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Direct linkHeather Coleman; Arlene Mannion; Sally Whelan; Megan Tones; Helen Heussler; Matthew Bellgard; Geraldine Leader – Journal of Autism and Developmental Disorders, 2025
Angelman Syndrome (AS) is a rare genetic disorder that impacts 1:20,000 people. Challenging behaviour, such as severe injurious behaviour, aggression and frequent unprovoked episodes of laughter are a significant problem among adults with AS that adversely impacts an individual's quality of life. This study, for the first time, aims understand the…
Descriptors: Genetic Disorders, Behavior Disorders, Adults, Predictor Variables
Bull, L. E.; Oliver, C.; Woodcock, K. A. – Journal of Autism and Developmental Disorders, 2021
Skin picking is highly prevalent in people with Prader-Willi syndrome (PWS). This study addressed the temporal (frequency, duration) and wider characteristics (e.g. type of skin picked, apparent motivations, or management strategies) of skin picking to inform intervention strategies. Nineteen parents/carers who observe skin picking shown by the…
Descriptors: Genetic Disorders, Intellectual Disability, Self Destructive Behavior, Incidence
Srivastava, Siddharth; Clark, Bennett; Landy-Schmitt, Colleen; Offermann, Elizabeth A.; Kline, Antonie D.; Wilkinson, Samuel T.; Grados, Marco A. – Journal of Autism and Developmental Disorders, 2021
Cornelia de Lange syndrome (CdLS) is associated with repetitive and self-injurious behaviors (RBs, SIB). Evaluating children with CdLS, this study: (1) characterizes the spectrum of RBs; (2) characterizes the impact and severity of RBs including SIB; (3) describes how age and adaptive functioning relate to RBs including SIB. Fifty children…
Descriptors: Behavior Problems, Self Destructive Behavior, Genetic Disorders, Intellectual Disability
Kurtz-Nelson, Evangeline C.; Tham, See Wan; Ahlers, Kaitlyn; Cho, Daniel; Wallace, Arianne S.; Eichler, Evan E.; Bernier, Raphael A.; Earl, Rachel K. – Journal of Autism and Developmental Disorders, 2021
Self-injurious behaviors (SIB) are elevated in autism spectrum disorder (ASD) and related genetic disorders, but the genetic and biological mechanisms that contribute to SIB in ASD are poorly understood. This study examined rates and predictors of SIB in 112 individuals with disruptive mutations to ASD-risk genes. Current SIB were reported in 30%…
Descriptors: Self Destructive Behavior, Injuries, Autism, Pervasive Developmental Disorders
Eckert, Eleanor M.; Dominick, Kelli C.; Pedapati, Ernest V.; Wink, Logan K.; Shaffer, Rebecca C.; Andrews, Howard; Choo, Tse-Hwei; Chen, Chen; Kaufmann, Walter E.; Tartaglia, Nicole; Berry-Kravis, Elizabeth M.; Erickson, Craig A. – Journal of Autism and Developmental Disorders, 2019
Using a dataset involving 415 individuals with irritability, aggression, agitation and self-injury (IAAS) behaviors from the fragile X syndrome (FXS) FORWARD database, we describe the psychopharmacologic management of IAAS and features of the population of persons with FXS treated with drug therapy for IAAS. Among those with FXS exhibiting IAAS,…
Descriptors: Genetic Disorders, Self Destructive Behavior, Aggression, Behavior Problems
Crawford, Hayley; Karakatsani, Efthalia; Singla, Gursharan; Oliver, Chris – Journal of Autism and Developmental Disorders, 2019
Self-injurious and aggressive behaviors are common in fragile X syndrome (FXS). However, little is known about the persistence of these behaviors and associated risk markers. We established the prevalence and persistence of self-injurious and aggressive behaviors over eight years in males with FXS, and associations with risk markers. Results…
Descriptors: Self Destructive Behavior, Aggression, Males, Genetic Disorders
Dykens, Elisabeth M. – Journal of Autism and Developmental Disorders, 2014
Although hyperphagia and compulsivity in Prader-Willi syndrome (PWS) are well described, recreation and adaptive skills are relatively unexplored. Parents of 123 participants with PWS (4--48 years) completed measures of their child's adaptive, recreation, and problem behaviors. Offspring received cognitive testing. Watching TV was the most…
Descriptors: Genetic Disorders, Developmental Disabilities, Body Composition, Measures (Individuals)
