The man who mistook his wife for a hat

by Oliver Sacks

  It came to me in this moment that such 'super-Touretters' must be placed, by an organic quirk, through no fault of their own, in a most extraordinary, indeed unique, existential position, which has some analogies to that of raging 'super-Korsakov's', but, of course, has a quite different genesis-and aim. Both can be driven to incoherence, to identity-delirium. The Korsakovian, perhaps mercifully, never knows it, but the Touretter perceives his plight with excruciating, and perhaps finally ironic, acuity, though he may be unable, or unwilling, to do much about it.

  For where the Korsakovian is driven by amnesia, absence, the Touretter is driven by extravagant impulse-impulse of which he is both the creator and the victim, impulse he may repudiate, but cannot disown. Thus he is impelled, as the Korsakovian is not, into an ambiguous relation with his disorder: vanquishing it, being vanquished by it, playing with it-there is every variety of conflict and collusion.

  Lacking the normal, protective barriers of inhibition, the normal, organically determined boundaries of self, the Touretter's ego is subject to a lifelong bombardment. He is beguiled, assailed, by impulses from within and without, impulses which are organic and convulsive, but also personal (or rather pseudo-personal) and seductive. How will, how can, the ego stand this bombardment? Will identity survive? Can it develop, in face of such a shattering, such pressures-or will it be overwhelmed, to produce a Tour-ettized soul' (in the poignant words of a patient I was later to see)? There is a physiological, an existential, almost a theological pressure upon the soul of the Touretter-whether it can be held whole and sovereign, or whether it will be taken over, possessed and dispossessed, by every immediacy and impulse.

  Hume, as we have noted, wrote:

  I venture to affirm . . . that [we] are nothing but a bundle or collection of different sensations, succeeding one another with inconceivable rapidity, and in a perpetual flux and movement.

  Thus, for Hume, personal identity is a fiction-we do not exist, we are but a consecution of sensations, or perceptions.

  This is clearly not the case with a normal human being, because he owns his own perceptions. They are not a mere flux, but his own, united by an abiding individuality or self. But what Hume describes may be precisely the case for a being as unstable as a super-Touretter, whose life is, to some extent, a consecution of random or convulsive perceptions and motions, a phantasmagoric fluttering with no centre or sense. To this extent he is a 'Humean' rather than a human being. This is the philosophical, almost theological, fate which lies in wait, if the ratio of impulse to self is too overwhelming. It has affinities to a 'Freudian' fate, which is also to be overwhelmed by impulse- but the Freudian fate has sense (albeit tragic), whereas a 'Humean' fate is meaningless and absurd.

  The super-Touretter, then, is compelled to fight, as no one else is, simply to survive-to become an individual, and survive as one, in face of constant impulse. He may be faced, from earliest

  childhood, with extraordinary barriers to individuation, to becoming a real person. The miracle is that, in most cases, he succeeds- for the powers of survival, of the will to survive, and to survive as a unique inalienable individual, are, absolutely, the strongest in our being: stronger than any impulses, stronger than disease. Health, health militant, is usually the victor.

  PART THREE

  TRANSPORTS

  Introduction

  While we have criticised the concept of function, even attempting a rather radical redefinition, we have adhered to it nevertheless, drawing in the broadest terms contrasts based on 'deficit' or 'excess'. But it is clear that wholly other terms also have to be used. As soon as we attend to phenomena as such, to the actual quality of experience or thought or action, we have to use terms more reminiscent of a poem or painting. How, say, is a dream intelligible in terms of function?

  We have always two universes of discourse-call them 'physical' and 'phenomenal', or what you will-one dealing with questions of quantitative and formal structure, the other with those qualities that constitute a 'world'. All of us have our own, distinctive mental worlds, our own inner journeyings and landscapes, and these, for most of us, require no clear neurological 'correlate'. We can usually tell a man's story, relate passages and scenes from his life, without bringing in any physiological or neurological considerations: such considerations would seem, at the least, supererogatory, if not frankly absurd or insulting. For we consider ourselves, and rightly, 'free'-at least, determined by the most complex human and ethical considerations, rather than by the vicissitudes of our neural functions or nervous systems. Usually, but not always: for sometimes a man's life may be cut across, transformed, by an organic disorder; and if so his story does require a physiological or neurological correlate. This, of course, is so with all the patients here described.

