The Case of Anna H. | The New Yorker

Why was she losing her ability to recognize familiar objects by sight?

In January of 1999, I received the following letter, from a woman I will call Anna H.:

Dear Dr. Sacks,

My (very unusual) problem, in one sentence, and in non-medical terms, is:

I can’t read. I can’t read music, or anything else.

In the ophthalmologist’s office, I can read the individual letters on the eye chart down to the last line. But I cannot read words, and music gives me the same problem. I have struggled with this for years, have been to the best doctors, and no one has been able to help.

I would be ever so happy and grateful if you could find the time to see me.

Sincerely yours,

Anna H.

I phoned Mrs. H.—this seemed to be the thing to do, although I normally would have written back—because although she apparently had no difficulty writing a letter, she had said that she could not read at all. I spoke to her and arranged to see her at the neurology clinic at New York University, where I work.

Mrs. H. came to the clinic soon afterward—a charming, vivacious sixty-seven-year-old woman, with a strong Prague accent—and was able to relate her story in much more detail. She was a pianist, she told me, and the first intimation of anything the matter had come during a concert in 1991. She was performing Mozart piano concertos, and there was a last-minute change in the program, from the nineteenth piano concerto to the twenty-first. But when she flipped open the score of the twenty-first she found it, to her bewilderment, completely unintelligible. Although she saw the staves, the lines, the individual notes, sharp and clear, none of it seemed to hang together, to make sense. But, whatever her eye problem—she assumed the difficulty must have something to do with her eyes—she went on to perform the concerto flawlessly, from memory, and the strange incident was dismissed as “one of those things.”

Several months later, the problem recurred, and Mrs. H. found that her ability to read musical scores fluctuated. If she was tired, or had a fever (she had had a bad bout of the flu just before the concert), she could hardly read them at all, but when she was fresh her sight-reading was as swift and easy as ever. But, in general, the problem worsened, and though she continued to teach, to give concerts around the world, and to record, she increasingly depended on her musical memory, her extensive repertoire, since it was now becoming impossible to learn new music by sight. “I used to be a fantastic sight reader,” she said, “easily able to play a Mozart concerto by sight, and now I couldn’t.”

Occasionally, at concerts, there were lapses of memory, though Mrs. H., a brilliant improviser, could usually cover these. When she was at ease, with friends or students, her playing seemed as good as ever. And thus, through inertia, or fear, or a sort of adjustment, it was possible for her to overlook her peculiar problems in reading music, for she had no other visual problems, and her memory and ingenuity still allowed her a full musical life.

In 1994, three years or so after she had first noticed problems reading music, Mrs. H. started to have problems with reading words. Here again, there were good days and bad, and even times when her ability to read seemed to change from moment to moment: a sentence would look strange, “like cuneiform or hieroglyphics” at first; then suddenly it would look fine, and she would have no difficulty reading it. (Similar “alexias,” sometimes lasting only a few seconds, can occur in the course of a migraine—I have sometimes had these myself.) Her ability to write, however, was quite unaffected, and she continued to maintain a large correspondence with former students and colleagues scattered throughout the world, though she depended increasingly on her husband to read letters she received, and even to reread her own.

An ability to write despite an alexia is not that uncommon. I recently received a letter from Howard Engel, a Canadian novelist, who told me that he had a somewhat similar problem following a stroke. “The area affected,” he relates, “was my ability to read. I can write, but I can’t read what I’ve just written. . . . So, I can write, but I can’t rewrite. . . . My vision for the most part is unaffected until I look at a text. Then, whatever I’m looking at turns into unfamiliar blocks of type that could at first glance be taken for Serbo-Croatian. Familiar words, including my own name, are unfamiliar blocks of type and have to be sounded out slowly. Each time a name recurs in an article or review, it hits me as unfamiliar on its last appearance as it does on the first. . . . I have just started [writing] a crime novel in which the hero has similar problems.” Though Engel was a fair sight reader, he told me later, he had no musical alexia.

Anna H., however, was beginning to develop additional visual problems. She noticed that she tended to “miss” objects to the right, and decided, after some minor mishaps, that she had best give up driving.

She had sometimes wondered whether her strange problem with reading might be neurological rather than ophthalmological in origin. “How can I recognize individual letters, even the tiny ones on the bottom line of the eye doctor’s chart, and yet be unable to read?” she had wondered. Then, in 1996, she started to make occasional embarrassing mistakes, such as failing to recognize old friends, and she found herself thinking of a case history of mine that she had read years before, entitled “The Man Who Mistook His Wife for a Hat.” She had chuckled when she read this, in 1986, but now she started to wonder whether her own difficulties might be eerily similar in nature.

Finally, five years or more after her original symptoms, she was referred to a department of neurology for a full workup. Given a battery of neuropsychological tests—tests of visual perception, of memory, of verbal fluency, etc.—Mrs. H. did particularly badly in the recognition of drawings: she called a violin a banjo, a glove a statue, a razor a pen, and pliers a banana. Asked to write a sentence, she wrote, “This is ridiculous.” There was a fluctuating inattention to the right, and very poor facial recognition (measured by recognition of photographs of famous public figures). She could read, but only slowly, letter by letter. She would read a “C,” an “A,” a “T,” and then, laboriously, “cat,” without recognizing the word as a whole. Yet, if she was shown words too quickly to decipher in this way, she could nonetheless correctly sort them into salient categories, such as “living” or “non-living,” even though she had no conscious idea of their meaning.

