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肌萎缩侧索硬化（ALS）是一种快速进展的神经系统变性疾病，以选择性上、下神经元受累为特征，下运动神经元受累导致的运动单位进行性丢失是ALS重要的病理改变之一。目前在ALS的早期诊断和疾病进展的监测中，仍缺乏特异生物标志物。近年来，随着电生理技术的发展，用于定量反映运动单位丢失的方法和技术也不断发展。本文主要对运动单位估数和运动单位数量指数检测技术在ALS中的研究进展进行综述。“,”Amyotrophic lateral sclerosis (ALS) is a rapid progressive neurodegenerative disease, characterized by the degeneration of both upper and lower motor neurons. The progressive motor unit loss due to lower motor neuron damage is one of the most important pathological process in ALS. However, there are lack of specific biomarkers for early diagnosis and progression monitoring of ALS. The relevant electrophysiologic techniques which quantify the number of motor unit have been developed rapidly in recent years. Among them, the motor unit number estimation (MUNE) and the motor unit number index (MUNIX) have been widely applied to quantify motor unit number loss in ALS. The most recent advances of MUNE and MUNIX in ALS were reviewed.