Krieger Scientist Sheds Light on
Cognition, Brain Development

People with a rare genetic condition called William’s syndrome are a study in contrasts. Their IQs place them in the mildly to moderately retarded range, yet they are generally strong readers who also use spoken language in often sophisticated ways. They are sociable and have an affinity (some call it a “gift”) for music. Yet many people with William’s cannot tie their own shoes or distinguish right from left, and if you ask them to copy a simple drawing of a bicycle, the wheels, chain, seat, frame, and pedals appear in disconnected bits all over the page.

It’s little wonder, then, that the paradoxes inherent in the syndrome, which afflicts one in every 20,000 children born in the United States, fascinate Barbara Landau, the Dick and Lydia Todd Professor of Cognitive Science at Johns Hopkins.

Landau finds William's syndrome compelling because it suggests that genetic material affects not only microscopic processes in the brain, but also higher-level functions, such as how people think and process information.

“This is very important as we go forward in trying to understand the relationships among gene, brain, mind, and behavior,” she says.

First identified in 1961, William’s syndrome results when a chunk of genetic material goes missing—for reasons that remain mysterious—from chromosome 7.

Of particular interest to Landau was that people with William’s combine normal language acquisition with an often severely impaired sense of spatial knowledge/relationships.

“For me, the key questions have always been: How do we learn language? How is it supported by our knowledge of objects and events around us, their spatial relationships, and so on?” says Landau, who first heard about William’s in the early 1990s and immediately grasped that the condition carried huge implications for her research.

Landau’s work on the syndrome has kept her busy for the past decade and made her Ames Hall lab something of a mecca for parents of children with William’s syndrome, who come from neighboring states to have their children participate in research studies.

“These parents are desperate to really understand this syndrome,” Landau says. “Until recently, William’s syndrome often went undiagnosed, and even when it was diagnosed, children were put into ‘special ed’ classes at school. No one really understands the special profile of WS … so there is no education tuned to their special strengths. But the parents are very interested in getting basic research done to understand what’s wrong, as well as what’s right.”

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