ERIC Number: EJ901394
Record Type: Journal
Publication Date: 2010-Nov
Abstractor: As Provided
Reference Count: 24
Adaptive Behaviour in Angelman Syndrome: Its Profile and Relationship to Age
Gasca, C. Brun; Obiols, J. E.; Bonillo, A.; Artigas, J.; Lorente, I.; Gabau, E.; Guitart, M.; Turk, J.
Journal of Intellectual Disability Research, v54 n11 p1024-1029 Nov 2010
Background: Angelman syndrome (AS) is a neurodevelopmental disorder usually caused by an anomaly in the maternally inherited chromosome 15. The main features are severe intellectual disability, speech impairment, ataxia, epilepsy, sleep disorder and a behavioural phenotype that reportedly includes happy disposition, attraction to/fascination with water and hypermotoric behaviour. Method: We studied the level of adaptive behaviour and the adaptive behavioural profile in the areas of "motor skills", "language and communication", "personal life skills" and "community life skills" in a group of 25 individuals with genetically confirmed AS, to determine whether there is a specific adaptive behaviour profile. Results and conclusions: None of the individuals, whatever their chronological age, had reached a developmental age of 3 years. A specific adaptive behaviour profile was found, with "personal life skills" emerging as relative strengths and "social and communication skills" as weaknesses.
Descriptors: Severe Mental Retardation, Profiles, Communication Skills, Adjustment (to Environment), Neurological Impairments, Genetic Disorders, Speech Impairments, Epilepsy, Sleep, Social Behavior, Psychomotor Skills, Language Skills, Daily Living Skills
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Publication Type: Journal Articles; Reports - Research
Education Level: N/A
Authoring Institution: N/A