The man who mistook his wife for a hat Page 2

  This dynamic, this 'striving to preserve identity', however strange the means or effects of such striving, was recognised in psychiatry long ago-and, like so much else, is especially associated with the work of Freud. Thus, the delusions of paranoia were seen by him not as primary but as attempts (however misguided) at restitution, at reconstructing a world reduced by complete chaos. In precisely the same way, Ivy McKenzie wrote:

  The pathological physiology of the Parkinsonian syndrome is the study of an organised chaos, a chaos induced in the first instance by destruction of important integrations, and reorganised on an unstable basis in the process of rehabilitation.

  As Awakenings was the study of 'an organised chaos' produced by a single if multiform disease, so what now follows is a series of similar studies of the organised chaoses produced by a great variety of diseases.

  In this first section, 'Losses', the most important case, to my mind, is that of a special form of visual agnosia: 'The Man Who Mistook His Wife for a Hat'. I believe it to be of fundamental importance. Such cases constitute a radical challenge to one of the most entrenched axioms or assumptions of classical neurology-in particular, the notion that brain damage, any brain damage, reduces or removes the 'abstract and categorical attitude' (in Kurt Goldstein's term), reducing the individual to the emotional and concrete. (A very similar thesis was made by Hughlings Jackson in the 1860s.) Here, in the case of Dr P., we see the very opposite of this-a man who has (albeit only in the sphere of the visual) wholly lost the emotional, the concrete, the personal, the 'real' . . . and been reduced, as it were, to the abstract and the categorical, with consequences of a particularly preposterous kind. What would Hughlings Jackson and Goldstein have said of this? I have often in imagination, asked them to examine Dr P., and then said, 'Gentlemen! What do you say now?'

  1

  The Man Who Mistook His Wife for a Hat

  Dr P. was a musician of distinction, well-known for many years as a singer, and then, at the local School of Music, as a teacher. It was here, in relation to his students, that certain strange problems were first observed. Sometimes a student would present himself, and Dr P. would not recognise him; or, specifically, would not recognise his face. The moment the student spoke, he would be recognised by his voice. Such incidents multiplied, causing embarrassment, perplexity, fear-and, sometimes, comedy. For not only did Dr P. increasingly fail to see faces, but he saw faces when there were no faces to see: genially, Magoo-like, when in the street he might pat the heads of water hydrants and parking meters, taking these to be the heads of children; he would amiably address carved knobs on the furniture and be astounded when they did not reply. At first these odd mistakes were laughed off as jokes, not least by Dr P. himself. Had he not always had a quirky sense of humour and been given to Zen-like paradoxes and jests? His musical powers were as dazzling as ever; he did not feel ill-he had never felt better; and the mistakes were so ludicrous-and so ingenious-that they could hardly be serious or betoken anything serious. The notion of there being 'something the matter' did not emerge until some three years later, when diabetes developed. Well aware that diabetes could affect his eyes, Dr P. consulted an ophthalmologist, who took a careful history and examined his eyes closely. 'There's nothing the matter with your eyes,' the doctor concluded. 'But there is trouble with the visual parts of your brain.

  You don't need my help, you must see a neurologist.' And so, as a result of this referral, Dr P. came to me.

  It was obvious within a few seconds of meeting him that there was no trace of dementia in the ordinary sense. He was a man of great cultivation and charm who talked well and fluently, with imagination and humour. I couldn't think why he had been referred to our clinic.

  And yet there was something a bit odd. He faced me as he spoke, was oriented towards me, and yet there was something the matter-it was difficult to formulate. He faced me with his ears, I came to think, but not with his eyes. These, instead of looking, gazing, at me, 'taking me in', in the normal way, made sudden strange fixations-on my nose, on my right ear, down to my chin, up to my right eye-as if noting (even studying) these individual features, but not seeing my whole face, its changing expressions, 'me', as a whole. I am not sure that I fully realised this at the time-there was just a teasing strangeness, some failure in the normal interplay of gaze and expression. He saw me, he scanned me, and yet . . .

