Department of Psychiatry, University of Cambridge, Cambridge, UK.
J Intellect Disabil Res. 2011 Jan;55(1):75-84. doi: 10.1111/j.1365-2788.2010.01348.x. Epub 2010 Dec 1.
People with Prader-Willi syndrome (PWS) may have mild intellectual impairments but less is known about their social cognition. Most parents/carers report that people with PWS do not have normal peer relationships, although some have older or younger friends. Two specific aspects of social cognition are being able to recognise other people's emotion and to then respond appropriately. In a previous study, mothers/carers thought that 26% of children and 23% of adults with PWS would not respond to others' feelings. They also thought that 64% could recognise happiness, sadness, anger and fear and a further 30% could recognise happiness and sadness. However, reports of emotion recognition and response to emotion were partially dissociated. It was therefore decided to test facial emotion recognition directly.
The participants were 58 people of all ages with PWS. They were shown a total of 20 faces, each depicting one of the six basic emotions and asked to say what they thought that person was feeling. The faces were shown one at a time in random order and each was accompanied by a reminder of the six basic emotions.
This cohort of people with PWS correctly identified 55% of the different facial emotions. These included 90% of happy faces, 55% each of sad and surprised faces, 43% of disgusted faces, 40% of angry faces and 37% of fearful faces. Genetic subtype differences were found only in the predictors of recognition scores, not in the scores themselves. Selective impairment was found in fear recognition for those with PWS who had had a depressive illness and in anger recognition for those with PWS who had had a psychotic illness.
The inability to read facial expressions of emotion is a deficit in social cognition apparent in people with PWS. This may be a contributing factor in their difficulties with peer relationships.
普拉德-威利综合征（PWS）患者可能存在轻度智力障碍，但他们的社会认知能力却知之甚少。大多数家长/照顾者报告称，PWS 患者没有正常的同伴关系，但也有一些患者有年龄较大或较小的朋友。社会认知的两个特定方面是能够识别他人的情绪，然后做出适当的反应。在之前的一项研究中，母亲/照顾者认为 26%的儿童和 23%的成年 PWS 患者不会对他人的感受做出反应。他们还认为，64%的患者能够识别快乐、悲伤、愤怒和恐惧，还有 30%的患者能够识别快乐和悲伤。然而，对情绪识别和对情绪的反应的报告部分是分离的。因此，决定直接测试面部情绪识别。
参与者为所有年龄段的 58 名 PWS 患者。他们总共观看了 20 张面孔，每张面孔都描绘了六种基本情绪之一，并被要求说出他们认为这个人的感受。这些面孔以随机顺序逐一显示，每张面孔都附有对六种基本情绪的提醒。
这组 PWS 患者正确识别了 55%的不同面部情绪。这包括 90%的快乐面孔、55%的悲伤和惊讶面孔、43%的厌恶面孔、40%的愤怒面孔和 37%的恐惧面孔。仅在识别分数的预测因素中发现了遗传亚型差异，而不是分数本身。仅在患有抑郁性疾病的 PWS 患者中发现了对恐惧的识别选择性损伤，而在患有精神病性疾病的 PWS 患者中发现了对愤怒的识别选择性损伤。
无法读取面部表情是 PWS 患者社会认知缺陷的一个表现。这可能是他们在同伴关系中遇到困难的一个因素。
