Paul Brianna M, Snyder Abraham Z, Haist Frank, Raichle Marcus E, Bellugi Ursula, Stiles Joan
Semel Institute for Neuroscience and Human Behavior, University of California, Los Angeles, CA 90095, USA.
Soc Cogn Affect Neurosci. 2009 Sep;4(3):278-85. doi: 10.1093/scan/nsp023. Epub 2009 Jul 24.
Individuals with Williams syndrome (WS), a genetically determined disorder, show relatively strong face-processing abilities despite poor visuospatial skills and depressed intellectual function. Interestingly, beginning early in childhood they also show an unusually high level of interest in face-to-face social interaction. We employed functional magnetic resonance imaging (fMRI) to investigate physiological responses in face-sensitive brain regions, including ventral occipito-temporal cortex and the amygdala, in this unique genetic disorder. Participants included 17 individuals with WS, 17 age- and gender-matched healthy adults (chronological age-matched controls, CA) and 17 typically developing 8- to 9-year-old children (developmental age controls, DA). While engaged in a face discrimination task, WS participants failed to recruit the amygdala, unlike both CA and DA controls. WS fMRI responses in ventral occipito-temporal cortex, however, were comparable to those of DA controls. Given the integral role of the amygdala in social behavior, the failure of WS participants to recruit this region during face processing may be a neural correlate of the abnormally high sociability that characterizes this disorder.
威廉姆斯综合征（WS）是一种由基因决定的疾病，患有该疾病的个体尽管视觉空间技能较差且智力功能低下，但面部处理能力相对较强。有趣的是，从童年早期开始，他们对面对面社交互动也表现出异常高的兴趣水平。我们采用功能磁共振成像（fMRI）来研究这种独特的遗传疾病中对面部敏感的大脑区域，包括枕颞叶腹侧皮质和杏仁核的生理反应。参与者包括17名患有WS的个体、17名年龄和性别匹配的健康成年人（实足年龄匹配对照组，CA）以及17名发育正常的8至9岁儿童（发育年龄对照组，DA）。在进行面部辨别任务时，与CA和DA对照组不同，患有WS的参与者未能激活杏仁核。然而，枕颞叶腹侧皮质中患有WS的参与者的fMRI反应与DA对照组相当。鉴于杏仁核在社交行为中的重要作用，患有WS的参与者在面部处理过程中未能激活该区域可能是这种疾病所特有的异常高社交性的神经关联。
