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.[8]
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?’
‘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.
8
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).