Ghaderi Sadegh, Mohammadi Sana, Fatehi Farzad
Neuromuscular Research Center, Department of Neurology, Shariati Hospital, Tehran University of Medical Sciences, Tehran 1416634793, Iran.
Department of Neuroscience and Addiction Studies, School of Advanced Technologies in Medicine, Tehran University of Medical Sciences, Tehran 1416634793, Iran.
Brain Sci. 2025 Jul 3;15(7):715. doi: 10.3390/brainsci15070715.
Despite extensive research, the underlying causes of amyotrophic lateral sclerosis (ALS) remain unclear. This umbrella review aims to synthesize a vast body of evidence from advanced magnetic resonance imaging (MRI) studies of ALS, encompassing a wide range of neuroimaging techniques and patient cohorts. Following the PRISMA guidelines, we conducted an extensive search of four databases (PubMed, Scopus, Web of Science, and Embase) for articles published until 3 December 2024. Data extraction and quality assessment were independently performed using the AMSTAR2 tool. This review included 18 studies that incorporated data from over 29,000 ALS patients. Structural MRI consistently showed gray matter atrophy in the motor and extra-motor regions, with significant white matter (WM) atrophy in the corticospinal tract and corpus callosum. Magnetic resonance spectroscopy revealed metabolic disruptions, including reduced N-acetylaspartate and elevated choline levels. Functional MRI studies have demonstrated altered brain activation patterns and functional connectivity, reflecting compensatory mechanisms and neurodegeneration. fMRI also demonstrated disrupted motor network connectivity and alterations in the default mode network. Diffusion MRI highlighted microstructural changes, particularly reduced fractional anisotropy in the WM tracts. Susceptibility-weighted imaging and quantitative susceptibility mapping revealed iron accumulation in the motor cortex and non-motor regions. Perfusion MRI indicated hypoperfusion in regions associated with cognitive impairment. Multiparametric MRI consistently highlights widespread structural, functional, and metabolic changes in ALS, reflecting neurodegeneration and compensatory mechanisms.
尽管进行了广泛的研究，但肌萎缩侧索硬化症（ALS）的潜在病因仍不清楚。本综述旨在综合来自ALS的先进磁共振成像（MRI）研究的大量证据，涵盖广泛的神经成像技术和患者队列。按照PRISMA指南，我们对四个数据库（PubMed、Scopus、科学网和Embase）进行了广泛搜索，以查找截至2024年12月3日发表的文章。使用AMSTAR2工具独立进行数据提取和质量评估。本综述纳入了18项研究，这些研究纳入了超过29000名ALS患者的数据。结构MRI始终显示运动和运动外区域的灰质萎缩，皮质脊髓束和胼胝体有明显的白质（WM）萎缩。磁共振波谱显示代谢紊乱，包括N-乙酰天门冬氨酸减少和胆碱水平升高。功能MRI研究表明大脑激活模式和功能连接发生改变，反映了代偿机制和神经退行性变。功能磁共振成像还显示运动网络连接中断和默认模式网络改变。扩散MRI突出了微观结构变化，特别是WM束中分数各向异性降低。磁敏感加权成像和定量磁敏感映射显示运动皮层和非运动区域有铁沉积。灌注MRI表明与认知障碍相关区域存在灌注不足。多参数MRI始终突出显示ALS中广泛的结构、功能和代谢变化，反映了神经退行性变和代偿机制。