  'What seems to be the matter?' I asked him at length.

  'Nothing that I know of,' he replied with a smile, 'but people seem to think there's something wrong with my eyes.'

  'But you don't recognise any visual problems?'

  'No, not directly, but I occasionally make mistakes.'

  I left the room briefly to talk to his wife. When I came back, Dr P. was sitting placidly by the window, attentive, listening rather than looking out. 'Traffic,' he said, 'street sounds, distant trains- they make a sort of symphony, do they not? You know Honegger's Pacific 234?'

  What a lovely man, I thought to myself. How can there be anything seriously the matter? Would he permit me to examine him?

  'Yes, of course, Dr Sacks.'

  I stilled my disquiet, his perhaps, too, in the soothing routine of a neurological exam-muscle strength, coordination, reflexes, tone. … It was while examining his reflexes-a trifle abnormal on the left side-that the first bizarre experience occurred. I had

  taken off his left shoe and scratched the sole of his foot with a key-a frivolous-seeming but essential test of a reflex-and then, excusing myself to screw my ophthalmoscope together, left him to put on the shoe himself. To my surprise, a minute later, he had not done this.

  'Can I help?' I asked.

  'Help what? Help whom?'

  'Help you put on your shoe.'

  'Ach,' he said, 'I had forgotten the shoe,' adding, sotto voce, 'The shoe? The shoe?' He seemed baffled.

  'Your shoe,' I repeated. 'Perhaps you'd put it on.'

  He continued to look downwards, though not at the shoe, with an intense but misplaced concentration. Finally his gaze settled on his foot: 'That is my shoe, yes?'

  Did I mis-hear? Did he mis-see?

  'My eyes,' he explained, and put a hand to his foot. 'This is my shoe, no?'

  'No, it is not. That is your foot. There is your shoe.'

  'Ah! I thought that was my foot.'

  Was he joking? Was he mad? Was he blind? If this was one of his 'strange mistakes', it was the strangest mistake I had ever come across.

  I helped him on with his shoe (his foot), to avoid further complication. Dr P. himself seemed untroubled, indifferent, maybe amused. I resumed my examination. His visual acuity was good: he had no difficulty seeing a pin on the floor, though sometimes he missed it if it was placed to his left.

  He saw all right, but what did he see? I opened out a copy of the National Geographic Magazine and asked him to describe some pictures in it.

  His responses here were very curious. His eyes would dart from one thing to another, picking up tiny features, individual features, as they had done with my face. A striking brightness, a colour, a shape would arrest his attention and elicit comment-but in no case did he get the scene-as-a-whole. He failed to see the whole, seeing only details, which he spotted like blips on a radar screen. He never entered into relation with the picture as a whole-never

  faced, so to speak, its physiognomy. He had no sense whatever of a landscape or scene.

  I showed him the cover, an unbroken expanse of Sahara dunes.

  'What do you see here?' I asked.

  'I see a river,' he said. 'And a little guest-house with its terrace on the water. People are dining out on the terrace. I see coloured parasols here and there.' He was looking, if it was 'looking', right off the cover into mid-air and confabulating nonexistent features, as if the absence of features in the actual picture had driven him to imagine the river and the terrace and the coloured parasols.

  I must have looked aghast,
but he seemed to think he had done rather well. There was a hint of a smile on his face. He also appeared to have decided that the examination was over and started to look around for his hat. He reached out his hand and took hold of his wife's head, tried to lift it off, to put it on. He had apparently mistaken his wife for a hat! His wife looked as if she was used to such things.

  I could make no sense of what had occurred in terms of conventional neurology (or neuropsychology). In some ways he seemed perfectly preserved, and in others absolutely, incomprehensibly devastated. How could he, on the one hand, mistake his wife for a hat and, on the other, function, as apparently he still did, as a teacher at the Music School?

  I had to think, to see him again-and to see him in his own familiar habitat, at home.

