โ Scribed by Greer, Margaret K.; Brown, Frank R.; Pai, G. Shashidhar; Choudry, Shazia H.; Klein, Alan J.
No coin nor oath required. For personal study only.
Williams syndrome is a genetic disorder linked to cognitive and behavioral patterns of varying consistency; this study was conducted to clarify further the strengths and weaknesses of children with Williams syndrome. Fifteen subjects with the characteristic features of Williams syndrome were evaluated using the Stanford-Binet Intelligence Scale for Children, Fourth Edition; the Vineland Adaptive Behavior Scales, Interview Edition; and the Child Behavior Checklist. Cognitive skills ranged from the Moderate Range of Mental Retardation to the Low Average range, with relative strengths in nonverbal and quantitative reasoning. Adaptive skills were delayed, with strengths in communication and socialization. Behaviorally, clinically significant levels of attention problems, borderline-significant levels of social and thought problems, and significantly low levels of social contacts and structured activities were found.
In contrast to the findings of many other studies of Williams syndrome, language skills and short-term memory skills were weak. Children with Williams syndrome may present a more evenly developed intellectual profile, with verbal and nonverbal skills being commensurate. In conclusion, a variety of cognitive, adaptive, and behavioral patterns have been shown to be possible in Williams syndrome; therefore, a single predictable cognitive or behavioral phenotype cannot be assumed. Am.
๐ SIMILAR VOLUMES
## To determine if persons with the Prader-Willi syndrome (PWS) have increased psyc h o p a t h o l o g y w h e n c o m p a r e d w i t h matched controls, and whether there is a specific behavior phenotype in PWS, the behavior of 46 persons with PWS was compared with that of control individuals d
In prospective studies of young, fragile X [fra(X)] males with the full mutation, cognitive abilities (IQ scores) and adaptive behavior levels (DQ scores) declined in most subjects tested. Little is known about longitudinal changes in IQ and DQ scores in young fra(X) females, although one earlier re
Clin Res 38:536Al reported stability of IQs in a group of 14 children with Williams-Beuren syndrome (WBS) over a 5-year period and concluded that they display a development rate similar to normal children. The aim of our study was to examine the stability of the development of nonverbal reasoning ab
Williams syndrome (WS) is associated with a deletion of the elastin gene in over 90% of cases. We report maternal serum alpha fetoprotein (MSAFP) levels in 5 women whose fetuses were later diagnosed as having WS. MSAFP levels ranged from 0.5-0.8 multiples of the median (MOM). Although further confir