by Oliver Sacks
'Aye, George is always the joker!' William quipped, apparently ignoring, or indifferent to, Bob's comment, and went on blathering of George in his excited, dead way, insensitive to truth, to reality, to propriety, to everything-insensitive too to the manifest distress of the living brother before him.
It was this which convinced me, above everything, that there was some ultimate and total loss of inner reality, of feeling and meaning, of soul, in William-and led me to ask the Sisters, as I had asked them of Jimmie G. 'Do you think William has a soul? Or has he been pithed, scooped-out, de-souled, by disease?'
This time, however, they looked worried by my question, as if something of the sort were already in their minds: they could not say 'Judge for yourself. See Willie in Chapel', because his wisecracking, his confabulations continued even there. There is an utter pathos, a sad sense of lostness, with Jimmie G. which one does not feel, or feel directly, with the effervescent Mr Thompson.
Jimmie has moods, and a sort of brooding (or, at least, yearning) sadness, a depth, a soul, which does not seem to be present in Mr Thompson. Doubtless, as the Sisters said, he had a soul, an immortal soul, in the theological sense; could be seen, and loved, as an individual by the Almighty; but, they agreed, something very disquieting had happened to him, to his spirit, his character, in the ordinary, human sense.
It is because Jimmie is 'lost' that he can be redeemed or found, at least for a while, in the mode of a genuine emotional relation. Jimmie is in despair, a quiet despair (to use or adapt Kierkegaard's term), and therefore he has the possibility of salvation, of touching base, the ground of reality, the feeling and meaning he has lost, but still recognises, still yearns for . . .
But for William-with his brilliant, brassy surface, the unending joke which he substitutes for the world (which if it covers over a desperation, is a desperation he does not feel); for William with his manifest indifference to relation and reality caught in an unending verbosity, there may be nothing 'redeeming' at all-his confabulations, his apparitions, his frantic search for meanings, being the ultimate barrier to any meaning.
Paradoxically, then, William's great gift-for confabulation- which has been called out to leap continually over the ever-opening abyss of amnesia-William's great gift is also his damnation. If only he could be quiet, one feels, for an instant; if only he could stop the ceaseless chatter and jabber; if only he could relinquish the deceiving surface of illusions-then (ah then!) reality might seep in; something genuine, something deep, something true, something felt, could enter his soul.
For it is not memory which is the final, 'existential' casualty here (although his memory is wholly devastated); it is not memory only which has been so altered in him, but some ultimate capacity for feeling which is gone; and this is the sense in which he is 'de-souled'.
Luria speaks of such indifference as 'equalisation'-and sometimes seems to see it as the ultimate pathology, the final destroyer of any world, any self. It exerted, I think, a horrified fascination on him, as well as constituting an ultimate therapeutic challenge. He was drawn back to this theme again and again-sometimes in
relation to Korsakov's and memory, as in The Neuropsychology of Memory, more often in relation to frontal-lobe syndromes, especially in Human Brain and Psychological Processes, which contains several full-length case-histories of such patients, fully comparable in their terrible coherence and impact to 'the man with a shattered world'-comparable, and, in a way, more terrible still, because they depict patients who do not realise that anything has befallen them, patients who have lost their own reality, without knowing it, patients who may not suffer, but be the most God-forsaken of all. Zazetsky (in The Man with a Shattered World) is constantly described as a fighter, always (even passionately) conscious of his state, and always fighting 'with the tenacity of the damned' to recover the use of his damaged brain. But William (like Luria's frontal-lobe patients-see next chapter) is so damned he does not know he is damned, for it is not just a faculty, or some faculties, which are damaged, but the very citadel, the self, the soul itself. William is 'lost', in this sense, far more than Jimmie-for all his brio; one never feels, or rarely feels, that there is a person remaining, whereas in Jimmie there is plainly a real, moral being, even if disconnected most of the time. In Jimmie, at least, re-connection is possible-the therapeutic challenge can be summed up as 'Only connect'.
