The Man Who Mistook His Wife For A Hat Part 3

Christina listened closely, with a sort of desperate attention.

'What I must do then,' she said slowly, 'is use vision, use my eyes, in every situation where I used-what do you call it?- proprioception before. I've already noticed,' she added, musingly, that I may "lose" my arms. I think they're one place, and I find they're another. This "proprioception" is like the eyes of the body, the way the body sees itself. And if it goes, as it's gone with me, it's like the body's blind. My body can't "see" itself if it's lost its eyes, right? So I have to watch it-be its eyes. Right?'

*Such sensory polyneuropathies occur, but are rare. What was unique in Chris-tina's case, to the best of our knowledge at the time (this was in 1977), was the extraordinary selectivity displayed, so that proprioceptive fibres, and these only, bore the brunt of the damage. But see Sterman (1979).

'Right,' I said, 'right. You could be a physiologist.'

'I'll have to be a sort of physiologist,' she rejoined, 'because my physiology has gone wrong, and may never naturally go right It was as well that Christina showed such strength of mind, from the start, for, though the acute inflammation subsided, and her spinal fluid returned to normal, the damage it did to her proprioceptive fibres persisted-so that there was no neurological recovery a week, or a year, later. Indeed there has been none in the eight years that have now pa.s.sed-though she has been able to lead a life, a sort of life, through accommodations and adjustments of every sort, emotional and moral no less than neurological.

That first week Christina did nothing, lay pa.s.sively, scarcely ate. She was in a state of utter shock, horror and despair. What sort of a life would it be, if there was not natural recovery? What sort of a life, every move made by artifice? What sort of a life, above all, if she felt disembodied?

Then life rea.s.serted itself, as it will, and Christina started to move. She could at first do nothing without using her eyes, and collapsed in a helpless heap the moment she closed them. She had, at first, to monitor herself by vision, looking carefully at each part of her body as it moved, using an almost painful conscientiousness and care. Her movements, consciously monitored and regulated, were at first clumsy, artificial, in the highest degree. But then-and here both of us found ourselves most happily surprised, by the power of an ever-increasing, daily increasing, automatism-then her movements started to appear more delicately modulated, more graceful, more natural (though still wholly dependent on use of the eyes).

Increasingly now, week by week, the normal, unconscious feedback of proprioception was being replaced by an equally unconscious feedback by vision, by visual automatism and reflexes increasingly integrated and fluent. Was it possible, too, that something more fundamental was happening? That the brain's visual model of the body, or body-image-normally rather feeble (it is, of course, absent in the blind), and normally subsidiary to the proprioceptive body-model-was it possible that this, now the proprioceptive body model was lost, was gaining, by way of compensation or subst.i.tution, an enhanced, exceptional, extraordinary force? And to this might be added a compensatory enhancement of the vestibular body-model or body-image, too . . . both to an extent which was more than we had expected or hoped for.*

Whether or not there was increased use of vestibular feedback, there was certainly increased use of her ears-auditory feedback. Normally this is subsidiary, and rather unimportant in speaking- our speech remains normal if we are deaf from a head cold, and some of the congenitally deaf may be able to acquire virtually perfect speech. For the modulation of speech is normally proprioceptive, governed by inflowing impulses from all our vocal organs. Christina had lost this normal inflow, this afference, and lost her normal proprioceptive vocal tone and posture, and there-tore had to use her ears, auditory feedback, instead.

Besides these new, compensatory forms of feedback, Christina also started to develop-it was deliberate and conscious in the first place, but gradually became unconscious and automatic-various forms of new and compensatory 'feed-forward' (in all this she was a.s.sisted by an immensely understanding and resourceful rehabilitative staff).

