Finally, Levitin, Bellugi, and others turned to investigating the functional correlates of musicality in Williams syndrome. Were the musicality and the emotional response to music in those with Williams syndrome, they wondered, subserved by the same sort of neurofunctional architecture as that in normal subjects or in professional musicians? They played a variety of music, from Bach cantatas to Strauss waltzes, to all three groups, and it was evident from brain imaging that people with Williams syndrome processed the music very differently from the others. They employed a much wider set of neural structures to perceive and respond to music, including regions of the cerebellum, brain stem, and amygdala which are scarcely activated at all in normal subjects. This very extensive brain activation, particularly of the amygdala, seemed to go with their almost helpless attraction to music and their sometimes overwhelming emotional reactions to it.
All of these studies, Bellugi feels, suggest that “the brains of Williams syndrome individuals are organized differently from normals, at both a macro and a micro level.” The very distinctive mental and emotional characteristics of people with Williams syndrome are reflected, very precisely and beautifully, in the peculiarities of their brains. Though this study of the neural basis of Williams syndrome is far from complete, it has nonetheless already made possible the most extensive correlation ever made between a multitude of mental and behavioral characteristics and their cerebral basis.
In people with Williams syndrome, it is now known, there is a “microdeletion” of fifteen to twenty-five genes on one chromosome. The deletion of this tiny gene cluster (less than a thousandth part of the twenty-five thousand or so genes in the human genome) is responsible for all of the features of Williams syndrome: the abnormalities of the heart and blood vessels (which have insufficient elastin); the unusual facial and bony features; and, not least, the unusual development of the brain— so well developed in some ways, so underdeveloped in others— which underlies the unique cognitive and personality profile of those with Williams.
More recent research has suggested differentiation within this gene cluster, but the most tantalizing part of the puzzle still eludes us. We think we know which genes are responsible for some of the cognitive deficits of Williams syndrome (such as the lack of visuospatial sense), but we do not know how such a deletion of genes can give rise to the special gifts of people with Williams syndrome. It is not even certain that these have a direct genetic basis; it is possible, for example, that some of these skills are simply spared by the vicissitudes of brain development in Williams syndrome, or that they may arise as a sort of compensation for the relative lack of other functions.
Freud once wrote, “Anatomy is destiny.” Now we tend to think that destiny is written in our genes. Certainly Williams syndrome affords an extraordinarily rich and precise view of how a particular genetic endowment can shape the anatomy of a brain and how this, in turn, will shape particular cognitive strengths and weaknesses, personality traits, and perhaps even creativity. And yet, beneath the superficial similarities among people with Williams syndrome, there is an individuality that, as with us all, is largely determined by experience.
* * *
IN 1994, I visited Heidi Comfort, a young girl with Williams syndrome, at her home in southern California. A very self-possessed eight-year-old, she immediately detected my own diffidence and said, encouragingly, “Don’t be shy, Mr. Sacks.” As soon as I arrived, she offered me some just-baked muffins. At one point, I covered the tray of muffins and asked her to tell me how many there were. She guessed three. I uncovered the tray and invited her to count them. She pointed to them, one by one, and came up with a total of eight; there were, in fact, thirteen. She showed me her room and her favorite things, as any eight-year-old might.
A few months later, we met again in Ursula Bellugi’s lab, then went out for a walk. We watched the kites and hang gliders sailing above the La Jolla cliffs; in town we gazed into the windows of a pastry shop and then stopped for lunch at a sandwich shop, where Heidi instantly befriended the half dozen workers behind the counter, learning all their names. At one point, she leaned so far over the counter, fascinated by the sandwich-making, that she nearly fell into the tuna fish. Her mother, Carol Zitzer-Comfort, told me that she had once warned her child not to speak to strangers and Heidi had replied, “There are no strangers, there are only friends.”
Heidi could be eloquent and funny, and she loved to spend hours listening to music and playing the piano; she was already composing little songs at eight. She had all the energy, impulsiveness, verbosity, and charm of Williams syndrome, and many of the problems. She could not form a simple geometric shape with some wooden blocks, as most children can do by nursery school. She had great difficulty placing a set of nesting cups into the right order. We went to the aquarium, where we saw a giant octopus, and I asked her how much it might weigh. “Thirty-two hundred pounds,” she replied. Later that day, she estimated that the creature had been “as big as a building.” Her cognitive impairments might be, I thought, quite disabling— both in school and in the world. And I could not avoid the feeling that there might be a sort of formulaic quality to her sociability, an automaticity. It was difficult for me to see her, at eight, as an individual separate from the superficial qualities of her Williams syndrome.
But ten years later, I received a letter from her mother. “Heidi has just had her eighteenth birthday,” Carol wrote. “I’m attaching a picture of her with her boyfriend at their homecoming dance. She is in her senior year at high school and has definitely come into her own as a young woman. Dr. Sacks, you were right when you predicted that the ‘who’ would emerge through the ‘what’ of Williams syndrome.”5
Heidi was nineteen now, and despite several brain surgeries to treat increased pressure (such procedures are occasionally necessary in some people with Williams syndrome), she was planning to leave home soon, to attend a residential college program where she would take academic courses, receive job coaching, and prepare to live independently. She planned to learn how to be a professional baker— she loved watching people decorate cakes and make desserts.
