I send you my very best wishes, this message of hope, and look forward to hearing from you,

  Oliver Sacks

  To Wendy[*11]

  College Student

  November 14, 2006

  2 Horatio St., New York

  Dear Wendy,

  I should have written before—I very much enjoyed meeting you, the gleaning together (how nice, how right, to meet in an activity, a shared activity), and talking together—but I have been totally occupied and preoccupied with my book, and couldn’t think of anything else. Now it is done!—was sent off to the publishers yesterday—I can start to catch up on everyone and everything else.

  Having said this, I am not quite sure how I can answer your long, moving letter in any adequate way. I don’t know, as a start, how much one can talk about genetic/evolutionary “mistakes,” for while there is natural selection there is no “purpose” (and perhaps no “progress”) in Nature. More to the point, things are very complex, and there can be unexpected spin-offs in both directions, all directions. Sickle-cell anemia is a fatal disease, but sickle-cell trait (to cite a famous example) confers resistance to malaria. (I want to avoid a dramatic way of putting this, like “Thousands die that millions may live”—for there is no “that” in Nature.) (Congenital) deafness was seen by Alexander Graham Bell in very negative terms—he felt that the deaf should not marry or be allowed to have children, lest they propagate an “inferior variety—of the human race,” but many deaf people enjoy rich, full, creative and happy lives, often enriched by the fact that they are deaf, and that there is a vital language and heritage and culture of the Deaf, and they would not wish to be otherwise.

  But you know all these arguments, and quote some of them in your letter. Also Kay Redfield Jamison’s notion that significant numbers of creative artists have bipolar cycles, and may do their best work when hypomanic (not manic). The other side, of course, is the devastating grimness of (psychotic) depression, the urge to suicide etc (which Jamison has also written about very powerfully—and must know in herself).

  What seems to me less stressed, and most in need of stressing, is that you are an individual—unique—with gifts and genes which no-one else in the world exactly duplicates—and that means you have a true place and role in evolution, and in the present. That you have bipolar disorder, in a sense are bipolar, does not begin to encompass the whole of you—it is a what, while you are a you. You have to hold to this sense of a personhood (“personality” is not quite the word, it has got too Hollywoodized)—Coleridge talked about “personeity,” which is deeper than any “condition” you have, and perhaps these (relatively) gentle years at Gould Farm will allow you to realize this (realize it, in both senses—understand and actualize). You have much to hope and to live for. So, my best to you & keep in touch.

  Kate too sends her warm regards—

  Oliver

  To V. S. Ramachandran

  Neuroscientist

  June 26, 2007

  2 Horatio St., New York

  Dear Rama,

  I hope when we meet in August, that we can also discuss another matter that has engaged your attention—and which now, through force of circumstance engages mine, namely some of the forms of “filling in” which occur with a (monocular) scotoma.

  The melanoma in my right eye has been persistent (persistence rather than “regrowth” is the soothing word my surgeon uses), and has required further lasering. And because it now envelops the fovea, this is obliterating central vision. I have lost the top half of this: when I look at people [with the damaged right eye only], for example, they are only visible up to about mid-chest. I will probably have further lasering next month, which may obliterate this remaining part of central vision, and then I will only have some peripheral vision, a sort of crescent from about 2 to 7 on a clockface. […]

  I have been puzzled at the different appearances of the scotoma—it tends, initially, to present as an intensely black, irregular “ink-blot,” roughly corresponding to the irregular […] shape of the melanoma […]. But if, say, I am looking at a colored surface—any color—the scotoma starts to change, to “match” its brightness and color so that, after a few seconds, it becomes almost invisible—so much so that I would have difficulty believing that it was there, and may have to intrude a hand or finger and see by its sudden “amputation” that the scotoma, tho’ now color- and brightness-matched, is still present. There is also—but this is slower, and may be less successful—a matching of texture. A fine texture, as of a wallpaper and some rugs, gets well-matched—especially a fine liney or pebbly texture. The textural filling-in tends to start at the periphery of the scotoma, like ice crystallizing at the edge of a pond in winter, and then moves in to the center. If I look at a page of print, a sort of “pseudoprint” will appear in the scotoma in fifteen seconds or so—I say “pseudoprint” because I cannot actually read it (curiously too it may sometimes be smaller, have a different “font-size,” or be topographically squashed). But with a gross chequer-board pattern, with a much coarser spatial frequency, the simulation or illusion (whatever word one should use) is much less successful. (I cannot help comparing myself to your flounders, or Roger Hanlon’s cuttlefish, camouflaging themselves against different surfaces; I cannot avoid the feeling of statistical evaluations being made—especially with random, “pebbly” surfaces—perhaps distinct from interpolations or extrapolations with more predictable patterns.) With the letters there is often a “ghostly” or “smokey” or “semi-transparent” quality to the illusion, whereas the fine pebbly patterns are scarcely distinguishable from perceptual reality.

  Another phenomenon, which seems to be quite different in quality (and presumably mechanism) from this slow, limited, gradual “filling-in” is an extraordinary heightening and perseveration (if this is the right word) of visual imagery—again forcing itself on my attention because my visual imagery (other than hypnopompic/gogic) has usually been so feeble.