  In the first half of this book we described cases of the obviously

  pathological-situations in which there is some blatant neurological excess or deficit. Sooner or later it is obvious to such patients, or their relatives, no less than to their doctors, that there is 'something (physically) the matter'. Their inner worlds, their dispositions, may indeed be altered, transformed; but, as becomes clear, this is due to some gross (and almost quantitative) change in neural function. In this third section, the presenting feature is reminiscence, altered perception, imagination, 'dream'. Such matters do not often come to neurological or medical notice. Such 'transports'-often of poignant intensity, and shot through with personal feeling and meaning-tend to be seen, like dreams, as psychical: as a manifestation, perhaps, of unconscious or preconscious activity (or, in the mystically-minded, of something 'spiritual'), not as something 'medical', let alone 'neurological'. They have an intrinsic dramatic, or narrative, or personal 'sense', and so are not apt to be seen as 'symptoms'. It may be in the nature of transports that they are more likely to be confided to psychoanalysts or confessors, to be seen as psychoses, or to be broadcast as religious revelations, rather than brought to physicians. For it never occurs to us at first that a vision might be 'medical'; and if an organic basis is suspected or found, this may be felt to 'devalue' the vision (though, of course, it does not-values, valuations, have nothing to do with etiology).

  All the transports described in this section do have more or less clear organic determinants (though it was not evident to begin with, but required careful investigation to bring out). This does not detract in the least from their psychological or spiritual significance. If God, or the eternal order, was revealed to Dostoievski in seizures, why should not other organic conditions serve as 'portals' to the beyond or the unknown? In a sense, this section is a study of such portals.

  Hughlings Jackson, in 1880, describing such 'transports', or 'portals', or 'dreamy states', in the course of certain epilepsies, used the general word 'reminiscence'. He wrote:

  I should never diagnose epilepsy from the paroxysmal occurrence of 'reminiscence', without other symptoms, although I

  should suspect epilepsy if that super-positive mental state began to occur very frequently … I have never been consulted for 'reminiscence' only . . .

  But J have been so consulted: for the forced or paroxysmal reminiscence of tunes, of 'visions', of 'presences', or scenes-not only in epilepsy, but in a variety of other organic conditions. Such transports or reminiscences are not uncommon in migraine (see 'The Visions of Hildegard', Chapter Twenty). This sense of 'going back', whether on an epileptic or toxic basis, suffuses 'A Passage to India' (Chapter Seventeen). A plainly toxic or chemical basis underlies 'Incontinent Nostalgia' (Chapter Sixteen) and the strange hyperosmia of Chapter Eighteen, 'The Dog Beneath the Skin'. Either seizure-activity or a frontal-lobe disinhibition determines the horrifying 'reminiscence' of 'Murder' (Chapter Nineteen).

  The theme of this section is the power of imagery and memory to 'transport' a person as a result of abnormal stimulation of the temporal lobes and limbic system of the brain. This may even teach us something of the cerebral basis of certain visions
and dreams, and of how the brain (which Sherrington called 'an enchanted loom') may weave a magic carpet to transport us.

  15

  Reminiscence

  Mrs O'C. was somewhat deaf, but otherwise in good health. She lived in an old people's home. One night, in January 1979, she dreamt vividly, nostalgically, of her childhood in Ireland, and especially of the songs they danced to and sang. When she woke up, the music was still going, very loud and clear. 'I must still be dreaming,' she thought, but this was not so. She got up, roused and puzzled. It was the middle of the night. Someone, she assumed, must have left a radio playing. But why was she the only person to be disturbed by it? She checked every radio she could find-they were all turned off. Then she had another idea: she had heard that dental fillings could sometimes act like a crystal radio, picking up stray broadcasts with unusual intensity. 'That's it,' she thought. 'One of my fillings is playing up. It won't last long. I'll get it fixed in the morning.' She complained to the night nurse, who said her fillings looked fine. At this point another notion occurred to Mrs O'C: 'What sort of radio-station,' she reasoned to herself, 'would play Irish songs, deafeningly, in the middle of the night? Songs, just songs, without introduction or comment? And only songs that I know. What radio station would play my songs, and nothing else?' At this point she asked herself: 'Is the radio in my head?'

