by Oliver Sacks
Miller et al. described a number of patients who showed heightenings of musical talents or, in some cases, the startling appearance of musical inclination and talents in previously “unmusical” people. Such patients had been described before in an anecdotal way, but no one before had seen and followed so many patients or explored their experiences in such depth and detail. I wanted to meet Dr. Miller and, if possible, some of his patients.
When we met, Miller first talked in general terms about frontotemporal dementia, how its symptoms and the underlying brain changes that caused them had been described in 1892 by Arnold Pick, even before Alois Alzheimer had described the better-known syndrome that now carries his name. For a time, “Pick’s disease” was considered relatively rare, but it is now becoming clear, Miller pointed out, that it is far from uncommon. Indeed, only about two-thirds of the patients Miller sees in his dementia clinic have Alzheimer’s disease; the remaining third have several other conditions, of which frontotemporal dementia is perhaps the most common.1
Unlike Alzheimer’s disease, which usually manifests itself with memory or cognitive losses, frontotemporal dementia often starts with behavioral changes— disinhibitions of one sort or another. This is perhaps a reason why relatives and physicians alike may be slow to recognize its onset. And, confusingly, there is no constant clinical picture but a variety of symptoms, depending on which side of the brain is chiefly affected and whether the damage is mainly in the frontal or the temporal lobes. The artistic and musical emergences that Miller and others have observed occur only in patients with damage chiefly in the left temporal lobe.
Miller had arranged for me to meet one of his patients, Louis F., whose story bore a striking resemblance to Vera B.’s. Even before I saw him, I heard Louis singing in the corridor, as, years before, I had heard Vera singing outside my clinic. When he entered the consulting room with his wife, there was barely a chance for hellos or handshakes, for he instantly burst into speech. “Near my house are seven churches,” he started. “I go to three churches on Sunday.” Then, presumably moved by the association of “church,” he burst into “We wish you a merry Christmas, we wish you a merry Christmas…” Seeing me sip a cup of coffee, he said, “Go on— when you’re old you can’t drink coffee,” and this then led to a little singsong: “A cup of coffee, coffee for me; a cup of coffee, coffee for me.” (I did not know if this was a “real” song or just the immediate thought of coffee transformed into a repetitive jingle.)
A plate of cookies attracted his attention; he took one and ate it voraciously, then another and another. “If you don’t take the plate away,” his wife said, “he’ll eat them all. He’ll say he’s full, but he’ll go on eating…. He’s put on twenty pounds.” He sometimes put nonfood items into his mouth, she added: “we had some bath salts shaped like candies, and he grabbed one, but had to spit it out.”
It was not so easy, however, to take the food away. I moved the plate, kept moving it into more and more inaccessible places, but Louis, without seeming to pay any attention to this, observed all my movements and would infallibly home in on the plate— under the desk, by my feet, in a drawer. (His ability to spot things was very acute, his wife told me; he would see coins or glittering objects in the street and pick up tiny crumbs from the floor.) Between eating and finding the cookie plate, Louis moved about restlessly and talked or sang nonstop. It was almost impossible to interrupt his speaking to have a conversation, or to get him to concentrate on any cognitive task— though he did, at one point, copy a complex geometrical figure and do an arithmetical calculation of a sort that would have been impossible for someone with advanced Alzheimer’s.
Louis works twice a week at a senior center, leading others in singing sessions. He loves this; his wife feels it may be the only thing that gives him any true pleasure now. He is only in his sixties, and he is not unconscious of what he has lost. “I don’t remember that stuff anymore, I don’t work anymore, I don’t do anything anymore— that’s why I help all the seniors,” he commented, but he said this with little emotional expression in his face or voice.
