Acceso usuarios
Michèle Fayasse, Jean-Pierre Thibaut
Williams syndrome (WS) is a genetically based developmental disorder resulting in a heterogeneous neuropsychological profile. Linguistic abilities are relatively spared whereas there are important deficits in the visuo-spatial domain. After a short description of WS persons' linguistic abilities, we focus on their visuo-constructive impairments. We describe the results obtained with the main experimental paradigms used in the field (drawing and block design tasks). Genetic, neurological and psychological interpretations of visuo- constructive problems are discussed. In the psychological domain, the role of factors such as perception, spatial analysis, short-term memory, mental imagery and executive functions are contrasted with the classical hypothesis of a general deficit in global processing and a new hypothesis is proposed : WS individuals have specific difficulties in disengaging from salient local parts when they have to reconstruct visual stimuli.