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In contrast to her severe visual problems, her speech comprehension, repetition, and verbal fluency were all normal. An MRI of the brain was also normal, but when a PET scan was performed—this can detect slight changes in the metabolism of different brain areas, even when they appear anatomically normal—Mrs. H. was found to have diminished metabolic activity in the posterior part of the brain, the visual cortex. This was more marked on the left side, which would account perhaps for her occasional failure to see things in her right visual field. The neurologists who had tested her felt that she must have a degenerative condition, a so-called “posterior cortical atrophy,” which would slowly continue to worsen.

The underlying disease was not treatable in any radical sense (at most, she might be given an anti-inflammatory drug like ibuprofen, which is thought to have some neuroprotective action in such conditions), but her neurologists felt that she might benefit from certain strategies: “guessing” words, for example, even when she could not read them in the ordinary way (for it was clear that she still possessed some mechanism that allowed unconscious, or preconscious, recognition of words). And, they felt, she might also use a deliberate and, so to speak, hyperconscious inspection of objects and faces, making particular note of their distinctive features, so that these could be identified in future encounters, even if the normal “automatic” recognition was impaired.

In the thirty months or so that had elapsed between this neurological exam and her first visit to me, Mrs. H. told me, she had continued to perform, though not as well, and not as frequently. She found her repertoire diminishing, because she could no longer check even familiar scores by vision. “My memory was no longer fed,” she remarked. Fed visually, she meant, for she felt that her auditory memory, her auditory orientation, had increased, so that she could now, to a much larger degree than before, learn and reproduce a piece by ear, sometimes after only a single hearing. She could not only reproduce a piece in this way; she could rearrange it in her mind. Nonetheless, there was, on balance, a shrinkage of her repertoire, and of her public concert performances. She continued to play in more informal settings, and to teach master classes at the music school.

Handing me the neurological report from 1996, she commented, “The doctors all say, ‘Posterior cortical atrophy of the left hemisphere, very atypical,’ and then they smile apologetically—but there’s nothing they can do.”

When I examined Mrs. H., I found that she had no problem matching colors or shapes, or recognizing movement or depth. But she showed gross problems in other areas. She was unable now to recognize individual letters or numerals (even though she still had no difficulty writing complete sentences). When I presented her with pictures to identify, it was difficult for her even to recognize pictures as pictures—she would sometimes look at a column of print or a white margin, thinking it was the picture I was quizzing her about. Of one such picture, she said, “I see a V, very elegant—two little dots here, then an oval, with little white dots in between. I don’t know what it’s supposed to be.” When I told her it was a helicopter, she laughed, embarrassed. (The V was a sling; the helicopter was unloading food supplies for refugees. The two little dots were wheels, the oval the helicopter’s body.) Thus she was now seeing only individual features of an object or picture, failing to synthesize them, to see them as a whole, much less to interpret them correctly. Shown a photograph of a face, she could perceive that the person was wearing glasses, nothing else. When I asked if she could see clearly, she said, “It’s not a blur, it’s a mush”—a mush consisting of clear, fine, sharp but unintelligible shapes and details.

Looking at the drawings in a standard neurological test booklet, she said of a pencil, “Could be so many things. Could be a violin . . . a pen.” A house, however, she immediately recognized. Regarding a whistle, she said, “I have no idea.” Shown a drawing of scissors, she looked steadfastly at the wrong place, at the white paper below the drawing.

When I asked Mrs. H. how she felt about herself and her situation, she said, “I think I am dealing with it very well . . . most of the time . . . knowing it is not getting better, but only slowly worse. . . . I’ve stopped seeing neurologists. I always hear the same thing. . . . But I am a very resilient person. I don’t tell my friends. I don’t want to burden them, and my little story is not very promising. A dead end. . . . I have a good sense of humor. And that’s it, in a nutshell. It is depressing, when I think of it, frustrations daily. But I have many good days and years ahead.”

After Mrs. H. had left, I was unable to find my medical bag—a black bag with some similarities (I now remembered) to one of the several bags she had brought. In the taxi she realized that she had taken my bag when she saw a red-tipped object sticking out of it (my long, red-tipped reflex hammer). It had attracted her attention, by its color and shape, when she saw it on my desk, and now she realized her mistake. Returning, breathless, to the clinic, she said, “I am the woman who mistook the doctor’s bag for her handbag.”

Mrs. H. had done so badly on the formal tests of visual recognition that I had difficulty imagining how she managed in daily life. How did she recognize a taxi, for example? How could she recognize her own home? How could she shop, as she told me she did, or recognize foods and serve them on a table? All this and much more—an active social life, travelling, going to concerts, and, above all, giving concerts—she did by herself when her husband, who was also a musician, went to Europe for weeks at a time. I could get no idea of how she accomplished this from seeing her dismal performance in the unnatural, artificial, impoverished atmosphere of a medical clinic. I had to see her in familiar surroundings, in her own context.

