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Williams Syndrome, the Brain, and Music

enes missing in people with Williams syndrome, whereas Debra Mills, an associate professor in the Department of Psychology at Emory University, concentrates on the neurophysiology, the electrical activity of behaving neural networks. Says Bellugi, who studies the cognitive aspects of the disorder: “Things are really starting to come together now.”

Identified more than 40 years ago, Williams syndrome arises from a faulty recombination event during the development of sperm or egg cells. As a result, almost invariably the same set of about 20 genes is deleted from one copy of chromosome seven, catapulting the carrier of the deletion into a world where people make much more sense than objects do.

“Williams syndrome is a perfect example where a genetic predisposition interacts with the environment to sculpt the brain in unique ways,” says Reiss. “It provides a unique window of understanding on how the brain develops under typical and atypical conditions,” he adds.

People with Williams syndrome are irresistibly drawn to strangers, remember names and faces with ease, show strong empathy and have fluent and exceptionally expressive language. Yet, they are confounded by the visual world around them: While they can’t scribble more than a few rudimentary lines to illustrate an elephant, they can verbally describe one in almost poetic detail.

“The discrepancy between their engaging social use of language and their poor visual-spatial skills is startling,” says Bellugi. “I am confident that once all the evidence is in, we will have identified genes and pathways in the Williams syndrome deletion that underlie these drastic differences in modalities,” she adds.

Despite whole brain volumes that are about 15 percent smaller than normal, the temporal lobe, which lies above the ear canal and, among other things, is involved in processing sounds and interpreting music and language, is of approximately normal volume in people
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