The Man Who Mistook His Wife For A Hat Part 4

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 b.u.t.ton. 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 want 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 d.a.m.ned!' 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 const.i.tute 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 s.p.a.ce-these have only been defined in our own century, and still hold many mysteries. Perhaps it will only be in this s.p.a.ce 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').

The old man suddenly became intent, his brows knitted, his lips pursed. He stood motionless, in deep thought, presenting the picture that I love to see: a patient in the actual moment of discovery-half-appalled, half-amused-seeing for the first time exactly what is wrong and, in the same moment, exactly what there is to be done. This is the therapeutic moment.

'Let me think, let me think,' he murmured, half to himself, drawing his s.h.a.ggy white brows down over his eyes and emphasising each point with his powerful, gnarled hands. 'Let me think. You think with me-there must be an answer! I tilt to one side, and I can't tell it, right? There should be some feeling, a clear signal, but it's not there, right?' He paused. 'I used to be a carpenter,' he said, his face lighting up. 'We would always use a spirit level to tell whether a surface was level or not, or whether it was tilted from the vertical or not. Is there a sort of spirit level in the brain?'

I nodded.

'Can it be knocked out by Parkinson's disease?'

I nodded again.

'Is this what has happened with me?'

I nodded a third time and said, 'Yes. Yes. Yes.'

In speaking of such a spirit level, Mr MacGregor had hit on a fundamental a.n.a.logy, a metaphor for an essential control system in the brain. Parts of the inner ear are indeed physically-literally-like levels; the labyrinth consists of semicircular ca.n.a.ls containing liquid whose motion is continually monitored. But it was not these, as such, that were essentially at fault; rather, it was his ability to use his balance organs, in conjunction with the body's sense of itself and with its visual picture of the world. Mr MacGregor's homely symbol applies not just to the labyrinth but also to the complex integration of the three secret senses: the labyrinthine, the proprioceptive, and the visual. It is this synthesis that is impaired in Parkinsonism.

The most profound (and most practical) studies of such integrations-and of their singular disintegrations in Parkinsonism- were made by the late, great Purdon Martin and are to be found in his remarkable book The Basal Ganglia and Posture (originally published in 1967, but continually revised and expanded in the ensuing years; he was just completing a new edition when he died recently). Speaking of this integration, this integrator, in the brain, Purdon Martin writes 'There must be some centre or "higher authority" in the brain . . . some "controller" we may say. This controller or higher authority must be informed of the state of stability or instability of the body.'

In the section on 'tilting reactions' Purdon Martin emphasises the threefold contribution to the maintenance of a stable and upright posture, and he notes how commonly its subtle balance is upset in Parkinsonism-how, in particular, 'it is usual for the labyrinthine element to be lost before the proprioceptive and the visual'. This triple control system, he implies, is such that one sense, one control, can compensate for the others-not wholly (since the senses differ in their capabilities) but in part, at least, and to a useful degree. Visual reflexes and controls are perhaps the least important-normally. So long as our vestibular and proprioceptive systems are intact, we are perfectly stable with our eyes closed. We do not tilt or lean or fall over the moment we close our eyes. But the precariously balanced Parkinsonian may do so. (One often sees Parkinsonian patients sitting in the most grossly tilted positions, with no awareness that this is the case. But let a mirror be provided, so they can see their positions, and they instantly straighten up.) Proprioception, to a considerable extent, can compensate for defects in the inner ears. Thus patients who have been surgically deprived of their labyrinths (as is sometimes done to relieve the intolerable, crippling vertigo of severe Meniere's disease), while at first unable to stand upright or take a single step, may learn to employ and to enhance their proprioception quite wonderfully; in particular, to use the sensors in the vast latissimus dorsi muscles of the back-the greatest, most mobile muscular expanse in the body-as an accessory and novel balance organ, a pair of vast, winglike proprioceptors. As the patients become practised, as this becomes second-nature, they are able to stand and walk-not perfectly, but with safety, a.s.surance, and ease.

Purdon Martin was endlessly thoughtful and ingenious in designing a variety of mechanisms and methods that made it possible for even severely disabled Parkinsonians to achieve an artificial normality of gait and posture-lines painted on the floor, counterweights in the belt, loudly ticking pacemakers-to set the cadence for walking. In this he always learned from his patients (to whom, indeed, his great book is dedicated). He was a deeply human pioneer, and in his medicine understanding and collaborating were central: patient and physician were coequals, on the same level, each learning from and helping the other and between them arriving at new insights and treatment. But he had not, to my knowledge, devised a prosthesis for the correction of impaired tilting and higher vestibular reflexes, the problem that afflicted Mr MacGregor.