Thus at the time of her catastrophe, and for about a month afterwards, Christina remained as floppy as a ragdoll, unable even to sit up. But three months later, I was startled to see her sitting very finely-too finely, statuesquely, like a dancer in mid-pose. And soon I saw that her sitting was, indeed, a pose, consciously or automatically adopted and sustained, a sort of forced or wilful or histrionic posture, to make up for the continuing lack of any genuine, natural posture. Nature having failed, she took to 'arti- *Contrast the fascinating case described by the late Purdon Martin in The Basal Ganglia and Posture (1967), p. 32: 'This patient, in spite of years of physiotherapy and training, has never regained the ability to walk in any normal manner. His greatest difficulty is in starting to walk and in propelling himself forward ... He is also unable to rise from a chair. He cannot crawl or place himself in the all-fours posture. When standing or walking he is entirely dependent on vision and falls down if he closes his eyes. At first he was unable to maintain his position on an ordinary chair when he closed his eyes, but he has gradually acquired the ability to do this.'

fice', but the artifice was suggested by nature, and soon became 'second nature'. Similarly with her voice-she had at first been almost mute.

This too was projected, as to an audience from a stage. It was a stagey, theatrical voice-not because of any histrionism, or perversion of motive, but because there was still no natural vocal posture. And with her face, too-this still tended to remain somewhat flat and expressionless (though her inner emotions were of full and normal intensity), due to lack of proprioceptive facial tone and posture, * unless she used an artificial enhancement of expression (as patients with aphasia may adopt exaggerated emphases and inflections).

But all these measures were, at best, partial. They made life possible-they did not make it normal. Christina learned to walk, to take public transport, to conduct the usual business of life-but only with the exercise of great vigilance, and strange ways of doing things-ways which might break down if her attention was diverted. Thus if she was eating while she was talking, or if her attention was elsewhere, she would grip the knife and fork with painful force-her nails and fingertips would go bloodless with pressure; but if there was any lessening of the painful pressure, she might nervelessly drop them straightaway-there was no in-between, no modulation, whatever.

Thus, although there was not a trace of neurological recovery (recovery from the anatomical damage to nerve fibres), there was, with the help of intensive and varied therapy-she remained in hospital, on the rehabilitation ward, for almost a year-a very considerable functional recovery, i.e., the ability to function using various subst.i.tutions and other such tricks. It became possible, finally, for Christina to leave hospital, go home, rejoin her children. She was able to return to her home-computer terminal, which she now learned to operate with extraordinary skill and efficiency, considering that everything had to be done by vision, *Purdon Martin, almost alone of contemporary neurologists, would often speak of facial and vocal 'posture', and their basis, finally, in proprioceptive integrity. He was greatly intrigued when 1 told him about Christina and showed him some films and tapes of her-many of the suggestions and formulations here are, in fact, his.

not feel. She had learned to operate-but how did she feel? Had the subst.i.tutions dispersed the disembodied sense she first spoke of?

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 a.n.a.logies 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 ident.i.ty-at least of that corporeal ident.i.ty, 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 Mitch.e.l.l 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 a.n.a.lyse 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 pa.s.sage 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 subst.i.tutions 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 quant.i.ties 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 amus.e.m.e.nt-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 rather 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 d.a.m.n 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 amus.e.m.e.nt, 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 G.o.d, 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 r.e.t.a.r.ded 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 G.o.dforsaken 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, pa.s.sive 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 ma.s.sive 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 'forgotten', 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 pa.s.sively 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').

After this first act, this first perception, progress was extremely rapid. As she had reached out to explore or touch a bagel, so now, in her new hunger, she reached out to explore or touch the whole world. Eating led the way-the feeling, the exploring, of different foods, containers, implements, etc. 'Recognition' had somehow to be achieved by a curiously roundabout sort of inference or guesswork, for having been both blind and 'handless' since birth, she was lacking in the simplest internal images (whereas Helen Keller at least had tactile images). Had she not been of exceptional intelligence and literacy, with an imagination filled and sustained, so to speak, by the images of others, images conveyed by language, by the word, she might have remained almost as helpless as a baby.

