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天使综合征、科里奥利-德朗热综合征和猫哭综合征的社会行为和自闭症谱系障碍特征。

Social behavior and characteristics of autism spectrum disorder in Angelman, Cornelia de Lange, and Cri du Chat syndromes.

机构信息

Cerebra Center for Neurodevelopmental Disorders, Psychology, University of Birmingham, Birmingham, Edgbaston B15 2TT, United Kingdom.

出版信息

Am J Intellect Dev Disabil. 2013 Jul;118(4):262-83. doi: 10.1352/1944-7558-118.4.262.

Abstract

We evaluated autism spectrum disorder (ASD) characteristics and social behavior in Angelman (AS; n  =  19; mean age  = 10.35 years), Cornelia de Lange (CdLS; n  =  15; mean age  = 12.40 years), and Cri du Chat (CdCS, also known as 5 p-syndrome; n  =  19; mean age  =  8.80 years) syndromes. The proportion of individuals meeting the ASD cutoff on the Social Communication Questionnaire was significantly higher in the AS and CdLS groups than in the CdCS group (p < .01). The groups demonstrated divergent social behavior profiles during social conditions in which adult availability, adult familiarity, and social demand were manipulated. Social enjoyment was significantly heightened in AS, whereas social approaches were heightened in individuals with CdCS. Social motivation, social communication, and enjoyment were significantly lower in CdLS. The findings highlight the importance of detailed observation when evaluating ASD and social behavior in genetic syndromes.

摘要

我们评估了 Angelman(AS;n = 19;平均年龄 = 10.35 岁)、Cornelia de Lange(CdLS;n = 15;平均年龄 = 12.40 岁)和 Cri du Chat(CdCS,也称为 5p 综合征;n = 19;平均年龄 = 8.80 岁)综合征的自闭症谱系障碍(ASD)特征和社会行为。在社交沟通问卷上达到 ASD 临界值的个体比例在 AS 和 CdLS 组中明显高于 CdCS 组(p <.01)。在社交条件下,当操纵成人可用性、成人熟悉度和社会需求时,各组表现出不同的社会行为特征。在 AS 中,社交享受显著提高,而在 CdCS 个体中,社交接近度提高。CdLS 中的社交动机、社交沟通和享受显著降低。这些发现强调了在评估遗传综合征中的 ASD 和社会行为时,详细观察的重要性。

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