'So that's it, is it?' asked Mr MacGregor. 'I can't use the spirit level inside my head. I can't use my ears, but I can use my eyes. Quizzically, experimentally, he tilted his head to one side: "Things look the same now-the world doesn't tilt." Then he asked for a mirror, and I had a long one wheeled before him. 'Now I see myself tilting,' he said. 'Now I can straighten up-maybe I could stay straight. . . But I can't live among mirrors, or carry one round with me.'

He thought again deeply, frowning in concentration-then suddenly his face cleared, and lit up with a smile. 'I've got it!' he exclaimed. 'Yeah, Doc, I've got it! I don't need a mirror-I just need a level. I can't use the spirit levels inside my head, but why couldn't I use levels outside my head-levels I could see, I could use with my eyes?' He took off his gla.s.ses, fingering them thoughtfully, his smile slowly broadening.

'Here, for example, in the rim of my gla.s.ses . . . This could tell me, tell my eyes, if I was tilting. I'd keep an eye on it at first; it would be a real strain. But then it might become second-nature, automatic. Okay, Doc, so what do you think?'

'I think it's a brilliant idea, Mr MacGregor. Let's give it a try.'

The principle was clear, the mechanics a bit tricky. We first experimented with a sort of pendulum, a weighted thread hung from the rims, but this was too close to the eyes, and scarcely seen at all. Then, with the help of our optometrist and workshop, we made a clip extending two nose-lengths forward from the bridge of the spectacles, with a miniature horizontal level fixed to each side. We fiddled with various designs, all tested and modified by Mr MacGregor. In a couple of weeks we had completed a prototype, a pair of somewhat Heath Robinsonish spirit spectacles: 'The world's first pair!' said Mr MacGregor, in glee and triumph. He donned them. They looked a bit c.u.mbersome and odd, but scarcely more so than the bulky hearing-aid spectacles that were coming in at the time. And now a strange sight was to be seen in our Home-Mr MacGregor in the spirit spectacles he had invented and made, his gaze intensely fixed, like a steersman eyeing the binnacle of his ship. This worked, in a fashion-at least he stopped tilting: but it was a continuous, exhausting exercise. And then, over the ensuing weeks, it got easier and easier; keeping an eye on his 'instruments' became unconscious, like keeping an eye on the instrument panel of one's car while being free to think, chat, and do other things.

Mr MacGregor's spectacles became the rage of St. Dunstan's. We had several other patients with Parkinsonism who also suffered from impairment of tilting reactions and postural reflexes-a problem not only hazardous but also notoriously resistant to treatment. Soon a second patient, then a third, were wearing Mr MacGregor's spirit spectacles, and now, like him, could walk upright, on the level.

8.

Eyes Right!

Mrs S., an intelligent woman in her sixties, has suffered a ma.s.sive stroke, affecting the deeper and back portions of her right cerebral hemisphere. She has perfectly preserved intelligence-and humour.

She sometimes complains to the nurses that tney have not put dessert or coffee on her tray. When they say, 'But, Mrs S., it is right there, on the left', she seems not to understand what they say, and does not look to the left. If her head is gently turned, so that the dessert comes into sight, in the preserved right half of her visual field, she says, 'Oh, there is it-it wasn't there before'. She has totally lost the idea of 'left', with regard to both the world and her own body. Sometimes she complains that her portions are too small, but this is because she only eats from the right half of the plate-it does not occur to her that it has a left half as well. Sometimes, she will put on lipstick, and make up the right half of her face, leaving the left half completely neglected: it is almost impossible to treat these things, because her attention cannot be drawn to them ('hemi-inattention'-see Battersby 1956) and she has no conception that they are wrong. She knows it intellectually, and can understand, and laugh; but it is impossible for her to know it directly.

