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March 2012




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By Traci Pedersen Associate News Editor
Reviewed by John M. Grohol, Psy.D. on March 25, 2012

Williams Syndrome, a personality type that features extremely extroverted yet anxious behavior, is triggered by the abnormal development of a circuit center buried deep in the front center of the brain, according to scientists at the National Institutes of Health.

Researchers used three different kinds of brain imaging to identify the suspect area in the brains of people with Williams Syndrome, a rare genetic disorder.

When the scans were matched to scores on a personality rating scale, it became clear that the stronger these personality/temperament traits appeared in an individual with Williams Syndrome, the more abnormalities there were in a specific brain structure, called the insula.

“Scans of the brain’s tissue composition, wiring, and activity produced converging evidence of genetically-caused abnormalities in the structure and function of the front part of the insula and in its connectivity to other brain areas in the circuit,” explained Karen Berman, M.D., of the NIH’s National Institute of Mental Health (NIMH).

In Williams Syndrome, there is a deletion of some 28 genes, many involved in brain development and behavior, in a specific part of chromosome 7. Individuals with the syndrome lack visual-spatial skills and a tendency to be overly friendly with people, while overly anxious about non-social events, such as spiders or heights. Often, those with the disorder are learning disabled, but some have normal IQs.

“This line of research offers insight into how genes help to shape brain circuitry that regulates complex behaviors — such as the way a person responds to others — and thus holds promise for unraveling brain mechanisms in other disorders of social behavior,” said NIMH Director Thomas R. Insel, M.D.

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