The Williams syndrome cognitive profile

Williams syndrome is a rare neurodevelopmental disorder caused by a hemizyg ous deletion of approximately 1.5 megabases on chromosome 7q11.73. In this article, we outline a Williams Syndrome Cognitive Profile (WSCP) that opera tionalizes the cognitive characteristics of the syndrome using measures of measure of absolute and relative performance on subtests of the Differentia l Abilities Scales (Elliot, 1990a). Testing confirmed excellent sensitivity and specificity scores for the WSCP. Seventy-four of 84 individuals with W illiams 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 var y greatly with chronological age or overall level of cognitive ability for individuals with Williams syndrome. Possible applications for the WSCP incl ude psychoeducational evaluation and empirical research such as the search for genotype/phenotype relations in this genetically based syndrome, (C) 20 00 Academic Press.