SOCIAL-EMOTIONAL AND BEHAVIORAL-ADJUSTMENT IN CHILDREN WITH WILLIAMS-BEUREN SYNDROME

In children with Williams-Beuren syndrome (WBS), disturbed behaviors ( neurotic, antisocial, and hyperactive) [Arnold et al., 1985: Dev Med C hild Neurol 27:49-69; Udwin et al., 1987: J Child Psychol Psychiat 28: 297-309] have been described. To study the behavior disturbances and s ocial-emotional adjustment in children with WBS, a group of N = 19 pat ients was compared with a control group, matched for age, gender, and nonverbal reasoning abilities. Parents were asked to assess the childr en's behavior in terms of a list of 20 items of the Child Behavior Che cklist (CBCL) [Achenbach and Edelbrock, 1983: Manual for the Child Beh avior Checklist] and the Vineland Social Maturity Scale (VSMS) [Luer e t al., 1972: Kurzform der Vineland Social Maturity Scale]. As compared with the control group, children with WBS differ significantly in the ir social behavior towards strangers. They exhibit no reserve or dista ncing behavior and would, for instance, follow a stranger without hesi tation. They are described as showing a hypersensitivity to sounds tha t is more pronounced than in the control group. Finally, they are foun d to be significantly less well-adjusted socially than the control ind ividuals. (C) 1994 Wiley Liss, Inc.