The following month, I visited Anna H. at home, home being a pleasant apartment in upper Manhattan, where she and her husband had lived for more than forty years. Josef was a charming, genial man, about the same age as his wife. They had met as music students nearly fifty years earlier, and had pursued their musical careers independently, but in parallel. The apartment had a friendly, cultured atmosphere, with a grand piano, a great many books, photographs of friends and family, abstract, modernist paintings on the wall, and mementos of their trips on every available surface. It was crowded—rich in personal history and significance, I imagined, but a nightmare, a complete chaos, for someone with visual agnosia. This, at least, was my first thought as I entered, negotiating my way between tables full of knickknacks. But Anna had no difficulty with the clutter, and threaded her way confidently through the obstacles.

Since she had had such difficulty on the drawing-recognition test, I brought a number of solid objects with me, wondering if she would do better with these. I started with some fruit and vegetables I had just bought, and here Anna did surprisingly well. She instantly recognized “a beautiful red pepper,” recognized it from across the room; a banana, too; she was momentarily uncertain whether the third object was an apple or a tomato, though she soon decided, correctly, on the former. When I showed her a small plastic model of a wolf (I keep a variety of such objects, for perceptual testing, in my medical bag), she exclaimed, “A marvellous animal! A baby elephant, perhaps?” When I asked her to look more closely, she decided it was “a kind of dog.”

Mrs. H.’s relative success in naming solid objects, as opposed to drawings of them, made me wonder whether she had a specific agnosia for representations. The recognition of representations requires a learning, the grasping of a code or convention, beyond that required for the recognition of objects. Thus primitive people who have never been exposed to photographs or drawings may fail to recognize that these are representations of something else; they may see paintings as mere colored surfaces. A complex system for recognition of representations must be specially constructed by the brain, and this ability may be lost through damage to that system by a stroke or disease, just as the learned understanding of writing, say, or any other acquired ability may be lost. Was Mrs. H.’s difficulty in recognizing drawings due simply to their “sketchiness,” their two-dimensionality, their poverty of information, or had she developed a specific agnosia for such visual representations?

I followed Anna into the kitchen, where she set about taking the kettle off the stove and pouring boiling water into the teapot. She seemed to navigate her crowded kitchen well, knowing, for instance, that all the skillets and pots were hung on hooks on one wall, various supplies kept in their regular places. When I pointed to a large cylindrical carton of table salt, she said, “I know what it is. If I didn’t know, I’d think it might be a carton for something—not a book.” When we opened the refrigerator and I quizzed her on the contents, she said, “O.J., milk, butter, on the top shelf—and a nice sausage, if you’re interested, one of those Austrian things . . . cheeses.” She recognized the eggs in the fridge door, and, when I asked her, counted them correctly, moving her finger from egg to egg as she counted. I could see at a glance that there were eight—two rows of four—but Anna, I suspect, could not perceive the eightness, the gestalt, at a glance, and had to enumerate the eggs one by one. And the spices, she said, were “a disaster.” They all came in identical red-topped bottles, and, of course, she could not read the labels. So—“I smell them! . . . And I call for help some of the time.” With the microwave oven, which she used often, she said, “I don’t see the numbers. I do it by feel—cook, try, see if it needs a bit more.”

Though Anna could scarcely recognize anything in the kitchen visually, she had organized it in such a way that mistakes rarely, if ever, occurred, utilizing a sort of informal classification system instead of a direct perceptual gnosis. Thus things were categorized not by meaning but by color, by size and shape, by position, by context, by association, somewhat as an illiterate person might arrange the books in a library, or identify, by its appearance, a passage in a book. Everything had its place, and she had memorized this.

Seeing how Anna inferred the character of objects about her in this way, using color, above all, as a marker, I wondered how she would do with objects of similar appearance, like the fish knives and steak knives, which looked almost the same. This was a problem, she confessed, and she often confused them. Perhaps, I suggested, she could use an artificial marker, a little green dot for the fish knives, a red one for the steak knives, so that she could see the difference at a glance. Anna said she had already thought about this but was not sure she wanted to “flaunt” her problem to others. What would her guests think of color-coded cutlery and dishes, or a color-coded apartment? (“Like a psychological experiment,” she said, “or an office.”) The “unnaturalness” of such an idea disturbed her, but if the agnosia got worse, she agreed, she might need it.

In some cases where Anna’s categorization system did not work, such as using the microwave, she could operate by trial and error. But if objects were not in their place, or were not a subject of knowledge and prediction, major difficulties could appear. This showed itself, startlingly, at the end of my visit. The three of us—Anna, Josef, and I—had sat down at the dining-room table. Anna had laid the table, put out biscotti and cakes, and now brought in a steaming pot of tea. She chatted as we ate, but retained a certain watchfulness, monitoring the position and movement of every dish, tracking everything (I later realized), so that it did not get “lost.” She got up to take the empty dishes into the kitchen, leaving only the biscotti, which she saw that I especially liked. Josef and I chatted for a few minutes—our first talk alone—pushing the plate of biscotti between us.

When Anna came back, and I packed my bag and prepared to go, she said, “You must take the rest of the biscotti with you”—but now, bizarrely, she could not find them, and became upset, almost frantic, at this. They were right on the table in their dish—but since the dish had been moved she no longer knew where they were, or even where to look. She seemed to have no strategy for looking. She was, however, quite startled to see my umbrella on the table, though she failed to see it as an umbrella, saw only that something curved and twisted had appeared—and wondered, for a half-serious moment, if it was a snake.

