Skip to main page content
U.S. flag

An official website of the United States government

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2010 May 15;154C(2):229-48.
doi: 10.1002/ajmg.c.30263.

Cognitive and behavioral characteristics of children with Williams syndrome: implications for intervention approaches

Affiliations
Review

Cognitive and behavioral characteristics of children with Williams syndrome: implications for intervention approaches

Carolyn B Mervis et al. Am J Med Genet C Semin Med Genet. .

Abstract

Portrayals of individuals with Williams syndrome (WS), a genetic disorder caused by a microdeletion of approximately 25 genes on chromosome 7q11.23, have reached the general public through a variety of media formats. These descriptions are often paradoxical in nature with individuals with WS repeatedly described as demonstrating near-normal language despite the presence of significant intellectual disability and as being extremely sociable and friendly in spite of their seemingly limited understanding of basic social norms. While this depiction of WS served to attract the interest of basic-science researchers, the results of subsequent studies have provided a more nuanced view. For example, rather than across-the-board "near-normal" language, children with WS demonstrate relative strengths in concrete vocabulary and verbal short-term memory, grammatical abilities at the level expected for general intellectual ability, and considerable weakness in relational/conceptual language and pragmatics (social use of language). To provide a more thorough characterization of the WS behavioral phenotype, we summarize recent findings related to intellectual ability, language development, memory development, executive function development, adaptive behavior skills, and behavior as it relates to learning by children with WS. Finally, we briefly discuss intervention approaches that may help children with WS to achieve their full potential.

PubMed Disclaimer

Similar articles

Cited by

References

    1. Abbeduto L, Murphy MM, Kover ST, Giles ND, Karadottir S, Amman A, et al. Signaling noncomprehension of language: A comparison of fragile X syndrome and Down syndrome. Am J Ment Retard. 2008;113:214–230. - PMC - PubMed
    1. Achenbach TM. Manual for the Revised Child Behavior Checklist. Burlington, BT: University of Vermont, Department of Psychiatry; 1991.
    1. American Association on Mental Retardation. Definitions, classifications, and systems of supports. 9. Washington DC: AAMR; 1992.
    1. Atkinson J, Braddick O, Anker S, Curran W, Andrew R, Wattam-Bell J, et al. Neurobiological models of visuospatial cognition in children with Williams syndrome: Measures for dorsal-stream and frontal function. Dev Neuropsychol. 2003;23:139–172. - PubMed
    1. Ayres AJ, Robbins J. Sensory integration and the child: Understanding hidden sensory challenges. Los Angeles, CA: Western Psychological Services; 2005.

Publication types