Our efforts to 're-connect' William all fail-even increase his confabulatory pressure. But when we abdicate our efforts, and let him be, he sometimes wanders out into the quiet and undemanding garden which surrounds the Home, and there, in its quietness, he recovers his own quiet. The presence of others, other people, excite and rattle him, force him into an endless, frenzied, social chatter, a veritable delirium of identity-making and -seeking; the presence of plants, a quiet garden, the non-human order, making no social or human demands upon him, allow this identity-delirium to relax, to subside; and by their quiet, non-human self-sufficiency and completeness allow him a rare quietness and self-sufficiency of his own, by offering (beneath, or beyond, all merely human identities and relations) a deep wordless communion with Nature itself, and with this the restored sense of being in the world, being real.
13
Yes, Father-Sister
Mrs B., a former research chemist, had presented with a rapid personality change, becoming 'funny' (facetious, given to wisecracks and puns), impulsive-and 'superficial' ('You feel she doesn't care about you,' one of her friends said. 'She no longer seems to care about anything at all.') At first it was thought that she might be hypomanic, but she turned out to have a cerebral tumour. At craniotomy there was found, not a meningioma as had been hoped, but a huge carcinoma involving the orbitofrontal aspects of both frontal lobes.
When I saw her, she seemed high-spirited, volatile-'a riot' (the nurses called her)-full of quips and cracks, often clever and funny.
'Yes, Father,' she said to me on one occasion.
'Yes, Sister,' on another.
'Yes, Doctor,' on a third.
She seemed to use the terms interchangeably.
'What am I?' I asked, stung, after a while.
'I see your face, your beard,' she said, 'I think of an Archimandrite Priest. I see your white uniform-I think of the Sisters. I see your stethoscope-I think of a doctor.'
'You don't look at all of me?'
'No, I don't look at all of you.'
'You realise the difference between a father, a sister, a doctor?'
'I know the difference, but it means nothing to me. Father, sister, doctor-what's the big deal?'
Thereafter, teasingly, she would say: 'Yes, father-sister. Yes, sister-doctor', and other combinations.
Testing left-right discrimination was oddly difficult, because she
said left or right indifferently (though there was not, in reaction, any confusion of the two, as when there is a lateralising defect of perception or attention). When I drew her attention to this, she said: 'Left/right. Right/left. Why the fuss? What's the difference?'
'Is there a difference?' I asked.
'Of course,' she said, with a chemist's precision. 'You could call them enantiomorphs of each other. But they mean nothing to me. They're no different for me. Hands . . . Doctors . . . Sisters . . . ' she added, seeing my puzzlement. 'Don't you understand? They mean nothing-nothing to me. Nothing means anything … at least to me.'
'And . . . this meaning nothing . . . ' I hesitated, afraid to go on. 'This meaninglessness . . . does this bother you? Does this mean anything to you?'
'Nothing at all,' she said promptly, with a bright smile, in the tone of one who makes a joke, wins an argument, wins at poker.
Was this denial? Was this a brave show? Was this the 'cover' of some unbearable emotion? Her face bore no deeper expression whatever. Her world had been voided of feeling and meaning. Nothing any longer felt 'real' (or 'unreal'). Everything was now 'equivalent' or 'equal'-the whole world
reduced to a facetious insignificance.
I found this somewhat shocking-her friends and family did too-but she herself, though not without insight, was uncaring, indifferent, even with a sort of funny-dreadful nonchalance or levity.
Mrs B., though acute and intelligent, was somehow not present- 'de-souled'-as a person. I was reminded of William Thompson (and also of Dr P.). This is the effect of the 'equalisation' described by Luria which we saw in the preceding chapter and will also see in the next.
Postscript
The sort of facetious indifference and 'equalisation' shown by this patient is not uncommon-German neurologists call it Witzel-sucht ('joking disease'), and it was recognised as a fundamental
form of nervous 'dissolution' by Hughlings Jackson a century ago. It is not uncommon, whereas insight is-and the latter, perhaps mercifully, is lost as the 'dissolution' progresses. I see many cases a year with similar phenomenology but the most varied etiologies. Occasionally I am not sure, at first, if the patient is just 'being funny', clowning around, or schizophrenic. Thus, almost at random, I find the following in my notes on a patient with cerebral multiple sclerosis, whom I saw (but whose case I could not follow up) in 1981:
She speaks very quickly, impulsively, and (it seems) indifferently … so that the important and the trivial, the true and the false, the serious and the joking, are poured out in a rapid, unselec-tive, half-confabulatory stream . . . She may contradict herself completely within a few seconds . . . will say she loves music, she doesn't, she has a broken hip, she hasn't . . .