  She was now thoroughly rattled-and the music continued deafening. Her last hope was her ENT man, the otologist she was seeing: he would reassure her, tell her it was just 'noises in the ear', something to do with her deafness, nothing to worry about. But when she saw him in the course of the morning, he said: 'No, Mrs O'C., I don't think it's your ears. A simple ringing or buzzing

  or rumbling, maybe: but a concert of Irish songs-that's not your ears. Maybe,' he continued, 'you should see a psychiatrist.' Mrs O'C. arranged to see a psychiatrist the same day. 'No, Mrs O'C.,' the psychiatrist said, 'it's not your mind. You are not mad-and the mad don't hear music, they only hear "voices". You must see a neurologist, my colleague, Dr Sacks.' And so Mrs O'C. came to me.

  Conversation was far from easy, partly because of Mrs O'C.'s deafness, but more because I was repeatedly drowned out by songs- she could only hear me through the softer ones. She was bright, alert, not delirious or mad, but with a remote, absorbed look, as of someone half in a world of their own. I could find nothing neurologically amiss. None the less, I suspected that the music was 'neurological'.

  What could have happened with Mrs O'C. to bring her to such a pass? She was 88 and in excellent general health with no hint of fever. She was not on any medications which might unbalance her excellent mind. And, manifestly, she had been normal the day before.

  'Do you think it's a stroke, Doctor?' she asked, reading my thoughts.

  'It could be,' I said, 'though I've never seen a stroke like this. Something has happened, that's for sure, but I don't think you're in danger. Don't worry, and hold on.'

  'It's not so easy to hold on,' she said, 'when you're going through what I'm going through. I know it's quiet here, but I am in an ocean of sound.'

  I wanted to do an electroencephalogram straightaway, paying special attention to the temporal lobes, the 'musical' lobes of the brain, but circumstances conspired to prevent this for a while. In this time, the music grew less-less loud and, above all, less persistent. She was able to sleep after the first three nights and, increasingly, to make and hear conversation between 'songs'. By the time I came to do an EEG, she heard only occasional brief snatches of music, a dozen times, more or less, in the course of a day. After we had settled her and applied the electrodes to her head, I asked her to lie still, say nothing and not 'sing to herself, but to

  raise her right forefinger slightly-which in itself would not disturb the EEG-if she heard any of her songs as we recorded. In the course of a two-hour recording, she raised her finger on three occasions, and each time she did this the EEG pens clattered, and transcribed spikes and sharp waves in the temporal lobes of the brain. This confirmed that she was indeed having temporal-lobe seizures, which, as Hughlings Jackson guessed and Wilder Pen-field proved, are the invariable basis of 'reminiscence' and experiential hallucinations. But why should she suddenly develop this strange symptom? I obtained a brainscan, and this showed that she had indeed had a small thrombosis or infarction in part of her right temporal lobe. The sudden onset of Irish songs in the night, the sudden activation of musical memory-traces in the cortex, were, apparently, the consequence of a stroke, and as it resolved, so the songs 'resolved' too.

  By mid-April the songs had entirely gone, and Mrs O'C. was herself once again. I asked her at this point how she felt about it all, and, in particular, whether she missed the paroxysmal songs she heard. 'It's funny you should ask that,' she said with a smile. 'Mostly, I would say, it is a great relief. But, yes, I do miss the old songs a little. Now, with lots of them, I can't even recall them. It was like being given back a forgotten bit of my childhood again. And some of the songs were really lovely.'

  I had heard similar sentiments from some of my patients on L-Dopa-the term I used was 'incontinent nostalgia'. And what Mrs O'C. told me, her obvious nostalgia, put me in mind of a poignant story of H.G. Wells, 'The Door in the Wall'. I told her the story. 'That's it,' she said. 'That captures the mood, the feeling, entirely. But my door is real, as my wall was real. My door leads to the lost and forgotten past.'

