The man who mistook his wife for a hat

by Oliver Sacks

  The answer is-not in the least. She continues to feel, with the continuing loss of proprioception, that her body is dead, not-real, not-hers-she cannot appropriate it to herself. She can find no words for this state, and can only use analogies derived from other senses: 'I feel my body is blind and deaf to itself … it has no sense of itself-these are her own words. She has no words, no direct words, to describe this bereftness, this sensory darkness (or silence) akin to blindness or deafness. She has no words, and we lack words too. And society lacks words, and sympathy, for such states. The blind, at least, are treated with solicitude-we can imagine their state, and we treat them accordingly. But when Christina, painfully, clumsily, mounts a bus, she receives nothing but uncomprehending and angry snarls: 'What's wrong with you, lady? Are you blind-or blind-drunk?' What can she answer-'I have no proprioception'? The lack of social support and sympathy is an additional trial: disabled, but with the nature of her disability not clear-she is not, after all, manifestly blind or paralysed, manifestly anything-she tends to be treated as a phoney or a fool. This is what happens to those with disorders of the hidden senses (it happens also to patients who have vestibular impairment, or who have been labyrinthectomised).

  Christina is condemned to live in an indescribable, unimaginable realm-though 'non-realm', 'nothingness', might be better words for it. At times she breaks down-not in public, but with me: 'If only I could feel!' she cries. 'But I've forgotten what it's like … I was normal, wasn't I? I did move like everyone else?'

  'Yes, of course.'

  'There's no "of course". I can't believe it. I want proof.'

  I show her a home movie of herself with her children, taken just a few weeks before her polyneuritis.

  'Yes, of course, that's me!' Christina smiles, and then cries: 'But I can't identify with that graceful girl any more! She's gone, I can't remember her, Icant even imagine her. It's like something's been scooped right out of me, right at the centre . . . that's what they

  do with frogs, isn't it? They scoop out the centre, the spinal cord, they pith them . . . That's what I am, pithed, like a frog . . . Step up, come and see Chris, the first pithed human being. She's no proprioception, no sense of herself-disembodied Chris, the pithed girl!' She laughs wildly, with an edge of hysteria. I calm her- 'Come now!'-while thinking, 'Is she right?'

  For, in some sense, she is 'pithed', disembodied, a sort of wraith. She has lost, with her sense of proprioception, the fundamental, organic mooring of identity-at least of that corporeal identity, or 'body-ego', which Freud sees as the basis of self: 'The ego is first and foremost a body-ego.' Some such depersonalisation or de-realisation must always occur, when there are deep disturbances of body perception or body image. Weir Mitchell saw this, and incomparably described it, when he was working with amputees and nerve-damaged patients in the American Civil War-and in a famous, quasi-fictionalised account, but still the best, phenom-enologically most accurate, account we have, said (through the mouth of his physician-patient, George Dedlow):

  'I found to my horror that at times I was less conscious of myself, of my own existence, than used to be the case. This sensation was so novel that at first it quite bewildered me. I felt like asking someone constantly if I were really George Dedlow or not; but, well aware of how absurd I should seem after such a question, I refrained from speaking of my case, and strove more keenly to analyse my feelings. At times the conviction of my want of being myself was overwhelming and most painful. It was, as well as I can describe it, a deficiency in the egoistic sentiment of individuality.'

  For Christina there is this general feeling-this 'deficiency in the egoistic sentiment of individuality'-which has become less with accommodation, with the passage of time. And there is this specific, organically based, feeling of disembodiedness, which remains as severe, and uncanny, as the day she first felt it. This is also felt, for example, by those who have high transections of the spinal cord-but they of course, are paralysed; whereas Christina, though 'bodiless', is up and about.

  There are brief, partial reprieves, when her skin is stimulated. She goes out when she can, she loves open cars, where she can feel the wind on her body and face (superficial sensation, light touch, is only slightly impaired). 'It's wonderful,' she says. 'I feel the wind on my arms and face, and then I know, faintly, I have arms and a face. It's not the real thing, but it's something-it lifts this horrible, dead veil for a while.'

