Chevalère, J. 1 , Postal, V. 1 , Jauregi, J. 2 , Copet, P. 3 , Laurier, V. 3 & Thuilleaux, D. 3
1 Laboratoire de Psychologie, Santé et Qualité de Vie EA 4139, Université Bordeaux Segalen, France.
2 Euskal Herriko Unibertsitatea- University of the Basque Country Psikologia Fakultatea, Donostia, Spain.
3 Hôpital Marin AP-AH, Unité Prader-Willi, Hendaye, France.
The Prader-Willi syndrome (PWS) is a genetically determined neurodevelopmental disorder characterized by a mild intellectual impairment and a maladaptative behaviour including hyperphagia, temper tantrums, and obsessive/compulsive traits. The aim of this study is to determine if the SPW is associated with a deficit of executive functions and whether the executive deficit is related to mental retardation or represents a clearly separable complaint. Thirteen adults with PWS were compared to age-mached control adults on three executive functions tasks. The results show that PWS individuals have poorer performance than controls in the updating task, in the planning task, and in the four subtests of the cognitive estimation task ; Weight estimation; Quantity estimation; Time estimation, Dimension estimation. After controlling Total IQ, the effect of group persists only on the Quantity estimation. The results confirm that the SPW have an impairment of executive functions which seems related to a mental retardation.