Tae Woo Suk, Sung Joo Hye, Baek Seol Hee, Lee Chan Nyoung, Kim Byung Jo
Brain Convergence Research Center, Korea University, Seoul, Korea.
Department of Neurology, Korea University Anam Hospital, Korea University College of Medicine, Seoul, Korea.
J Clin Neurol. 2020 Oct;16(4):592-598. doi: 10.3988/jcn.2020.16.4.592.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that mainly affects the pyramidal motor system. However, recent studies have suggested that degeneration of the extramotor system plays a role in the disability experienced by patients with ALS. We investigated the local shape changes and mean volumes of the subcortical nuclei in sporadic ALS patients with preserved cognition.
The participants comprised 32 patients with ALS and 43 age- and sex-matched healthy controls. Three-dimensional T1-weighted structural images were acquired. Surface-based vertex analysis was performed with fully automated segmentation of both amygdalae, hippocampi, caudate nuclei, nuclei accumbens, putamina, pallida, and thalami, and the brainstem. The scalar distances from the mean surfaces of the individual subcortical nuclei were compared between groups, and correlations of the local shape distances with initial Amyotrophic Lateral Sclerosis Functional Rating Scale Revised (ALS-FRS-R) scores and the delta FRS-R and with the disease duration were analyzed.
ALS patients showed regional shape contractions on the lateral surfaces of both pallida, the lateroposterior surface of the right putamen, and the anterior basal surface of the right accumbens. Delta FRS-R scores were negatively correlated with local shape distances in the right hippocampus and the putamina. However, the initial ALS-FRS-R score and disease duration were not correlated with local shape distances.
Subcortical gray-matter structures are involved in the neurodegenerative process of ALS before cognitive impairment becomes evident.
肌萎缩侧索硬化症（ALS）是一种主要影响锥体运动系统的神经退行性疾病。然而，最近的研究表明，运动外系统的退化在ALS患者的残疾中起作用。我们研究了认知功能保留的散发性ALS患者皮质下核团的局部形状变化和平均体积。
参与者包括32例ALS患者和43例年龄及性别匹配的健康对照者。采集三维T1加权结构图像。对杏仁核、海马体、尾状核、伏隔核、壳核、苍白球和丘脑以及脑干进行全自动分割，进行基于表面的顶点分析。比较两组之间各个皮质下核团平均表面的标量距离，并分析局部形状距离与初始修订的肌萎缩侧索硬化功能评定量表（ALS-FRS-R）评分、FRS-R变化值以及病程的相关性。
ALS患者在双侧苍白球的外侧表面、右侧壳核的后外侧表面和右侧伏隔核的前基底表面出现区域形状收缩。FRS-R变化值与右侧海马体和壳核的局部形状距离呈负相关。然而，初始ALS-FRS-R评分和病程与局部形状距离无关。
在认知障碍明显出现之前，皮质下灰质结构就参与了ALS的神经退行性过程。
