Musicophilia: Tales of Music and the Brain (35 page)

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.

I
n 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 hand, 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.)

Anne, the eldest at forty-six, had undergone many surgeries to treat the physical problems that can come with Williams syndrome. She looked much older than her age, but she also conveyed a sense of wisdom and insight, and often seemed to be regarded by the others as a sort of adviser and honored elder. She favored Bach, and played some of the
Forty-eight Preludes and Fugues
for me on the piano. Anne lived quasi-independently, with some help; she had her own apartment and her own phone— though with her Williams loquacity, she said, she would often run up huge phone bills. Very important to Anne was her close relationship with her music teacher, who seemed to have a most sensitive ability to help her find musical expression for her feelings— as well as helping with the technical challenges of piano playing, which were exacerbated by Anne’s medical problems.

Even as toddlers, children with Williams syndrome are extraordinarily responsive to music, as I later saw at a Williams syndrome clinic at the Children’s Hospital at Montefiore in the Bronx. Here people of all ages go for periodic medical evaluation, but also to see one another and to make music with a gifted music therapist, Charlotte Pharr, whom they seem to adore. Majestic, a small three-year-old, was withdrawn and unresponsive to everyone and everything in his environment. He was making odd noises of every sort, but Charlotte began to imitate his noises, immediately catching his attention. The two of them began to exchange a volley of noises, which soon became rhythmic patterns, then musical tones and short improvised melodies. With this, Majestic was transformed in a remarkable way— he became fully engaged and even grabbed Charlotte’s guitar (it was bigger than he was) and plucked its strings one by one for himself. His eyes were constantly fixed on Charlotte’s face, drawing encouragement, support, and orientation from her. But when the session was over and Charlotte left, he soon reverted to the unresponsive state he had been in before.

Deborah, an engaging seven-year-old, was diagnosed with Williams syndrome before she was a year old. Storytelling and playacting were as important for Debbie as music— she always wanted a dramatic accompaniment of words and actions, rather than “pure” music. She knew by heart all the songs from her synagogue, but when her mother began to demonstrate this, she inadvertently sang a melody from her own childhood. “No!” said Debbie. “I want to do the song from
my
synagogue!” And she proceeded to sing it. (The songs from the synagogue are, of course, charged with meaning and narrative, the drama of ritual and liturgy— it is not coincidental that some cantors, like Richard Tucker, have become opera singers, going from the drama of the synagogue to that of the stage.)

Tomer, at six, was a strong, energetic boy, with a tough, outgoing personality to match. He adored drumming and seemed intoxicated by rhythms. When Charlotte demonstrated various complex rhythms, he got these instantly— indeed, he could simultaneously drum different rhythms with each hand. He anticipated rhythmic phrases and could improvise easily. At one point, the exuberance of drumming so overcame him that he flung the drumsticks down and started dancing instead. When I asked him about the names of different types of drums, he rapidly reeled off twenty different kinds from around the world. With training, Charlotte thought, he could certainly become a professional drummer when he grew up.

Pamela, at forty-eight, was, like Anne at the camp, the eldest, and she was highly, at times heartbreakingly, articulate. She became tearful at one point, speaking of the group home where she lived with other “disabled” people. “They call me all kinds of hurtful things,” she said. They did not understand her, could not comprehend, she said, how she could be so articulate and yet so disabled in other ways. She longed for a friend, for someone else with Williams syndrome with whom she could feel at ease, talk, and make music. “But there are not enough of us,” she said, “so I’m the only Williams in the place.” I had the feeling, as I had had with Anne, that Pamela had acquired a painful wisdom, a larger perspective, with age.

Pamela’s mother mentioned that she liked the Beatles, so I started singing “Yellow Submarine,” and Pamela joined in, bursting into a loud, joyful rendition and smiling broadly. “She comes alive with music,” her mother said. She had a huge repertoire, from Yiddish folk songs to Christmas hymns, and once she had started, there was no stopping her. She sang sensitively, always getting the emotion, and yet— I was surprised by this— she was often off-key, sometimes without any clear tonal center whatever. Charlotte, too, had observed this and had difficulty accompanying Pamela with her guitar. “People with Williams all love music,” she said, “they are all deeply moved by it, but not all of them are geniuses, not all of them are musically talented.”

W
ILLIAMS SYNDROME IS
very rare, affecting perhaps one child in ten thousand, and it was not formally described in the medical literature until 1961, when J. C. P. Williams, a New Zealand cardiologist, published a paper on it; the following year, it was independently described by Alois J. Beuren and his colleagues in Europe. (In Europe, therefore, people tend to refer to Williams-Beuren syndrome, but in the United States it is usually known as Williams syndrome.) They each described a syndrome characterized by defects of the heart and great vessels, unusual facial conformations, and retardation.

The term “retardation” suggests an overall or global intellectual defect, one that impairs language ability along with all other cognitive powers. But in 1964, G. von Arnim and P. Engel, who noted the elevated calcium levels that seemed to go along with Williams syndrome, also observed a curiously uneven profile of abilities and disabilities. They spoke of the children’s “friendly and loquacious personalities” and “their unusual command of language”— the last thing one would expect to find in a “retarded” child. (They noted too, though only in passing, that these children seemed to have a strong attachment to music.)

Individual parents of these children, similarly, were often struck by the unusual constellation of strengths and intellectual disabilities their children displayed and found it very difficult to find appropriate environments or schooling for them, as they were not “retarded” in the usual sense. In the early 1980s, a group of such parents in California discovered one another and came together to form the nucleus of what would become the Williams Syndrome Association.
1

At much the same point, Ursula Bellugi, a cognitive neuroscientist who had pioneered research on deafness and sign language, became fascinated by Williams syndrome. She had met Crystal, a fourteen-year-old girl with Williams syndrome, in 1983 and was intrigued and charmed by her, not least by her ready improvisations of songs and lyrics. Bellugi arranged to see Crystal every week for a year, and this was the start of an enormous enterprise.

Bellugi is a linguist, albeit one as much attuned to the emotional powers of speech, and all the poetic uses of language, as to its formal linguistic character. She was fascinated by the large vocabularies and unusual words that youngsters with Williams syndrome would use, despite their low IQs— words like “canine,” “abort,” “abrasive,” “evacuate,” and “solemn.” When asked to name as many animals as she could, one child’s first responses were “newt, saber-tooth tiger, ibex, antelope.”
2
And it was not only a large and unusual vocabulary, but all communicative powers that seemed highly developed in these children, especially in contrast to IQ-matched youngsters with Down’s syndrome. Those with Williams particularly showed a special feeling for narrative. They would use vivid sound effects and other devices to convey feeling and heighten the impact of what they said; Bellugi called these “audience-hookers”— locutions like “All of a sudden,” “Lo and behold!,” and “Guess what happened next?” It became increasingly clear to Bellugi that this narrative skill went with their hypersociability— their longing to connect and bond with others. They were minutely aware of personal details, they seemed to study people’s faces with extraordinary attention, and they showed great sensitivity in reading others’ emotions and moods.

They seemed strangely indifferent, though, to the nonhuman in their environments. Indifferent and inept— in some cases, children with Williams syndrome were unable to tie their shoes, to judge obstacles and steps, to “get” how things were arranged in the house. (This was in striking contrast to autistic children, who might fixate on inanimate objects and seemed indifferent to the emotions of others. In some ways, Williams seemed to be the exact opposite of severe autism.) Some children with Williams syndrome were utterly unable to put simple Lego blocks together— toys that IQ-matched children with Down’s syndrome could easily assemble. And many children with Williams syndrome were unable to draw even a simple geometric shape.