A bagel was recognised as round bread, with a hole in it; a fork as an elongated flat object with several sharp tines. But then this preliminary a.n.a.lysis gave way to an immediate intuition, and objects were instantly recognised as themselves, as immediately familiar in character and 'physiognomy', were immediately recognised as unique, as 'old friends'. And this sort of recognition, not a.n.a.lytic, but synthetic and immediate, went with a vivid delight, and a sense that she was discovering a world full of enchantment, mystery and beauty.

The commonest objects delighted her-delighted her and stimulated a desire to reproduce them. She asked for clay and started to make models: her first model, her first sculpture, was of a shoehorn, and even this was somehow imbued with a peculiar power and humour, with flowing, powerful, chunky curves reminiscent of an early Henry Moore.

And then-and this was within a month of her first recognitions-her attention, her appreciation, moved from objects to people. There were limits, after all, to the interest and expressive possibilities of things, even when transfigured by a sort of innocent, ingenuous and often comical genius. Now she needed to explore the human face and figure, at rest and in motion. To be 'felt' by Madeleine was a remarkable experience. Her hands, only such a little while ago inert, doughy, now seemed charged with a preternatural animation and sensibility. One was not merely being recognised, being scrutinised, in a way more intense and searching than any visual scrutiny, but being 'tasted' and appreciated meditatively, imaginatively and aesthetically, by a born (a newborn) artist. They were, one felt, not just the hands of a blind woman exploring, but of a blind artist, a meditative and creative mind, just opened to the full sensuous and spiritual reality of the world. These explorations too pressed for representation and reproduction as an external reality.

She started to model heads and figures, and within a year was locally famous as the Blind Sculptress of St. Benedict's. Her sculptures tended to be half or three-quarters life size, with simple but recognisable features, and with a remarkably expressive energy. For me, for her, for all of us, this was a deeply moving, an amazing, almost a miraculous, experience. Who would have dreamed that basic powers of perception, normally acquired in the first months of life, but failing to be acquired at this time, could be acquired in one's sixtieth year? What wonderful possibilities of late learning, and learning for the handicapped, this opened up. And who could have dreamed that in this blind, palsied woman, hidden away, inactivated, over-protected all her life, there lay the germ of an astonis.h.i.+ng artistic sensibility (unsuspected by her, as by others) that would germinate and blossom into a rare and beautiful reality, after remaining dormant, blighted, for sixty years?

Postscript The case of Madeleine J., however, as I was to find, was by no means unique. Within a year I had encountered another patient (Simon K.) who also had cerebral palsy combined with profound impairment of vision. While Mr K. had normal strength and sensation in his hands, he scarcely ever used them-and was extraordinarily inept at handling, exploring, or recognising anything. Now we had been alerted by Madeleine J., we wondered whether he too might not have a similar 'developmental agnosia'-and, as such, be 'treatable' in the same way. And, indeed, we soon found that what had been achieved with Madeleine could be achieved with Simon as well. Within a year he had become very 'handy' in all ways, and particularly enjoyed simple carpentry, shaping plywood and wooden blocks, and a.s.sembling them into simple wooden toys. He had no impulse to sculpt, to make reproductions-he was not a natural artist like Madeleine. But still, after a half-century spent virtually without hands, he enjoyed their use in all sorts of ways.

This is the more remarkable, perhaps, because he is mildly r.e.t.a.r.ded, an amiable simpleton, in contrast to the pa.s.sionate and highly gifted Madeleine J. It might be said that she is extraordinary, a Helen Keller, a woman in a million-but nothing like this could possibly be said of simple Simon. And yet the essential achievement-the achievement of hands-proved wholly as possible for him as for her. It seems clear that intelligence, as such, plays no part in the matter-that the sole and essential thing is use.

