Abstract
BackgroundWilliams syndrome (WS) is a neurodevelopmental disease characterized by compelling psychological phenotypes. The symptoms span multiple cognitive domains and include a distinctive pattern of social behavior. The goal of this study was to explore adaptive behavior in WS patients in China.MethodsWe conducted a structured interview including the Infants-Junior Middle School Students Social-life Abilities Scale in three participant groups: children with WS (n = 26), normally-developing children matched for mental age (MA, n = 30), and normally-developing children matched for chronological age (CA, n = 40). We compared the mean scores for each domain between the three groups.ResultsChildren with WS had more siblings than children in the two control groups. The educational level of the caregivers of WS children was lower than that of the control children. We found no differences in locomotion, work skill, socialization, or self-management between the WS and MA groups. WS children obtained higher scores of self-dependence (df = 54, Z = −2.379, p = 0.017) and had better communication skills (df = 54, Z = −2.222, p = 0.026) compared with MA children. The CA children achieved higher scores than the WS children for all dimensions of adaptive behavior.ConclusionsWS children have better adaptive behavior skills regarding communication and self-dependence than normal children matched for mental age. Targeted intervention techniques should be designed to promote social development in this population.
Highlights
Williams syndrome (WS) is a neurodevelopmental disease characterized by compelling psychological phenotypes
Using some form of the Vineland Adaptive Behavior Scales (VABS) [3], children with WS have been found to have strong socialization and communication skills, and poor daily living and motor skills, relative to their overall level of adaptive functioning
Participants A total of 96 individuals participated in the study, including 26 children with WS, 30 children matched for mental age (MA group) and 40 children matched for chronological age (CA group)
Summary
Williams syndrome (WS) is a neurodevelopmental disease characterized by compelling psychological phenotypes. The symptoms span multiple cognitive domains and include a distinctive pattern of social behavior. Individuals with WS exhibit compelling psychological phenotypes, including cognitive strengths and weaknesses, and display unique patterns of social behavior [1] An increased appetitive drive toward social interaction is one of the most significant social phenotypes of WS [2]. Evaluation of adaptive behavior includes several aspects, such as communication, socialization, daily living, and motor skills. Using some form of the Vineland Adaptive Behavior Scales (VABS) [3], children with WS have been found to have strong socialization (especially interpersonal skills related to initiating social interaction) and communication skills, and poor daily living and motor skills, relative to their overall level of adaptive functioning. Some researchers have reported that the adaptive behaviors of people with WS are poor compared with those of the normal population [4,5,6]
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