Hypersociability in Williams syndrome
Hypersociability in Williams syndrome
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DOI：
10.1162/089892900561968
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发表时间：
2000-01-01
影响因子：
3.2
通讯作者：
Adolphs, R
中科院分区：
文献类型：
--
作者：
Jones, W;Bellugi, U;Adolphs, R
对异常人群的研究为研究认知、基因型和脑神经生物学之间的关系提供了难得的机会，允许在这些不同水平的分析中进行比较。在我们的研究中，我们调查了患有罕见的遗传性疾病威廉姆斯综合征（WMS）的个体，以建立这些水平之间的联系。这种跨领域工作的一个关键组成部分是清楚地描绘所讨论的疾病的表型。本文中特别感兴趣的是WMS中相对未探索的不寻常的社会表型，包括过度友好和迷人的个性。四项研究测量不同方面的超社会行为在WMS，每个探讨特定方面的WMS婴儿，幼儿，学龄儿童和成人。过度社交行为的异常特征是WMS与其他发育障碍区别的表型的重要组成部分。此外，这些研究表明，在各种年龄段都可以观察到这种情况，并且在多种实验范式中一致出现。这些对WMS中超社会行为的研究有望为基因-大脑-行为关系的跨学科分析提供基础。
Studies of abnormal populations provide a rare opportunity for examining relationships between cognition, genotype and brain neurobiology, permitting comparisons across these different levels of analysis. In our studies, we investigate individuals with a rare, genetically based disorder called Williams syndrome (WMS) to draw links among these levels. A critical component of such a cross-domain undertaking is the clear delineation of the phenotype of the disorder in question. Of special interest in this paper is a relatively unexplored unusual social phenotype in WMS that includes an overfriendly and engaging personality. Four studies measuring distinct aspects of hypersocial behavior in WMS are presented, each probing specific aspects in WMS infants, toddlers, school age children, and adults. The abnormal profile of excessively social behavior represents an important component of the phenotype that may distinguish WMS from other developmental disorders. Furthermore, the studies show that the profile is observed across a wide range of ages, and emerges consistently across multiple experimental paradigms. These studies of hypersocial behavior in WMS promise to provide the ground-work for crossdisciplinary analyses of gene-brain-behavior relationships.