%0 Book Section %T Neuropsychological profile of people with Williams Syndrome (WS) %+ Laboratoire de Psychologie : Cognition, Comportement, Communication (LP3C - EA1285) %A Pezzino, Anne-Sophie %A Marec-Breton, Nathalie %A Lacroix, Agnès %B Williams Syndrome : Features, Management and Research. Publisher: Nova Publisher Edition %I Nova Publisher Edition %8 2018 %D 2018 %Z Humanities and Social Sciences %Z Humanities and Social Sciences/PsychologyBook sections %X Williams syndrome (WS) is a genetic neurodevelopmental disorder (prevalence close to 1 in 20,000-30,000 births) resulting from the deletion of 16-25 genes on the long arm of Chromosome 7 (Scherer & Osborne, 2006). Individuals with WS have an intelligence quotient of 40-70 (Howlin, Davies, & Udwin, 1998). Theirs is a unique neuropsychological profile, characterized by an apparent dissociation between cognition and language, as language is relatively well preserved, compared with other cognitive skills (Karmiloff-Smith, et al., 2004; Martens, Wilson, & Reutens, 2008). However, a more complex profile is now emerging, with good lexical, short-term memory (especially auditory-verbal) and face processing skills, but visuospatial (especially local processing of information), executive (planning and inhibition), memory (working memory and long-term) and attentional deficits (Bellugi, Lichtenberger, Jones, Lai, & George, 2000; Schmitt, Eliez, Warsofsky, Bellugi, & Reiss, 2001; Fayasse & Thibaut, 2003; Menghini, Addona, Costanzo, & Vicari, 2010; Costanzo et al., 2013; Dessalegn, Landau & Rapp, 2013). %G English %L hal-02296571 %U https://univ-rennes2.hal.science/hal-02296571 %~ SHS %~ UNIV-BREST %~ UR2-HB %~ UNIV-UBS %~ UNIV-RENNES2 %~ LPC3 %~ UNIV-RENNES %~ LP3C %~ IBSHS