Journal
CURRENT DIRECTIONS IN PSYCHOLOGICAL SCIENCE
Volume 16, Issue 2, Pages 99-104Publisher
SAGE PUBLICATIONS INC
DOI: 10.1111/j.1467-8721.2007.00484.x
Keywords
Williams syndrome; genotype-phenotype correlations; cognition; language; sociability
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Williams syndrome (WS) is a rare genetic disorder characterized by intellectual impairment and a distinctive physical and neuropsychological profile. Relative to their level of intellectual functioning, individuals with WS exhibit strengths in language and face recognition, with deficits in visual-spatial cognition. A heightened appetitive drive toward social interaction is a strong behavioral feature. Relative to other neurodevelopmental disorders, WS has a clearly defined genetic basis, together with a consistent neurocognitive profile of strengths and deficits. Thus, this disorder offers unique opportunities for elucidating gene-brain-behavior relationships. We focus on manifestations of the unusual social profile in WS, by examining data within and across levels of cognition, brain, and molecular genetics.
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