Knowing it intellectually, knowing it inferentially, she has worked out strategies for dealing with her imperception. She cannot look left, directly, she cannot turn left, so what she does is to turn right-and right through a circle. Thus she requested, and was given, a rotating wheelchair. And now if she cannot find something which she knows should be there, she swivels to the right, through a circle, until it comes into view. She finds this signally successful if she cannot find her coffee or dessert. If her portions seem too small, she will swivel to the right, keeping her eyes to the right, until the previously missed half now comes into view; she will eat this, or rather half of this, and feel less hungry than before. But if she is still hungry, or if she thinks on the matter, and realises that she may have perceived only half of the missing half, she will make a second rotation till the remaining quarter comes into view, and, in turn, bisect this yet again. This usually suffices-after all, she has now eaten seven-eighths of the portion-but she may, if she is feeling particularly hungry or obsessive, make a third turn, and secure another sixteenth of her portion (leaving, of course, the remaining sixteenth, the left sixteenth, on her plate). 'It's absurd,' she says. 'I feel like Zeno's arrow-I never get there. It may look funny, but under the circ.u.mstances what else can I do?'

It would seem far simpler for her to rotate the plate than rotate herself. She agrees, and has tried this-or at least tried to try it. But it is oddly difficult, it does not come naturally, whereas whizzing round in her chair does, because her looking, her attention, her spontaneous movements and impulses, are all now exclusively and instinctively to the right.

Especially distressing to her was the derision which greeted her when she appeared only half made-up, the left side of her face absurdly void of lipstick and rouge. 'I look in the mirror,' she said, 'and do all I see.' Would it be possible, we wondered, for her to have a 'mirror' such that she would see the left side of her face on the right? That is, as someone else, facing her, would see her. We tried a video system, with camera and monitor facing her, and the results were startling, and bizarre. For now, using the video screen as a 'mirror', she did see the left side of her face to her right, an experience confounding even to a normal person (as anyone knows who has tried to shave using a video screen), and doubly confounding, uncanny, for her, because the left side of her face and body, which she now saw, had no feeling, no existence, for her, in consequence of her stroke. 'Take it away!' she cried, in distress and bewilderment, so we did not explore the matter further. This is a pity because, as R. L. Gregory also wonders, there might be much promise in such forms of video feedback for such patients with hemi-inattention and left hemi-field extinction. The matter is so physically, indeed metaphysically, confusing that only experiment can decide.

Postscript Computers and computer games (not available in 1976, when I saw Mrs S.) may also be invaluable to patients with unilateral neglect in monitoring the 'missing' half, or teaching them to do this themselves; I have recently (1986) made a short film of this. I could not make reference, in the original edition of this book, to a very important book which came out almost simultaneously: Principles of Behavioral Neurology (Philadelphia: 1985), edited by M. Ma.r.s.el Mesulam. I cannot forbear quoting Mesulam's eloquent formulation of 'neglect': When the neglect is severe, the patient may behave almost as if one half of the universe had abruptly ceased to exist in any meaningful form. . . . Patients with unilateral neglect behave not only as if nothing were actually happening in the left hem-is.p.a.ce, but also as if nothing of any importance could be expected to occur there.

9.

The President's Speech What was going on? A roar of laughter from the aphasia ward, just as the President's speech was coming on, and they had all been so eager to hear the President speaking . . .

There he was, the old Charmer, the Actor, with his practised rhetoric, his histrionisms, his emotional appeal-and all the patients were convulsed with laughter. Well, not all: some looked bewildered, some looked outraged, one or two looked apprehensive, but most looked amused. The President was, as always, moving-but he was moving them, apparently, mainly to laughter. What could they be thinking? Were they failing to understand him? Or did they, perhaps, understand him all too well?

It was often said of these patients, who though intelligent had the severest receptive or global aphasia, rendering them incapable of understanding words as such, that they none the less understood most of what was said to them. Their friends, their relatives, the nurses who knew them well, could hardly believe, sometimes, that they were aphasic.

This was because, when addressed naturally, they grasped some or most of the meaning. And one does speak 'naturally', naturally.

Thus, to demonstrate their aphasia, one had to go to extraordinary lengths, as a neurologist, to speak and behave un-naturally, to remove all the extraverbal cues-tone of voice, intonation, suggestive emphasis or inflection, as well as all visual cues (one's expressions, one's gestures, one's entire, largely unconscious, personal repertoire and posture): one had to remove all of this (which might involve total concealment of one's person, and total deper-sonalisation of one's voice, even to using a computerised voice synthesiser) in order to reduce speech to pure words, speech totally devoid of what Frege called 'tone-colour' (Klangenfarben) or 'evocation'. With the most sensitive patients, it was only with such a grossly artificial, mechanical speech-somewhat like that of the computers in Star Trek-that one could be wholly sure of their aphasia.

