Towards the neural basis for hypersociability in a genetic syndrome

Towards the neural basis for hypersociability in a genetic syndrome
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DOI:
10.1097/00001756-199906030-00006
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发表时间:
1999-06-03
期刊:
影响因子:
1.7
通讯作者:
Chiles, M
Chiles, M
中科院分区:
医学4区
文献类型:
--
作者:
Bellugi, U;Adolphs, R;Chiles, M

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威廉姆斯综合征(WMS)是一种罕见的疾病,具有独特的医学,心理,神经生理学和神经解剖学特征,由约20个基因的半合子缺失引起。该表型在高级认知功能中表现出特定的解离:一般认知缺陷,但保留了语言能力;极端的空间认知缺陷,但完整的面部处理。特别有趣的是,WMS中有一种不寻常的社会表型:过于友好、迷人的个性以及与陌生人的过度社交。在WMS的社会行为的第一个实验研究中,我们报告说,WMS的主题显示出异常的积极偏见,在他们的社会判断不熟悉的人,符合他们的行为在真实的生活。我们的发现有助于理解人类社会行为的神经和遗传基础。NeuroReport 10:1653-1657(C)1999 Lippincott威廉姆斯和威尔金斯。
WILLIAMS syndrome (WMS), a rare disorder with a distinctive profile of medical, psychological, neurophysiological and neuroanatomical characteristics, results from hemizygous deletion of about 20 genes. The phenotype exhibits specific dissociations in higher cognitive functions: general cognitive deficits but spared linguistic abilities; extreme spatial cognitive deficits, but intact face processing. Of special, interest is an unusual social phenotype in WMS: an overly friendly, engaging personality and excessive sociability with strangers. In this first experimental study of social behavior in WMS, we report that WMS subjects show an abnormal positive bias in their social judgments of unfamiliar individuals, consistent with their behavior in real life. Our findings contribute to an understanding of the neural and genetic bases of human social behavior. NeuroReport 10:1653-1657 (C) 1999 Lippincott Williams & Wilkins.