzpah, his spunk. He has come to feel, increasingly, that something is missing.
Most important, and disabling, because this was vital for him- as a means of both support and self-expression-he found that on Haldol he was musically 'dull', average, competent, but lacking energy, enthusiasm, extravagance and joy. He no longer had tics
or compulsive hitting of the drums-but he no longer had wild and creative surges.
As this pattern became clear to him, and after discussing it with me, Ray made a momentous decision: he would take Haldol 'dutifully' throughout the working week, but would take himself off it, and 'let fly', at weekends. This he has done for the past three years. So now there are two Rays-on and off Haldol. There is the sober citizen, the calm deliberator, from Monday to Friday; and there is 'witty ticcy Ray', frivolous, frenetic, inspired, at weekends. It is a strange situation, as Ray is the first to admit:
Having Tourette's is wild, like being drunk all the while. Being on Haldol is dull, makes one square and sober, and neither state is really free . . . You 'normals', who have the right transmitters in the right places at the right times in your brains, have all feelings, all styles, available all the time-gravity, levity, whatever is appropriate. We Touretters don't: we are forced into levity by our Tourette's and forced into gravity when we take Haldol. You are free, you have a natural balance: we must make the best of an artificial balance.
Ray does make the best of it, and has a full life, despite Tourette's, despite Haldol, despite the 'unfreedom' and the 'artifice', despite being deprived of that birthright of natural freedom which most of us enjoy. But he has been taught by his sickness and, in a way, he has transcended it. He would say, with Nietzsche: 'I have traversed many kinds of health, and keep traversing them . . . And as for sickness: are we not almost tempted to ask whether we could get along without it? Only great pain is the ultimate liberator of the spirit.' Paradoxically, Ray-deprived of natural, animal physiological health-has found a new health, a new freedom, through the vicissitudes he is subject to. He has achieved what Nietzsche liked to call 'The Great Health'-rare humour, valour, and resilience of spirit: despite being, or because he is, afflicted with Tourette's.
11
Cupid's Disease
A bright woman of ninety, Natasha K., recently came to our clinic. Soon after her eighty-eighth birthday, she said, she noticed 'a change'. What sort of change? we queried.
'Delightful!' she exclaimed. 'I thoroughly enjoyed it. I felt more energetic, more alive-I felt young once again. I took an interest in the young men. I started to feel, you might say, "frisky"-yes, frisky.'
'This was a problem?'
'No, not at first. I felt well, extremely well-why should I think anything was the matter?'
'And then?'
'My friends started to worry. First they said, "You look radiant- a new lease on life!", but then they started to
think it was not quite-appropriate. "You were always so shy," they said, "and now you're a flirt. You giggle, you tell jokes-at your age, is that right?" '
'And how did you feel?'
'I was taken aback. I'd been carried along, and it didn't occur to me to question what was happening. But then I did. I said to myself, "You're 89, Natasha, this has been going on for a year. You were always so temperate in feeling-and now this extravagance! You are an old woman, nearing the end. What could justify such a sudden euphoria?" And as soon as I thought of euphoria, things took on a new complexion . . . "You're sick, my dear," I said to myself. "You're feeling too well, you have to be ill!" '
'Ill? Emotionally? Mentally ill?'
'No, not emotionally-physically ill. It was something in my
body, my brain, that was making me high. And then I thought- goddam it, it's Cupid's Disease!'
'Cupid's Disease?' I echoed, blankly. I had never heard of the term before.
'Yes, Cupid's Disease-syphilis, you know. I was in a brothel in Salonika, nearly seventy years ago. I caught syphilis-lots of the girls had it-we called it Cupid's Disease. My husband saved me, took me out, had it treated. That was years before penicillin, of course. Could it have caught up with me after all these years?'
