The Williams syndrome cognitive profile. Brain Cogn 2000 Dec;44(3):604-28
Date
12/06/2000Pubmed ID
11104544DOI
10.1006/brcg.2000.1232Scopus ID
2-s2.0-0034537430 (requires institutional sign-in at Scopus site) 326 CitationsAbstract
Williams syndrome is a rare neurodevelopmental disorder caused by a hemizygous deletion of approximately 1.5 megabases on chromosome 7q11.23. In this article, we outline a Williams Syndrome Cognitive Profile (WSCP) that operationalizes the cognitive characteristics of the syndrome using measures of absolute and relative performance on subtests of the Differential Abilities Scales (Elliot, 1990a). Testing confirmed excellent sensitivity and specificity scores for the WSCP. Seventy-four of 84 individuals with Williams syndrome fit the WSCP while only 4 participants in a contrast group met all of the WSCP criteria. It was also found that the WSCP does not vary greatly with chronological age or overall level of cognitive ability for individuals with Williams syndrome. Possible applications for the WSCP include psychoeducational evaluation and empirical research such as the search for genotype/phenotype relations in this genetically based syndrome.
Author List
Mervis CB, Robinson BF, Bertrand J, Morris CA, Klein-Tasman BP, Armstrong SCAuthor
Bonita Klein-Tasman BA,MA,PhD Professor in the Psychology department at University of Wisconsin - MilwaukeeMESH terms used to index this publication - Major topics in bold
AdolescentAdult
Brain
Child
Child, Preschool
Chromosome Deletion
Chromosomes, Human, Pair 7
Cognition Disorders
Female
Humans
Male
Middle Aged
Neuropsychological Tests
Sensitivity and Specificity
Severity of Illness Index
Space Perception
Visual Perception
Williams Syndrome