Before I left, I asked Anna to go to the piano, asked if she might play something for me. She hesitated. It was clear that she had lost a good deal of her confidence. She started beautifully, on a Bach fugue, but broke off, apologetically, after a few bars. Seeing a volume of Chopin mazurkas on the piano, I asked about those, and now, encouraged, she closed her eyes, and played two of the Opus 50 mazurkas without faltering and with great brio and feeling.

She told me afterward that the printed music was just “lying around,” and said, “It throws me off to see the score, people turning pages, my hands, or the keyboard,” and that, in these circumstances, she might make mistakes, especially in her right hand. (I was reminded of a blind woman, a contemporary of Mozart and a most remarkable pianist, who, it is said, could no longer play after she regained some sight.) Thus Anna had to close her eyes and perform non-visually, using only her kinesthesia, her motor patterns, and her fine ear. Despite the loss of her ability to visualize the score, her playing had lost none of its brilliance.

Josef was due to leave for Europe in a few days, where he would teach master classes and judge competitions for several weeks—but he felt that he could leave Anna with complete confidence, knowing that she would manage perfectly well in his absence; she was quite able to get around in familiar surroundings, despite her agnosia.

But how would she manage in circumstances less familiar and predictable, how would she deal with the challenge of novel neighborhoods and streets? This I would have to investigate the next time I saw her.

And what could I say about the nature and progress of her strange disease? It had clearly advanced somewhat since her original neurological examination, in 1996, and there were hints—though no more than hints—that her problems might no longer be purely visual, or confined to the visual, occipital parts of the brain. In particular, she occasionally had difficulty naming objects, and would speak of a “thingmy” when she could not get the word, although I was not sure how to interpret this difficulty.

I had ordered a new MRI to compare with an earlier one, and it showed that there was now some shrinkage of the visual areas on both sides of the brain. Was there any sign of real damage elsewhere? I could not decide, although I suspected that there might have been some shrinkage in the hippocampi, too—parts of the brain crucial for the registration of new memories. But, it was clear, the damage was still largely, if not wholly, confined to the occipital lobes, and the rate of advance was very slow.

When I discussed these MRI findings with Josef, he stressed that in my conversation with Anna I should avoid certain terms, above all the frightening label of Alzheimer’s disease. “It’s not Alzheimer’s disease, is it?” he said. Clearly, this had been much on their minds.

“I’m not sure,” I said. “Not in the ordinary sense. One should see it as something much rarer, kinder, more benign.”

Posterior cortical atrophy, PCA, so called because of the characteristic atrophy of the visual-association areas at the back of the brain, was described by Frank Benson, Jeffrey Davis, and Bruce Snyder in the Archives of Neurology in 1988. Occasional cases were probably seen, but not recognized for what they were, for a century before this. It takes an original eye to recognize a disease or syndrome for the first time, but a condition once recognized is more readily recognized when seen again, and dozens more cases of PCA have come to light in the fourteen years since Benson’s description.

Such patients tend to present initially with complex visual disturbances—difficulties reading (alexias) or recognizing faces and objects (agnosias)—while more elementary forms of visual perception, such as movement and color perception, are perfectly preserved. Anna H. is the only patient, to my knowledge, whose first symptom was a musical alexia.

Visual difficulties may become profound quite early in the illness, and all of the patients originally described found themselves becoming disoriented and lost in their own neighborhoods, and even in their own homes. (Benson called this an “environmental agnosia.”) Other difficulties commonly follow: left-right confusion, difficulty in writing and calculation, even an agnosia for one’s own fingers (a tetrad of problems sometimes called Gerstmann’s syndrome). One such patient, a touch typist, became unable to type. Sometimes patients with PCA may recognize objects but find themselves unable to name them, a so-called anomia.

In contrast to these difficulties, memory, intelligence, insight, and personality tend to be preserved until late in the course of the disease. Every patient described by Benson, he writes, “could present his or her own history, was aware of current events, and showed considerable insight into his or her predicament.”

Thus, although PCA is clearly a degenerative brain disease, it seems quite different in character from the commonest forms of Alzheimer’s, where gross changes in memory and thinking, in the comprehension and use of language, and often in behavior and personality tend to dominate the picture, and insight into what is happening (perhaps mercifully) is generally lost early on.

In Anna H.’s case, the course of the disease seemed to have been particularly benign, for even now she had no environmental agnosia, did not get lost in her own home or neighborhood. She seemingly had no sign of Gerstmann’s syndrome, either, for she could add grocery bills in her head and write letters, had no left-right confusion, and remained as dexterous as ever with her fingers on the keyboard.

I could not help making a comparison, as Anna herself had, with my patient Dr. P., “the man who mistook his wife for a hat.” Both of them were highly gifted professional musicians; both developed severe visual agnosias, while being remarkably intact in many other ways; and both had discovered or developed ingenious ways around their problems, so that it was possible for them to keep teaching at the highest level in music colleges, despite what might appear to be quite devastating disabilities.

