Relations Between Lab-Based and Parent-Reported Executive Functioning in Children and Adolescents with Williams Syndrome

Abstract

Williams syndrome (WS) is a rare genetic disorder characterized by lowered cognitive abilities and significant attention and executive functioning (EF) difficulties. The current study constitutes the first investigating the relevance of performance on an EF task measuring one or more of the “core” EF’s (inhibition, shifting, working memory) to EF behaviors observed by parents of youth with WS. Parent-ratings of their children indicated more EF difficulties in all domains compared to the general population. Performance on the EF task (correct trials during the last phase of the Dimensional Change Card Sort) predicted parent reported general EF difficulties, metacognition, working memory and inhibition difficulties but not shifting difficulties after controlling for age, gender and nonverbal ability. Performance on this EF card sorting task appears to have some relevance to everyday executive functioning difficulties of youth with WS.