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 lang uage and face recognition and severe difficulties in other visuospatial tas ks has been hypothesized in children with WS. This cognitive-linguistic pro file has emerged through comparisons of WS participants with other populati ons with a similar degree of mental retardation. In this article, performan ce by WS participants is compared with that of younger normally developing children on linguistic and visuospatial tasks. Results show that the partic ipants included in our study obtained very different profiles in their neur opsychological performance. Overall our results seem to support the hypothe sis that children with WS have a complex neuropsychological profile charact erized by atypical developments in both the cognitive and the linguistic do main.