Children with Williams Syndrome: Is there a single neuropsychological profile?

Williams Syndrome (WS) is a rare genetic condition characterized by mental retardation, typical facial dysmorphology, and several medical anomalies. A specific neuropsychological profile with both proficient abilities in language and face recognition and severe difficulties in other visuospatial tasks has been hypothesized in children with WS. This cognitive-linguistic profile has emerged through comparisons of WS participants with other populations with a similar degree of mental retardation. In this article, performance by WS participants is compared with that of younger normally developing children on linguistic and visuospatial tasks. Results show that the participants included in our study obtained very different profiles in their neuropsychological performance. Overall our results seem to support the hypothesis that children with WS have a complex neuropsychological profile characterized by atypical developments in both the cognitive and the linguistic domain.