The actual ways in which Anna and Dr. P. coped with their illnesses were very different, a reflection in part of the severity of their symptoms, and in part of differences in temperament and training. Dr. P. was already in grave trouble when I saw him, barely three years after his initial symptoms. He not only had visual difficulties but tactile ones, too—witness his grasping his wife’s head and mistaking it for a hat; he showed a sort of levity or indifference, and little insight into the fact that he was ill, and he often confabulated to make up for the fact that he could not identify what he was seeing. This was in strong contrast to Anna, who, nine years after her first symptoms, had no substantial problems outside her visual ones, was still able to travel and teach, and showed acute insight into her own condition. Anna could still identify objects by inference, using her intact perceptions of color, shape, texture, and movement, along with her memory and intelligence. Dr. P. could not. He could not, for instance, identify a glove by sight or by feel (despite being able to describe it in almost absurdly abstract terms, as “a continuous surface infolded on itself [with] five outpouchings, if this is the word . . . a container of some sort?”)—until, by accident, he got it onto his hand. Dr. P., in general, was dependent on action, and came to a bewildered halt if the flow of action was stopped. Moreover, he was a professional singer, for whom singing was the most natural, irrepressible, spontaneous activity. This allowed his crucial discovery that he could bypass his agnosia to some extent by the use of songs; he would hum or sing dressing songs, shaving songs, action songs of all sorts. Music, he had found, could organize his activities, his daily life, to a remarkable degree.

I had seen Dr. P. in 1978, ten years before Benson and his colleagues described PCA. I was puzzled by the picture Dr. P. presented, the paradoxes of his illness. Clearly, he had a degenerative brain disease, yet it seemed quite different from any form of Alzheimer’s disease I had seen. But, if not Alzheimer’s, then what did he have? When I read about PCA in 1988—Dr. P. had died in the meantime—I wondered whether this in fact could have been his diagnosis.

But PCA is only an anatomical diagnosis; it denotes the part of the brain affected most, but says nothing of the underlying disease process, nothing of why these parts of the brain are damaged. It is similar, in a sense, to a clinical diagnosis (say, of autism) which indicates only a confluence of certain characteristic symptoms or signs or behaviors—a syndrome—but not the actual disease process (genetic, chemical, infectious, or whatever) that causes the syndrome.

When Benson described PCA in 1988, he had no information regarding the underlying pathology. His patients might have Alzheimer’s disease, he thought, but if so, it was Alzheimer’s with a strikingly atypical presentation. They might have Pick’s disease, a degenerative brain disorder more commonly affecting the frontal and temporal lobes of the brain. (Maurice Ravel is thought to have had this, so that he became unable to speak, or, tragically, compose.) They might even, Benson speculated, have vascular rather than degenerative disease, an accumulation of small blockages in the watershed zone between the posterior and carotid circulations of the brain.

In the early nineteen-nineties, it was established by other researchers that certain patients with PCA, upon autopsy, showed the characteristic microscopic plaques and tangles of Alzheimer’s disease, predominantly clustered in the posterior, visual parts of the brain. Not all PCA patients have Alzheimer’s, but the syndrome is now generally regarded as a “visual variant” of Alzheimer’s. It may, or may not, be that Anna H. has this form of the disease.

The very names of certain diseases inspire dread. This is especially so of Alzheimer’s disease—and its diagnosis may be taken almost as a death sentence. But this does not take into account the enormous variation there may be in the character and course of the disease, nor the varied adaptations and strategies by which life may be maintained and enjoyed despite the disease’s advance. I have seen malignant forms of Alzheimer’s that lead to profound dementia and death in a year, while other patients may have years of good life and decline only very slowly; I have seen patients in whom intellectual loss and personality change are prominent from the start, and others in whom such changes may not occur until the very last stages of the illness. Very slow decline, with preservation of insight and personality until very late, seems to be characteristic of the visual variant of Alzheimer’s. And in Anna H.’s case—if this is what she has—the course has been slower and more benign than in any other patient yet described.

In June of 1999, I revisited Anna and Josef in their apartment—Josef was just back from his weeks in Europe, and Anna, I gathered, had been moving freely within a four-block radius of their apartment, going to her favorite restaurant, shopping, etc. When I arrived, I saw that Anna had been sending cards to her friends all over the world—there were envelopes addressed to Korea, to Germany, to Australia, to Brazil, scattered all over the table. Her alexia, clearly, had not diminished her correspondence, though the names and addresses sometimes straggled over the envelope.

“Let’s go out, let’s wander,” I said. Anna immediately started singing “Der Wanderer”—she loves Schubert—and then the elaboration of this in the “Wanderer” Fantasy.

In the elevator, she greeted some neighbors. It was not clear to me that she recognized them visually; it may have been that they spoke to her first. She instantly recognized voices, noises of all sorts; indeed, she seemed hyperacute here, or hyperattentive, as she did to colors and shapes. They had assumed a special importance as cues.

She had no difficulty crossing the street. She could not read the “Walk” and “Don’t Walk” signs, but she knew their relative position and color; knew, too, that she could walk when the sign was blinking. She pointed out a synagogue on the corner opposite, and other shops she identified by their geometric patterns or colors, as with her favorite diner, which had alternating black and white tiles. At one point, we passed an enormous dog, which made me think of the Hound of the Baskervilles. Anna did not notice it at first, but when I drew her attention to it she thought its expression “patient and sweet” (I thought it murderous), and would have patted it had I not deflected her.

We went into a supermarket and got a cart—she headed instantly to the alcove where these were. She had no difficulty in finding the fruit-and-vegetable section, or in identifying apples, pears, carrots, yellow peppers, and asparagus. She said of a leek, “Is it a cousin of an onion?” and then got the word “leek.” She was puzzled by a kiwifruit, until I let her handle it. (She thought it “delightfully furry, like a little mouse.”) I reached up for an object hung above the fruit. “What is this?” I asked. Anna squinted, hesitated. “Is it edible? Paper?” When I let her touch it, she burst into somewhat embarrassed laughter. “It’s an oven glove, a pot sticker,” she said. “How could I be so silly?”