For the most part, left to his own devices, he will sing upbeat songs with great gusto. I thought that he sang a variety of such songs with sense and sensibility, but Miller cautioned me about assuming too much. For while Louis sang “My Bonnie Lies over the Ocean” with great conviction, he could not say, when asked, what an “ocean” was. Indre Viskontas, a cognitive neuroscientist working with Miller, demonstrated Louis’s indifference to the meaning of words by giving him a nonsensical but phonemically and rhythmically similar version to sing:
My bonnie lies over the ocean,
My bonnie lies under the tree,
My bonnie lies table and then some,
Oh, bring tact my bonnie to he.
Louis sang this with the same animation, the same emotion and conviction, as he had sung the original.
This loss of knowledge, of categories, is characteristic of the “semantic” dementia which such patients develop. When I started him singing “Rudolph, the Red-Nosed Reindeer,” he continued it perfectly. But he was not able to say what a reindeer was or to recognize a drawing of one— so it was not just the verbal or visual representation of reindeers that was impaired, but the idea of a reindeer. He could not say, when I asked him, what “Christmas” was, but instantly reverted to singing, “We wish you a merry Christmas.”
In some sense, then, it seemed to me that Louis existed only in the present, in the act of singing or speaking or performing. And, perhaps because of this abyss of nonbeing which yawned beneath him, he talked, he sang, he moved ceaselessly.
Patients like Louis often seem quite bright and intellectually intact, unlike patients with comparably advanced Alzheimer’s disease. On formal mental testing, they may, indeed, achieve normal or superior scores, at least in the earlier stages of their illness. So it is not really a dementia that such patients have but an amnesia, a loss of factual knowledge, such as the knowledge of what a reindeer is, or Christmas, or an ocean. This forgetting of facts— a “semantic” amnesia— is in striking contrast to their vivid memories for events and experiences in their own lives, as Andrew Kertesz has commented. It is the reverse, in a way, of what one sees in most patients with amnesia, who retain factual knowledge but lose autobiographical memories.
Miller has written about “empty speech” with regard to patients with frontotemporal dementia, and most of what Louis said was repetitive, fragmentary, and stereotyped. “Every utterance, I’ve heard before,” his wife remarked. And yet there were islands of meaning, moments of lucidity, as when he had spoken of not working, not remembering, not doing anything— which were surely real, and heartbreaking, even though they lasted only a second or two before they were forgotten, swept away in the torrent of his distraction.
Louis’s wife, who has seen this deterioration descend on her husband over the last year, looked frail and exhausted. “I wake at night,” she said, “and see him there, but he is not really there, not really present…. When he dies, I will miss him very much, but in some sense, he is already no longer here— he is not the same vibrant person I knew. It is a slow grieving, all the way through.” She fears, too, that with his impulsive, restless behavior, he will sooner or later have an accident. What Louis himself feels at this stage, it is difficult to know.
Louis has never had any formal musical education or vocal training, though he had occasionally sung in choruses. But now music and singing dominate his life. He sings with great energy and gusto, it obviously gives him pleasure, and, between songs, he likes to invent little jingles, like the “coffee” song. When his mouth is occupied in eating, his fingers will find rhythms, improvise, tap. It is not just the feeling, the emotion of songs— which I am sure he “gets,” despite his dementia— but musical patterns that excite and enchant him and, perhaps, hold him together. When they play cards in the evening, Mrs. F. said, “he loves to listen to music, taps his fingers or foot or sings while he plans his next m
ove…. He likes country music or golden oldies.”
Bruce Miller had perhaps chosen Louis F. for me to see because I had spoken of Vera, her disinhibition, her incessant babbling and singing. But there were many other ways, Miller said, that musicality could emerge and come to take over a person’s life in the course of a frontotemporal dementia. He had written about several such patients.
Miller has described one man who developed frontotemporal dementia in his early forties (the onset of frontotemporal dementia is often considerably earlier than that of Alzheimer’s) and who constantly whistled. He became known as “the Whistler” at work, mastering a great range of classical and popular pieces and inventing and singing songs about his bird.
