Adaptive behaviour in Angelman syndrome: Its profile and relationship to age

C. Brun Gasca, J. E. Obiols, A. Bonillo, J. Artigas, I. Lorente, E. Gabau, M. Guitart, J. Turk

Research output: Contribution to journalArticleResearchpeer-review

13 Citations (Scopus)

Abstract

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. © 2010 The Authors. Journal of Intellectual Disability Research © 2010 Blackwell Publishing Ltd.
Original languageEnglish
Pages (from-to)1024-1029
JournalJournal of Intellectual Disability Research
Volume54
DOIs
Publication statusPublished - 1 Nov 2010

Keywords

  • Adaptive behaviour
  • Angelman syndrome
  • Behavioural phenotype
  • ICAP
  • Intellectual disability

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