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Williams Syndrome

Williams Syndrome

Research explores 'uneven cognitive profiles' of individuals with disorder

People with Williams syndrome frequently are described as having extraordinary musical and verbal skills, despite a profound inability to conceptualize spatial information. But recent research at the University of Delaware (UD) in Newark has shown that language use in these children may, in fact, be directly affected by their cognitive deficits related to spatial events.

The university also studied eye movement in this population. This research suggests that some of this spatial deficit may result, at least in part, from "their tendency to allocate attention to smaller regions of space than normal children, as well as their difficulties encoding an object's properties and location," stated Barbara Landau, a professor of psychology at the university and director of its Language and Cognition Laboratory.

The research on language use was presented earlier this month during the Conference on Language Development at Boston University, in Boston, MA. The findings of the eye movement study will be presented Friday at the 39th Annual Meeting of the Psychonomic Society in Dallas, Texas.

The UD research was directed by Landau in collaboration with research specialist Andrea L. Zukowski, professor James Hoffman and others. It is shedding light on how Williams syndrome, a genetic disorder that affects an estimated one in every 25,000 individuals, impacts the brain and cognitive development.

Ultimately, Landau said, a better understanding of the disorder "may suggest strategies to help people with Williams syndrome organize their world and learn more effectively by emphasizing their strengths."

People with this disorder may be unable to solve simple mathematical problems or draw a circle that is half red and half green, but some adults with Williams syndrome have been known to memorize thousands of songs in many different languages. Understanding such "uneven cognitive profiles" is at the heart of Landau's research.

Zukowski compared the spatial language skills of eight children, ages 7-14, with Williams syndrome to 12 non-affected children, ages 4-7, and 12 adults. To learn how children with Williams syndrome use motion verbs and other spatial language, the subjects were asked to view 80 short videotaped events. After each event researchers asked, "What happened?"

When describing such events as a doll falling into a bowl, children with Williams syndrome, like other subjects, correctly identified the moving object. However, they were less likely to mention the bowl or other stable reference object in the scenes, especially when the main object moved away from the reference object, Zukowski reported.

Their overall use of verbs was "essentially uncompromised," Landau said, although some verb differences were observed among the subject groups.

For example, the children with Williams syndrome demonstrated the greatest difficulty using prepositions to describe the path of the moving object. Specifically, they rarely used terms such as "off," "through" or "away from," according to Zukowski.

Such language traits may be closely related to the way that children with Williams syndrome process spatial information, Landau said. "Children with Williams syndrome don't seem to have much trouble describing a scene in which one object is moving toward another object--going 'into' or 'onto' the object; but when the children look at scenes in which one object is moving away--'out of' or 'off of' another object--then they often fail to mention the stable object, and they fail to describe the path of the moving object."

The problem is that children with Williams syndrome have difficulty grasping the spatial relationships between objects, she said. "If something is on top of something and the two objects are close together, that's easy; but if they're far apart, the child may be less likely to be able to conceptualize it, less likely to store it in memory, and possibly even less likely to mention it."

Children with Williams syndrome don't seem to have widespread, major spatial vocabulary problems, Landau reported. Cases that do show clear difficulties, however, "may be a consequence of their impaired, nonlinguistic knowledge."

Hoffman, Landau and graduate student Barney Pagani investigated the eye movement of individuals with Williams syndrome, a project funded by the March of Dimes and the National Science Founda-tion.

The UD researchers used a special, eye-tracking device with a tiny camera hidden in the visor of a cap placed on the head of the subjects. They studied the eye movements of children as they attempted to solve a set of block construction problems.

The subjects with Williams syndrome "made many more errors than the controls, particularly on complex problems composed of multi-part blocks," Landau reported. Eye recordings "suggest that at least part of their spatial deficit is due to their allocation of attention to single blocks rather than to configurations."

She said the goal of the research at the university is to pinpoint which aspects of language and learning are "uncompromised" among people with Williams syndrome.

Such knowledge might make it possible to improve educational opportunities for people who have the disorder. *



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