In the present study we investigated emotion recognition in pure motor amyotrophic lateral sclerosis (ALS) patients and its relationship with the integrity of basal ganglia, hippocampus and amygdala. Twenty ALS patients without either cognitive or behavioural impairment, and 52 matched healthy controls performed a neuropsychological assessment including the Comprehensive Affect Testing System (CATS) investigating emotion recognition. All participants underwent also a 3T brain MRI. Volumes of basal ganglia, hippocampus and amygdala bilaterally were measured using FIRST in FSL. Sociodemographic, cognitive and MRI data were compared between groups. In ALS patients, correlations between CATS significant findings, brain volumes, cognition, mood and behaviour were explored. ALS patients showed altered performances at the CATS total score and, among the investigated emotions, patients were significantly less able to recognize disgust compared with controls. No brain volumetric differences were observed between groups. In ALS patients, a lower performance in disgust recognition was related with a reduced volume of the left pallidum and a lower performance on the Edinburgh Cognitive and Behavioural ALS Screen. Cognitively/behaviourally unimpaired ALS patients showed impaired disgust recognition, which was associated with pallidum volume. The association with cognitive alterations may suggest impaired disgust recognition as an early marker of cognitive decline.

在本研究中，我们调查了纯运动性肌萎缩侧索硬化症 (ALS) 患者的情绪识别及其与基底神经节、海马和杏仁核完整性的关系。20 名没有认知或行为障碍的 ALS 患者和 52 名匹配的健康对照进行了神经心理学评估，包括调查情绪识别的综合情感测试系统 (CATS)。所有参与者还接受了 3T 脑部 MRI 检查。在 FSL 中使用 FIRST 测量双侧基底神经节、海马和杏仁核的体积。比较了各组之间的社会人口统计学、认知和 MRI 数据。在 ALS 患者中，探索了 CATS 显着发现、脑容量、认知、情绪和行为之间的相关性。ALS 患者的 CATS 总分表现有所改变，并且，在所调查的情绪中，与对照组相比，患者识别厌恶的能力显着降低。组间未观察到脑容量差异。在 ALS 患者中，厌恶识别的较低表现与左侧苍白球体积减少和爱丁堡认知和行为 ALS 筛查的较低表现有关。认知/行为未受损的 ALS 患者表现出厌恶识别受损，这与苍白球体积有关。与认知改变的关联可能表明厌恶识别受损是认知下降的早期标志。厌恶识别的较低表现与左侧苍白球体积减少和爱丁堡认知和行为 ALS 筛查的较低表现有关。认知/行为未受损的 ALS 患者表现出厌恶识别受损，这与苍白球体积有关。与认知改变的关联可能表明厌恶识别受损是认知下降的早期标志。厌恶识别的较低表现与左侧苍白球体积减少和爱丁堡认知和行为 ALS 筛查的较低表现有关。认知/行为未受损的 ALS 患者表现出厌恶识别受损，这与苍白球体积有关。与认知改变的关联可能表明厌恶识别受损是认知下降的早期标志。