Adaptive Functioning in Williams Syndrome: A Systematic Review

Abstract

Literature on the level of adaptive functioning and relative strengths and weaknesses in functioning of individuals with Williams syndrome (WS) was reviewed. The electronic databases PsycINFO, PubMed, Expanded Academic, Web of Science, Scopus and ProQuest were searched electronically for relevant articles and dissertations using the search terms ‘Williams syndrome’ or ‘Williams-Beuren syndrome’ combined with ‘adaptive function*’, ‘adaptive behavio*’, ‘independ*’ and ‘autonomy’. Selection criteria included English language articles, theses and book chapters, participants with a diagnosis of Williams syndrome and inclusion of a standardised assessment of adaptive functioning. Twenty-two published articles and two PhD. dissertations (one subsequently published as a poster presentation) met the selection criteria for inclusion in the review. Fifteen investigated adaptive functioning in children and adolescents aged up to 19 years of age, five investigated adaptive functioning in adults and four included participants across a wider age range and included both children and adults. Along with identifying methodological issues, the review addressed the following areas: overall level of adaptive functioning in WS, domain strengths and weaknesses, evidence of heterogeneity, relationship to intellectual ability, changes with chronological age, relationship with maladaptive behaviour, gender differences and the potential influence of environmental factors which may be related to adaptive functioning in WS.