Motor unit number estimation in neuromuscular disease.
Motor unit number estimation (MUNE) is an electrophysiological method designed to quantify motor unit loss in target muscles of interest. Most of the techniques are noninvasive and are therefore well suited for longitudinal monitoring. In this brief review, we describe the more commonly used techniques and their applications in amyotrophic lateral sclerosis, poliomyelitis, spinal muscular atrophy and hereditary sensorimotor neuropathies. Findings in some of these studies offer important pathophysiological insights. Since conventional electrophysiologic methods are not sensible measures of motor neuronal loss, MUNE could play a potentially important role in the diagnosis, monitoring of disease progression and response to treatment in neuromuscular diseases in which motor unit loss is a major feature.
Rashidipour
Omid
O
Centre for Neuroscience, Division of Physical Medicine and Rehabilitation, Faculty of Medicine, University of Alberta, Edmonton, Alberta, Canada.
Chan
K Ming
KM
D004558:Electric Stimulation / Q000379:methods; D004576:Electromyography / Q000379:methods; D019054:Evoked Potentials, Motor / Q000502:physiology; D006801:Humans; D009046:Motor Neurons / Q000473:pathology*; D018482:Muscle, Skeletal / Q000503:physiopathology; D009468:Neuromuscular Diseases / Q000473:pathology*