  Thus, walking yesterday with Ralph along 8th Avenue, I saw two men in white shirts walking towards us. Closing my eyes for a few seconds (I dared not do so for longer!), I could “see” them with almost undiminished intensity, continuing to walk towards us. So this was not an “after-image” in the usual sense—e.g. a brief continuation, positive or negative, such as one might have with a lighted filament, an illuminated figure, etc. It was like, and I do not know whether to use terms like “perseveration” or “computational,” but what they were doing, walking towards us, vividly persisted. It was similar with traffic, including traffic moving in both directions. This sort of imagery tended to fade, or diffuse, or lose its sharpness, after a few seconds—but the brilliance and verisimilitude was so striking that sometimes I wondered if I had actually closed my eyes. […] I also find myself thinking in terms of a perceptual “cinema,” with the movie going on even though there is now no visual input. […]

  I regret that my retina (and my brain) should be the seat of all this—but since it is occurring to me, I feel I should try and explore it a bit. […] I hope we can discuss some of these phenomena—I think you probably have extensive knowledge (and all sorts of thoughts) here, and I would be grateful if you could send me your (early) paper(s) with Richard Gregory, and anything you may have published on the subject.

  I hope you are well, have a grand return-visit to India…and to see you in August.

  Best,

  Oliver

  To Orrin Devinsky

  Neurologist[*12]

  July 11, 2007

  2 Horatio St., New York

  Dear Orrin,

  […] I have received a very full narrative of B’s life from her sister, P, who accompanies her today (along with Tabitha, who works at Randall House and has known B there for the last fifteen years).[*13] […] I have [past records of her consultations and tests], none of which show any progressive pathology.

  The overall history is as follows: B was grossly premature, weighing only a little more than 2 lbs. at birth. Her first weeks were spent in an incubator, and she became blind (from retrolental fibroplasia) in consequence of the high oxygen levels to which she was exposed. When she was two and a half, she showed remarkable musical gifts—“savant” talents. She had shown behavioral problems prior to this time: would scream, hurl objects, bang her head, could not be calmed, except when music was played (she would explode within seconds of a record finishing, and her mother had to get a special phonograph which moved from one record to another with scarcely any delay). Thus (in an account from her mother), “one day I was at the piano. I was playing some nursery songs and she was on my lap. She pushed my left hand away and she filled in the left hand. She didn’t want any help.”

  Although lessons were attempted (her sister writes), not much formal progress was made due to her behavioral difficulties. Her piano playing was not merely repetitive, it could be creative. She was able to play songs she hadn’t heard played in a particular style in the style of other artists she had heard, to transpose pieces into different keys, and also to create her own songs if asked. She could play one song with one hand and another with the other. Of course, having perfect pitch, she could name any note played.

  In an existence often sadly restricted by her limited intelligence (estimated IQ around 70) and behavioral difficulties—impulsiveness, violence, self-destructiveness (and, later, seizures), B’s musical ability, and her prowess at the piano provided an oasis, an island of stability and pleasure, in her life. She enjoyed playing for herself, she enjoyed playing for others (she sometimes gave recitals), and she enjoyed playing with others—especially with her sisters: P, who played the guitar, and her younger sister J, who played the flute, though they were not as gifted as she was.

  She also showed other savant talents at a very early age, being able to name the day of any date in the past (or next) two centuries, and possessing an apparently limitless memory for dates and what happened on them. This too was a source of pride, and sometimes amusement, for her—and, along with music, gave her a special place, a special role, in Randall House (“a Home to 15 men and women who face the dual challenges of visual impairment and mental retardation or developmental disability”).

  Despite her modest intelligence, B learned Braille, and became fond of reading both books and newspapers. When I asked her what she most liked, she said biographies. I asked if she had read a biography of Helen Keller. “Not a biography,” she said. “Her autobiography.” (This made me wonder whether she had greater intellectual capacities than her IQ tests might have suggested.) She is right handed, and reads Braille with her right index finger. If this is cut or hurt, she will continue to use this finger if she can, for she cannot read with any other finger. (I mentioned here that the reading finger of Braille readers had an especially large representation in the brain—B seemed to understand this, at least the general import of this finger being special, and nodded.)

  There seemed no special physical problems outside B’s blindness and hearing impairments (this came partly from having had mastoiditis as a child, and partly from a cholesteatoma she developed as an adult). Her left ear was the functional one, and could be brought up to par with a hearing aid. B enjoyed the garden at Randall House, enjoyed outings (with someone) in the City, and enjoyed visits from and to her relatives, who were devoted to her, as her two sisters and elderly mother (who is in her ninetieth year) remain.

  Her closest relationship, however, was with her father—he seemed to understand her, and to be able to joke with her, to bring out her lighter side, better than anyone else. And it was when he died [three years ago] that B descended into a tormented and obsessed state which she has been in ever since. At the same time two fellow residents of Randall House, whom she was friends with, and had known for decades, also died. And this triple bereavement pushed her overboard, or so it seemed.