Musical tastes, too, may be affected. C. Geroldi et al. described two patients whose lifelong musical tastes changed with the onset of frontotemporal dementia. One of them, an elderly lawyer with a strong preference for classical music and an antipathy to pop music (which he regarded as “mere noise”), developed a passion for what he previously hated and would listen to Italian pop music at full volume for many hours each day. B. F. Boeve and Y. E. Geda described another patient with frontotemporal dementia who developed a consuming passion for polka music.
At a much deeper level, a level beyond action, improvisation, and performance, Miller and his colleagues described (in a 2000 paper in the British Journal of Psychiatry) an elderly man with very little musical training or background who at sixty-eight began composing classical music. Miller emphasized that what occurred, suddenly and spontaneously, to this man were not musical ideas but musical patterns— and it was from these, by elaboration and permutation, that he built up his compositions.2 His mind, Miller wrote, was “taken over” during composition, and his compositions were of real quality (several were publicly performed). He continued composing even when his loss of language and other cognitive skills became severe. (Such creative concentration would not be possible for Vera or Louis, because they had severe frontal lobe damage early in their illnesses, and thus were deprived of the integrative and executive powers needed to reflect on the musical patterns rushing through their heads.)
Maurice Ravel, the composer, suffered in the last years of his life from a condition that was sometimes called Pick’s disease and would probably now be diagnosed as a form of frontotemporal dementia. He developed a semantic aphasia, an inability to deal with representations and symbols, abstract concepts, or categories. His creative mind, though, remained teeming with musical patterns and tunes— patterns and tunes which he could no longer notate or put on paper. Théophile Alajouanine, Ravel’s physician, was quick to realize that his illustrious patient had lost musical language but not his musical inventiveness. One wonders, indeed, whether Ravel was on the cusp of a dementia when he wrote his Bolero, a work characterized by the relentless repetition of a single musical phrase dozens of times, waxing in loudness and orchestration but with no development. While such repetition was always part of Ravel’s style, in his earlier works it formed a more integral part of much larger musical structures, whereas in Bolero, it could be said, there is the reiterative pattern and nothing else.
* * *
FOR HUGHLINGS JACKSON a hundred and fifty years ago (and for Freud, an ardent Jacksonian, a few years later) the brain was not a static mosaic of fixed representations or points, but incessantly active and dynamic, with certain potentials being actively suppressed or inhibited— potentials that could be “released” if this inhibition was lifted. Among such release phenomena, Jackson included epilepsy and chorea (and Freud the violent affects and impulses of the “id,” if it was uncapped by psychosis).
Normally there is a balance in each individual, an equilibrium between excitatory and inhibitory forces. But if there is damage to the (more recently evolved) anterior temporal lobe of the dominant hemisphere, then this equilibrium may be upset, and there may be a disinhibition or release of the perceptual powers associated with the posterior parietal and temporal areas of the non-dominant hemisphere.3 This, at least, is the hypothesis which Miller and others entertain, a hypothesis which is now gaining support from brain-imaging studies.
This hypothesis gains support clinically, too, from cases in which there is an emergence of musical or artistic talent following strokes or other forms of damage to the left hemisphere. This seemed to have been the case with a patient described by Daniel E. Jacome in 1984. Jacome’s patient had a postsurgical stroke causing extensive damage in the dominant left hemisphere— especially the anterior frontotemporal areas— which produced not only severe difficulties with expressive language (aphasia) but a strange access of musicality, with incessant whistling and singing and a passionate interest in music, a profound change in a man whom Jacome described as “musically naïve” before his stroke.
But the strange change did not last; it diminished, Jacome wrote, “in parallel with very good recovery of verbal skills.” These findings, he felt, “seem to support the greater role of the non-dominant hemisphere in music, somehow normally dormant and ‘released’ by dominant hemisphere damage.”
