肌萎缩侧索硬化症患者神经影像学研究进展  

Neuroimaging advances in patients with amyotrophic lateral sclerosis

作　　者：张秋丽[1] 张明[1] ZHANG Qiuli;ZHANG Ming(Department of Medical Imaging,The First Affiliated Hospital of Xi’an Jiaotong University,Xi’an 710061,China)

机构地区：[1]西安交通大学第一附属医院医学影像科,陕西西安710061

出　　处：《西安交通大学学报（医学版）》2023年第5期668-673,共6页Journal of Xi’an Jiaotong University（Medical Sciences）

基　　金：陕西省2020年重点研发计划项目(No.2020SF-098);陕西省自然科学基础研究计划(No.2022JQ-964)。

摘　　要：肌萎缩侧索硬化症(amyotrophic lateral sclerosis,ALS)是一类累及运动系统及运动系统外脑区的多系统退变疾病。复杂的临床表型以及上运动神经元损伤客观标记物的缺乏是导致ALS诊断困难的主要原因;同时,非运动系统损伤的极大异质性以及药物疗效的争议,增加了疾病管理及治疗的困难。多参数功能磁共振技术为全面探索ALS诊断、疾病管理及疗效评价机制提供了客观证据。本文阐述了功能磁共振在ALS运动系统、非运动系统损伤中的研究进展,以及其在疾病分期及药物疗效评价等方面的潜在价值。以疾病复杂表现为核心,联合大脑脊髓成像,为疾病诊疗提供直接证据是未来研究的重点。Amyotrophic lateral sclerosis(ALS)is a multi-system neurodegenerative disease characterized with degeneration of both motor and non-motor areas.Complicated clinical manifestations and lack of objective biomarkers for upper motor neuron deficits challenged the early diagnosis of ALS.Meanwhile,heterogeneous non-motor symptoms and conflicted treatment effects exacerbated the management and therapy of the disease.The multiparametric functional MRI has the potential to address all the needs for diagnosis,management,and disease modified therapy in ALS.The present paper summarizes the research progress in both motor and non-motor impairment in ALS,as well as their potential value in visualizing disease stages and drug effect evaluation.Focusing on the heterogeneity of the disease and combining with brain and spinal cord imaging may provide direct evidence for disease diagnosis and treatment and be the priority in the future for ALS.

关 键 词：肌萎缩侧索硬化症 上运动神经元损伤 认知障碍 行为异常 神经影像 

分 类 号：R445.2[医药卫生—影像医学与核医学]