Why all this? Because speech-natural speech-does not consist of words alone, nor (as Hughlings Jackson thought) 'propositions' alone. It consists of utterance-an uttering-forth of one's whole meaning with one's whole being-the understanding of which involves infinitely more than mere word-recognition. And this was the clue to aphasiacs' understanding, even when they might be wholly uncomprehending of words as such. For though the words, the verbal constructions, per se, might convey nothing, spoken language is normally suffused with 'tone', embedded in an expressiveness which transcends the verbal-and it is precisely this expressiveness, so deep, so various, so complex, so subtle, which is perfectly preserved in aphasia, though understanding of words be destroyed. Preserved-and often more: preternaturally enhanced . . .

This too becomes clear-often in the most striking, or comic, or dramatic way-to all those who work or live closely with aphasiacs: their families or friends or nurses or doctors. At first, perhaps, we see nothing much the matter; and then we see that there has been a great change, almost an inversion, in their understanding of speech. Something has gone, has been devastated, it is true- but something has come, in its stead, has been immensely enhanced, so that-at least with emotionally-laden utterance-the meaning may be fully grasped even when every word is missed. This, in our species h.o.m.o loquens, seems almost an inversion of the usual order of things: an inversion, and perhaps a reversion too, to something more primitive and elemental. And this perhaps is why Hughlings Jackson compared aphasiacs to dogs (a comparison that might outrage both!) though when he did this he was chiefly thinking of their linguistic incompetences, rather than their remarkable, and almost infallible, sensitivity to 'tone' and feeling. Henry Head, more sensitive in this regard, speaks of 'feeling-tone'

in his (1926) treatise on aphasia, and stresses how it is preserved, and often enhanced, in aphasiacs.*

Thus the feeling I sometimes have-which all of us who work closely with aphasiacs have-that one cannot lie to an aphasiac. He cannot grasp your words, and so cannot be deceived by them; but what he grasps he grasps with infallible precision, namely the expression that goes with the words, that total, spontaneous, involuntary expressiveness which can never be simulated or faked, as words alone can, all too easily . . .

We recognise this with dogs, and often use them for this purpose-to pick up falsehood, or malice, or equivocal intentions, to tell us who can be trusted, who is integral, who makes sense, when we-so susceptible to words-cannot trust our own instincts.

And what dogs can do here, aphasiacs do too, and at a human and immeasurably superior level. 'One can lie with the mouth,' Nietzsche writes, 'but with the accompanying grimace one nevertheless tells the truth.' To such a grimace, to any falsity or impropriety in bodily appearance or posture, aphasiacs are preternatur-ally sensitive. And if they cannot see one-this is especially true of our blind aphasiacs-they have an infallible ear for every vocal nuance, the tone, the rhythm, the cadences, the music, the subtlest modulations, inflections, intonations, which can give-or remove-verisimilitude to or from a man's voice.

In this, then, lies their power of understanding-understanding, without words, what is authentic or inauthentic. Thus it was the grimaces, the histrionisms, the false gestures and, above all, the false tones and cadences of the voice, which rang false for these wordless but immensely sensitive patients. It was to these (for them) most glaring, even grotesque, incongruities and improprieties that "Feeling-tone' is a favourite term of Head's, which he uses in regard not only to aphasia but to the affective quality of sensation, as it may be altered by thalmic or peripheral disorders. Our impression, indeed, is that Head is continually half-uncon-sciously drawn towards the exploration of 'feeling-tone'-towards, so to speak, a neurology of feeling-tone, in contrast or complementarity to a cla.s.sical neurology of proposition and process. It is, incidentally, a common term in the U.S.A., at least among blacks in the South: a common, earthy and indispensable term. 'You see, there's such a thing as a feeling tone . . . And if you don't have this, baby, you've had it' (cited by Studs Terkel as epigraph to his 1967 oral history Division Street: America).

my aphasic patients responded, undeceived and undeceivable by words.

This is why they laughed at the President's speech.