That musicality might be not only spared but heightened with damage to the language functions of the left hemisphere was suggested by Hughlings Jackson as early as 1871, when he wrote of singing in aphasic children. For him, this was an example— one of many— of normally suppressed brain functions being released by damage to others. Such dynamic explanations also seem very plausible in relation to other strange emergences and excesses: the musical hallucinations sometimes “released” by deafness, the synesthesia sometimes “released” by blindness, and the savant functions sometimes “released” by damage to the left hemisphere.
There have been many other stories, both in the medical literature and in the popular press, of people who have developed artistic talent following left-hemisphere strokes, or whose art has changed in character following such strokes— often becoming less constrained formally and freer emotionally. Such emergences or changes are often rather sudden.
One must wonder, too, about the “Grandma Moses” phenomenon— the unexpected and sometimes sudden appearance of artistic or musical powers in old age. Without speaking of “pathology” (perhaps, indeed, one should speak of “health” here), there may be a variety of inhibitions— psychological, neurological, and social— which may, for one reason or another, relax in one’s later years and allow a creativity as surprising to oneself as to others.
The musical or artistic powers that may be released in frontotemporal dementia do not come out of the blue; they are, one must presume, potentials or propensities that are already present but inhibited— and undeveloped. Once released by damage to these inhibitory factors, musical or artistic powers can potentially be developed, nurtured, and exploited to produce a work of real artistic value— at least as long as frontal lobe function, with its executive and planning powers, is intact. This, then, may provide a brief, brilliant interlude as the frontotemporal degeneration advances. The degenerative process, unfortunately, does not come to a halt, and sooner or later, all is lost— but for a brief time, for some, there can at least be music or art, with some of the fulfillment, the pleasure and joy it can so uniquely provide.
28
A Hypermusical Species: Williams Syndrome
In 1995 I visited a special summer camp in Lenox, Massachusetts, to spend a few days with a unique group of people, all of whom had a congenital disorder called Williams syndrome, which results in a strange mixture of intellectual strengths and deficits (most have an IQ of less than 60). They all seemed extraordinarily sociable and inquisitive, and though I had met none of these campers before, they instantly greeted me in the most friendly and familiar way— I could have been an old friend or an uncle, rather than a stranger. They were effusive and chatty, asking about my journey there, whether I had family, what colors and music I liked best. None of them was reticent— even the younger ones, at an age when most children are shy or wary of strangers, felt free to come up, take me by the h
and, look deep into my eyes, and converse with me with an adeptness that belied their years.
Most were in their teens or twenties, though there were a few younger children, as well as a woman of forty-six. But age and sex made relatively little difference in their appearance— all of them had wide mouths and upturned noses, small chins, and round, curious, starry eyes. Despite their individuality, they seemed like members of a single tribe marked by an extraordinary loquacity, effervescence, fondness for telling stories, reaching out to others, fearlessness of strangers, and, above all, a love of music.
Soon after I arrived, the campers trooped to a big tent, pulling me along with them, excited at the thought of a Saturday evening dance. Almost all of them would be performing and dancing. Steven, a stocky fifteen-year-old, was practicing on his trombone— the pure, assertive, brassy sounds of this, it was clear, satisfied him deeply. Meghan, a romantic and outgoing soul, was strumming her guitar and singing soft ballads. Christian, a tall, gangly youth wearing a beret, had a very good ear and was able to pick out and reproduce on the piano songs he had never heard before. (It was not just music that the campers were so sensitive, so attuned to; there seemed to be an extraordinary sensitivity to sounds generally— or, at least, attention to them. Tiny background sounds the rest of us did not hear or were not conscious of were immediately picked up and often imitated by them. One boy could identify the make of a car by the sound of its engine as it approached. As I walked in the woods with another boy the next day, we chanced on a beehive, and he was enchanted by this and started his own humming, which lasted the rest of the day. The sensitivity to sounds is highly individual and can vary moment by moment. One child at the camp might be enthralled by the noise of a particular vacuum cleaner, while another would be unable to stand it.)