The man who mistook his wife for a hat

by Oliver Sacks

  The autistic, by their nature, are seldom open to influence. It is their 'fate' to be isolated, and thus original. Their 'vision', if it can be glimpsed, comes from within and appears aboriginal. They seem to me, as I see more of them, to be a strange species in our midst, odd, original, wholly inwardly directed, unlike others.

  Autism was once seen as a childhood schizophrenia, but phe-nomenologically the reverse is the case. The schizophrenic's complaint is always of 'influence' from the outside: he is passive, he is played upon, he cannot be himself. The autistic would complain-if they complained-of absence of influence, of absolute isolation.

  'No man is an island, entire of itself,' wrote Donne. But this is precisely what autism is-an island, cut off from the main. In 'classical' autism, which is manifest, and often total, by the third year of life, the cutting off is so early there may be no memory of

  the main. In 'secondary' autism, like Jose's, caused by brain disease at a later stage in life, there is some memory, perhaps some nostalgia, for the main. This may explain why Jose was more accessible than most, and why, at least in drawing, he may show interplay taking place.

  Is being an island, being cut off, necessarily a death? It may be a death, but it is not necessarily so. For though 'horizontal' connections with others, with society and culture, are lost, yet there may be vital and intensified 'vertical' connections, direct connections with nature, with reality, uninfluenced, unmediated, untouchable, by any others. This 'vertical' contact is very striking with Jose, hence the piercing directness, the absolute clarity of his perceptions and drawings, without a hint or shade of ambiguity or indirection, a rocklike power uninfluenced by others.

  This brings us to our final question: is there any 'place' in the world for a man who is like an island, who cannot be acculturated, made part of the main? Can 'the main' accommodate, make room for, the singular? There are similarities here to the social and cultural reactions to genius. (Of course I do not suggest that all autists have genius, only that they share with genius the problem of singularity.) Specifically: what does the future hold for Jose? Is there some 'place' for him in the world which will employ his autonomy, but leave it intact?

  Could he, with his fine eye, and great love of plants, make illustrations for botanical works or herbals? Be an illustrator for zoology or anatomy texts? (See the drawing overleaf he made for me when I showed him a textbook illustration of the layered tissue called 'ciliated epithelium'.) Could he accompany scientific expeditions, and make drawings (he paints and makes models with equal facility) of rare species? His pure concentration on the thing before him would make him ideal in such situations.

  Or, to take a strange but not illogical leap, could he, with his peculiarities, his idiosyncrasy, do drawings for fairy tales, nursery tales, Bible tales, myths? Or (since he cannot read, and sees letters only as pure and beautiful forms) could he not illustrate, and elaborate, the gorgeous capitals of manuscript breviaries and missals? He has done beautiful altarpieces, in mosaic and stained

  wood, for churches. He has carved exquisite lettering on tombstones. His current 'job' is hand-printing sundry notices for the ward, which he does with the flourishes and elaborations of a latter-day Magna Carta. All this he could do, and do very well. And it would be of use and delight to others, and delight him too. He could do all of these-but, alas, he will do none, unless someone very understanding, and with opportunities and means, can guide and employ him. For, as the stars stand, he will probably do nothing, and spend a useless, fruitless life, as so many other autistic people do, overlooked, unconsidered, in the back ward of a state hospital.

  Postscript

  After publication of this piece, I again received many offprints and letters, the most interesting being from Dr C.C. Park. It is indeed clear (as Nigel Dennis suspected) that even though 'Nadia' may

  Ciliated epithelium from the trachea of a kitten (magnified 255 times).

  have been unique-a sort of Picasso-artistic gifts of fairly high order are not uncommon among the autistic. Testing for artistic potential, as in the Goodenough 'Draw-a-Man' intelligence test, is almost useless: there must occur, as with 'Nadia', Jose and the Parks' 'Ella', a spontaneous production of striking drawings.

  In an important and richly illustrated review of'Nadia', Dr Park (1978) brings out, on the basis of experience with her own child, no less than from a perusal of the world literature, what seem to be the cardinal characteristics of such drawings. These include 'negative' characteristics, such as derivativeness and stereotypy, and 'positive' ones, such as an unusual capacity for delayed rendition, and for rendering the object as perceived (not as conceived): hence the sort of inspired naivete especially seen. She also notes a relative indifference to display of others' reactions, which might seem to render such children untrainable. And yet, manifestly, this need not be the case. Such children are not necessarily unresponsive to teaching or attention, though this may need to be of a very special type.