If one cannot lie to an aphasiac, in view of his special sensitivity to expression and 'tone', how is it, we might ask, with patients- if there are such-who lack any sense of expression and 'tone', while preserving, unchanged, their comprehension for words: patients of an exactly opposite kind? We have a number of such patients, also on the aphasia ward, although, technically, they do not have aphasia, but, instead, a form of agnosia, in particular a so-called 'tonal' agnosia. For such patients, typically, the expressive qualities of voices disappear-their tone, their timbre, their feeling, their entire character-while words (and grammatical constructions) are perfectly understood. Such tonal agnosias (or 'apro-sodias') are a.s.sociated with disorders of the right temporal lobe of the brain, whereas the aphasias go with disorders of the left temporal lobe.

Among the patients with tonal agnosia on our aphasia ward who also listened to the President's speech was Emily D., with a glioma in her right temporal lobe. A former English teacher, and poetess of some repute, with an exceptional feeling for language, and strong powers of a.n.a.lysis and expression, Emily D. was able to articulate the opposite situation-how the President's speech sounded to someone with tonal agnosia. Emily D. could no longer tell if a voice was angry, cheerful, sad-whatever. Since voices now lacked expression, she had to look at people's faces, their postures and movements when they talked, and found herself doing so with a care, an intensity, she had never shown before. But this, it so happened, was also limited, because she had a malignant glaucoma, and was rapidly losing her sight too.

What she then found she had to do was to pay extreme attention to exactness of words and word use, and to insist that those around her did just the same. She could less and less follow loose speech or slang-speech of an allusive or emotional kind-and more and more required of her interlocutors that they speak prose-'proper words in proper places'. Prose, she found, might compensate, in some degree, for lack of perceived tone or feeling. In this way she was able to preserve, even enhance, the use of 'expressive' speech-in which the meaning was wholly given by the apt choice and reference of words-despite being more and more lost with 'evocative' speech (where meaning is wholly given in the use and sense of tone).

Emily D. also listened, stony-faced, to the President's speech, bringing to it a strange mixture of enhanced and defective perceptions-precisely the opposite mixture to those of our aphasiacs. It did not move her-no speech now moved her-and all that was evocative, genuine or false completely pa.s.sed her by. Deprived of emotional reaction, was she then (like the rest of us) transported or taken in? By no means. 'He is not cogent,' she said. 'He does not speak good prose. His word-use is improper. Either he is braindamaged, or he has something to conceal.' Thus the President's speech did not work for Emily D. either, due to her enhanced sense of formal language use, propriety as prose, any more than it worked for our aphasiacs, with their word-deafness but enhanced sense of tone.

Here then was the paradox of the President's speech. We normals-aided, doubtless, by our wish to be fooled, were indeed well and truly fooled ('Populus vult decipi, ergo decipiatur'). And so cunningly was deceptive word-use combined with deceptive tone, that only the brain-damaged remained intact, undeceived.

PART TWO.

EXCESSES.

Introduction.

'Deficit', we have said, is neurology's favourite word-its only word, indeed, for any disturbance of function. Either the function (like a capacitor or fuse) is normal-or it is defective or faulty: what other possibility is there for a mechanistic neurology, which is essentially a system of capacities and connections?

What then of the opposite-an excess or superabundance of function? Neurology has no word for this-because it has no concept. A function, or functional system, works-or it does not: these are the only possibilities it allows. Thus a disease which is 'ebullient' or 'productive' in character challenges the basic mechanistic concepts of neurology, and this is doubtless one reason why such disorders-common, important, and intriguing as they are-have never received the attention they deserve. They receive it in psychiatry, where one speaks of excited and productive disorders- extravagances of fancy, of impulse . . . of mania. And they receive it in anatomy and pathology, where one speaks of hypertrophies, monstrosities-of teratoma. But physiology has no equivalent for this-no equivalent of monstrosities or manias. And this alone suggests that our basic concept or vision of the nervous system- as a sort of machine or computer-is radically inadequate, and needs to be supplemented by concepts more dynamic, more alive.

This radical inadequacy may not be apparent when we consider only loss-the privation of functions we considered in Part One. But it becomes immediately obvious if we consider their excesses- not amnesia, but hypermnesia; not agnosia, but hypergnosia; and all the other 'hypers' we can imagine.