  Prior to this time the chief problem (outside her “behavior problems”) were seizures—petit mal (which had first occurred in infancy) and then grand mal attacks (from about the age of five). Various drugs were used, and it was soon found that mysoline (primidone) seemed to provide optimal seizure control with a minimum of adverse effects. In 1987 she was taken off this, as a trial, and immediately had a return of seizures. In one grand mal attack, she fell down an entire flight of stairs and received severe head and other injuries.

  She is now on other medications in addition to mysoline—seroquil, depakote, and trileptal, a complex situation which I will leave to [you] to advise on. She has been seizure free, clinically, for several months, although there is some spiking etc. in EEG.

  In the past two years postural and motor problems have developed associated with a severe (and at times painful) scoliosis—this was very marked today. […]

  Following her father’s death, and other deaths, at the end of 2006 B’s state showed a striking and unprecedented deterioration. She became suspicious, agitated, frightened. Fearful that her sisters or mother would die, or had died, she kept making phone calls to “check” on this. Feared the cat would die. Feared other residents at the Home would die. Feared that she herself would die. These fears assumed delusional intensity and “rationalization.” When she had a D and C (for minor problems) in 2005, she told her sister that without this she would have died. She fears she may have a grand mal seizure, an unstoppable series of grand mal seizures (as perhaps happened, with drug withdrawal, in 1987), though she is, in fact, seizure free at this time. She lives in mortal fear of a sudden heart attack—her father died of a heart attack in 2004.

  She thinks that another resident at the home, Lisa, follows her around (she felt that Lisa had made her way to [my office] today). Lisa’s special way of tormenting her is to play music nonstop in her room—nonstop, B clarified, meant 24 hours a day, for 365 (or, if called for, 366) days a year. I asked what music she played. B says that it is just two pieces, endlessly repeated: “The Heart Attack Song” and “The Grand Mal Song.” She has occasionally hummed these; both, her sister says, are “atonal and disturbing,” with no resemblance to classical music or the popular songs B used to play and enjoy. These hallucinatory songs are fraught with horror and threat. They seem addressed to her: they are “telling” her that grand mal seizures, or a heart attack, are imminent, may come at any time.

  In addition to these songs, which she feels are “aimed” at her, B has been subjected, in the past 2–3 years, to a range of invariably negative suggestions and commands from other people. She will not allow that these are “voices”—indeed got angry when I used the term. She says they are “people,” and that this is what they said (though, when pressed, she cannot say exactly when or where or how they said such things to her). These ostensibly well-meaning (but in reality malicious) “people”—physicians, others in the Home, perhaps fellow residents—have intimated to her that she is a “major epileptic,” and that as a “major epileptic” she cannot, and must not, play the piano.

  They have also forbidden her to exercise her calendar or calculating talents. They have forbidden her to take exercise or have physiotherapy.

  In response to them B has ceased to play the piano, her great solace, her one real pleasure and talent, in an always narrow and dependent life; and they have forbidden everything else which would give her any pleasure or any relief. They are also preventing her from taking the needed physical care she requires for her worsening scoliosis and pain.

  It is partly in response to them that she has ceased to use her hearing aids—she feels these may provide a portal for voices and “bad” music; but taking them out also reduces her possibilities of real interaction with others, and she was always, despite her tantrums and frustrations, a sociable creature who enjoyed chatting and hearing about other people’s lives. […]

  When, after formal neurological examination, I took B to the piano, she became very anxious, and refused to play anything. […] She kept voicing the fear that “something”—a heart attack? A grand mal seizure? might happen if she played anything. She said, “It’s too risky,” but she was also tempted, and wondered if she dared “take a chance.” […] When I asked her about favorite composers, she immediately said, “Bach, Beethoven, Brahms.” Any more B’s, I enquired? She added Bizet and Berlioz (but not Bellini or Britten). She then asked me what I liked, and I said Chopin. She asked if I played any of his études. (Her fingers were moving when she asked me, as if they itched to play an étude themselves.)

  Later when I spoke, by mistake, into her right ear, which is deaf, and then said that I had right/left confusion, and had to feel which side my heart was on, I elaborated this, and told her how, as a medical student, I was asked to examine a patient and got frantic because I did not hear his heart on his left side. And how he saved me by whispering, “Listen on the right side, Doc—I’ve got dextrocardia.” B laughed at this, showing her “light” side, her nice sense of humor. This was, in fact, the only “easy” moment during the time we met.

  * * *

  —

  In clinical terms, one would have to say that this unfortunate patient has a psychotic depression, with great anxiety, phobias, nihilistic delusions, musical (and perhaps vocal) hallucinations, etc. A psychosis of a particularly vicious and malignant kind, one which makes life almost unbearable for her. Dr. D. wondered whether this psychiatric symptomology, while originating from loss of a beloved figure, indeed the simultaneous loss of three important figures, might “ultimately have an organic basis.” A 5-day video EEG […] showed no correlation of psychotic muttering or hallucinations etc. with epileptic activity (this does not wholly exclude a more general connection).