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Language, communication, and neural systems in Williams syndrome

✍ Scribed by Bellugi, Ursula ;Lai, Zona ;Wang, Paul

Publisher
John Wiley and Sons
Year
1997
Tongue
English
Weight
221 KB
Volume
3
Category
Article
ISSN
1080-4013

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✦ Synopsis

Purpose: Williams syndrome, a rare, genetically based disorder, results in a distinctive facies, a specific heart defect, mental retardation, but remarkably spared language. We discuss results of a research program designed to forge links between cognition, brain, and gene in the context of new directions in cognitive neuroscience.

Findings: Illustration of the relevance of this approach begins with the separation of language from concept formation which challenges important theories of the development of cognition. When compared to Down syndrome (another genetically based syndrome), dissociations in cognition, linguistic, and memory functions are discussed. Next, dissociations within the visual-processing capabilities, specifically spared face processing, and impaired visualspatial processing in Williams syndrome are characterized. Third, developmental trajectories of specific aspects of cognition including receptive language, visual-spatial function, and face processing in both syndromes are contrasted. Finally, the neurobiological bases of Williams syndrome is reviewed, including neurophysiological, neuroanatomical, and molecular genetic characteristics.

Conclusions: The special characteristics of Williams syndrome provide an opportunity to challenge and develop theories of cognition and, on a larger scale, forge links between specific neuropsychological, neuroanatomical, and genetic underpinnings relevant to developmental cognitive neuroscience.

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