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Behavioral Phenotype in Adults with Prader-Willi Syndrome
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Sinnema, Margje; Einfeld, Stewart L.; Schrander-Stumpel, Constance T. R. M.; Maaskant, Marian A.; Boer, Harm; Curfs, Leopold M. G. – Research in Developmental Disabilities: A Multidisciplinary Journal, 2011
Prader-Willi syndrome (PWS) is characterized by temper tantrums, impulsivity, mood fluctuations, difficulty with change in routine, skinpicking, stubbornness and aggression. Many studies on behavior in PWS are limited by sample size, age range, a lack of genetically confirmed diagnosis of PWS and inconsistent assessment of behavior. The aim of…
Descriptors: Check Lists, Behavior Problems, Disability Identification, Genetics
Behavioral Features of Williams Beuren Syndrome Compared to Fragile X Syndrome and Subjects with Intellectual Disability without Defined Etiology
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Perez-Garcia, D.; Granero, R.; Gallastegui, F.; Perez-Jurado, L. A.; Brun-Gasca, C. – Research in Developmental Disabilities: A Multidisciplinary Journal, 2011
Williams-Beuren syndrome (WBS) is a genetically determined neurodevelopmental disorder caused by a heterozygous deletion of 26-28 genes on chromosome band 7q11.23. During the past few years, researchers and clinicians have significantly contributed to define the phenotype of the syndrome, including its cognitive and behavioral aspects. However, it…
Descriptors: Check Lists, Delinquency, Aggression, Mental Retardation