The neurocognitive profile of Williams syndrome: A complex pattern of strengths and weaknesses

The rare, genetically based disorder, Williams syndrome (WMS), produces a c onstellation of distinctive cognitive, neuroanatomical, and electrophysiolo gical features which we explore through the series of studies reported here . In this paper, we focus primarily on the cognitive characteristics of WMS and begin to forge links among these characteristics, the brain, and the g enetic basis of the disorder. The distinctive cognitive profile of individu als with WMS includes relative strengths in language and facial processing and profound impairment in spatial cognition. The cognitive profile of abil ities, including what is 'typical' for individuals with WMS is discussed, b ut we also highlight areas of variability across the group of individuals w ith WMS that we have studied. Although the overall cognitive abilities (IQs ) of individuals with WMS are typically in the mild-to-moderate range of me ntal retardation, the peaks and valleys within different cognitive domains make this syndrome especially intriguing to study across levels. Understand ing the brain basis (and ultimately the genetic basis) for higher cognitive functioning is the goal we have begun to undertake with this line of inter disciplinary research.