Yuan Yanchun, Fu Yan, Wang Xueying, Hu Fan, Zhao Qianqian, He Cailin, Tang Linxin, Li Yongchao, Bu Yue, Song Xinyu, Liu Qing, Liu Ziqin, Xu Renshi, Cao Wenfeng, Zhang Yuanchao, Yi Xiaoping, Wang Junling, Chen Bihong T
Xiangya Hospital, Central South University, Jiangxi (National Regional Center for Neurological Diseases), Nanchang, Jiangxi, P. R. China.
Department of Radiology, Xiangya Hospital, Central South University, Changsha, Hunan, P.R. China.
Brain Behav. 2025 May;15(5):e70495. doi: 10.1002/brb3.70495.
Neuroimaging has been increasingly used to assess brain structural alterations in patients with amyotrophic lateral sclerosis (ALS). We aimed to investigate alterations in brain sub-cortical structures and to identify potential neuroimaging biomarkers for disease progression for patients with ALS.
A total of 61 patients with ALS were prospectively enrolled and were divided into three subgroups according to disease progression, i.e., fast, intermediate, and slow progression. Sixty-one matched healthy controls (HCs) were also recruited. All participants acquired a brain structural magnetic resonance imaging scan for subcortical volumetric and shape analyses. Neuropsychological testing and functional assessment were performed.
Patients with fast progression showed significant shape alterations in basal ganglia and brainstem as compared to the HCs group. In ALS patients with fast progression, shape contractions with atrophic changes were noted in bilateral nucleus accumbens, left caudate, left thalamus, and brainstem; while shape expansion with hypertrophy was noted in the left caudate, left thalamus, and left pallidum (all p < 0.05). There were significant positive correlations of the shape changes of the left thalamus with the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALS-FRS-R) total and limb scores and with disease duration (all p < 0.05). There were positive correlations of left pallidum with anxiety or with disease duration, and of left nucleus accumbens with ALS-FRS-R total or bulbar score, and of brainstem with mini-mental state examination score (all p < 0.05).
Extensive shape alterations of subcortical nuclei were noted in patients with fast progression of ALS, implicating subcortical shape being a potential neuroimaging biomarker for ALS progression.
神经影像学已越来越多地用于评估肌萎缩侧索硬化症（ALS）患者的脑结构改变。我们旨在研究脑皮质下结构的改变，并确定ALS患者疾病进展的潜在神经影像学生物标志物。
前瞻性纳入61例ALS患者，并根据疾病进展分为三个亚组，即快速进展、中度进展和缓慢进展亚组。还招募了61名匹配的健康对照者（HCs）。所有参与者均进行了脑结构磁共振成像扫描，以进行皮质下体积和形状分析。进行了神经心理学测试和功能评估。
与HCs组相比，快速进展的患者基底节和脑干出现明显的形状改变。在快速进展的ALS患者中，双侧伏隔核、左侧尾状核、左侧丘脑和脑干出现萎缩性形状收缩；而左侧尾状核、左侧丘脑和左侧苍白球出现肥大性形状扩张（均p<0.05）。左侧丘脑形状改变与修订的肌萎缩侧索硬化功能评定量表（ALS-FRS-R）总分和肢体评分以及疾病持续时间均呈显著正相关（均p<0.05）。左侧苍白球与焦虑或疾病持续时间呈正相关，左侧伏隔核与ALS-FRS-R总分或延髓评分呈正相关，脑干与简易精神状态检查评分呈正相关（均p<0.05）。
快速进展的ALS患者皮质下核出现广泛的形状改变，提示皮质下形状是ALS进展的潜在神经影像学生物标志物。
