HAL will be down for maintenance from Friday, June 10 at 4pm through Monday, June 13 at 9am. More information
Skip to Main content Skip to Navigation
Book sections

Neuropsychological profile of people with Williams Syndrome (WS)

Abstract : 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).
Complete list of metadata

https://hal.univ-rennes2.fr/hal-02296571
Contributor : Laurence Leroux Connect in order to contact the contributor
Submitted on : Wednesday, September 25, 2019 - 12:10:26 PM
Last modification on : Monday, December 13, 2021 - 12:10:02 PM

Identifiers

  • HAL Id : hal-02296571, version 1

Citation

Anne-Sophie Pezzino, Nathalie Marec-Breton, Agnès Lacroix. Neuropsychological profile of people with Williams Syndrome (WS). Williams Syndrome : Features, Management and Research. Publisher: Nova Publisher Edition, Nova Publisher Edition, 2018. ⟨hal-02296571⟩

Share

Metrics

Record views

178