Department of Psychology, Stanford University, Stanford, California 94305, USA.
J Neurosci. 2010 May 12;30(19):6700-12. doi: 10.1523/JNEUROSCI.4268-09.2010.
Williams syndrome (WS) is a genetic condition characterized by atypical brain structure, cognitive deficits, and a life-long fascination with faces. Face recognition is relatively spared in WS, despite abnormalities in aspects of face processing and structural alterations in the fusiform gyrus, part of the ventral visual stream. Thus, face recognition in WS may be subserved by abnormal neural substrates in the ventral stream. To test this hypothesis, we used functional magnetic resonance imaging and examined the fusiform face area (FFA), which is implicated in face recognition in typically developed (TD) individuals, but its role in WS is not well understood. We found that the FFA was approximately two times larger among WS than TD participants (both absolutely and relative to the fusiform gyrus), despite apparently normal levels of face recognition performance on a Benton face recognition test. Thus, a larger FFA may play a role in face recognition proficiency among WS.
威廉姆斯综合征（WS）是一种以非典型大脑结构、认知缺陷和对人脸的终身着迷为特征的遗传疾病。尽管在面部处理的各个方面存在异常以及梭状回（腹侧视觉流的一部分）结构改变，但 WS 中的人脸识别相对不受影响。因此，WS 中的人脸识别可能由腹侧流中的异常神经基质提供。为了验证这一假设，我们使用功能磁共振成像检查了梭状回面孔区（FFA），该区域在正常发育的个体（TD）中参与人脸识别，但在 WS 中的作用尚不清楚。我们发现，WS 参与者的 FFA 比 TD 参与者大约大两倍（无论是绝对值还是相对于梭状回），尽管他们在本顿面部识别测试中的面部识别表现似乎正常。因此，较大的 FFA 可能在 WS 中的人脸识别熟练程度中发挥作用。
