Porter Melanie A, Coltheart Max, Langdon Robyn
Macquarie Centre for Cognitive Science, Macquarie University, NSW 2109, Australia.
Neuropsychologia. 2007 Sep 20;45(12):2839-49. doi: 10.1016/j.neuropsychologia.2007.05.006. Epub 2007 May 21.
People with Williams syndrome (WS) display indiscriminate approach toward strangers in everyday life. People with Down syndrome (DS) can also do so, but to a lesser degree. Inappropriate approach behavior is also characteristic of people with acquired amygdala damage and people with acquired frontal lobe impairment; given this, the developmental disorder of social approach seen in WS and perhaps also DS might be due to poor emotion recognition (due to abnormal amygdala functioning) or poor control of behavior (due to frontal lobe abnormality). A third account of this developmental disorder of social cognition can be couched in terms of heightened salience for social stimuli. We explored these three hypotheses by testing emotion recognition, social approach and frontal lobe functioning in people with WS and DS. Overall, our results were inconsistent with predictions from the amygdala and social salience hypotheses. In contrast, results from a battery of neuropsychological tasks suggested that abnormal social approach in WS and DS in everyday life is best explained by frontal lobe impairment, in particular, poor response inhibition.
患有威廉姆斯综合征（WS）的人在日常生活中对陌生人表现出不加区分的亲近态度。患有唐氏综合征（DS）的人也会如此，但程度较轻。不适当的亲近行为也是患有后天杏仁核损伤的人和患有后天额叶损伤的人的特征；鉴于此，在WS以及可能在DS中出现的社交亲近方面的发育障碍可能是由于情绪识别能力差（由于杏仁核功能异常）或行为控制能力差（由于额叶异常）。关于这种社会认知发育障碍的第三种解释可以用对社会刺激的显著增强来表述。我们通过测试WS和DS患者的情绪识别、社交亲近和额叶功能来探究这三种假设。总体而言，我们的结果与杏仁核和社会显著性假设的预测不一致。相比之下，一系列神经心理学任务的结果表明，WS和DS患者在日常生活中异常的社交亲近行为最好用额叶损伤来解释，特别是反应抑制能力差。
