scholarly journals The Role of the Statistical Method of Motor Unit Number Estimation (MUNE) to Assess the Potential Therapeutic Benefits of Riluzole on Patients with Pre-symptomatic Familial Amyotrophic Lateral Sclerosis

Author(s):  
Arun Aggarwal
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.


2010 ◽  
Vol 107 (26) ◽  
pp. E107-E107 ◽  
Author(s):  
S. Millecamps ◽  
S. Da Barroca ◽  
C. Cazeneuve ◽  
F. Salachas ◽  
P.-F. Pradat ◽  
...  

2010 ◽  
Vol 42 (2) ◽  
pp. 239-244 ◽  
Author(s):  
Johannes P. van Dijk ◽  
Helenius J. Schelhaas ◽  
Ivo N. Van Schaik ◽  
Henny M.H.A. Janssen ◽  
Dick F. Stegeman ◽  
...  

2010 ◽  
Vol 25 (9) ◽  
pp. 1359 ◽  
Author(s):  
Suk-Won Ahn ◽  
Su-Hyun Kim ◽  
Dong-Hoon Oh ◽  
Sung-Min Kim ◽  
Kyung Seok Park ◽  
...  

Author(s):  
James M. Powers ◽  
Dikran S. Horoupian ◽  
Herbert H. Schaumburg

SUMMARYThe neuropathological findings of a Farr family member consist of neuronal loss in the anterior horns and dorsal nuclei of Clarke, neuronal intracytoplasmic inclusions and posterior and lateral column demyelination. This report supports the role of familial amyotrophic lateral sclerosis as a link between common motor neuron disease and classical spinocerebellar degeneration.


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