The Williams syndrome behavioral phenotype: the ‘whole person’ is missing


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Abstract

Purpose of reviewOf the more than 1000 known genetic causes of mental retardation, Williams syndrome was selected for review because it aptly demonstrates both amazing advances and glaring omissions in the field of behavioral phenotypes.Recent findingsResearch on the Williams syndrome behavioral phenotype remains rather lopsided, with an abundance of publications on cognitive and linguistic profiles. Recent progress in these domains, especially in visual-spatial functioning, is summarized. In contrast, data are scant on psychosocial factors that predict successful life outcomes, including social competence and adaptation, psychopathology, families, and educational and vocational interventions. By examining one of these non-cognitive domains - psychopathology - the review highlights findings that are just as startling as the cognitive peaks and valleys that have long dominated the Williams syndrome phenotype landscape.SummaryWilliams syndrome demonstrates the need for a more integrated, ‘whole-person’ program of research. Specific recommendations are made that address this need.

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