Gray J M, Young A W, Barker W A, Curtis A, Gibson D
Neurobehavioural Service, St Nicholas Hospital, Gosforth, UK.
Brain. 1997 Nov;120 ( Pt 11):2029-38. doi: 10.1093/brain/120.11.2029.
Face processing and facial expression recognition were investigated in the earliest stages of Huntington's disease, by studying 40 people who presented for genetic testing. Twenty-three of these 'at risk' individuals turned out not to carry the gene for Huntington's disease (the AR- group). Seventeen were found to be gene carriers (the AR+ group); 15 from genetic testing, and two who showed signs of early stages of Huntington's disease. A number of standard tasks were used to provide background information, including recognition memory for words, picture naming, verbal fluency, and figure copying; none revealed significant differences between the AR+ and AR- groups. Face processing abilities were investigated using tests of identification of familiar (famous) faces, unfamiliar face matching, recognition memory for faces, and recognition of facial expressions of emotion. No statistically significant differences between the AR+ and AR- groups were found for any of these tests, but the AR+ group showed a borderline overall impairment in recognizing facial expressions of emotion (0.05 < P < 0.1). When recognition of each of the six basic emotions used was examined separately, only disgust was found to be significantly impaired. This highly selective deficit in the recognition of disgust was confirmed in the subgroup of 15 individuals shown by genetic testing to be Huntington's gene carriers; it was therefore found in people who were free from clinical symptoms and did not perform significantly more poorly than non-carriers on any of the background tests, on any of the other face processing tasks, and even for recognition of any other basic emotion. This points strongly to the importance of the basal ganglia in the emotion of disgust.
通过对40名前来进行基因检测的人进行研究，在亨廷顿舞蹈症的最早阶段对面部处理和面部表情识别进行了调查。这些“有风险”的个体中，有23人被证明不携带亨廷顿舞蹈症基因（AR-组）。17人被发现是基因携带者（AR+组）；其中15人通过基因检测确诊，2人表现出亨廷顿舞蹈症早期症状。使用了一些标准任务来提供背景信息，包括单词识别记忆、图片命名、语言流畅性和图形复制；没有一项显示AR+组和AR-组之间存在显著差异。使用识别熟悉（著名）面孔、匹配不熟悉面孔、面孔识别记忆以及识别面部情绪表情的测试来研究面部处理能力。在这些测试中，AR+组和AR-组之间均未发现具有统计学意义的差异，但AR+组在识别面部情绪表情方面总体上有接近显著水平的损伤（0.05 < P < 0.1）。当分别检查所使用的六种基本情绪中的每一种的识别情况时，仅发现厌恶情绪的识别存在显著损伤。在通过基因检测显示为亨廷顿基因携带者的15人亚组中，证实了这种对厌恶情绪识别的高度选择性缺陷；因此，在没有临床症状且在任何背景测试、任何其他面部处理任务甚至在识别任何其他基本情绪方面表现并不比非携带者差得多的人群中也发现了这种情况。这有力地表明了基底神经节在厌恶情绪中的重要性。