Cla.s.sical, 'Jacksonian' neurology never considers such disorders of excess-that is, primary superabundances or burgeonings of functions (as opposed to so-called 'releases'). Hughlings Jackson himself, it is true, did speak of 'hyper-physiological' and 'super-positive' states. But here, we might say, he is letting himself go, being playful, or, simply, just being faithful to his clinical experience, though at odds with his own mechanical concepts of function (such contradictions were characteristic of his genius, the chasm between his naturalism and his rigid formalism).

We have to come almost to the present day to find a neurologist who even considers an excess. Thus Luria's two clinical biographies are nicely balanced: The Man with a Shattered World is about loss, The Mind of a Mnemonist about excess. I find the latter by far the more interesting and original of the two, for it is, in effect, an exploration of imagination and memory (and no such exploration is possible to cla.s.sical neurology).

In Awakenings there was an internal balance, so to speak, between the terrible privations seen before L-Dopa-akinesia, abou-lia, adynamia, anergia, etc.-and the almost equally terrible excesses after L-Dopa-hyperkinesia, hyperboulia, hyperdynamia, etc.

And in this we see the emergence of a new sort of term, of terms and concepts other than those of function-impulse, will, dynamism, energy-terms essentially kinetic and dynamic (whereas those of cla.s.sical neurology are essentially static). And, in the mind of the Mnemonist, we see dynamisms of a much higher order at work-the thrust of an ever-burgeoning and almost uncontrollable a.s.sociation and imagery, a monstrous growth of thinking, a sort of teratoma of the mind, which the Mnemonist himself calls an 'It'.

But the word 'It', or automatism, is also too mechanical. 'Burgeoning' conveys better the disquietingly alive quality of the process. We see in the Mnemonist-or in my own overenergised, galvanised patients on L-Dopa-a sort of animation gone extravagant, monstrous, or mad-not merely an excess, but an organic proliferation, a generation; not just an imbalance, a disorder of function, but a disorder of generation.

We might imagine, from a case of amnesia or agnosia, that there is merely a function or competence impaired-but we see from patients with hypermnesias and hypergnosias that mnesis and gnosis are inherently active, and generative, at all times; inherently, and-potentially-monstrously as well. Thus we are forced to move from a neurology of function to a neurology of action, of life. This crucial step is forced upon us by the diseases of excess- and without it we cannot begin to explore the 'life of the mind'. Traditional neurology, by its mechanicalness, its emphasis on deficits, conceals from us the actual life which is instinct in all cerebral functions-at least higher functions such as those of imagination, memory and perception. It conceals from us the very life of the mind. It is with these living (and often highly personal) dispositions of brain and mind-especially in a state of enhanced, and thus illuminated, activity-that we shall be concerned now.

Enhancement allows the possibilities not only of a healthy fullness and exuberance, but of a rather ominous extravagance, aberration, monstrosity-the sort of 'too-muchness' which continually loomed in Awakenings, as patients, over-excited, tended to disintegration and uncontrol; an overpowering by impulse, image and will; possession (or dispossession) by a physiology gone wild.

This danger is built into the very nature of growth and life. Growth can become over-growth, life 'hyper-life'. All the 'hyper' states can become monstrous, perverse aberrations, 'para' states: hyperkinesia tends towards parakinesia-abnormal movements, ch.o.r.ea, tics; hypergnosia readily becomes paragnosia-perversions, apparitions, of the morbidly-heightened senses; the ardours of 'hyper' states can become violent pa.s.sions.

The paradox of an illness which can present as wellness-as a wonderful feeling of health and well-being, and only later reveal its malignant potentials-is one of the chimaeras, tricks and ironies of nature. It is one which has fascinated a number of artists, especially those who equate art with sickness: thus it is a theme-at once Dionysiac, Venerean and Faustian-which persistently recurs in Thomas Mann-from the febrile tuberculous highs of The Magic Mountain, to the spirochetal inspirations in Dr Faustus and the aphrodisiac malignancy in his last tale, The Black Swan.

I have always been intrigued by such ironies, and have written of them before. In Migraine I spoke of the high which may precede, or const.i.tute the start of, attacks-and quoted George Eliot's remark that feeling 'dangerously well' was often, for her, the sign or harbinger of an attack. 'Dangerously well'-what an irony is this: it expresses precisely the doubleness, the paradox, of feeling 'too well'.

