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The newspaper of The Johns Hopkins University September 19, 2005 | Vol. 35 No. 3
Researcher Awarded $1.7 Million to Study Rare Brain Disorder

Barbara Landau

By Lisa De Nike

Johns Hopkins has received a five-year $1.7 million grant from the National Institutes of Health to support research into the cognitive problems experienced by people with a rare genetic disorder known as Williams syndrome.

Barbara Landau, a professor of cognitive science, will be principal investigator. The grant, from NIH's National Institute of Neurological Disorders and Stroke, will fund her team's investigation of the origins of spatial impairments suffered by people with Williams. Those people have difficulty with tasks such as assembling simple puzzles, copying basic patterns and navigating their bodies through the physical world.

"We are specifically looking into the hypothesis that certain areas of the brain — the parietal regions, in particular — are the key to those deficits," said Landau, the Dick and Lydia Todd Professor in the Department of Cognitive Science in the Krieger School of Arts and Sciences.

Williams syndrome, caused when a small amount of genetic material is missing from one human chromosome, occurs in approximately one of every 20,000 live births. The parietal regions are the lobes in each cerebral hemisphere of the brain that lie between the rear, called the occipital, and the frontal lobes.

"Our work is primarily experimental," Landau said, "and our experiments will ask whether certain kinds of spatial tasks — those that are thought to involve parietal regions — show significant breakdown in people with Williams, compared with other kinds of spatial tasks that involve different regions of the brain."

The researchers will test individuals with Williams syndrome who are between 8 and 15 years old, as well as adults, to ascertain whether the spatial deficits will resolve in adulthood, though Landau hypothesizes that they will not.

"Another part of our hypothesis is that the unusual spatial profile we see in people with Williams syndrome — what have been called 'peaks and valleys,' or an uneven profile of spatial abilities — is similar to what we would see among young, normally developing children," Landau said.

"In our past work, we have seen great similarities between children and adults with Williams syndrome and normal children of about 4 or 5. The idea is that normal children then go on to develop these spatial abilities more fully, whereas people with Williams syndrome stop at about the functional level of a 4- or 5-year-old," she said. "Perhaps the most important part of our research will ask whether there are ways to promote learning and development past this level in people with Williams syndrome."

The grant will fund a research assistant, graduate and undergraduate students and compensation for the families of individuals who participate in the experiments.

Landau's research also is supported by the March of Dimes Foundation, which continues to fund her work on language in people with Williams syndrome. Individuals with Williams are quite competent verbally, especially when those abilities are contrasted with the severe spatial impairment seen in this syndrome.


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