OBJECTIVE Cerebellar neurodegenerative disorders (CDs) are a heterogeneous group of disorders. It is known that the cerebellum plays a role not only in motor, but also in cognitive and social cognitive functions. The aim of this study was to investigate social cognition in patients with different CDs. MATERIALS AND METHODS Social cognition was examined in 34 patients, 12 with spinocerebellar ataxia type 1 (SCA1), 6 with spinocerebellar ataxia type 2 (SCA2), and 16 with idiopathic late onset cerebellar ataxia (ILOCA). All patients were clinically evaluated using the Scale for the Rating and Assessment of Ataxia. In addition, 34 age, sex, and education-matched healthy control (HC) subjects were similarly analyzed. Social cognition was studied using two tests: the Faux Pas Recognition Test and the Reading the Mind in the Eyes Test (RMET). An appropriate array of neuropsychological tests was used to assess the global cognitive status as well as the frontal functions and mood. RESULTS CD patients achieved significantly worse results on both tests of social cognition compared to the HCs. The SCA1 + 2 group achieved the poorest results on the Faux Pas Recognition Test and exhibited poor performance on all cognitive tests, but was only significantly worse compared to the ILOCA group on the Free and Cued Selective Reminding Test (FCSRT) - recognition. The patients in the SCA1 + 2 and ILOCA groups obtained similar scores on RMET. In the SCA1 + 2 group the findings significantly correlated with clinical parameters of disease severity and duration and executive functions (EFs), and with mood and executive functions in the ILOCA group. In the SCA group EFs appeared as the only significant predictor of RMET achievement. The Boston Naming Test (BTN) was a significant predictor of the CD patients' achievement on RMET, while the BTN, the Trail Making Test Part A and FCSRT - Delayed free recall predicted their performance on the Faux Pas Recognition Test. CONCLUSION Patients with CD have social cognitive impairments as demonstrated by the Faux Pas Test and the RMET test results. The SCA1 and 2 patients exhibited a more pronounced impairment compared with the ILOCA patients. The independent cognitive predictors of social cognition impairment were EFs and language.

中文翻译：

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小脑神经退行性疾病患者的社会认知。

目的 小脑神经退行性疾病 (CDs) 是一组异质性疾病。众所周知，小脑不仅在运动中起作用，而且在认知和社会认知功能中也起作用。本研究的目的是调查不同 CD 患者的社会认知。材料和方法 对 34 名患者进行社会认知检查，其中 12 名脊髓小脑性共济失调 1 型 (SCA1)、6 名脊髓小脑性共济失调 2 型 (SCA2) 和 16 名特发性迟发性小脑共济失调 (ILOCA)。使用共济失调评定和评估量表对所有患者进行临床评估。此外，对 34 名年龄、性别和教育匹配的健康对照 (HC) 受试者进行了类似分析。社会认知使用两项测试进行研究：失礼识别测试和眼中读心测试 (RMET)。一系列适当的神经心理学测试用于评估整体认知状态以及额叶功能和情绪。结果 与 HC 相比，CD 患者在两项社会认知测试中取得了明显更差的结果。SCA1 + 2 组在失礼识别测试中取得了最差的结果，并且在所有认知测试中表现出较差的表现，但与 ILOCA 组相比，在自由和提示选择性提醒测试 (FCSRT) - 识别中仅显着更差。SCA1 + 2 和 ILOCA 组的患者在 RMET 上获得了相似的分数。在 SCA1 + 2 组中，这些发现与疾病严重程度和持续时间和执行功能 (EF) 的临床参数显着相关，并且与 ILOCA 组中的情绪和执行功能显着相关。在 SCA 组中，EFs 似乎是 RMET 成就的唯一重要预测指标。波士顿命名测试 (BTN) 是 CD 患者在 RMET 上取得成就的重要预测指标，而 BTN、Trail Making Test Part A 和 FCSRT - 延迟自由召回预测了他们在失礼识别测试中的表现。结论 CD 患者存在社交认知障碍，如 Faux Pas 测试和 RMET 测试结果所示。与 ILOCA 患者相比，SCA1 和 2 名患者表现出更明显的损伤。社会认知障碍的独立认知预测因子是 EFs 和语言。Trail Making Test Part A 和 FCSRT - 延迟自由召回预测了他们在失礼识别测试中的表现。结论 CD 患者存在社交认知障碍，如 Faux Pas 测试和 RMET 测试结果所示。与 ILOCA 患者相比，SCA1 和 2 名患者表现出更明显的损伤。社会认知障碍的独立认知预测因子是 EFs 和语言。Trail Making Test Part A 和 FCSRT - 延迟自由召回预测了他们在失礼识别测试中的表现。结论 CD 患者存在社交认知障碍，如 Faux Pas 测试和 RMET 测试结果所示。与 ILOCA 患者相比，SCA1 和 2 名患者表现出更明显的损伤。社会认知障碍的独立认知预测因子是 EFs 和语言。