  A few days later I called on Dr P. and his wife at home, with the score of the Dichterliebe in my briefcase (I knew he liked Schumann), and a variety of odd objects for the testing of perception. Mrs P. showed me into a lofty apartment, which recalled fin-de-siecle Berlin. A magnificent old Bosendorfer stood in state in the centre of the room, and all around it were music stands, instruments, scores. . . . There were books, there were paintings, but the music was central. Dr P. came in, a little bowed, and, distracted, advanced with outstretched hand to the grandfather clock, but, hearing my voice, corrected himself, and shook hands with me. We exchanged greetings and chatted a little of current

  concerts and performances. Diffidently, I asked him if he would sing.

  The Dichterliebe!' he exclaimed. 'But I can no longer read music. You will play them, yes?'

  I said I would try. On that wonderful old piano even my playing sounded right, and Dr P. was an aged but infinitely mellow Fischer-Dieskau, combining a perfect ear and voice with the most incisive musical intelligence. It was clear that the Music School was not keeping him on out of charity.

  Dr P. 's temporal lobes were obviously intact: he had a wonderful musical cortex. What, I wondered, was going on in his parietal and occipital lobes, especially in those areas where visual processing occurred? I carry the Platonic solids in my neurological kit and decided to start with these.

  'What is this?' I asked, drawing out the first one.

  'A cube, of course.'

  'Now this?' I asked, brandishing another.

  He asked if he might examine it, which he did swiftly and systematically: 'A dodecahedron, of course. And don't bother with the others-I'll get the icosahedron, too.'

  Abstract shapes clearly presented no problems. What about faces? I took out a pack of cards. All of these he identified instantly, including the jacks, queens, kings, and the joker. But these, after all, are stylised designs, and it was impossible to tell whether he saw faces or merely patterns. I decided I would show him a volume of cartoons which I had in my briefcase. Here, again, for the most part, he did well. Churchill's cigar, Schnozzle's nose: as soon as he had picked out a key feature he could identify the face. But cartoons, again, are formal and schematic. It remained to be seen how he would do with real faces, realistically represented.

  I turned on the television, keeping the sound off, and found an early Bette Davis film. A love scene was in progress. Dr P. failed to identify the actress-but this could have been because she had never entered his world. What was more striking was that he failed to identify the expressions on her face or her partner's, though in the course of a single torrid scene these passed from sultry yearning through passion, surprise, disgust, and fury to a melting reconcil-

  iation. Dr P. could make nothing of any of this. He was very unclear as to what was going on, or who was who or even what sex they were. His comments on the scene were positively Martian.

  It was just possible that some of his difficulties were associated with the unreality of a celluloid, Hollywood world; and it occurred to me that he might be more successful in identifying faces from his own life. On the walls of the apartment there were photographs of his family, his colleagues, his pupils, himself. I gathered a pile of these together and, with some misgivings, presented them to him. What had been funny, or farcical, in relation to the movie, was tragic in relation to real life. By and large, he recognised nobody: neither his family, nor his colleagues, nor his pupils, nor himself. He recognised a portrait of Einstein because he picked up the characteristic hair and moustache; and the same thing happened with one or two other people. 'Ach, Paul!' he said, when shown a portrait of his brother. 'That square jaw, those big teeth- I would know Paul anywhere!' But was it Paul he recognised, or one or two of his features, on the basis of which he could make a reasonable guess as to the subject's identity? In the absence of obvious 'markers', he was utterly lost. But it was not merely the cognition, the gnosis, at fault; there was something radically wrong with the whole way he proceeded. For he approached these faces- even of those near and dear-as if they were abstract puzzles or tests. He did not relate to them, he did not behold. No face was familiar to him, seen as a 'thou', being just identified as a set of features, an 'it'. Thus, there was formal, but no trace of personal, gnosis. And with this went his indifference, or blindness, to expression. A face, to us, is a person looking out-we see, as it were, the person through his persona, his face. But for Dr P. there was no persona in this sense-no outward persona, and no person within.

  I had stopped at a florist on my way to his apartment and bought myself an extravagant red rose for my buttonhole. Now I removed this and handed it to him. He took it like a botanist or morphol-ogist given a specimen, not like a person given a flower.