But a few months ago, I received another letter from her mother, telling me that Heidi had started a new job— and it sounds as though she may have found another calling:
She is working at a convalescent home and just loves it. The patients say that Heidi’s bright smile cheers them up and helps them feel better. Heidi enjoys the socializing so much that she has asked if she can visit the patients on the weekends. She plays bingo, paints their fingernails, gets them coffee, and, of course, talks and listens. This job is a perfect fit for her.
29
Music and Identity: Dementia and Music Therapy
Of the five hundred or so neurological patients at my hospital, about half have dementia of various sorts— from multiple strokes, from cerebral hypoxia, from toxic or metabolic abnormalities, from brain injuries or infections, from frontotemporal degeneration, or, most commonly, from Alzheimer’s disease.
Some years ago, Donna Cohen, a colleague of mine, after studying our large population of patients with Alzheimer’s, coauthored a book called The Loss of Self. For various reasons, I deplored the title (though it is a very good book as a resource for families and caregivers) and set myself to contradicting it, lecturing here and there on “Alzheimer’s Disease and the Preservation of Self.” And yet, I am not sure that we were in real disagreement.
Certainly someone with Alzheimer’s loses many of his powers or faculties as the disease advances (though this process may take many years). The loss of certain forms of memory is often an early indicator of Alzheimer’s, and this may progress to a profound amnesia. Later there may be impairment of language and, with the involvement of the frontal lobes, loss of subtler and deeper powers, like judgment, foresight, and the ability to plan. Eventually a person with Alzheimer’s may lose some fundamental aspects of self-awareness, in particular the awareness of their own incapacities. But does the loss of one’s
self-awareness, or some aspects of mind, constitute loss of self?
Shakespeare’s Jaques, in As You Like It, considering the seven ages of man, sees the final one as “sans everything.” Yet though one may be profoundly reduced and impaired, one is never sans everything, never a tabula rasa. Someone with Alzheimer’s may undergo a regression to a “second childhood,” but aspects of one’s essential character, of personality and personhood, of self, survive— along with certain, almost indestructible forms of memory— even in very advanced dementia. It is as if identity has such a robust, widespread neural basis, as if personal style is so deeply ingrained in the nervous system, that it is never wholly lost, at least while there is still any mental life present at all. (This, indeed, is what one might expect if perceptions and actions, feelings and thoughts, have molded the structure of one’s brain from the start.) This is poignantly clear in such memoirs as John Bayley’s Elegy for Iris.
In particular, the response to music is preserved, even when dementia is very advanced. But the therapeutic role of music in dementia is quite different from what it is in patients with motor or speech disorders. Music that helps patients with parkinsonism, for example, must have a firm rhythmic character, but it need not be familiar or evocative. With aphasics it is crucial to have songs with lyrics or intoned phrases, and interaction with a therapist. The aim of music therapy in people with dementia is far broader than this— it seeks to address the emotions, cognitive powers, thoughts, and memories, the surviving “self” of the patient, to stimulate these and bring them to the fore. It aims to enrich and enlarge existence, to give freedom, stability, organization, and focus.
This might seem a very tall order— nearly impossible, one would think, seeing patients with advanced dementia, who may sit in a seemingly mindless, vacant torpor or scream agitatedly in incommunicable distress. But music therapy with such patients is possible because musical perception, musical sensibility, musical emotion, and musical memory can survive long after other forms of memory have disappeared.1 Music of the right kind can serve to orient and anchor a patient when almost nothing else can.
I see this continually with my patients, and I hear of it constantly in the letters I receive. One man wrote to me about his wife:
Although my wife has Alzheimer’s— diagnosed at least seven years ago— the essential person miraculously remains…. She plays piano several hours daily, very well. Her present ambition is to memorize the Schumann A-minor Piano Concerto.
And yet this is a woman who is, in most other spheres, grossly forgetful and disabled. (Nietzsche, too, continued to improvise at the piano long after he had been rendered mute, demented, and partially paralyzed by neurosyphilis.)
The extraordinary neural robustness of music is also brought out in the following letter I was sent, about a well-known pianist:
[He] is 88 now and has lost language…but he plays every day. When we read through Mozart, he points back and ahead well in advance of the repeats. Two years ago we recorded the complete four-hand repertoire of Mozart that he had recorded…in the 1950s. While his language has begun to fail him, I love his recent playing and conception even more than the earlier recording.
Especially moving here is not merely the preservation but the apparent heightening of musical powers and sensitivity, as other powers wane. My correspondent concluded: “The extremes of musical accomplishment and illness are so plainly evident in his case; a visit becomes miraculous as he transcends the disease with music.”