  But her situation is, and remains, a 'Wittgensteinian' one. She does not know 'Here is one hand'-her loss of proprioception, her de-afferentation, has deprived her of her existential, her epistemic, basis-and nothing she can do, or think, will alter this fact. She cannot be certain of her body-what would Wittgenstein have said, in her position?

  In an extraordinary way, she has both succeeded and failed. She has succeeded in operating, but not in being. She has succeeded to an almost incredible extent in all the accommodations that will, courage, tenacity, independence and the plasticity of the senses and the nervous system will permit. She has faced, she faces, an unprecedented situation, has battled against unimaginable difficulties and odds, and has survived as an indomitable, impressive human being. She is one of those unsung heroes, or heroines, of neurological affliction.

  But still and forever she remains defective and defeated. Not all the spirit and ingenuity in the world, not all the substitutions or compensations the nervous system allows, can alter in the least her continuing and absolute loss of proprioception-that vital sixth sense without which a body must remain unreal, unpossessed.

  Poor Christina is 'pithed' in 1985 as she was eight years ago and will remain so for the rest of her life. Her life is unprecedented. She is, so far as I know, the first of her kind, the first 'disembodied' human being.

  Postscript

  Now Christina has company of a sort. I understand from Dr H.H. Schaumburg, who is the first to describe the syndrome, that large numbers of patients are turning up everywhere now with severe

  sensory neuronopathies. The worst affected have body-image disturbances like Christina. Most of them are health faddists, or are on a megavitamin craze, and have been taking enormous quantities of vitamin B6 (pyridoxine). Thus there are now some hundreds of 'disembodied' men and women-though most, unlike Christina, can hope to get better as soon as they stop poisoning themselves with pyridoxine.

  4

  The Man Who Fell out of Bed

  When I was a medical student many years ago, one of the nurses called me in considerable perplexity, and gave me this singular story on the phone: that they had a new patient-a young man- just admitted that morning. He had seemed very nice, very normal, all day-indeed, until a few minutes before, when he awoke from a snooze. He then seemed excited and strange-not himself in the least. He had somehow contrived to fall out of bed, and was now sitting on the floor, carrying on and vociferating, and refusing to go back to bed. Could I come, please, and sort out what was happening?

  When I arrived I found the patient lying on the floor by his bed and staring at one leg. His expression contained anger, alarm, bewilderment and amusement-bewilderment most of all, with a hint of consternation. I asked him if he would go back to bed, or if he needed help, but he seemed upset by these suggestions and shook his head. I squatted down beside him, and took the history on the floor. He had come in, that morning, for some tests, he said. He had no complaints, but the neurologists, feeling that he had a 'lazy' left leg-that was the very word they had used- thought he should come in. He had felt fine all day, and fallen asleep towards evening. When he woke up he felt fine too, until he moved in the bed. Then he found, as he put it, 'someone's leg' in the bed-a severed human leg, a horrible thing! He was stunned, at first, with amazement and disgust-he had never experienced, never imagined, such an incredible thing. He felt the

  leg gingerly. It seemed perfectly formed, but 'peculiar' and cold. At this point he had a brainwave. He now realised what had happened: it was all a joke!A rat
her monstrous and improper, but a very original, joke! It was New Year's Eve, and everyone was celebrating. Half the staff were drunk; quips and crackers were flying; a carnival scene. Obviously one of the nurses with a macabre sense of humour had stolen into the Dissecting Room and nabbed a leg, and then slipped it under his bedclothes as a joke while he was still fast asleep. He was much relieved at the explanation; but feeling that a joke was a joke, and that this one was a bit much, he threw the damn thing out of the bed. But-and at this point his conversational manner deserted him, and he suddenly trembled and became ashen-pale-when he threw it out of bed, he somehow came after it-and now it was attached to him.

  'Look at it!' he cried, with revulsion on his face. 'Have you ever seen such a creepy, horrible thing? I thought a cadaver was just dead. But this is uncanny! And somehow-it's ghastly-it seems stuck to me!' He seized it with both hands, with extraordinary violence, and tried to tear it off his body, and, failing, punched it in an access of rage.

  'Easy!' I said. 'Be calm! Take it easy! I wouldn't punch that leg like that.'

  'And why not?' he asked, irritably, belligerently.

  'Because it's your leg,' I answered. 'Don't you know your own leg?'

