Motor unit number estimation in neurologic disease.
Since its introduction 30 years ago, MUNE technologies have been increasingly refined and applied to a wide variety of neuromuscular disorders. Differences of opinion remain among MUNE investigators as to which method should be used; however, statistical and MPS MUNE currently enjoy the most widespread use. A number of methodological issues remain, including the development of detailed universal standards for each technique and modifications for the further enhancement of reproducibility. These issues are the subject of ongoing investigation. However, despite technical variability, the MUNE values obtained with different methods show good agreement, both in studies of healthy subjects and in patients with a variety of neurogenic processes. MUNE has been most successfully applied to patients with ALS and in animal models of motor neuron disease, providing significant insight into the pathophysiology of these disorders. These techniques are being increasingly incorporated into clinical therapeutic trials. MUNE is a technology offering important promise in the study of neuromuscular disease, enabling the collection of novel data in the living patient unobtainable by any other method.
Shefner
Jeremy M
JM
Department of Neurology, Clinical Neurophysiology Laboratory and MDA/ALS Research and Treatment Center, Upstate Medical University, State University of New York, Syracuse, New York 13210, USA.
Gooch
Clifton L
CL
D000200:Action Potentials; D002452:Cell Count / Q000379:methods*; D006801:Humans; D009046:Motor Neurons / Q000473:pathology* / Q000502:physiology; D009468:Neuromuscular Diseases / Q000473:pathology*