The man who mistook his wife for a hat

by Oliver Sacks

  "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 classical 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 associated 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 analysis 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 passed 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.

  Classical, '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 classical 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 classical 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 association 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 fo
rced 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, chorea, tics; hypergnosia readily becomes paragnosia-perversions, apparitions, of the morbidly-heightened senses; the ardours of 'hyper' states can become violent passions.

  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 constitute 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 association, 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 passed from repletion to excess: 'his abundance of health and energy-of "grace", as he called it-became too abundant, and started to assume 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 nobody, absolutely nobody, 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 spacious 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 association and invention which went with it,

  while others might indeed be 'possessed' and scarcely able to achieve real identity 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.

  Charcot and his pupils, who included Freud and Babinski as well as Tourette, were among the last of their profession with a combined vision of body and soul, 'It', and T, neurology and psychiatry. By the turn of the century, a split had occurred, into a soulless neurology and a bodiless psychology, and with this any understanding of Tourette's disappeared. In fact, Tourette's syndrome itself seemed to have disappeared, and was scarcely at all reported in the first half of this century. Some physicians, indeed, regarded it as 'mythical', a product of Tourette's colourful imagination; most had never heard of it. It was as forgotten as the great sleepy-sickness epidemic of the 1920s.

  The forgetting of sleepy-sickness (encephalitis lethargica) and the forgetting of Tourette's have much in common. Both disorders were extraordinary, and strange beyond belief-at least, the beliefs of a contracted medicine. They could not be accommodated in the conventional frameworks of medicine, and therefore they were forgotten and mysteriously 'disappeared'. But there is a much mor
e intimate connection, which was hinted at in the 1920s, in the hyperkinetic or frenzied forms which the sleepy-sickness sometimes took: these patients tended, at the beginning of their illness, to show a mounting excitement of mind and body, violent movements, tics, compulsions of all kinds. Some time afterwards, they were overtaken by an opposite fate, an all-enveloping trance-like 'sleep'-in which I found them forty years later.

  In 1969, I gave these sleepy-sickness or post-encephalitic patients L-Dopa, a precursor of the transmitter dopamine, which was greatly lowered in their brains. They were transformed by it. First they were 'awakened' from stupor to health: then they were driven towards the other pole-of tics and frenzy. This was my first experience of Tourette-like syndromes: wild excitements, violent impulses, often combined with a weird, antic humour. I started to speak of 'Tourettism', although I had never seen a patient with Tourette's.

  Early in 1971, the Washington Post, which had taken an interest in the 'awakening' of my post-encephalitic patients, asked me how they were getting on. I replied, 'They are ticcing', which prompted them to publish an article on 'Tics'. After the publication of this article, I received countless letters, the majority of which I passed on to my colleagues. But there was one patient I did consent to see-Ray.

  The day after I saw Ray, it seemed to me that I noticed three Touretters in the street in downtown New York. I was confounded, for Tourette's syndrome was said to be excessively rare. It had an incidence, I had read, of one in a million, yet I had apparently seen three examples in an hour. I was thrown into a turmoil of bewilderment and wonder: was it possible that I had been overlooking this all the time, either not seeing such patients or vaguely dismissing them as 'nervous', 'cracked', 'twitchy'? Was it possible that everyone had been overlooking them? Was it possible that Tourette's was not a rarity, but rather common-a thousand times more common, say, than previously supposed? The next day, without specially looking, I saw another two in the street. At this point I conceived a whimsical fantasy or private joke: suppose (I said to myself) that Tourette's is very common but fails to be recognised but once recognised is easily and constantly seen.* Suppose one such Touretter recognises another, and these two a third, and these three a fourth, until, by incrementing recognition, a whole band of them is found: brothers and sisters in pathology, a new species in our midst, joined together by mutual recognition and concern? Could there not come together, by such spontaneous aggregation, a whole association of New Yorkers with Tourette's?