When we moved to the next section, Anna called out, “Salad dressings on the left, oils on the right,” in the manner of a department-store elevator operator. She obviously had the entire supermarket in her head. Wanting a particular tomato sauce, one of a dozen different brands, she picked it out because it had “a deep-blue rectangle and below that a yellow circle” on its label. “Color is of the essence,” she emphasized again. This is her most immediately visible cue, recognizable when nothing else is. (For that reason, fearing we might be separated, I had dressed entirely in red for our visit, knowing that it would allow her to spot me instantly if she got lost.)

But color was not always enough. If confronted with a plastic container, she might have no idea whether it contained peanut butter or cantaloupe. Often, she found that the simplest strategy was to bring in a used can or carton, and ask someone for help in matching it.

As we left the market, she accidentally crashed the shopping cart into a pile of shopping baskets to her right—such accidents, when they happen, are always to the right, because of the impaired visual awareness to this side.

Some months later, I arranged to see Mrs. H. in my own office rather than at the clinic, where she had come before. She arrived promptly, having made her way to Greenwich Village from Penn Station. She had been in New Haven the night before, where her husband had given a concert, and he had seen her onto a train that morning. “I know Penn Station like the back of my hand,” she said, so she did not have problems there. But outside, in the melee of people and traffic, “there were many moments when I had to ask.”

When I asked how she had been doing, she said her agnosia was getting worse. “When you and I went to the market together, there were many things I could recognize easily. Now, if I want to buy the same things, I have to ask people.” In general, she had to ask others to identify objects for her, and sometimes to help her if there were awkward steps, sudden changes of level, or irregularities in the ground. She depended more on touch and on hearing (to make sure, for instance, that she was facing the right way). And she depended increasingly on her memory, her thinking, and her logic and common sense to help negotiate what would otherwise be—visually—an unintelligible world.

Yet, in my office, she immediately recognized herself on a CD cover, playing Chopin. “It looks slightly familiar,” she said, with a smile.

I asked her what she saw on the wall of my office. First, she turned her chair not to the wall but to the window, and said, “I see buildings.” Then I rotated her chair for her until she faced the wall. I had to take her through it bit by bit—“Do you see lights?” Yes, there, and there. It took a little while to establish that she was looking at a sofa under the lights, though its color was commented on at once. She observed something green lying on the sofa, and astonished me by saying, correctly, that it was a stretch cord. She said she had been given such a cord by her physiotherapist. Asked what she saw above the couch (a painting with abstract geometric forms), she said, “I see yellow . . . and black.” What is it? I asked. Something to do with the ceiling, Anna hazarded. Or a fan. A clock. Then she added, “I haven’t really found out whether it is one item or many.” It was in fact a painting done by another patient, a color-blind painter. But clearly Mrs. H. had no idea that it was a painting, was not even sure that it was a single object, and thought that it might even be part of the structure of the room.

I found all this puzzling. How was it that she could not clearly distinguish a striking painting from the wall itself, yet instantly recognize a small photograph of herself on a CD? How could she identify a slender green stretch cord while failing to see, or recognize, the sofa it was on? And there had been innumerable such inconsistencies before.

I wondered how she could read the time, since she was wearing a wristwatch. She could not read the numbers, she said, but could judge the position of the hands. I then showed her, mischievously, a strange clock I have, in which the numbers are replaced by the symbols of elements (H, He, Li, Be, etc.). She did not perceive anything the matter with this, as for her the chemical abbreviations were no more or less unintelligible than numerals would have been.

We went out for a walk, I in a bright-colored hat for recognition. Stopping at the first shopwindow, I asked Anna what she saw. “Straw hats,” she said. (I had just shown her a large Mexican straw hat I had brought back from Oaxaca, and she had instantly recognized it visually.) The shopwindow, however, contained not straw hats but straw baskets. She was most bewildered by the objects in an adjacent window—but so was I. This was a Tibetan-handicrafts shop, but they could have been Martian handicrafts, given the exotic unfamiliarity of everything. The shop next to this, curiously, she recognized at once, and mentioned having passed it on her way to my office. It was a clock shop, with dozens of clocks of different sizes and shapes. She told me later that her father had had a passion for clocks.

A padlock on another store was a total puzzle, though Anna thought it might be something “to open up . . . like a hydrant.” The moment she touched it, she knew what it was.

We stopped briefly for coffee, then I took her to my apartment, in the next block. I wanted her to try my grand piano, an 1894 Bechstein. Entering my apartment, she immediately recognized the grandfather clock in the hall. (Dr. P., by contrast, had tried to shake hands with a grandfather clock.) I played a couple of Chopin mazurkas for her, and she said, “Each one of them is a world of its own. . . . They are miniatures which are gigantic.”

