Motor unit number estimation in the assessment of performance and function in motor neuron disease.

Motor unit number estimation (MUNE) is a unique electrophysiologic test used to estimate the number of surviving motor units in a muscle or group of muscles. It is used most frequently to monitor lower motor neuron loss in amyotrophic lateral sclerosis and spinal muscle atrophy. Of particular interest is its use as an endpoint measure in clinical trials for these diseases. This article describes the principles of MUNE and the factors that need to be considered, and reviews several techniques that have been used in clinical trials and in monitoring progression. It then reviews experience with MUNE in clinical trials for amyotrophic lateral sclerosis and spinal muscle atrophy and discusses how MUNE correlates with measures of function.