I concluded my observation on a note of uncertainty:
How much is cryptamnesia-confabulation, how much frontal-lobe indifference-equalisation, how much some strange schizophrenic disintegration and shattering-flattening?
Of all forms of 'schizophrenia' the 'silly-happy', the so-called 'hebephrenic', most resembles the organic amnestic and frontal lobe syndromes. They are the most malignant, and the least imaginable-and no one returns from such states to tell us what they were like.
In all these states-'funny' and often ingenious as they appear- the world is taken apart, undermined, reduced to anarchy and chaos. There ceases to be any 'centre' to the mind, though its formal intellectual powers may be perfectly preserved. The end point of such states is an unfathomable 'silliness', an abyss of superficiality, in which all is ungrounded and afloat and comes apart. Luria once spoke of the mind as reduced, in such states, to 'mere Brownian movement'. I share the sort of horror he clearly felt about them (though this incites, rather than impedes, their
accurate description). They make me think, first, of Borges' 'Funes', and his remark, 'My memory, Sir, is like a garbage-heap', and finally, of the Dunciad, the vision of a world reduced to Pure Silliness-Silliness as being the End of the World:
Thy hand, great Anarch, lets the curtain fall; And Universal Darkness buries All.
14
The Possessed
In Witty Ticcy Ray (Chapter Ten), I described a relatively mild form of Tourette's syndrome, but hinted that there were severer forms 'of quite terrible grotesqueness and violence'. I suggested that some people could accommodate Tourette's within a commodious personality, while others 'might indeed be "possessed", and scarcely able to achieve real identity amid the tremendous pressure and chaos of Tourettic impulses'.
Tourette himself, and many of the older clinicians, used to recognise a malignant form of Tourette's, which might disintegrate the personality, and lead to a bizarre, phantasmagoric, pantomimic and often impersonatory form of 'psychosis' or frenzy. This form of Tourette's-'super-Tourette's'-is quite rare, perhaps fifty times rarer than ordinary Tourette's syndrome, and it may be qualitatively different, as well as far more intense than any of the ordinary forms of the disorder. This 'Tourette psychosis', this singular identity-frenzy, is quite different from ordinary psychosis, because of its underlying, and unique, physiology and phenomenology. None the less, it has affinities, on the one hand, to the frenzied motor psychoses sometimes induced by L-Dopa and, on the other, to the confabulatory frenzies of Korsakov's psychosis (see above, Chapter Twelve). And like these it can almost overwhelm the person.
The day after I saw Ray, my first Touretter, my eyes and mind opened, as I mentioned earlier, when, in the streets of New York, I saw no less than three Touretters-all as characteristic as Ray, though more florid. It was a day of visions for the neurological eye. In swift vignettes I witnessed what it might mean to have
Tourette's syndrome of ultimate severity, not only tics and convulsions of movement, but tics and convulsions of perception, imagination, the passions-of the entire personality.
Ray himself had shown what might happen in the street. But it is not enough to be told. You must see for yourself. And a doctor's clinic or ward is not always the best place for observing disease-at least, not for observing a disorder which, if organic in origin, is expressed in impulse, imitation, impersonation, reaction, interaction, raised to an extreme and almost incredible degree. The clinic, the laboratory, the ward are all designed to restrain and focus behaviour, if not indeed to exclude it altogether. They are for a systematic and scientific neurology, reduced to fixed tests and tasks, not for an open, naturalistic neurology. For this one must see the patient unselfconscious, unobserved, in the real world, wholly given over to the spur and play of every impulse, and one must oneself, the observer, be unobserved. What could be better, for this purpose, than a street in New York-an anonymous public street in a vast city-where the subject of extravagant, impulsive disorders can enjoy and exhibit to the full the monstrous liberty, or slavery, of their condition.
