肌萎缩侧索硬化(amyotrophic lateral sclerosis, ALS)是一种进行性发展的神经退行性疾病，由于缺乏有效的生物标志物，疾病的早期诊断、进展监测及预后评估均受到影响。近年来基于ALS患者体液的生物标志物研究取得了重要进展，为该病的早期诊断和进展监测提供了一定的理论基础及实践的可行性，同时也为研究其发病机制提供了可靠的线索。本文总结了ALS患者脑脊液、血液和尿液等不同来源的体液生物标志物及其意义。“,”Amyotrophic lateral sclerosis (ALS)is a relentless, progressive, and presently incurable neurodegenerative disease. Its drug development has been hampered by the lack of effective biomarkers for early diagnosis, progression and prognosis. Recently, significant progress has been made for the identification of body fluid biomarkers for ALS, which conferred both theoretical and practical feasibility for the early diagnosis and progression monitoring. Meanwhile, it also facilitated identification of genes and/or pathways for the pathogenesis of ALS. This review summarized biomarkers identified from cerebrospinal fluid, blood and urine of ALS patients and their clinical implications.