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Am J Med Genet C Semin Med Genet. 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.

Author information

1
Department of Psychological and Brain Sciences, 317 Life Sciences Building, University of Louisville, Louisville, KY 40292, USA. cbmervis@louisville.edu

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.

PMID:
20425784
PMCID:
PMC2922953
DOI:
10.1002/ajmg.c.30263
[Indexed for MEDLINE]
Free PMC Article

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