Kobayakawa Mutsutaka
Department of Informatics, Faculty of Informatics, Tokyo University of Information Sciences.
Brain Nerve. 2016 Feb;68(2):137-44. doi: 10.11477/mf.1416200363.
Myotonic dystrophy type 1 (DM 1) is a heritable, multisystem disease that affects not only the muscles but also the brain. DM 1 is often accompanied by developmental behavioral disorders, such as autism spectrum disorders. The autistic traits in DM 1 may be related to social cognitive dysfunction. The social cognitive function of patients with DM 1 was examined with respect to facial emotion recognition and theory of mind, which is the specific cognitive ability to understand the mental states of other people. With respect to facial emotion recognition, the sensitivities to disgust and anger were lower among patients with DM 1 than among healthy subjects, and this difference could not be attributed to visual impairment. To examine the theory of mind ability, the "Reading the Mind in the Eyes" test and the faux pas recognition test were used. Patients with DM 1 were found to be impaired in both tests, but the results were not attributed to visual ability and lexical comprehension. The possible causes of social cognitive dysfunction in DM 1 are the l cerebral atrophy and white matter abnormalities in the temporal, frontal, and insular cortex. Dysfunctions in these areas may affect the emotional and theory of mind abilities in DM 1, which result in the behavioral and communication disorders.
1型强直性肌营养不良症（DM 1）是一种遗传性多系统疾病，不仅会影响肌肉，还会影响大脑。DM 1常伴有发育行为障碍，如自闭症谱系障碍。DM 1中的自闭症特征可能与社会认知功能障碍有关。研究人员从面部表情识别和心理理论（即理解他人心理状态的特定认知能力）方面对DM 1患者的社会认知功能进行了检查。在面部表情识别方面，DM 1患者对厌恶和愤怒的敏感度低于健康受试者，且这种差异不能归因于视力障碍。为了检验心理理论能力，研究人员使用了“读心术（眼睛测试）”和失礼识别测试。结果发现，DM 1患者在这两项测试中均表现受损，但结果不能归因于视觉能力和词汇理解能力。DM 1中社会认知功能障碍的可能原因是大脑萎缩以及颞叶、额叶和岛叶皮质的白质异常。这些区域的功能障碍可能会影响DM 1患者的情绪和心理理论能力，进而导致行为和沟通障碍。
