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

被引：15

作者：

Brun Gasca, C. ^{[1
]}

Obiols, J. E. ^{[1
]}

Bonillo, A. ^{[1
]}

Artigas, J. ^{[2
]}

Lorente, I. ^{[2
]}

Gabau, E. ^{[2
]}

Guitart, M. ^{[2
]}

Turk, J. ^{[3
,4
]}

机构：

[1] Univ Autonoma Barcelona, Fac Psicol, Bellaterra, Spain

[2] Hosp Tauli Sabadell, Sabadell, Spain

[3] S London & Maudsley Fdn NHS Trust, Southwark Child & Adolescent Mental Hlth Dev Neur, London, England

[4] St Georges Univ London, London, England

来源：

JOURNAL OF INTELLECTUAL DISABILITY RESEARCH | 2010年 / 54卷

关键词：

adaptive behaviour; Angelman syndrome; behavioural phenotype; ICAP; intellectual disability; MENTAL-RETARDATION; WILLIAMS-SYNDROME; CHILDREN; PHENOTYPE; ADULTS;

D O I：

10.1111/j.1365-2788.2010.01331.x

中图分类号：

G76 [特殊教育];

学科分类号：

040109 ;

摘要：

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.

引用

页码：1024 / 1029

页数：6

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