Such cases of developmental agnosia may be rare, but one commonly sees cases of acquired agnosia, which ill.u.s.trate the same fundamental principle of use. Thus I frequently see patients with a severe 'glove-and-stocking' neuropathy, so-called, due to diabetes. If the neuropathy is sufficiently severe, patients go beyond feelings of numbness (the 'glove-and-stocking' feeling), to a feeling of complete nothingness or de-realisation. They may feel (as one patient put it) 'like a basket-case', with hands and feet completely 'missing'. Sometimes they feel their arms and legs end in stumps, with lumps of 'dough' or 'plaster' somehow 'stuck on'. Typically this feeling of de-realisation, if it occurs, is absolutely sudden . . . and the return of reality, if it occurs, is equally sudden. There is, as it were, a critical (functional and ontological) threshold. It is crucial to get such patients to use their hands and feet-even, if necessary, to 'trick' them into so doing. With this there is apt to occur a sudden re-realisation-a sudden leap back into subjective reality and 'life' . . . provided there is sufficient physiological potential (if the neuropathy is total, if the distal parts of the nerves are quite dead, no such re-realisation is possible).

For patients with a severe but sub-total neuropathy, a modic.u.m of use is literally vital, and makes all the difference between being a 'basket-case' and reasonably functional (with excessive use, there may be fatigue of the limited nerve function, and sudden de-realisation again).

It should be added that these subjective feelings have precise objective correlates: one finds 'electrical silence', locally, in the muscles of the hands and feet; and, on the sensory side, a complete absence of any 'evoked potentials', at every level up to the sensory cortex. As soon as the hands and feet are re-realised, with use, there is a complete reversal of the physiological picture.

A similar feeling of deadness and unrealness is described above in Chapter Three, 'The Disembodied Lady'.

6.

Phantoms A 'phantom', in the sense that neurologists use, is a persistent image or memory of part of the body, usually a limb, for months or years after its loss. Known in antiquity, phantoms were described and explored in great detail by the great American neurologist Silas Weir Mitch.e.l.l, during and following the Civil War.

Weir Mitch.e.l.l described several sorts of phantom-some strangely ghost-like and unreal (these were the ones he called 'sensory ghosts'); some compellingly, even dangerously, life-like and real; some intensely painful, others (most) quite painless; some photographically exact, like replicas or facsimiles of the lost limb, others grotesquely foreshortened or distorted ... as well as 'negative phantoms', or 'phantoms of absence'. He also indicated, clearly, that such 'body-image' disorders-the term was only introduced (by Henry Head) fifty years later-might be influenced by either central factors (stimulation or damage to the sensory cortex, especially that of the parietal lobes), or peripheral ones (the condition of the nerve-stump, or neuromas; nerve-damage, nerve-block or nerve-stimulation; disturbances in the spinal nerve-roots or sensory tracts in the cord). I have been particularly interested, myself, in these peripheral determinants.

The following pieces, extremely short, almost anecdotal, come from the 'Clinical Curio' section of the British Medical Journal.

Phantom Finger A sailor accidentally cut off his right index finger. For forty years afterwards he was plagued by an intrusive phantom of the finger rigidly extended, as it was when cut off. Whenever he moved his hand toward his face-for example, to eat or to scratch his nose- he was afraid that this phantom finger would poke his eye out. (He knew this to be impossible, but the feeling was irresistible.) He then developed severe sensory diabetic neuropathy and lost all sensation of even having any fingers. The phantom finger disappeared too.

It is well known that a central pathological disorder, such as a sensory stroke, can 'cure' a phantom. How often does a peripheral pathological disorder have the same effect?

Disappearing Phantom Limbs All amputees, and all who work with them, know that a phantom limb is essential if an artificial limb is to be used. Dr Michael Kremer writes: 'Its value to the amputee is enormous. I am quite certain that no amputee with an artificial lower limb can walk on it satisfactorily until the body-image, in other words the phantom, is incorporated into it.'

