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Nag, Heidi Elisabeth; Naerland, Terje – Journal of Intellectual Disabilities, 2021
Smith-Magenis syndrome (SMS) is a genetic syndrome most often caused by a deletion on chromosome 17 or more rarely by a mutation in the retinoic acid-induced 1 gene. The aim of this study was to investigate the Developmental Behavior Checklist (DBC) profile of persons with SMS and the associations between behavioural and emotional problems, age,…
Descriptors: Genetic Disorders, Behavior Problems, Emotional Problems, Age Differences
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
Wulffaert, Josette; Scholte, Evert M.; Van Berckelaer-Onnes, Ina A. – Journal of Intellectual & Developmental Disability, 2010
Background: Parenting stress was investigated in mothers with a child with Angelman syndrome (AS) or Prader-Willi syndrome (PWS), which are genetically related. Method: Mothers of 24 children with AS and 23 children with PWS (2-12 years) completed the Nijmegen Parenting Stress Index-Short, Developmental Behaviour Checklist, and Vineland Screener…
Descriptors: Mothers, Mental Retardation, Family Programs, Child Rearing
Wulffaert, Josette; Van Berckelaer-Onnes, Ina A.; Scholte, Evert M. – Autism: The International Journal of Research and Practice, 2009
According to the major classification systems it is not possible to diagnose a comorbid autistic disorder in persons with Rett syndrome. However, this is a controversial issue, and given the level of functioning of persons with Rett syndrome, the autistic disorder is expected to be present in a comparable proportion as in people with the same…
Descriptors: Check Lists, Severe Mental Retardation, Autism, Communication Disorders