She then sat at the piano and played a piece—a piece that I found puzzling, for it seemed familiar to me in a way, yet unfamiliar, too. When I said this, Anna told me it was a Haydn quartet that she had heard on the radio and been enchanted by a couple of years before, and which, immediately, she longed to play herself. So she had arranged it for the piano, and had done this entirely in her head, overnight. She had occasionally arranged pieces for the piano before, using manuscript paper and with the original score in front of her, but when this became impossible because of her alexia she found that she could do it wholly by ear. She felt that her musical memory, her musical imagery, had become stronger, more tenacious, but also more flexible, so that she could hear the most complex music in her mind, hold it, alter it, rearrange it, and replay it mentally, in a way that would have been impossible before her alexia. Her continually strengthening powers of musical memory and imagery had indeed become crucial to her, kept her going, since the onset of her visual difficulties, nine years earlier.

I was reminded, when Anna told me this, of a patient I had seen in the hospital some years before, who had overnight become totally paralyzed from a fulminating myelitis. When it became evident that no recovery was forthcoming, she fell into despair, felt that her life was over—not only the great things of life but the little familiar pleasures of each day, like doing the Times crossword, to which she was addicted. She requested that the Times be brought to her each day, so that at least she could look at the crossword, get its configuration, run her eyes along the clues. And when she did this she found her mind doing something extraordinary—for now, as she looked at the clues, the answers seemed to write themselves, like hallucinations, in their spaces. Her visual imagery strengthened over the next few weeks, until she found that she was able to hold the entire crossword and its clues in her mind after a single, intense inspection; and then she could solve it, mentally, at her leisure later in the day. This became a source of great solace to her, in her paralysis; she had no idea, she later told me, that such powers of memory and imagery were available to her. (I now suspect that such powers are not too uncommon in visual thinkers; I know one artist, a highly geometric thinker, who does diagramless crosswords mentally, manipulating the letters and their squares in his head while he is painting.)

Anna’s obvious confusion about what was what in my office, and in the little streets and shops around it, brought home to me how dependent she was on the familiar, the memorized; how anchored, in a sense, to her own apartment and her own neighborhood. In time, no doubt, if she were to visit a place frequently, she would gradually become more familiar with it, but this would be an almost unimaginably complex challenge, demanding great patience and resourcefulness, a whole new enterprise of categorization and memorization. It was clear to me, after this one visit of Anna’s to my office, that in future I should stick to house calls, visiting her in her own apartment, where she felt organized, in control, at home. Going out, for her, was becoming an increasingly surreal visual adventure, full of fantastic and sometimes frightening misperceptions.

Anna H. wrote to me again in August of 2001, expressing growing concern. She said that she hoped I might be able to come soon for a visit, and I suggested the following weekend.

Anna stood by her door to welcome me, knowing, as she does, my own (congenital) defects of visual and topographic memory, my confusion of left and right, and my inability to find my way around in buildings. She welcomed me with great warmth, but also a touch of anxiety, which seemed to hover throughout the visit.

“Life is difficult,” she began, after she had seated me and given me a glass of seltzer. She had difficulty finding the seltzer in her refrigerator, and, not seeing the bottle, which was “hidden” behind a jug of orange juice, she had taken to exploring the refrigerator by hand, groping for a bottle of the right shape. “It is not getting better. . . . The eyes are very bad.” (She knows, of course, that her eyes are fine, and that it is the visual parts of the brain that are declining in function—indeed, she realized this before anyone else—but she finds it easier, more natural, to refer to her “bad eyes.”) When I had gone shopping with her two years before, she seemed to recognize almost everything she saw, or at least had it coded by shape and color and location, so that she hardly ever needed help. At that time, too, she moved infallibly about her kitchen, never losing anything, working efficiently. Today, she “lost” both the seltzer and the schmaltz herrings— a losing that entailed not only not remembering where she put them but not recognizing them when she saw them. I also observed that the kitchen was less organized than it had been before—and organization is crucial in Mrs. H.’s situation.

Anna’s anomia, her problems with word finding, had increased, too. When I showed her some kitchen matches, she recognized them at once, visually, but could not say the word “match,” saying, instead, “That is to make fire.” The Sweet ’N Low, similarly, she could not name, but identified as “Better than Sugar.” And at one point, looking for some condiments, she got worried because she could not find the “little red things” in the kitchen—these turned out to be the red-topped spice bottles. Anna was well aware of these difficulties, and her strategies for dealing with them. “When I can’t say something,” she explained, “I circumscribe.” But it is only the names of visual objects, objects with appearances, which sometimes elude her. She has no difficulty with proper names, abstract nouns, musical terms, or the names for textures or smells or shapes or colors. Nor is there any compromise in her fluency or the organization of her speech, no larger linguistic problems. Her anomia, it seems, is integrally related to her visual problems, and not a form of aphasia in the usual sense.

When I asked her about travel and life generally, she said that although she had recently travelled to Ontario, to Colorado, and to Connecticut with her husband, she would not have been able to do this by herself, as she had only a few years before. But she said she remained quite capable of looking after herself at home when Josef was away. Still, she said, “When I am alone, it is lousy. I’m not complaining—I’m describing.”

While Anna was in the kitchen at one point, I asked Josef how he felt about these problems, and he expressed sympathy and understanding, but added, “My impatience is provoked sometimes when I think that some of her weaknesses may be exaggerated. I’ll give you an example. I get puzzled, annoyed sometimes, because Anna’s ‘blindness’ is sometimes ‘selective.’ Last Friday, she noticed that a painting was hung lopsidedly by a few millimetres. And sometimes she comments on people’s facial expressions in tiny photographs. She will touch a spoon and ask, ‘What is this?’ and then five minutes later look at a vase and say, ‘We have a similar one.’ I have found no pattern, only inconsistency. What should my attitude be when she grabs a cup and says, ‘What’s this?’ I sometimes don’t tell her. But this may be wrong, and the effect disastrous. What should I say?”