Thus the disappearance of a phantom may be disastrous, and its recovery, its re-animation, a matter of urgency. This may be effected in all sorts of ways: Weir Mitch.e.l.l describes how, with faradisation of the brachial plexus, a phantom hand, missing for twenty-five years, was suddenly 'resurrected'. One such patient, under my care, describes how he must 'wake up' his phantom in the mornings: first he flexes the thigh-stump towards him, and then he slaps it sharply-'like a baby's bottom'-several times. On the fifth or sixth slap the phantom suddenly shoots forth, rekindled, fulgurated, by the peripheral stimulus. Only then can he put on his prosthesis and walk. What other odd methods (one wonders) are used by amputees?

Positional Phantoms A patient, Charles D., was referred to us for stumbling, falls and vertigo-there had been unfounded suspicions of labyrinthine disorder. It was evident on closer questioning that what he experi- enced was not vertigo at all, but a flutter of ever-changing positional illusions-suddenly the floor seemed further, then suddenly nearer, it pitched, it jerked, it tilted-in his own words 'like a s.h.i.+p in heavy seas'. In consequence he found himself lurching and pitching, unless he looked down at his feet. Vision was necessary to show him the true position of his feet and the floor-feel had become grossly unstable and misleading-but sometimes even vision was overwhelmed by feel, so that the floor and his feet looked frightening and s.h.i.+fting.

We soon ascertained that he was suffering from the acute onset of tabes-and (in consequence of dorsal root involvement) from a sort of sensory delirium of rapidly fluctuating 'proprioceptive illusions'. Everyone is familiar with the cla.s.sical end-stage of tabes, in which there may be virtual proprioceptive 'blindness' for the legs. Have readers encountered this intermediate stage-of positional phantoms or illusions-due to an acute (and reversible) tabetic delirium?

The experience this patient recounts reminds me of a singular experience of my own, occurring with the recovery from a proprioceptive scotoma. This was described (in A Leg to Stand On) as follows: I was infinitely unsteady, and had to gaze down. There and then I perceived the source of the commotion. The source was my leg-or, rather, that thing, that featureless cylinder of chalk which served as my leg-that chalky-white abstraction of a leg. Now the cylinder was a thousand feet long, not a matter of two millimetres; now it was fat, now it was thin; now it was tilted this way, now tilted that. It was constantly changing in size and shape, in position and angle, the changes occurring four or five times a second. The extent of transformation and change was immense-there could be a thousandfold switch between successive 'frames' . . .

Phantoms-Dead or Alive?

There is often a certain confusion about phantoms-whether they should occur, or not; whether they are pathological, or not; whether they are 'real', or not. The literature is confusing, but patients are not-and they clarify matters by describing different sorts of phantoms.

Thus a clear-headed man, with an above-the-knee amputation, described this to me: There's this thing, this ghost-foot, which sometimes hurts like h.e.l.l-and the toes curl up, or go into spasm. This is worst at night, or with the prosthesis off, or when I'm not doing any- '' thing. It goes away, when I strap the prosthesis on and walk. I ; still feel the leg then, vividly, but it's a good phantom, differ- , ent-it animates the prosthesis, and allows me to walk.

With this patient, with all patients, is not use all-important, in dispelling a 'bad' (or pa.s.sive, or pathological) phantom, if it exists; and in keeping the 'good' phantom-that is, the persisting personal limb-memory or limb-image-alive, active, and well, as they need?

Postscript Many (but not all) patients with phantoms suffer 'phantom pain', or pain in the phantom. Sometimes this has a bizarre quality, but often it is a rather 'ordinary' pain, the persistence of a pain previously present in the limb, or the onset of a pain that might be expected were the limb actually present. I have-since the original publication of this book-received many fascinating letters from patients about this: one such patient speaks of the discomfort of an ingrown toenail, which had not been 'taken care of before amputation, persisting for years after the amputation; but also of an entirely different pain-an excruciating root-pain or 'sciatica' in the phantom-following an acute 'slipped disc', and disappearing with removal of the disc and spinal fusion. Such problems, not at all uncommon, are in no sense 'imaginary', and may indeed be investigated by neurophysiological means.