目的: 通过MRI皮层厚度分析法，探讨肌萎缩侧索硬化(amyotrophic lateral sclerosis, ALS)患者脑结构变化，并分析其与认知功能改变的相关性。方法: 纳入ALS患者18例，以及性别、年龄、教育程度匹配的正常对照者18例，对所有研究对象进行3D磁化准备快速递度回波(magnetization prepared rapid gradient echo imaging, MPRAGE)序列MRI扫描，进行皮层厚度分析，同时对所有ALS患者进行神经心理学测评，包括简易精神状态检查(mini-mental state examination，MMSE)、言语流畅性试验、Stroop色词试验、前瞻性记忆、情绪图片感知及再认、失言识别测验。结果: 经过认知评估，2例ALS患者存在认知功能障碍。比较18例ALS患者和18例正常对照的顶点水平全脑皮层厚度，ALS组左侧大脑半球内侧眶额叶后部、颞叶内侧皮层厚度明显减低(P < 0.05)；区域水平比较，ALS组左内嗅区、左颞下回、左内侧眶额叶、左岛叶皮层厚度明显减低(P < 0.05)。认知功能正常的16例ALS患者与正常对照相比较，两组间顶点水平和区域水平的皮层厚度差异均无统计学意义(P>0.05)。前瞻性记忆、情绪图片感知及再认、失言识别测验评分与对应脑区皮层厚度值存在相关性(P < 0.05)。结论: ALS患者皮层厚度与神经心理学检查具有相关性，可能反映认知测评所对应的脑区皮层结构改变，可能为ALS患者认知改变的早期诊断提供帮助。

Objective: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with high morbidity and mortality. There are about 5%-15% of ALS patients combining with frontotemporal lobe degeneration (FTLD) at the same time and 50% of patients combing with cognitive function changes. The analysis of cortical thickness based on MRI is an important imaging method to evaluate brain structure. The aim of the study was to explore the changes of brain structure in ALS patients by cortical thickness analysis, and to explore the correlation between the brain structure and cognitive function. Methods: In the study, 18 ALS patients treated in Department of Neurology, Peking University Third Hospital and 18 normal controls (age, gender and education level matched) were included. 3D magnetization prepared rapid gradient echo imaging (MPRAGE) sequence MRI was performed and the cortical thickness was analyzed. At the same time, all the ALS patients took neuropsychology assessments, including: mini-mental state examination (MMSE), verbal fluency test (VFT), Stroop color word test (SCWT), prospective memory (PM), emotional picture perception and recognition, and faux pas story test. Results: After cognitive assessment, two ALS patients had cognitive impairment. One was in accordance with ALS-frontotemporal dementia (FTD) diagnosis and the other one was in accordance with ALS cognitive impairment (ALSci) diagnosis. In all the 18 ALS patients and 18 normal controls, the cortical thickness of the left medial orbitofrontal lobe and the medial temporal lobe were significantly reduced (P < 0.05) in ALS group by the vertex-wise comparison. Cortical thickness of the left entorhinal cortex, the left inferior temporal gyrus, the left medial orbitofrontal lobe and the left insular lobe was significantly reduced (P < 0.05) by the region-wise comparison. However, when only concluded the 16 ALS non-cognitive impairment patients, there was no significant difference between the two groups (P>0.05). There were correlations between the scores of prospective memory, emotional picture perception and recognition, faux pas story test and the cortical thickness of their corresponding regions (P < 0.05). Conclusion: The cortical thickness of ALS patients are correlated with neuropsychological scores which may reflect the changes of cortical structure corresponding to the cognitive assessment, and may provide help for the early diagnosis of cognitive changes in ALS patients.