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Williams Syndrome: New UD Study May Shed Light On Rare Genetic Disorder-And Normal Development

Children with Williams Syndrome are delightful and engaging, with elfin-like features and often-extraordinary verbal skills but severe spatial deficits, and a new University of Delaware study may reveal the cognitive impacts of the rare genetic disorder.

"Understanding the details of the cognitive profile in this syndrome will likely be extremely complex," says Barbara Landau, a professor of psychology and director of UD's Language and Cognition Laboratory. "But ultimately, it will shed light on how brain and cognitive development become compromised by small genetic defects. This, in turn, will enhance our understanding of how normal development occurs."

The musical and verbal skills of children with Williams Syndrome are extraordinary. But when they see a circle that is half red and half green, they are at a loss to replicate it. They may correctly select a red crayon and a green one, but their drawings will not even remotely resemble the original two-tone circle.

A recent report on "60 Minutes" described a similar grown-up who can sing nearly 2,000 songs memorized in more than 20 foreign languages, yet is unable to solve simple mathematical problems.

Such are the mystifying intellectual discrepancies of those diagnosed with Williams Syndrome. First recognized as a separate syndrome in 1961, it has only been in the last 30 years that persons with Williams have been recognized as a group with a unique cognitive profile.

In particular, individuals with Williams Syndrome have very large discrepancies across their cognitive abilities. One striking discrepancy is that between language and spatial skills: Their language is, in many ways, quite normal, but they show profound deficiencies in certain spatial skills. Landau, an expert in the field of spatial cognition, was intrigued by this riddle.

With her colleague James Hoffman, a professor of psychology, and her team of graduate and undergraduate researchers, Landau recently received a $59,208 gran
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Contact: Beth Thomas
beth.thomas@mvs.udel.edu
302-831-8749
University of Delaware
3-Jul-1998


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