COGNITIVE, ADAPTIVE, AND BEHAVIORAL-CHARACTERISTICS OF WILLIAMS-SYNDROME

Williams syndrome is a genetic disorder linked to cognitive and behavi oral patterns of varying consistency; this study was conducted to clar ify further the strengths and weaknesses of children with Williams syn drome, Fifteen subjects with the characteristic features of Williams s yndrome were evaluated using the Stanford-Binet Intelligence Scale for Children, Fourth Edition; the Vineland Adaptive Behavior Scales, Inte rview Edition; and the Child Behavior Checklist. Cognitive skills rang ed from the Moderate Range of Mental Retardation to the Low Average ra nge, with relative strengths in nonverbal and quantitative reasoning, Adaptive skills were delayed, with strengths in communication and soci alization. Behaviorally, clinically significant levels of attention pr oblems, borderline-significant levels of social and thought problems, and significantly low levels of social contacts and structured activit ies were found, In contrast to the findings of many other studies of W illiams syndrome, language skills and short-term memory skills were we ak, Children with Williams syndrome may present a more evenly develope d intellectual profile, with verbal and nonverbal skills being commens urate, In conclusion, a variety of cognitive, adaptive, and behavioral patterns have been shown to be possible in Williams syndrome; therefo re, a single predictable cognitive or behavioral phenotype cannot be a ssumed. (C) 1997 Wiley-Liss, Inc.