* * *
MARY ELLEN GEIST, a writer, contacted me a few months ago about her father, Woody, who began to show signs of Alzheimer’s thirteen years ago, at the age of sixty-seven. Now, she said,
The plaque has apparently invaded a large amount of his brain, and he can’t remember much of anything about his life. However, he remembers the baritone part to almost every song he has ever sung. He has performed with a twelve-man a capella singing group for almost forty years…. Music is one of the only things that keep him grounded in this world.
He has no idea what he did for a living, where he is living now, or what he did ten minutes ago. Almost every memory is gone. Except for the music. In fact, he opened for the Radio City Music Hall Rockettes in Detroit this past November…. The evening he performed, he had no idea how to tiea tie…he got lost on the way to the stage— but the performance? Perfect…. He performed beautifully and remembered all the parts and words.
A few weeks later, I had the pleasure of meeting Mr. Geist, his daughter, and his wife, Rosemary. Mr. Geist was, in fact, carrying a newspaper, a neatly furled New York Times— though he did not know it was the New York Times, nor (apparently) what a “newspaper” was.2 He was well-groomed and neatly dressed, though this, his daughter later told me, had needed supervision, for left alone he might put on his pants backwards, not recognize his shoes, shave with toothpaste, and so on. When I asked Mr. Geist how he was, he replied, pleasantly, “I think I am in good health.” This reminded me of how Ralph Waldo Emerson, after he became severely demented, would answer such questions by saying, “Quite well; I have lost my mental faculties but am perfectly well.”3
Indeed, there was an Emersonian sweetness and reasonableness and serenity in Woody (as he immediately introduced himself)— he was profoundly demented, without doubt, but he had preserved his character, his courtesy, his thoughtfulness. Despite the manifest ravages of Alzheimer’s— his loss of event memory and of general knowledge, his disorientation, his cognitive defects— the behaviors of civility, it seemed, were ingrained, perhaps at a much deeper and older level. I wondered whether these were merely habits, mimicries, residues of once-meaningful behavior, now empty of feeling and meaning. But Mary Ellen had never thought this— she felt her father’s civility and courtesy, his sensitive and thoughtful behavior, to be “almost telepathic.”
“The way he reads my mother’s face to find out how she is doing,” she wrote, “the way he reads her mood, the way he reads people in social situations and acts accordingly…is beyond mimicking.”
Woody seemed to be tiring of questions to which he could not supply an answer (such as “Can you read this?” or “Where were you born?”), so I asked him to sing. Mary Ellen had told me how, since she could first remember, the whole family— Woody, Rosemary, and the three daughters— had sung together, and how singing had always been a central part of family life. Woody had been whistling when he came in, whistling “Somewhere over the Rainbow,” so I asked him to sing it. Rosemary and Mary Ellen joined in, and the three of them sang beautifully, each harmonizing in different ways. When Woody sang, he showed all the expressions, emotions, and postures appropriate to the song, and to singing in a group— turning to the others, awaiting their cues, and so on. This was so with all the songs they sang— whether they were exuberant, jazzy, lyrical and romantic, funny, or sad.
Mary Ellen had brought along a CD Woody had recorded years before with his a cappella group, the Grunyons, and when we played this, Woody sang along beautifully. His musicality, at least his performing musicality, like his civility and equanimity, was completely intact— but again, I wondered if it could be just a mimesis, just a performance, representing feelings and meanings he no longer had. Certainly Woody looked more “present” when singing than at any other time. I asked Rosemary whether she felt that he, the man she had known and loved for fifty-five years, was totally present in his singing. She said, “I think he probably is.” Rosemary looked tired, exhausted, from her almost nonstop caring for her husband, and the inch-by-inch way in which she was being widowed, as he lost more and more of what used to constitute his self. But she was least sad, least widowed, when they all sang together. He seemed so present at such times that his absence a few minutes later, his forgetting that he had sung (or could sing), would always come as a shock.
Given her father’s powerful musical memory, Mary Ellen asked, “Why can’t we use this as an opening…embed shopping lists, information about himself, in his songs?” I said I feare
d this would not work.
Mary Ellen had, in fact, found this out already for herself. “Why couldn’t we sing him his life story?” she had written in her journal in 2005. “Or the directions from one room to the next? I’ve tried— it doesn’t work.” I too had had this thought, in relation to Greg, an intelligent, very musical, very amnesic patient I had seen years before. Writing about him in The New York Review of Books in 1992, I observed:
It is easy to show that simple information can be embedded in songs; thus we can give Greg the date every day, in the form of a jingle, and he can readily isolate this, and say it when asked— give it, that is, without the jingle. But what does it mean to say, “This is December the 19th, 1991,” when one is sunk in the profoundest amnesia, when one has lost one’s sense of time and history, when one is existing from moment to moment in a sequenceless limbo? “Knowing the date” means nothing in these circumstances. Could one, however, through the evocativeness and power of music, perhaps using songs with specially written lyrics— songs which relate something valuable about himself or the current world— accomplish something more lasting, deeper? Give Greg not only “facts,” but a sense of time and history, of the relatedness (and not merely the existence) of events, an entire (if synthetic) framework for thinking? This is something which Connie Tomaino and I are trying to do now. We hope to have an answer in a year.