  In addition to experience with her own child, who is now an accomplished adult artist, Dr Park cites also the fascinating and insufficiently known experiences of the Japanese, especially Mor-ishima and Motzugi, who have had remarkable success in bringing autists from an untutored (and seemingly unteachable) childhood giftedness to professionally accomplished adult artistry. Morishima favours special instructional techniques ('highly structured skill training'), a sort of apprenticeship in the classical Japanese cultural tradition, and encouragement of drawing as a means of communication. But such formal training, though crucial, is not enough. A most intimate, empathic relationship is required. The words with which Dr Park concludes her review may properly conclude 'The World of the Simple':

  The secret may lie elsewhere, in the dedication that led Motzugi to live with another retarded artist in his home, and to write: 'The secret in developing Yanamura's talent was to share his spirit. The teacher should love the beautiful, honest retarded person, and live with a purified, retarded world.'

  Bibliography

  GENERAL REFERENCES

  Hughlings Jackson, Kurt Goldstein, Henry Head, A. R. Luria-these are the fathers of neurology who lived intensely, and thought intensely, about patients and problems not so dissimilar to our own. They are always present, in the neurologist's mind, and they haunt the pages of this book. There is a tendency to reduce complex figures to stereotypes, to disallow the fullness, and often the rich contradictoriness, of their thought. Thus 1 often talk about classical "Jacksonian" neurology, but the Hughlings Jackson who wrote of "dreamy states" and "reminiscence" was very different from the Jackson who saw all thought as propositional calculus. The former was a poet, the latter a logician, and yet they are one and the same man. Henry Head the diagram-maker, with his passion for schematics, was very different from the Head who wrote poignantly of "feeling-tone." Goldstein, who wrote so abstractly of "the Abstract," delighted in the rich concreteness of individual cases. In Luria, finally, the doubleness was conscious: he had, he felt, to write two sorts of books: formal, structural books (like Higher Cortical Functions in Man) and biographical "novels" (like The Mind of a Mnemonist). The first he called "Classical Science," the second, "Romantic Science."

  Jackson, Goldstein, Head, and Luria-they constitute the essential axis of neurology, and certainly they are the axis of my own thinking and of this book. My first references must therefore be to them-ideally to everything they wrote, for what is most characteristic is always suffused through a life's work, but for the sake of practicality to certain key-works which are the most accessible to English-speaking readers.

  Hughlings Jackson

  There are wonderful descriptions of cases before Hughlings Jackson- such as Parkinson's "Essay on the Shaking Palsy," as early as 1817-but no general vision or systemization of nervous function. Jackson is the founder of neurology as a science. One can browse through the basic volumes of Jacksoniana: Taylor, J., Selected Writings of John Hughlings Jackson. London: 1931; rep
r. New York: 1958. These writings are not easy reading, though often evocative and dazzlingly clear in parts. A further selection, with records of Jackson's conversations and a memoir, had been almost completed by Purdon Martin at the time of his recent death, and will, it is Jioped, be published in this sesquicentennial year of Jackson's birth.

  Henry Head

  Head, like Weir Mitchell (see below under Chapter 6), is a marvellous writer, and his heavy volumes, unlike Jackson's, are always a delight to read:

  Studies in Neurology. 2 vols. Oxford: 1920.

  Aphasia and Kindred Disorders of Speech. 2 vols. Cambridge: 1926.

  Kurt Goldstein

  Goldstein's most accessible general book is Der Aufbau des Organis-mus (The Hague: 1934), translated as The Organism: A Holistic Approach to Biology Derived from Pathological Data in Man (New York: 1939). See also Goldstein, K. and Sheerer, M., "Abstract amp; concrete behaviour," Psychol. Monogr. 53 (1941).

  Goldstein's fascinating case-histories, scattered through many books and journals, await collection.

  A. R. Luria

  The greatest neurological treasure of our time, for both thought and case description, is the works of A. R. Luria. Most of Luria's books have been translated into English. The most accessible are:

  The Man with a Shattered World. New York: 1972. The Mind of a Mnemonist. New York: 1968.

  Speech amp; the Development of Mental Processes in the Child. London: 1959. A study of mental defect, speech, play, and twins.