This was, indeed, a very delicate matter. How much should he intervene when she was faced with perceptual bewilderment? How much should we prompt a friend, or a patient, when he has forgotten someone’s name? How much do I myself—with no sense of direction—wish to be told of my error, or to battle out the right way by myself? How much do any of us like to be “told” anything? The question was especially vexing with Anna, for, while she needed to work things out, fend for herself, her sensory difficulties were becoming more severe all the time, and they sometimes threatened, as Josef observed, to throw her into an abyss of bewilderment, a disintegrating terror. I could suggest no rule, I said to Josef, except that of tact: each situation would call for its own solution.

But I, too, was puzzled when Josef spoke of the extraordinary variations in Anna’s visual function. Some of them, it seemed, went with the reduced and unstable function of her damaged visual cortex—just as, ten years earlier, when the first problems appeared, her ability to read music would come and go. Some of the variations might reflect fluctuations in blood flow. But some of the variations seemed to go with a decreasing ability, for whatever reason, to compensate in her usual way. Her ability to make use of her memory and her intellectual powers in place of direct visual recognition, I now felt, might also be diminishing at this point. Thus it was more important than ever to “code” things, to provide easily used sensory clues—above all, color, to which Anna remained intensely sensitive.

What intrigued me especially was Josef’s mention of Anna’s sudden abilities—her ability, for example, to recognize facial expressions on a tiny photograph, since most of the time she had the greatest difficulty recognizing people at all. I could not help wondering whether this was an example of the preconscious abilities she had shown on earlier testing—her ability, for example, to instantly categorize pictures as “living” or “non-living” before she could consciously recognize them (and even when she could not recognize them at all). Such unconscious recognition (so-called “blindsight”) might still be possible to some extent despite her agnosia, despite her cortical damage, because it made use of more primitive mechanisms in the visual system which, presumably, were still intact.

Anna has been ingenious, and resilient, since her illness started, eleven or twelve years ago. She has brought brilliant resources of every kind to her own aid—visual, musical, emotional, intellectual. Her family, her friends, her husband above all, but also her students and colleagues, helpful people in the supermarket or on the street—everyone has helped her cope. Her adaptations to the agnosia have been extraordinary—a lesson in what can be done to hold together a life in the face of severe perceptual and cognitive challenge. But it is in her art, her music, that Mrs. H. not only copes with disease but transcends it. This is clear when she plays the piano, an art that both demands and provides a sort of superintegration, a total integration of sense and muscle, of body and mind, of memory and fantasy, of intellect and emotion, of one’s whole self, of being alive. Her musical powers, mercifully, have remained untouched by her disease. (“Nothing wrong with my ears or my hands, thank God.”)

So I had a special sense of sadness during this visit in the summer of 2001, for Anna had injured her back in a fall, and sitting was painful, and I could not even ask her to play the piano, as I usually did. Her piano playing had always added a transcendent note to my visits, and it recalled her, no less crucially, to her identity as an artist, and showed the joy she could still get and give, whatever other problems were now closing in on her.

When I revisited Mr. and Mrs. H. not long ago, I found the apartment full of balloons. “It was my birthday,” Anna explained. “Three days ago.” She did not look well, and seemed somewhat frail, although her voice and her warmth were entirely unchanged. She said that her visual powers had deteriorated further, and this was all too evident as she groped for a chair to sit down on, walked in the wrong direction, or got lost inside her own apartment—something of which there had been no hint before. Her behavior now looked much more “blind,” reflecting not only her increasing inability to decipher what faced her but her complete lack of visual orientation and searching and gaze. She had difficulty picking out any shapes now, though her sense of color and motion were as acute as ever.

She is still able to write letters, but reading, even the painfully slow letter-by-letter reading that she could do a few years ago, has become impossible. She adores being read to—Josef reads her items from the papers and from books—and I promised to send her some audiotapes. She goes out a little, walking around the block on her husband’s arm. The two of them are very close, closer than ever now, with her increasing disability.

Despite all this, Anna feels her ear is as good as it always was, and she has been able to continue a little teaching, with students from the music college coming to the house. Apart from this, she says, she does very little playing now.

And yet, when I mentioned the Haydn quartet she had played for me before, her face lit up. “I was absolutely enthralled,” she said. “I’d never heard it before. It’s never played.” And she described for me again how, unable to get it out of her head, she had arranged it, mentally, for the piano, overnight. I asked her to play it for me again. Anna first demurred, and then, persuaded, encouraged, started for the piano, but went in the wrong direction. Josef corrected her, gently. At the piano, she first blundered, hitting wrong notes, and seemed anxious and confused. “Where am I?” she cried, and I thought, Oh, God, she can no longer play. But then, oriented to the keyboard, she found her place and began to play beautifully, the sound soaring up, melting, twisting into itself. Even Josef was amazed and moved by this. “She hasn’t played at all for two or three weeks,” he whispered to me. As she played, Anna stared upward, singing the melody softly to herself. She played with consummate artistry, with all the power and feeling she had shown before, when, as the Haydn drew to its final, resolving chords, after a furious turbulence, a musical altercation, she said, simply, “All is forgiven.” ♦