Equipe de Neuropsychologie Interventionnelle, Institut Mondor de Recherche Biomédicale, Créteil, France.
Cortex. 2013 Feb;49(2):549-58. doi: 10.1016/j.cortex.2011.12.003. Epub 2011 Dec 16.
Patients with Huntington's disease (HD), a neurodegenerative disorder that causes major motor impairments, also show cognitive and emotional deficits. While their deficit in recognising emotions has been explored in depth, little is known about their ability to express emotions and understand their feelings. If these faculties were impaired, patients might not only mis-read emotion expressions in others but their own emotions might be mis-interpreted by others as well, or thirdly, they might have difficulties understanding and describing their feelings. We compared the performance of recognition and expression of facial emotions in 13 HD patients with mild motor impairments but without significant bucco-facial abnormalities, and 13 controls matched for age and education. Emotion recognition was investigated in a forced-choice recognition test (FCR), and emotion expression by filming participants while they mimed the six basic emotional facial expressions (anger, disgust, fear, surprise, sadness and joy) to the experimenter. The films were then segmented into 60 stimuli per participant and four external raters performed a FCR on this material. Further, we tested understanding of feelings in self (alexithymia) and others (empathy) using questionnaires. Both recognition and expression were impaired across different emotions in HD compared to controls and recognition and expression scores were correlated. By contrast, alexithymia and empathy scores were very similar in HD and controls. This might suggest that emotion deficits in HD might be tied to the expression itself. Because similar emotion recognition-expression deficits are also found in Parkinson's Disease and vascular lesions of the striatum, our results further confirm the importance of the striatum for emotion recognition and expression, while access to the meaning of feelings relies on a different brain network, and is spared in HD.
亨廷顿病（HD）患者是一种神经退行性疾病，会导致严重的运动障碍，同时还表现出认知和情感缺陷。虽然他们识别情绪的缺陷已经被深入研究，但对于他们表达情绪和理解自身感受的能力知之甚少。如果这些功能受损，患者不仅可能误解他人的情绪表达，还可能导致他人误解他们自己的情绪，或者他们可能难以理解和描述自己的感受。我们比较了 13 名 HD 患者（轻度运动障碍但无明显面口异常）和 13 名年龄和教育程度匹配的对照组在识别和表达面部表情方面的表现。使用强制选择识别测试（FCR）来评估情绪识别，通过拍摄参与者模仿愤怒、厌恶、恐惧、惊讶、悲伤和喜悦等六种基本面部表情来评估情绪表达，然后将影片分成每个参与者 60 个刺激，由四名外部评估者对该材料进行 FCR。此外，我们使用问卷测试了自我（情感表达不能）和他人（同理心）的情感理解。与对照组相比，HD 患者在不同情绪下的识别和表达都受到了损害，且识别和表达评分呈正相关。相比之下，HD 患者和对照组的情感表达不能和同理心评分非常相似。这可能表明 HD 中的情绪缺陷与表达本身有关。由于帕金森病和纹状体血管病变也存在类似的情绪识别-表达缺陷，我们的结果进一步证实了纹状体在情绪识别和表达中的重要性，而对感受意义的获取则依赖于不同的大脑网络，在 HD 中不受影响。
