scholarly journals Protection of Motor Neurons in Pre-Symptomatic Individuals Carrying SOD 1 Mutations: Results of Motor Unit Number Estimation (MUNE) Electrophysiology

10.5772/30259 ◽  
2012 ◽  
Author(s):  
Arun Aggarwal
2012 ◽  
Vol 123 (10) ◽  
pp. 2080-2091 ◽  
Author(s):  
S.T. Ngo ◽  
F. Baumann ◽  
P.G. Ridall ◽  
A.N. Pettitt ◽  
R.D. Henderson ◽  
...  

2009 ◽  
Vol 83 (9) ◽  
pp. 4251-4261 ◽  
Author(s):  
Venkatraman Siddharthan ◽  
Hong Wang ◽  
Neil E. Motter ◽  
Jeffery O. Hall ◽  
Robert D. Skinner ◽  
...  

ABSTRACT To investigate the hypothesis that neurological sequelae are associated with persistent West Nile virus (WNV) and neuropathology, we developed an electrophysiological motor unit number estimation (MUNE) assay to measure the health of motor neurons temporally in hamsters. The MUNE assay was successful in identifying chronic neuropathology in the spinal cords of infected hamsters. MUNE was suppressed at days 9 to 92 in hamsters injected subcutaneously with WNV, thereby establishing that a long-term neurological sequela does occur in the hamster model. MUNE suppression at day 10 correlated with the loss of neuronal function as indicated by reduced choline acetyltransferase staining (R 2 = 0.91). Between days 10 and 26, some α-motor neurons had died, but further neuronal death was not detected beyond day 26. MUNE correlated with disease phenotype, because the lowest MUNE values were detected in paralyzed limbs. Persistent WNV RNA and foci of WNV envelope-positive cells were identified in the central nervous systems of all hamsters tested from 28 to 86 days. WNV-positive staining colocalized with the neuropathology, which suggested that persistent WNV or its products contributed to neuropathogenesis. These results established that persistent WNV product or its proteins cause dysfunction, that WNV is associated with chronic neuropathological lesions, and that this neurological sequela is effectively detected by MUNE. Inasmuch as WNV-infected humans can also experience a poliomyelitis-like disease where motor neurons are damaged, MUNE may also be a sensitive clinical or therapeutic marker for those patients.


Author(s):  
Omid Rashidipour ◽  
K. Ming Chan

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.


2018 ◽  
Vol 129 ◽  
pp. e80
Author(s):  
Christina S. Nielsen ◽  
Michael Vaeggemose ◽  
Anna B. Jacobsen ◽  
Anders Fuglsang-Frederiksen ◽  
Henning Andersen ◽  
...  

2012 ◽  
Vol 123 (9) ◽  
pp. e94
Author(s):  
Mana Higashihara ◽  
Masahiro Sonoo ◽  
Tomotaka Yamamoto ◽  
Yu Nagashima ◽  
Yasuo Terao ◽  
...  

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