"Rita Levi Montalcini" Department of Neuroscience, Amyotrophic Lateral Sclerosis Center, University of Turin, Turin, Italy.
Neurology 1, City of Health and Science University Hospital of Turin, Turin, Italy.
Eur J Neurol. 2022 Aug;29(8):2211-2219. doi: 10.1111/ene.15388. Epub 2022 May 30.
Social cognition (SC) deficits are included in amyotrophic lateral sclerosis (ALS)-frontotemporal spectrum disorder revised diagnostic criteria. However, SC performance among ALS patients is heterogeneous due to the phenotypic variability of the disease and the wide range of neuropsychological tools employed. The aim of the present study was to assess facial emotion recognition and theory of mind in ALS patients compared to controls and to evaluate correlations with the other cognitive domains and degree of motor impairment.
Eighty-three patients and 42 controls underwent a cognitive evaluation and SC assessment through the Ekman 60 Faces Test (EK-60F), the Reading the Mind in the Eyes Test-36 Faces (RMET-36), and the Story-Based Empathy Task (SET).
ALS patients showed significantly worse performance compared to controls in EK-60F global score (p < 0.001), recognition of disgust (p = 0.032), anger (p = 0.038), fear (p < 0.001), and sadness (p < 0.001); RMET-36 (p < 0.001), and SET global score (p < 0.001). Also, cognitively normal patients (ALS-CN) showed significantly worse performance compared to controls in EK-60F global score (p < 0.001), recognition of fear (p = 0.002), sadness (p < 0.001), and SET (p < 0.001). RMET-36 showed a significant correlation with the Category Fluency Test (p = 0.041). SC tests showed no correlation with motor impairment expressed by Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised.
ALS patients, also when categorized as ALS-CN, may show impairment in SC performance. The frequent identification of early SC impairment in ALS patients supports the need to routinely assess SC for its impact on end-of-life decisions and its potential influence on patients' quality of life.
社会认知(SC)缺陷已被纳入肌萎缩侧索硬化症(ALS)-额颞叶谱障碍修订后的诊断标准。然而,由于疾病表型的变异性和所采用的神经心理学工具范围广泛,ALS 患者的 SC 表现存在异质性。本研究旨在评估 ALS 患者与对照组相比的面部情绪识别和心理理论能力,并评估其与其他认知领域和运动损伤程度的相关性。
83 名患者和 42 名对照者接受了认知评估和 SC 评估,采用 Ekman 60 张面孔测试(EK-60F)、36 张面孔的读心测试(RMET-36)和基于故事的同理心任务(SET)。
与对照组相比,ALS 患者在 EK-60F 总评分(p < 0.001)、识别厌恶(p = 0.032)、愤怒(p = 0.038)、恐惧(p < 0.001)和悲伤(p < 0.001);RMET-36(p < 0.001)和 SET 总评分(p < 0.001)方面表现明显更差。此外,认知正常的患者(ALS-CN)在 EK-60F 总评分(p < 0.001)、识别恐惧(p = 0.002)、悲伤(p < 0.001)和 SET(p < 0.001)方面的表现也明显差于对照组。RMET-36 与类别流畅性测试(p = 0.041)呈显著相关性。SC 测试与改良 ALS 功能评定量表(ALSFRS-R)所表示的运动损伤无相关性。
ALS 患者,即使归类为 ALS-CN,也可能表现出 SC 表现受损。ALS 患者早期 SC 受损的频繁发现支持了常规评估 SC 的必要性,以评估其对生命末期决策的影响及其对患者生活质量的潜在影响。