The man who mistook his wife for a hat (8 page)

   She was admitted to hospital three days before the operation date, and placed on an antibiotic for microbial prophylaxis. This was purely routine, a precaution, no complications of any sort being expected at all. Christina understood this, and being a sensible soul had no great anxieties.

   The day before surgery Christina, not usually given to fancies or dreams, had a disturbing dream of peculiar intensity. She was swaying wildly, in her dream, very unsteady on her feet, could hardly feel the ground beneath her, could hardly feel anything in

   her hands, found them flailing to and fro, kept dropping whatever she picked up.

   She was distressed by this dream. ('I never had one like it,' she said. 'I can't get it out of my mind.')-so distressed that we requested an opinion from the psychiatrist. 'Pre-operative anxiety,' he said. 'Quite natural, we see it all the time.'

   But later that day
the dream came true.
Christina did find herself very unsteady on her feet, with awkward flailing movements, and dropping things from her hands.

   The psychiatrist was again called-he seemed vexed at the call, hut also, momentarily, uncertain and bewildered. 'Anxiety hysteria,' he now snapped, in a dismissive tone. 'Typical conversion symptoms-you see them all the while.'

   But the day of surgery Christina was still worse. Standing was impossible-unless she looked down at her feet. She could hold nothing in her hands, and they 'wandered'-unless she kept an eye on them. When she reached out for something, or tried to feed herself, her hands would miss, or overshoot wildly, as if some essential control or coordination was gone.

   She could scarcely even sit up-her body 'gave way'. Her face was oddly expressionless and slack, her jaw fell open, even her vocal posture was gone.

   'Something awful's happened,' she mouthed, in a ghostly flat voice. 'I can't feel my body. I feel weird-disembodied.'

   This was an amazing thing to hear, confounded, confounding. 'Disembodied'-was she crazy? But what of her physical state then? The collapse of tone and muscle posture, from top to toe; the wandering of her hands, which she seemed unaware of; the flailing and overshooting, as if she were receiving no information from the periphery, as if the control loops for tone and movement had catastrophically broken down.

   'It's a strange statement,' I said to the residents. 'It's almost impossible to imagine what might provoke such a statement.'

   'But it's hysteria, Dr Sacks-didn't the psychiatrist say so?'

   'Yes, he did. But have you ever seen a hysteria like this? Think phenomenologically-take what you see as genuine phenomenon, in which her state-of-body and state-of-mind are not fictions, but

   a psychophysical whole. Could anything give such a picture of undermined body and mind?

   'I'm not testing you,' I added. 'I'm as bewildered as you are. I've never seen or imagined anything quite like this before . . . '

   I thought, and they thought, we thought together.

   'Could it be a biparietal syndrome?' one of them asked.

   'It's an "as if',' I answered:
'as if
the parietal lobes were not getting their usual sensory information. Let's
do
some sensory testing-and test parietal lobe function, too.

   We did so, and a picture began to emerge. There seemed to be a very profound, almost total, proprioceptive deficit, going from the tips of her toes to her head-the parietal lobes were working,
but had nothing to work with.
Christina might have hysteria, but she had a great deal more, of a sort which none of us had ever seen or conceived before. We put in an emergency call now, not to the psychiatrist, but to the physical medicine specialist, the physiatrist.

   He arrived promptly, responding to the urgency of the call. He opened his eyes very wide when he saw Christina, examined her swiftly and comprehensively, and then proceeded to electrical tests of nerve and muscle function. 'This is quite extraordinary,' he said. 'I have never seen or read about anything like this before. She has lost all proprioception-you're right-from top to toe. She has no muscle or tendon or joint sense whatever. There is slight loss of other sensory modalities-to light touch, temperature, and pain, and slight involvement of the motor fibres, too. But it is predominantly position-sense-proprioception-which has sustained such damage.'

   'What's the cause?' we asked.

   'You're the neurologists. You find out.'

   By afternoon, Christina was still worse. She lay motionless and toneless; even her breathing was shallow. Her situation was grave- we thought of a respirator-as well as strange.

   The picture revealed by spinal tap was one of an acute polyneuritis, but a polyneuritis of a most exceptional type: not like Guillain-Barre syndrome, with its overwhelming motor involvement, but a purely (or almost purely) sensory neuritis, affecting

   the sensory roots of spinal and cranial nerves throughout the neu-raxis. *

   Operation was deferred; it would have been madness at this time. Much more pressing were the questions: 'Will she survive? What can we do?'

   'What's the verdict?' Christina asked, with a faint voice and fainter smile, after we had checked her spinal fluid.

   'You've got this inflammation, this neuritis . . . ' we began, and told her all we knew. When we forgot something, or hedged, her clear questions brought us back.

   'Will it get better?' she demanded. We looked at each other, and at her: 'We have no idea.'

   The sense of the body, I told her, is given by three things: vision, balance organs (the vestibular system), and proprioception-which she'd lost. Normally all of these worked together. If one failed, (he others could compensate, or substitute-to a degree. In particular, I told of my patient Mr MacGregor, who, unable to employ his balance organs, used his eyes instead (see below, Chapter Seven). And of patients with neurosyphilis,
tabes dorsalis,
who had similar symptoms, but confined to the legs-and how they too had to compensate by use of their eyes (see 'Positional Phantoms' in Chapter Six). And how, if one asked such a patient to move his legs, he was apt to say: 'Sure, Doc, as soon as I find them.'

   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 passed-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 passively, 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 reasserted 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 substitution, 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 assisted 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 substitutions 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.