'Street-neurology', indeed, has respectable antecedents. James Parkinson, as inveterate a walker of the streets of London as Charles Dickens was to be, forty years later, delineated the disease that bears his name, not in his office, but in the teeming streets of London. Parkinsonism, indeed, cannot be fully seen, comprehended, in the clinic; it requires an open, complexly interactional space for the full revelation of its peculiar character (beautifully shown in Jonathan Miller's film Ivan). Parkinsonism has to be seen, to be fully comprehended, in the world, and if this is true of Parkinsonism, how much truer must it be of Tourette's. Indeed an extraordinary description from within of an imitative and antic ticqueur in the streets of Paris is given in 'Les confidences d'un ticqueur' which prefaces Meige and Feindel's great book Tics (1901), and a vignette of a manneristic ticqueur, also in the streets of Paris, is provided by the poet Rilke in The Notebook of Malte Laurids Brigge. Thus it was not just seeing Ray in my office but what I
saw the next day that was such a revelation to me. And one scene, in particular, was so singular that it remains in my memory today as vivid as it was the day I saw it.
My eye was caught by a grey-haired woman in her sixties, who was apparently the centre of a most amazing disturbance, though what was happening, what was so disturbing, was not at first clear to me. Was she having a fit? What on earth was convulsing her- and, by a sort of sympathy or contagion, also convulsing everyone whom she gnashingly, ticcily passed?
As I drew closer I saw what was happening. She was imitating the passers-by-if 'imitation' is not too pallid, too passive, a word. Should we say, rather, that she was caricaturing everyone she passed? Within a second, a split-second, she 'had' them all.
I have seen countless mimes and mimics, clowns and antics, but nothing touched the horrible wonder I now beheld: this virtually instantaneous, automatic and convulsive mirroring of every face and figure. But it was not just an imitation, extraordinary as this would have been in itself. The woman not only took on, and took in, the features of countless people, she took them off. Every mirroring was also a parody, a mocking, an exaggeration of salient gestures and expressions, but an exaggeration in itself no less convulsive than intentional-a consequence of the violent acceleration and distortion of all her motions. Thus a slow smile, monstrously accelerated, would become a violent, milliseconds-lo
ng grimace; an ample gesture, accelerated, would become a farcical convulsive movement.
In the course of a short city-block this frantic old woman frenetically caricatured the features of forty or fifty passers-by, in a quick-fire sequence of kaleidoscopic imitations, each lasting a second or two, sometimes less, and the whole dizzying sequence scarcely more than two minutes.
And there were ludicrous imitations of the second and third order; for the people in the street, startled, outraged, bewildered by her imitations, took on these expressions in reaction to her; and those expressions, in turn, were re-reflected, re-directed, re-distorted, by the Touretter, causing a still greater degree of outrage and shock. This grotesque, involuntary resonance, or mutuality, by which everyone was drawn into an absurdly amplifying inter-
action, was the source of the disturbance I had seen from a distance. This woman who, becoming everybody, lost her own self, became nobody. This woman with a thousand faces, masks, per-sonae-how must it be for her in this whirlwind of identities? The answer came soon-and not a second too late; for the build-up of pressures, both hers and others', was fast approaching the point of explosion. Suddenly, desperately, the old woman turned aside, into an alley-way which led off the main street. And there, with all the appearances of a woman violently sick, she expelled, tremendously accelerated and abbreviated, all the gestures, the postures, the expressions, the demeanours, the entire behavioural repertoires, of the past forty or fifty people she had passed. She delivered one vast, pantomimic egurgitation, in which the engorged identities of the last fifty people who had possessed her were spewed out. And if the taking-in had lasted two minutes, the throwing-out was a single exhalation-fifty people in ten seconds, a fifth of a second or less for the time-foreshortened repertoire of each person.
I was later to spend hundreds of hours, talking to, observing, taping, learning from, Tourette patients. Yet nothing, I think, taught me as much, as swiftly, as penetratingly, as overwhelmingly as that phantasmagoric two minutes in a New York street.
