Part 3 (2/2)

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.

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