  I did not see a similar case until June last year, when I was asked to see Mrs O'M., who was now a resident at the same home. Mrs O'M. was also a woman in her eighties, also somewhat deaf, also bright and alert. She, too, heard music in the head and sometimes a ringing or hissing or rumbling; occasionally she heard 'voices talking', usually 'far away' and 'several at once', so that she could never catch what they were saying. She hadn't mentioned

  these symptoms to anybody, and had secretly worried, for four years, that she was mad. She was greatly relieved when she heard from the Sister that there had been a similar case in the Home some time before, and very relieved to be able to open up to me.

  One day, Mrs O'M. recounted, while she was grating parsnips in the kitchen, a song started playing. It was 'Easter Parade', and was followed, in swift succession, by 'Glory, Glory, Hallelujah' and 'Good Night, Sweet Jesus'. Like Mrs O'C., she assumed that a radio had been left on, but quickly discovered that all the radios were off. This was in 1979, four years earlier. Mrs O'C. recovered in a few weeks, but Mrs O'M.'s music continued, and got worse and worse.

  At first she would hear only these three songs-sometimes spontaneously, out of the blue, but for certain if she chanced to think of any of them. She tried, therefore, to avoid thinking of them, but the avoidance of thinking was as provocative as the thinking.

  'Do you like these particular songs?' I asked, psychiatrically. 'Do they have some special meaning for you?'

  'No,' she answered promptly. 'I never specially liked them, and I don't think they had any special meaning for me.'

  'And how did you feel when they kept going on?'

  'I came to hate them,' she replied with great force. 'It was like some crazy neighbour continually putting on the same record.'

  For a year or more, there was nothing but these songs, in maddening succession. After this-and though it was worse in one way, it was also a relief-the inner music became more complex and various. She would hear countless songs-sometimes several simultaneously; sometimes she would hear an orchestra or choir; and, occasionally, voices, or a mere hubbub of noises.

  When I came to examine Mrs O'M. I found nothing abnormal except in her hearing, and here what I found was of singular interest. She had some inner-ear deafness, of a commonplace sort, but over and above this she had a peculiar difficulty in the perception and discrimination of tones of a kind which neurologists call amusia, and which is especially correlated with impaired function in the auditory (or temporal) lobes of the brain. She herself complained that recently the hymns in the chapel see
med more

  and more alike so that she could scarcely distinguish them by tone or tune, but had to rely on the words, or the rhythm.* And although she had been a fine singer in the past when I tested her she sang flat and out of key. She mentioned, too, that her inner music was most vivid when she woke up, becoming less so as other sensory impressions crowded in; and that it was least likely to occur when she was occupied-emotionally, intellectually, but especially visually. In the hour or so she was with me, she heard music only once-a few bars of 'Easter Parade', played so loud, and so suddenly, she could hardly hear me through it.

  When we came to do an EEG on Mrs O'M. it showed strikingly high voltage and excitability in both temporal lobes-those parts of the brain associated with the central representation of sounds and music, and with the evocation of complex experiences and scenes. And whenever she 'heard' anything, the high voltage waves became sharp, spike-like, and frankly convulsive. This confirmed my thought that she had too a musical epilepsy, associated with disease of the temporal lobes.

  But what was going on with Mrs O'C. and Mrs O'M.? 'Musical epilepsy' sounds like a contradiction in terms: for music, normally, is full of feeling and meaning, and corresponds to something deep in ourselves, 'the world behind the music', in Thomas Mann's phrase-whereas epilepsy suggests quite the reverse: a crude, random physiological event, wholly unselective, without feeling or meaning. Thus a 'musical epilepsy' or a 'personal epilepsy' would seem a contradiction in terms. And yet such epilepsies do occur, though solely in the context of temporal lobe seizures, epilepsies of the reminiscent part of the brain. Hughlings Jackson described these a century ago, and spoke in this context of 'dreamy states', 'reminiscence', and 'physical seizures':

  It is not very uncommon for epileptics to have vague and yet exceedingly elaborate mental states at the onset of epileptic seizures . . . The elaborate mental state, or so-called intellectual aura, is always the same, or essentially the same, in each case.