Olney R K, Lomen-Hoerth C
Department of Neurology, University of California at San Francisco, 94143-0114, USA.
Amyotroph Lateral Scler Other Motor Neuron Disord. 2000 Jun;1 Suppl 2:S41-4. doi: 10.1080/146608200300079473.
Motor unit number estimation (MUNE) is a type of electrophysiological technique that measures the approximate number of lower motor neurons (LMNs) innervating a single muscle or a small group of muscles. Low MUNE counts provide evidence of LMN degeneration, but a single MUNE study does not determine if this loss is ongoing, recent or remote in time. Sequential change of MUNE count provides evidence for ongoing degeneration. Furthermore, sequential change in MUNE from a normal to abnormally low count provides evidence for progressive spread of signs within a region or to another region. MUNE has no established ability to identify other diseases that may provide a non-ALS explanation for the signs of LMN degeneration. If MUNE studies were to be incorporated into a future revision of the diagnostic criteria for ALS, prospective studies will be important to define more clearly the sensitivity and specificity of MUNE in patients with ALS and in patients with weakness that does not involve LMN degeneration. In addition to its potential contributions toward the diagnosis of ALS, MUNE may have greater potential in quantifying the rate of progression in studies of the natural history of ALS and the response to experimental treatment.
运动单位数量估计（MUNE）是一种电生理技术，用于测量支配单个肌肉或一小群肌肉的下运动神经元（LMN）的大致数量。低MUNE计数提供了LMN变性的证据，但单次MUNE研究无法确定这种损失是正在发生、近期发生还是时间上较为久远。MUNE计数的连续变化为正在进行的变性提供了证据。此外，MUNE从正常计数到异常低计数的连续变化为体征在一个区域内或向另一个区域的进行性扩散提供了证据。MUNE尚无确定的能力来识别可能为LMN变性体征提供非肌萎缩侧索硬化（ALS）解释的其他疾病。如果MUNE研究要纳入未来修订的ALS诊断标准中，前瞻性研究对于更明确地界定MUNE在ALS患者和无LMN变性的肌无力患者中的敏感性和特异性将很重要。除了其对ALS诊断的潜在贡献外，MUNE在量化ALS自然史研究中的进展速度以及对实验性治疗的反应方面可能具有更大的潜力。
