Musicophilia: Tales of Music and the Brain (36 page)

Bellugi showed me how Crystal, despite her IQ of 49, had given a vivid, quirky description of an elephant, but the drawing of an elephant she had done a few minutes earlier bore no resemblance to an elephant or, indeed, to anything at all; none of the features she had painstakingly described had actually made their way into the drawing.
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O
BSERVANT
and often puzzled parents, while noting the problems and difficulties of their children, also noted their unusual social attention and friendliness, their reaching out to others. Many were struck by how, even as infants, their children listened with great attention to music and began to reproduce melodies accurately by singing or humming, even before they could speak. Some parents observed that their children would be so completely absorbed in music that they were unable to attend to anything else; other children were extremely sensitive to the emotions expressed in music and might burst into tears at a sad song. Others would play their instruments for hours each day, or might learn songs in three or four other languages, if they liked the melody and beat.

This was very much the case with Gloria Lenhoff, a young woman with Williams syndrome who learned to sing operatic arias in more than thirty languages. In 1988,
Bravo, Gloria,
a documentary about Gloria’s remarkable musical abilities, was aired on public television. Soon after, her parents, Howard and Sylvia Lenhoff, were surprised to get a phone call from someone who had seen the documentary, saying, in effect, “That was a wonderful film— but why did you not mention that Gloria has Williams syndrome?” The viewer, a parent, had identified Gloria right away from the characteristic facial features and behaviors of Williams syndrome. This was the first the Lenhoffs had heard of the syndrome; their daughter was thirty-three.

Since then, Howard and Sylvia Lenhoff have been instrumental in bringing awareness to the condition. In 2006, they collaborated with the writer Teri Sforza on
The Strangest Song,
a book about Gloria’s remarkable life. In this, Howard described Gloria’s musical precocity. At the age of one, he said, “Gloria could listen to ‘The Owl and the Pussycat’ and ‘Baa Baa Black Sheep’ over and over again— rhythm and rhyme delighted her.” In her second year, she became able to respond to rhythm.

“When Howard and Sylvia played their records,” Sforza wrote, “Gloria grew excited and focused all at once, pulling herself up in the crib, holding onto the railings, and bouncing up and down…keeping time to the beat.” Howard and Sylvia encouraged Gloria’s passion for rhythm by giving her tambourines, drums, and a xylophone, which she played with to the exclusion of all other toys. By her third year, Gloria could carry and sing a tune, and by her fourth year, Sforza noted, she was “ravenous for language…greedily picked up bits of Yiddish, Polish, Italian, whatever she heard…absorbed them like a sponge, and started to sing little songs in other languages.” She did not know these languages, but she had learned their prosody, their intonations and stresses, by listening to records, and could reproduce them fluently. Already then, at four, there was something extraordinary about Gloria, prefiguring the opera singer she was to become. In 1992, when Gloria was thirty-eight, Howard wrote to me:

Early in 1993, I met Gloria and accompanied her on the piano while she sang a few numbers from
Turandot,
which she performed, as always, with brilliance and an impeccable ear. Gloria, despite her deficits, is a dedicated professional who spends most of her time perfecting and expanding her repertoire. “We know she is ‘retarded,’ ” her father says, “but in comparison to her and others with Williams syndrome, are not most of us ‘retarded’ when it comes to learning and retaining complex music?”

Gloria’s talents are extraordinary, but not unique. At much the same time as her talents were emerging, another unusual youngster, Tim Baley, was showing a similar picture of striking musical abilities and fluent speech, along with severe intellectual impairments in many other respects. His musicality, and the support of his parents and teachers, allowed him, like Gloria, to become a performing musician (in his case, a pianist), and in 1994 Gloria and Tim joined three other musically talented people with Williams syndrome to form the Williams Five. They had their debut in Los Angeles, an event that led to features in the
Los Angeles Times
and on NPR’s
All Things Considered.

While all this delighted Howard Lenhoff, it left him dissatisfied. He was a biochemist, a scientist— and what had science to say about the musical gifts of his daughter and others like her? There had been no scientific attention to the musical passions and talents of people with Williams syndrome. Ursula Bellugi was primarily a linguist, and though she had been struck by the musicality of people with Williams syndrome, she had not made a systematic study of it. Lenhoff pressed her and other researchers to investigate this.

Not all people with Williams syndrome are as musically talented as Gloria— few “normal” people are. But virtually all share her passion for music and are extraordinarily responsive to music on an emotional level. Lenhoff felt, therefore, that there needed to be a proper arena, a musical arena, where people with Williams could meet and interact. He played a crucial role in setting up, in 1994, the camp in Massachusetts, where people with Williams could socialize and make music together, and receive formal training in music. In 1995, Ursula Bellugi went to the camp for a week; she returned the next year, accompanied by Daniel Levitin, a neuroscientist and professional musician. Bellugi and Levitin were thus able to put together and publish the first survey of rhythm in such a musical community, in which they wrote:

That the whole panoply of musical talents could be so strikingly developed in people who were deficient (sometimes severely so) in general intelligence showed, like the isolated powers of musical savants, that one could indeed speak of a specific “musical intelligence,” as Howard Gardner had postulated in his theory of multiple intelligences.

The musical talents of people with Williams syndrome differ from those of musical savants, however, for savant talents often seem to emerge full-blown, to have something of a mechanical quality, to require little reinforcement by learning or practice, and to be largely independent of influence by others. In children with Williams syndrome, by contrast, there is always a strong desire to play music with and for others. This was very clear with several young people I observed, including Meghan, whom I watched during one of her music lessons. She was clearly much attached to her teacher, listened to him carefully, and worked assiduously on suggestions he made.

Such engagement manifests itself in many ways, as Bellugi and Levitin found when they visited the music camp:

The three dispositions which are so heightened in people with Williams syndrome— the musical, the narrative, and the social— seem to go together, distinct yet intimately associated elements of the ardent expressive and communicative drive that is absolutely central in Williams syndrome.

G
IVEN SO EXTRAORDINARY
a constellation of cognitive talents and deficits, Bellugi and others started to explore what their cerebral basis might be. Brain imaging, along with, more rarely, autopsy reports, revealed remarkable divergences from the normal. The brains of people with Williams syndrome were, on average, twenty percent smaller than normal brains, and their shape was quite unusual, for the decrease in size and weight seemed to be exclusively at the back of the brain, in the occipital and parietal lobes, whereas the temporal lobes were of normal and sometimes supernormal size. This corresponded with what had been so clear in the uneven cognitive abilities of those with Williams— the devastating impairments of visuospatial sense could be attributed to the underdevelopment of parietal and occipital areas, while the strong auditory, verbal, and musical abilities could, in general terms, be attributed to the large size and rich neuronal networks of the temporal lobes. The primary auditory cortex was larger in people with Williams syndrome, and there seemed to be significant changes in the planum temporale— a structure known to be crucial for the perception of both speech and music, as well as for absolute pitch.
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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.

I
N 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.”