Neuroscience Area, Biodonostia Research Institute, Donostia-San Sebastian, Gipuzkoa, Spain.
Personality, Assessment and Psychological Treatment Department, Psychology Faculty, University of the Basque Country (UPV/EHU), Donostia-San Sebastian, Gipuzkoa, Spain.
PLoS One. 2018 Sep 24;13(9):e0204227. doi: 10.1371/journal.pone.0204227. eCollection 2018.
The cognitive profile of Myotonic Dystrophy type 1 (DM1) has been described in recent decades. Moreover, DM1 patients show lowered social engagement and difficulties in social-cognitive functions. The aim of the present study is to explore whether social cognition impairment is present in DM1 taking into account the overall cognitive condition.
38 patients and a control group paired in age and gender participated in the study. All the participants had an IQ within the normal range. Subjects were administered an abbreviated neuropsychological battery which comprised a facial emotion recognition test (POFA) and Faux Pas Test, as well as a self-report questionnaire on cognitive and affective empathy (TECA).
Statistically significant differences were found only for facial emotion recognition (U = 464.0, p = .006) with a moderate effect size (.31), with the controls obtaining a higher score than the patients. Analyzing each emotion separately, DM1 patients scored significantly lower than controls on the recognition of anger and disgust items. Emotion recognition did not correlate with genetic load, but did correlate negatively with age. No differences were found between patients and controls in any of the other variables related to Theory of Mind (ToM) and empathy.
DM1 does not manifest specific impairments in ToM since difficulties in this area predominantly rely on the cognitive demand of the tasks employed. However, a more basic process such as emotion recognition appears as a core deficit. The role of this deficit as a marker of aging related decline is discussed.
几十年来，人们已经描述了 1 型肌强直性营养不良（DM1）的认知特征。此外，DM1 患者表现出社交参与度降低和社会认知功能障碍。本研究的目的是探讨是否存在社会认知障碍，同时考虑到整体认知状况。
38 名患者和一组年龄和性别匹配的对照组参加了这项研究。所有参与者的智商均在正常范围内。研究对象接受了一项简短的神经心理学测试，包括面部情绪识别测试（POFA）和失态测试，以及认知和情感同理心自我报告问卷（TECA）。
仅在面部情绪识别方面存在统计学上的显著差异（U = 464.0，p =.006），具有中等效应大小（.31），对照组的得分高于患者。对每个情绪进行单独分析，DM1 患者在识别愤怒和厌恶项目方面的得分明显低于对照组。情绪识别与遗传负荷无关，但与年龄呈负相关。在与心理理论（ToM）和同理心相关的其他变量方面，患者与对照组之间没有差异。
DM1 没有表现出特定的 ToM 障碍，因为该领域的困难主要依赖于所使用任务的认知需求。然而，像情绪识别这样更基本的过程似乎是核心缺陷。讨论了这种缺陷作为与衰老相关衰退的标志物的作用。
