Part 3 (1/2)

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 . . .