  He gazed at me with a look compounded of stupefaction, incredulity, terror and amusement, not unmixed with a jocular sort of suspicion, 'Ah Doc!' he said. 'You're fooling me! You're in cahoots with that nurse-you shouldn't kid patients like this!'

  'I'm not kidding,' I said. 'That's your own leg.'

  He saw from my face that I was perfectly serious-and a look of utter terror came over him. 'You say it's my leg, Doc? Wouldn't you say that a man should know his own leg?'

  'Absolutely,' I answered. 'He should know his own leg. I can't imagine him not knowing his own leg. Maybe you're the one who's been kidding all along?'

  'I swear to God, cross my heart, I haven't … A man should know his own body, what's his and what's not-but this leg, this thing'-another shudder of distaste-'doesn't feel right, doesn't feel real-and it doesn't look part of me.'

  'What does it look like?' I asked in bewilderment, being, by this time, as bewildered as he was.

  'What does it look like?' He repeated my words slowly. 'I'll tell you what it looks like. It looks like nothing on earth. How can a thing like that belong to me? I don't know where a thing like that belongs . . . ' His voice trailed off. He looked terrified and shocked.

  'Listen,' I said. 'I don't think you're well. Please allow us to return you to bed. But I want to ask you one final question. If this-this thing-is not your left leg' (he had called it a 'counterfeit' at one point in our talk, and expressed his amazement that someone had gone to such lengths to 'manufacture' a 'facsimile') 'then where is your own left leg?'

  Once more he became pale-so pale that I thought he was going to faint. 'I don't know, he said. 'I have no idea. It's disappeared. It's gone. It's nowhere to be found . . .

  Postscript

  Since this account was published (in A Leg to Stand On, 1984), I received a letter from the eminent neurologist Dr Michael Kre-mer, who wrote:

  I was asked to see a puzzling patient on the cardiology ward. He had atrial fibrillation and had thrown off a large embolus giving him a left hemiplegia, and I was asked to see him because he constantly fell out of bed at night for which the cardiologists could find no reason.

  When I asked him what happened at night he said quite openly that when he woke in the night he always found that there was a dead, cold, hairy leg in bed with him which he could not understand but could not tolerate and he, therefore,

  with his good arm and leg pushed it out of bed and naturally, of course, the rest of him followed.

  He was such an excellent example of this complete loss of awareness of his hemiplegic limb but, interestingly enough, I could not get him to tell me whether his own leg on that side was in bed with him because he was so caught up with the unpleasant foreign leg that was there.

  5

  Hands

  Madeleine J. was admitted to St. Benedict's Hospital near New York City in 1980, her sixtieth year, a congenitally blind woman with cerebral palsy, who had been looked after by her family at home throughout her life. Given this history, and her pathetic condition-with spasticity and athetosis, i.e., involuntary movements of both hands, to which was added a failure of the eyes to develop-I expected to find her both retarded and regressed.

  She was neither. Quite the contrary: she spoke freely, indeed eloquently (her speech, mercifully, was scarcely affected by spasticity), revealing herself to be a high-spirited woman of exceptional intelligence and literacy.

  'You've read a tremendous amount,' I said. 'You must be really at home with Braille.'

  'No, I'm not,' she said. 'All my reading has been done for me- by talking-books or other people. I can't read Braille, not a single word. I can't do anything with my hands-they are completely useless.'

  She held them up, derisively. 'Useless godforsaken lumps of dough-they don't even feel part of me.'

  I found this very startling. The hands are not usually affected by cerebral palsy-at least, not essentially affected: they may be somewhat spastic, or weak, or deformed, but are generally of considerable use (unlike the legs, which may be completely paralysed-in that variant called Little's disease, or cerebral diplegia).

  Miss J.'s hands were mildly spastic and athetotic, but her sensory capacities-as I now rapidly determined-were completely intact: she immediately and correctly identified light touch, pain, tern-

  perature, passive movement of the fingers. There was no impairment of elementary sensation, as such, but, in dramatic contrast, there was the profoundest impairment of perception. She could not recognise or identify anything whatever-I placed all sorts of objects in her hands, including one of my own hands. She could not identify-and she did not explore; there were no active 'inter-ogatory' movements of her hands-they were, indeed, as inactive, as inert, as useless, as 'lumps of dough'.

