Grolez G, Moreau C, Danel-Brunaud V, Delmaire C, Lopes R, Pradat P F, El Mendili M M, Defebvre L, Devos D
Department of Movement Disorders and Neurology, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France.
INSERM U1171, Lille University Hospital, Faculty of Medicine, University of Lille, Lille, France.
BMC Neurol. 2016 Aug 27;16(1):155. doi: 10.1186/s12883-016-0672-6.
Amyotrophic lateral sclerosis (ALS) is a fatal, rapidly progressive neurodegenerative disease that mainly affects the motor system. A number of potentially neuroprotective and neurorestorative disease-modifying drugs are currently in clinical development. At present, the evaluation of a drug's clinical efficacy in ALS is based on the ALS Functional Rating Scale Revised, motor tests and survival. However, these endpoints are general, variable and late-stage measures of the ALS disease process and thus require the long-term assessment of large cohorts. Hence, there is a need for more sensitive radiological biomarkers. Various sequences for magnetic resonance imaging (MRI) of the brain and spinal cord have may have value as surrogate biomarkers for use in future clinical trials. Here, we review the MRI findings in ALS, their clinical correlations, and their limitations and potential role as biomarkers.
The PubMed database was screened to identify studies using MRI in ALS. We included general MRI studies with a control group and an ALS group and longitudinal studies even if a control group was lacking.
A total of 116 studies were analysed with MRI data and clinical correlations. The most disease-sensitive MRI patterns are in motor regions but the brain is more broadly affected.
Despite the existing MRI biomarkers, there is a need for large cohorts with long term MRI and clinical follow-up. MRI assessment could be improved by standardized MRI protocols with multicentre studies.
肌萎缩侧索硬化症（ALS）是一种致命的、快速进展的神经退行性疾病，主要影响运动系统。目前有多种具有潜在神经保护和神经修复作用的疾病修饰药物正处于临床开发阶段。目前，评估一种药物在ALS中的临床疗效是基于修订后的ALS功能评定量表、运动测试和生存期。然而，这些终点指标是ALS疾病进程的一般性、可变且晚期的测量指标，因此需要对大量队列进行长期评估。因此，需要更敏感的影像学生物标志物。脑和脊髓的各种磁共振成像（MRI）序列可能作为替代生物标志物用于未来的临床试验。在此，我们综述了ALS的MRI表现、它们与临床的相关性、局限性以及作为生物标志物的潜在作用。
对PubMed数据库进行筛选，以识别使用MRI研究ALS的文献。我们纳入了有对照组和ALS组的一般MRI研究以及即使没有对照组的纵向研究。
共分析了116项具有MRI数据和临床相关性的研究。对疾病最敏感的MRI表现位于运动区域，但大脑受到的影响更为广泛。
尽管已有MRI生物标志物，但仍需要大量队列进行长期的MRI和临床随访。通过标准化的MRI方案和多中心研究可以改进MRI评估。