  Human Brain and Psychological Process. New York: 1966. Case histories of patients with frontal lobe syndromes.

  The Neuropsychology of Memory. New York: 1976.

  Higher Cortical Functions in Man. 2nd ed. New York: 1980. Luria's magnum opus-the greatest synthesis of neurological work and thought in our century.

  The Working Brain. Harmondsworth: 1973. A condensed and highly readable version of the above. The best available introduction to neuropsychology.

  CHAPTER REFERENCES

  1. The Man Who Mistook His Wife for a Hat

  Macrae, D. and Trolle, E. "The defect of function in visual agnosia." Brain (1956) 77: 94-110.

  Kertesz, A. "Visual agnosia: the dual deficit of perception and recognition." Cortex (1979) J 5: 403-19.

  Marr, D. See below under Chapter 15.

  Damasio, A. R. "Disorders in Visual Processing," in M. M. Mesulam (1985), pp. 259-88. (See below under Chapter 8.)

  2. The Lost Mariner

  Korsakov's original (1887) contribution and his later works have not been translated. A full bibliography, with translated excerpts and discussion, is given in A. R. Luria's Neuropsychology of Memory (op. cit.), which itself provides many striking examples of amnesia akin to that of "The Lost Mariner." Both here, and in the preceding case history, I refer to Anton, Potzl, and Freud. Of these only Freud's monograph-a work of great importance-has been translated into English.

  Anton, G. "Uber die Selbstwarnehmung der Herderkrankungen des Gehirns durch den Kranken." Arch. Psychiat. (1899) 32.

  Freud, S. Zur Auffassung der Aphasia. Leipzig: 1891. Authorized English tr., by E. Stengel, as On Aphasia: A Critical Study. New York: 1953.

  Potzl, O. Die Aphasielehre vom Standpunkt der klinischen Psychiatrie: Die Optische-agnostischen Storungen. Leipzig: 1928. The syndrome Potzl describes is not merely visual, but may extend to a complete unawareness of parts, or one half, of the body. As such it is also relevant to the themes

  of Chapters 3, 4, and 8. It is also referred to in my book A Leg to Stand On (1984).

  3. The Disembodied Lady

  Sherrington, C. S. The Integrative Action of the Nervous System. Cambridge: 1906. Esp. pp. 335-43.

  –----. Man on His Nature. Cambridge: 1940. Ch. 11, esp. pp.

  328-9, has the most direct relevance to this patient's condition.

  Purdon Martin, J. The Basal Ganglia and Posture. London: 1967. This important book is more extensively referred to in Chapter 7.

  Weir Mitchell, S. See below under Chapter 6.

  Sterman, A. B. et al. "The acute sensory neuronopathy syndrome." Annals of Neurology (1979) 7: 354-8.

  4. The Man Who Fell out of Bed

  Potzl, O. Op. cit.

  5. Hands

  Leont'ev, A. N. and Zaporozhets, A. V. Rehabilitation of Hand Function. Eng. tr. Oxford: 1960.

  6. Phantoms

  Sterman, A. B. et al. Op. cit.

  Weir Mitchell, S. Injuries of Nerves. 1872; Dover repr. 1965. This great book contains Weir Mitchell's classic accounts of phantom limbs, reflex paralysis, etc. from the American Civil War. It is wonderfully vivid and easy to read, for Weir Mitchell was a novelist no less than a neurologist. Indeed, some of his most imaginative neurological writings (such as "The Case of George Dedlow") were published not in scientific journals but in the Atlantic Monthly in the 1860s and 1870s, and are therefore not very accessible now, though they enjoyed an immense readership at the time.

  7. On the Level

  Purdon Martin, J. Op. cit. Esp. ch. 3, pp. 36-51.

  8. Eyes Right!

  Battersby, W. S. et al. "Unilateral 'spatial agnosia' (inattention) in patients with cerebral lesions." Brain (1956) 79: 68-93.

  Mesulam, M. M. Principles of Behavioral Neurology (Philadelphia: 1985), pp. 259-88.

  9. The President's Speech

  The best discussion of Frege on "tone" is to be found in Dummett, M., Frege: Philosophy of Language (London: 1973), esp. pp. 83-89.