  This is very strange, I said to myself. How can one make sense of all this? There is no gross sensory 'deficit'. Her hands would seem to have the potential of being perfectly good hands-and yet they are not. Can it be that they are functionless-'useless'-because she had never used them? Had being 'protected', 'looked after', 'babied' since birth prevented her from the normal exploratory use of the hands which all infants learn in the first months of life? Had she been carried about, had everything done for her, in a manner that had prevented her from developing a normal pair of hands? And if this was the case-it seemed far-fetched, but was the only hypothesis I could think of-could she now, in her sixtieth year, acquire what she should have acquired in the first weeks and months of life?

  Was there any precedent? Had anything like this ever been described-or tried? I did not know, but I immediately thought of a possible parallel-what was described by Leont'ev and Zapo-rozhets in their book Rehabilitation of Hand Function (Eng. tr. 1960). The condition they were describing was quite different in origin: they described a similar 'alienation' of the hands in some two hundred soldiers following massive injury and surgery-the injured hands felt 'foreign', 'lifeless', 'useless', 'stuck on', despite elementary neurological and sensory intactness. Leont'ev and Za-porozhets spoke of how the 'gnostic systems' that allow 'gnosis', or perceptive use of the hands, to take place could be 'dissociated' in such cases as a consequence of injury, surgery and the weeks- or months-long hiatus in the use of the hands that followed. In Madeleine's case, although the phenomenon was identical-'useless-ness', 'lifelessness', 'alienation'-it was lifelong. She did not need

  just to recover her hands, but to discover them-to acquire them, to achieve them-for the first time: not just to regain a dissociated gnostic system, but to construct a gnostic system she had never had in the first place. Was this possible?

  The injured soldiers described by Leont'ev and Zaporozhets had normal hands before injury. All they had to do was to 'remember' what had been 'forg
otten', or 'dissociated', or 'inactivated', through severe injury. Madeleine, in contrast, had no repertoire of memory for she had never used her hands-and she felt she had no hands- or arms either. She had never fed herself, used the toilet by herself, or reached out to help herself, always leaving it for others to help her. She had behaved, for sixty years, as if she were a being without hands.

  This then was the challenge that faced us: a patient with perfect elementary sensations in the hands, but, apparently, no power to integrate these sensations to the level of perceptions that were related to the world and to herself; no power to say, 'I perceive, I recognise, I will, I act', so far as her 'useless' hands went. But somehow or other (as Leont'ev and Zaporozhets found with their patients), we had to get her to act and to use her hands actively, and, we hoped, in so doing, to achieve integration: 'The integration is in the action,' as Roy Campbell said.

  Madeleine was agreeable to all this, indeed fascinated, but puzzled and not hopeful. 'How can I do anything with my hands,' she asked, 'when they are just lumps of putty?'

  'In the beginning is the deed,' Goethe writes. This may be so when we face moral or existential dilemmas, but not where movement and perception have their origin. Yet here too there is always something sudden: a first step (or a first word, as when Helen Keller said 'water'), a first movement, a first perception, a first impulse- total, 'out of the blue', where there was nothing, or nothing with sense before. 'In the beginning is the impulse.' Not a deed, not a reflex, but an 'impulse', which is both more obvious and more mysterious than either . . . We could not say to Madeleine, 'Do it!' but we might hope for an impulse; we might hope for, we might solicit, we might even provoke one . . .

  I thought of the infant as it reached for the breast. 'Leave Ma-

  deleine her food, as if by accident, slightly out of reach on occasion,' I suggested to her nurses. 'Don't starve her, don't tease her, but show less than your usual alacrity in feeding her.' And one day it happened-what had never happened before: impatient, hungry, instead of waiting passively and patiently, she reached out an arm, groped, found a bagel, and took it to her mouth. This was the first use of her hands, her first manual act, in sixty years, and it marked her birth as a 'motor individual' (Sherrington's term for the person who emerges through acts). It also marked her first manual perception, and thus her birth as a complete 'perceptual individual'. Her first perception, her first recognition, was of a bagel, or 'bagelhood'-as Helen Keller's first recognition, first utterance, was of water ('waterhood').