2008
DOI: 10.1017/s0317167100008568
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Motor Unit Number Estimation in Neuromuscular Disease

Abstract: Motoneuronal death is a major feature in many neuromuscular diseases. As a result of the remarkable ability of motor axons to sprout and to regenerate, the extent of motoneuronal loss often does not become clinically apparent until the loss is well advanced. This discrepancy is particularly striking in chronic, slowly progressive disorders in which surviving motoneurons have ample time to expand their innervation field. In those conditions, motor unit number estimation (MUNE) can be particularly valuable in ga… Show more

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Cited by 25 publications
(20 citation statements)
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“…Electromyography and motor unit number estimation play an important role in detecting LMNs and are regarded to be the most accurate confirmatory tests for ALS, but these techniques cannot evaluate disease development or the effects of treatment [6,7]. For the UMNs, neuroimaging techniques are a promising way to diagnose ALS or evaluate disease progression, and these techniques include conventional MRI, DTI, MRS and functional MRI [8].…”
Section: Discussionmentioning
confidence: 99%
“…Electromyography and motor unit number estimation play an important role in detecting LMNs and are regarded to be the most accurate confirmatory tests for ALS, but these techniques cannot evaluate disease development or the effects of treatment [6,7]. For the UMNs, neuroimaging techniques are a promising way to diagnose ALS or evaluate disease progression, and these techniques include conventional MRI, DTI, MRS and functional MRI [8].…”
Section: Discussionmentioning
confidence: 99%
“…The temporal development of neuronal cell damage in the spinal cord in the acute infection followed by the chronic infection is reminiscent in some ways of ALS and poliomyelitis. The motor units consisting of cell bodies, axons, and the muscle fibers are diminished, probably due to cell body damage followed by axonal degeneration (30). Suppression of MUNE was coincident with the presence of WNV-induced neuropathology, whereas MUNE was not suppressed in hamsters infected with yellow fever virus or Punta Toro virus, which cause nonneurological diseases.…”
mentioning
confidence: 96%
“…The loss of motor neurons is detected in other neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS) (30,36) and poliomyelitis (25), by using electrophysiological motor unit number estimation (MUNE) (5), in which a motor unit consists of an ␣-motor neuron and all the muscle fibers it innervates. A presumptive use of MUNE in the human WNV infection was identified in an uncontrolled clinical study (2) in which MUNE appeared to be a marker for degrees of weakness and recovery in patients with neurological sequelae.…”
mentioning
confidence: 99%
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“…Le vieillissement spécifique des unités motrices [12] est, quant à lui, de mieux en mieux précisé, et s'intègre dans la discussion étiopathogénique du « syndrome post-polio » (réduction des motoneurones alpha, surtout de grande taille).…”
Section: Maintien Des Acquisunclassified