Face recognition ability is often reported to be a relative strength in Williams syndrome (WS). Yet methodological issues associated with the supporting research, and evidence that atypical face processing mechanisms may drive outcomes 'in the typical range', challenge these simplistic characterisations of this important social ability. Detailed investigations of face processing abilities in WS both at a behavioural and neural level provide critical insights. Here, we behaviourally characterised face recognition ability in 18 individuals with WS comparatively to typically developing children and adult control groups. A subset of 11 participants with WS as well as chronologically age matched typical adults further took part in an EEG task where they were asked to attentively view a series of upright and inverted faces and houses. State-of-the-art multivariate pattern analysis (MVPA) was used alongside standard ERP analysis to obtain a detailed characterisation of the neural profile associated with 1) viewing faces as an overall category (by examining neural activity associated with upright faces and houses), and to 2) the canonical upright configuration of a face, critically associated with expertise in typical development and often linked with holistic processing (upright and inverted faces). Our results show that while face recognition ability is not on average at a chronological age-appropriate level in individuals with WS, it nonetheless appears to be a relative strength within their cognitive profile. Furthermore, all participants with WS revealed a differential pattern of neural activity to faces compared to objects, showing a distinct response to faces as a category, as well as a differential neural pattern for upright vs. inverted faces. Nonetheless, an atypical profile of face orientation classification was found in WS, suggesting that this group differs from typical individuals in their face processing mechanisms. Through this innovative application of MVPA, alongside the high temporal resolution of EEG, we provide important new insights into the neural processing of faces in WS.

中文翻译：

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通过多元模式分析和与事件相关的电位来表征威廉姆斯综合症面部处理的神经信号。

人们通常认为面部识别能力是威廉姆斯综合征（WS）的相对优势。然而，与支持性研究相关的方法论问题以及非典型面部处理机制可能在“典型范围内”推动结果的证据，挑战了这一重要社交能力的这些简单化特征。在行为和神经层面对WS中的面部处理能力进行详细研究，可以提供重要的见解。在这里，我们的行为特征是与典型的发育中的儿童和成人对照组相比，WS的18个人具有面部识别能力。11名患有WS以及年龄相匹配的典型成年人的参与者进一步参加了EEG任务，要求他们仔细观察一系列直立和倒置的面孔和房屋。将最新的多元模式分析（MVPA）与标准ERP分析一起使用，以获取与以下特征相关的神经特征的详细特征：1）将面部作为一个整体类别（通过检查与直立的面部和房屋相关的神经活动） ，以及2）脸部的规范直​​立配置，与典型开发中的专业知识紧密相关，并且通常与整体处理（直立和倒置的脸部）相关联。我们的结果表明，虽然WS个体的面部识别能力在按年龄顺序排列的年龄水平上并不是平均水平，但它似乎是他们认知能力的相对强度。此外，所有参与WS的参与者都发现与对象相比，面部神经活动的差异模式，显示出对脸部的明显反应，以及直立和倒立脸部的差异神经模式。但是，在WS中发现了一个非典型的面部朝向分类，这表明该组的面部处理机制与典型个体有所不同。通过MVPA的这一创新应用，以及EEG的高时间分辨率，我们为WS中人脸的神经处理提供了重要的新见解。