For 'wellness', naturally, is no cause for complaint-people relish it, they enjoy it, they are at the furthest pole from complaint. People complain of feeling ill-not well. Unless, as George Eliot does, they have some intimation of 'wrongness', or danger, either through knowledge or a.s.sociation, or the very excess of excess. Thus, though a patient will scarcely complain of being 'very well', they may become suspicious if they feel 'too well'.

This was a central, and (so to speak) cruel, theme in Awakenings, that patients profoundly ill, with the profoundest deficits, for many decades, might find themselves, as by a miracle, suddenly well, only to move from there into the hazards, the tribulations, of excess, functions stimulated far beyond 'allowable' limits. Some patients realised this, had premonitions-but some did not. Thus Rose R., in the first flush and joy of restored health, said, 'It's fabulous, it's gorgeous!', but as things accelerated towards uncon-trol said, 'Things can't last. Something awful is coming.' And similarly, with more or less insight, in most of the others-as with Leonard L., as he pa.s.sed from repletion to excess: 'his abundance of health and energy-of "grace", as he called it-became too abundant, and started to a.s.sume an extravagant form. His sense of harmony and ease and effortless control was replaced by a sense of too-muchness ... a great surplus, a great pressure of . . . [every kind]', which threatened to disintegrate him, to burst him asunder.

This is the simultaneous gift and affliction, the delight, the anguish, conferred by excess. And it is felt, by insightful patients, as questionable and paradoxical: 'I have too much energy,' one Tourette patient said. 'Everything is too bright, too powerful, too much. It is a feverish energy, a morbid brilliance.'

'Dangerous wellness', 'morbid brilliance', a deceptive euphoria with abysses beneath-this is the trap promised and threatened by excess, whether it be set by Nature, in the form of some intoxicating disorder, or by ourselves, in the form of some excitant addiction.

The human dilemmas, in such situations, are of an extraordinary kind: for patients are here faced with disease as seduction, something remote from, and far more equivocal than, the traditional theme of illness as suffering or affliction. And n.o.body, absolutely n.o.body, is exempt from such bizarrenesses, such indignities. In disorders of excess there may be a sort of collusion, in which the self is more and more aligned and identified with its sickness, so that finally it seems to lose all independent existence, and be nothing but a product of sickness. This fear is expressed by Witty Ticcy Ray in Chapter Ten when he says: 'I consist of tics-there is nothing else', or when he envisages a mind-growth- a 'Tourettoma'-which might engulf him. For him, with his strong ego, and relatively mild Tourette's syndrome, there was not, in reality, any such danger. But for patients with weak or undeveloped egos, coupled with overwhelmingly strong disease, there is a very real risk of such 'possession' or 'dispossession'. I do no more than touch on this in 'The Possessed'.

10.

Witty Ticcy Ray In 1885 Gilles de la Tourette, a pupil of Charcot, described the astonishing syndrome which now bears his name. Tourette's syndrome', as it was immediately dubbed, is characterised by an excess of nervous energy, and a great production and extravagance of strange motions and notions: tics, jerks, mannerisms, grimaces, noises, curses, involuntary imitations and compulsions of all sorts, with an odd elfin humour and a tendency to antic and outlandish kinds of play. In its 'highest' forms, Tourette's syndrome involves every aspect of the affective, the instinctual and the imaginative life; in its 'lower', and perhaps commoner, forms, there may be little more than abnormal movements and impulsivity, though even here there is an element of strangeness. It was well recognised and extensively reported in the closing years of the last century, for these were years of a s.p.a.cious neurology which did not hesitate to conjoin the organic and the psychic. It was clear to Tourette, and his peers, that this syndrome was a sort of possession by primitive impulses and urges: but also that it was a possession with an organic basis-a very definite (if undiscovered) neurological disorder.

In the years that immediately followed the publication of Tourette's original papers many hundreds of cases of this syndrome were described-no two cases ever being quite the same. It became clear that there were forms which were mild and benign, and others of quite terrible grotesqueness and violence. Equally, it was clear that some people could 'take' Tourette's, and accommodate it within a commodious personality, even gaining advantage from the swiftness of thought and a.s.sociation and invention which went with it, while others might indeed be 'possessed' and scarcely able to achieve real ident.i.ty amid the tremendous pressure and chaos of Tourettic impulses. There was always, as Luria remarked of his mnemonist, a fight between an 'It' and an T.