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    J Intellect Disabil Res. 2010 Nov;54(11):1024-9. doi: 10.1111/j.1365-2788.2010.01331.x. Epub 2010 Sep 20.

    Adaptive behaviour in Angelman syndrome: its profile and relationship to age.

    Source

    Universitat Autonoma de Barcelona, Facultat de Psicologia, Bellaterra, Spain. carme.brun@uab.cat

    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.

    PMID:
    20854288
    [PubMed - indexed for MEDLINE]

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