Takeda Akitoshi, Kobayakawa Mutsutaka, Suzuki Atsunobu, Tsuruya Natsuko, Kawamura Mitsuru
Department of Neurology, Showa University School of Medicine, Tokyo, Japan.
J Neurol Sci. 2009 May 15;280(1-2):35-9. doi: 10.1016/j.jns.2009.01.014. Epub 2009 Feb 15.
It has been observed that patients with myotonic dystrophy type 1 (DM1) exhibit social-cognitive impairment. However, the cognitive and neurological bases of the social-cognitive impairment in DM1 have not been adequately investigated.
We studied cognitive deficits and impairment in facial expression recognition in two DM1 patients (one man and one woman). We measured the sensitivity of these patients to basic emotions and compared the results with those from magnetic resonance imaging and single photon emission computed tomography.
The DM1 patients showed lower sensitivity to fearful, disgusted, and angry faces than did the healthy controls. They also had lesions in the anterior temporal white matter, the amygdala, and the insular and orbitofrontal cortices.
The results of this study revealed that the DM1 patients had subcortical lesions in the anterior temporal areas, including the amygdala and the insular and orbitofrontal cortices. The limbic system, which includes these areas of the brain, plays an important role in emotional processing. Hence, the social-cognitive impairment in DM1 patients could be associated with a decreased sensitivity to facial expressions owing to lesions in the limbic system.
据观察，1型强直性肌营养不良（DM1）患者存在社会认知障碍。然而，DM1患者社会认知障碍的认知和神经学基础尚未得到充分研究。
我们研究了两名DM1患者（一名男性和一名女性）的认知缺陷及面部表情识别障碍。我们测量了这些患者对基本情绪的敏感度，并将结果与磁共振成像和单光子发射计算机断层扫描的结果进行比较。
与健康对照相比，DM1患者对恐惧、厌恶和愤怒面孔的敏感度较低。他们在颞前白质、杏仁核、岛叶和眶额皮质也有病变。
本研究结果显示，DM1患者在颞前区域存在皮质下病变，包括杏仁核、岛叶和眶额皮质。包括大脑这些区域的边缘系统在情绪处理中起重要作用。因此，DM1患者的社会认知障碍可能与边缘系统病变导致的面部表情敏感度降低有关。
