by Oliver Sacks
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 analysis 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 analytic, 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 astonishing 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 assembling 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 retarded, an amiable simpleton, in contrast to the passionate 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 illustrate 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 modicum 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 Mitchell, during and following the Civil War.
Weir Mitchell 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 piec
es, 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 Mitchell 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 ship 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 shifting.
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 classical 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
hell-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 passive, 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.
Thus, Dr Jonathan Cole, a former student of mine and now a spinal neurophysiologist, describes how in a woman with persistent phantom leg pain, anaesthesia of the spinous ligament with Lig-nocaine caused the phantom to be anaesthetized (indeed to disappear) briefly; but that electrical stimulation of the spinal roots
produced a sharp tingling pain in the phantom quite different from the dull one which was usually present; whilst stimulation of the spinal cord higher up reduced the phantom pain (personal communication). Dr Cole has also presented detailed electrophysiological studies of a patient with a sensory polyneuropathy of fourteen years' duration, very similar in many respects to Christina, the "Disembodied Lady" (see Proceedings of the Physiological Society, February 1986, p. 5IP).
7
On the Level
It is nine years now since I met Mr MacGregor, in the neurology clinic of St. Dunstan's, an old-people's home where I once worked, but I remember him-I see him-as if it were yesterday.
'What's the problem?' I asked, as he tilted in.
'Problem? No problem-none that I know of . . . But others keep telling me I lean to the side: "You're like the Leaning Tower of Pisa," they say. "A bit more tilt, and you'll topple right over." '
'But you don't feel any tilt?'
'I feel fine. I don't know what they mean. How could I be tilted without knowing I was?'
'It sounds a queer business,' I agreed. 'Let's have a look. I'd like to see you stand and take a little stroll-just from here to that wall and back. I want to see for myself, and 1 want you to see too. We'll take a videotape of you walking and play it right back.'
'Suits me, Doc,' he said, and, after a couple of lunges, stood up. What a fine old chap, I thought. Ninety-three-and he doesn't look a day past seventy. Alert, bright as a button. Good for a hundred. And strong as a coal-heaver, even if he does have Parkinson's disease. He was walking, now, confidently, swiftly, but canted over, improbably, a good twenty degrees, his centre of gravity way off to the left, maintaining his balance by the narrowest possible margin.
'There!' he said with a pleased smile. 'See! No problems-I walked straight as a die.'
'Did you, indeed, Mr MacGregor?' I asked. 'I w
ant you to judge for yourself.'
I rewound the tape and played it back. He was profoundly shocked when he saw himself on the screen. His eyes bulged, his jaw dropped, and he muttered, 'I'll be damned!' And then, 'They're right, I am over to one side. I see it here clear enough, but I've no sense of it. I don't feel it.'
'That's it,' I said. 'That's the heart of the problem.'
We have five senses in which we glory and which we recognise and celebrate, senses thar constitute the sensible world for us. But there are other senses-secret senses, sixth senses, if you will- equally vital, but unrecognised, and unlauded. These senses, unconscious, automatic, had to be discovered. Historically, indeed, their discovery came lare: what the Victorians vaguely called 'muscle sense'-the awareness of the relative position of trunk and limbs, derived from receptors in the joints and tendons-was only really defined (and named 'proprioception') in the 1890s. And the complex mechanisms and controls by which our bodies are properly aligned and balanced in space-these have only been defined in our own century, and still hold many mysteries. Perhaps it will only be in this space age, with the paradoxical license and hazards of gravity-free life, that we will truly appreciate our inner ears, our vestibules and all the other obscure receptors and reflexes that govern our body orientation. For normal man, in normal situations, they simply do not exist.
Yet their absence can be quite conspicuous. If there is defective (or distorted) sensation in our overlooked secret senses, what we then experience is profoundly strange, an almost incommunicable equivalent to being blind or being deaf. If proprioception is completely knocked out, the body becomes, so to speak, blind and deaf to itself-and (as the meaning of the Latin root proprius hints) ceases to 'own' itself, to feel itself as itself (see Chapter Three, 'The Disembodied Lady').