Williams Syndrome

by F.

ScienceDaily has an interesting piece on Williams Syndrome:

What is it?

Children with Williams syndrome, a rare genetic disorder, just love music and will spend hours listening to or making music. Despite averaging an IQ score of 60, many possess a great memory for songs, an uncanny sense of rhythm, and the kind of auditory acuity, than can discern differences between different vacuum cleaner brands.

What are the other effects?

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.

What part of the brain is affected?

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 with Williams syndrome. In their study, the researchers tried to answer the question of whether an atypical development of the planum temporale, which is part of the temporal lobe and thought to be involved many auditory tasks, including perfect pitch, may underlie the unusual musical and language skills.

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