  Head's discussion of speech and language, in particular its "feeling-tone," is best read in his treatise on aphasia (op. cit.). Hughlings Jackson's work on speech was widely scattered, but much was brought together posthumously in "Hughlings Jackson on aphasia and kindred affections of speech, together with a complete bibliography of his publications of speech and a reprint of some of the more important papers," Brain (1915) 38: 1-190.

  On the complex and confused subject of the auditory agnosias, see Hecaen, H. and Albert, M. L., Human Neuropsychology (New York: 1978), pp. 265-76.

  10. Witty Ticcy Ray

  In 1885 Gilles de la Tourette published a two-part paper in which he described with extreme vividness (he was a playwright as well as a neurologist) the syndrome that now bears his name: "Etude sur an affection nerveuse caracterisee par l'incoordination motrice accompagnee d'echo-lalie et de coprolalie," Arch. Neurol. 9: 19-42, 158-200. The first English translation of these papers, with interesting editorial comments, is provided by: Goetz, C. G. and Klawans, H. L., Gilles de la Tourette on Tourette Syndrome (New York: 1982).

  In Meige and Feidel's great Les Tics et leur traitement (1902), brilliantly translated by Kinnier Wilson in 1907, there is a wonderful opening personal memoir by a patient, "Les confidences d'un ticqueur," which is unique of its kind.

  11. Cupid's Disease

  As with Tourette's syndrome, we must go back to the older literature to find full clinical descriptions. Kraepelin, Freud's contemporary, provides many striking vignettes of neurosyphilis. The interested reader might

  consult: Kraepelin, E., Lectures on Clinical Psychiatry (Eng. tr. London: 1904), in particular chs. 10 and 12 on megalomania and delirium in general paralysis.

  12. A Matter of Identity See Luria (1976).

  13. Yes, Father-Sister

  See Luria (1966).

  14. The Possessed

  See above under Chapter 10.

  15. Reminiscence

  Alajouanine, T. "Dostoievski's epilepsy." Brain (1963) 86: 209-21.

  Critchley, M. and Henson, R. A., eds. Music and the Brain: Studies in the Neurology of Music. London: 1977. Esp. chs. 19 and 20.

  Penfield, W. and Perot, P. "The brain's record of visual and auditory experience: a final summary and discussion." Brain (1963) 86: 595-696. I regard this magnificent 100-page paper, the culmination of nearly thirty years' profound observation, experiment, and thought, as one of the most ori
ginal and important in all neurology. It stunned me when it came out in 1963 and was constantly in my mind when I wrote Migraine in 1967. It is the essential reference and inspiration to the whole of this section. More readable than many novels, it has a wealth and strangeness of material which any novelist would envy.

  Salaman, E. A Collection of Moments. London: 1970.

  Williams, D. "The structure of emotions reflected in epileptic experiences." Brain (1956) 79: 29-67.

  Hughlings Jackson was the first to address himself to "psychical seizures," to describe their almost novelistic phenomenology and to identify their anatomical loci in the brain. He wrote several papers on the subject. Most pertinent are those published in Volume 1 of his Selected Writings (1931), pp. 251ff. and 274ff., and the following (not included in that volume):

  Jackson, J. H. "On right- or left-sided spasm at the onset of epileptic

  paroxysms, and on crude sensation warnings, and elaborate mental states." Brain (1880) 3: 192-206.

  –----. "On a particular variety of epilepsy ('Intellectual Aura')."

  Brain (1888) I J: 179-207.

  Purdon Martin has provided an intriguing suggestion that Henry James met Hughlings Jackson, discussed such seizures with him, and employed this knowledge in his depiction of the uncanny apparitions in The Turn of the Screw. "Neurology in fiction: The Turn of the Screw," British Medical]. (1973)4:717-21.

  Marr, D. Vision: A Computational Investigation of Visual Representation in Man. San Francisco: 1982. This is a work of extreme originality and importance, published posthumously (Marr contracted leukemia while still a young man). Penfield shows us the forms of the brain's final representations-voices, faces, tunes, scenes-the "iconic": Marr shows us what is not intuitively obvious, or ever normally experienced-the form of the brain's initial representations. Perhaps I should have given this reference in Chapter 1-it is certain that Dr. P. had some "Marr-like" deficits, difficulties in forming what Marr calls a "primal sketch" in addition to, or underlying, his physiognomonic difficulties. Probably no neurological study of imagery, or memory, can dispense with the considerations raised by Marr.