  About six inches in length,' he commented. 'A convoluted red form with a linear green attachment.'

  'Yes,' I said encouragingly, 'and what do you think it is, Dr P.?'

  'Not easy to say.' He seemed perplexed. 'It lacks the simple symmetry of the Platonic solids, although it may have a higher symmetry of its own. … I think this could be an inflorescence or flower.'

  'Could be?' I queried.

  'Could be,' he confirmed.

  'Smell it,' I suggested, and he again looked somewhat puzzled, as if I had asked him to smell a higher symmetry. But he complied courteously, and took it to his nose. Now, suddenly, he came to life.

  'Beautiful!' he exclaimed. 'An early rose. What a heavenly smell!' He started to hum 'Die Rose, die Lillie . . .' Reality, it seemed, might be conveyed by smell, not by sight.

  I tried one final test. It was still a cold day, in early spring, and I had thrown my coat and gloves on the sofa.

  'What is this?' I asked, holding up a glove.

  'May I examine it?' he asked, and, taking it from me, he proceeded to examine it as he had examined the geometrical shapes.

  'A continuous surface,' he announced at last, 'infolded on itself. It appears to have'-he hesitated-'five outpouchings, if this is the word.'

  'Yes,' I said cautiously. You have given me a description. Now tell me what it is.'

  'A container of some sort?'

  Yes,' I said, 'and what would it contain?'

  'It would contain its contents!' said Dr P., with a laugh. 'There are many possibilities. It could be a change purse, for example, for coins of five sizes. It could . . .'

  I interrupted the barmy flow. 'Does it not look familiar? Do you think it might contain, might fit, a part of your body?'

  No light of recognition dawned on his face.*

  No child would have the power to see and speak of 'a contin-

  * Later, by accident, he got it on, and exclaimed, 'My God, it's a glove!' This was reminiscent of Kurt Goldstein's patient 'Lanuti', who could only recognise objects by trying to use them in action.

  uous surface . . . infolded on itself,' but any child, any infant, would immediately know a glove as a glove, see it as familiar, as going with a hand. Dr P. didn't. He saw nothing as familiar. Visually, he was lost in a world of lifeless abstractions. Indeed, he did not have a real visual world, as he did not have a real visual self. He could speak about things, but
did not see them face-to-face. Hughlings Jackson, discussing patients with aphasia and left-hemisphere lesions, says they have lost 'abstract' and 'propositional' thought-and compares them with dogs (or, rather, he compares dogs to patients with aphasia). Dr P., on the other hand, functioned precisely as a machine functions. It wasn't merely that he displayed the same indifference to the visual world as a computer but-even more strikingly-he construed the world as a computer construes it, by means of key features and schematic relationships. The scheme might be identified-in an 'identi-kit' way-without the reality being grasped at all.

  The testing I had done so far told me nothing about Dr P.'s inner world. Was it possible that his visual memory and imagination were still intact? I asked him to imagine entering one of our local squares from the north side, to walk through it, in imagination or in memory, and tell me the buildings he might pass as he walked. He listed the buildings on his right side, but none of those on his left. I then asked him to imagine entering the square from the south. Again he mentioned only those buildings that were on the right side, although these were the very buildings he had omitted before. Those he had 'seen' internally before were not mentioned now; presumably, they were no longer 'seen'. It was evident that his difficulties with leftness, his visual field deficits, were as much internal as external, bisecting his visual memory and imagination.

  What, at a higher level, of his internal visualisation? Thinking of the almost hallucinatory intensity with which Tolstoy visualises and animates his characters, I questioned Dr P. about Anna Kar-enina. He could remember incidents without difficulty, had an undiminished grasp of the plot, but completely omitted visual characteristics, visual narrative, and scenes. He remembered the words of the characters but not their faces; and though, when

  asked, he could quote, with his remarkable and almost verbatim memory, the original visual descriptions, these were, it became apparent, quite empty for him and lacked sensorial, imaginal, or emotional reality. Thus, there was an internal agnosia as well*