Motor unit number estimation (MUNE) in diseases of the motor neuron: utility and comparative analysis in a multimodal biomarker study1

2009 ◽  
pp. 153-162 ◽  
Author(s):  
Clifton L. Gooch ◽  
Seth L. Pullman ◽  
Dikoma C. Shungu ◽  
Aziz M. Uluğ ◽  
Stephen Chan ◽  
...  
Keyword(s):  
Comparative Analysis ◽  
Motor Neuron ◽  
Motor Unit ◽  
Motor Unit Number Estimation

Following disease progression in motor neuron disorders with 3 motor unit number estimation methods

2018 ◽  
Vol 59 (1) ◽  
pp. 82-87 ◽  
Author(s):  
Anna Bystrup Jacobsen ◽  
Hugh Bostock ◽  
Hatice Tankisi
Keyword(s):  
Motor Neuron ◽  
Disease Progression ◽  
Motor Unit ◽  
Estimation Methods ◽  
Motor Unit Number Estimation

scholarly journals Bioengineered model of the human motor unit with physiologically functional neuromuscular junctions

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Rowan P. Rimington ◽  
Jacob W. Fleming ◽  
Andrew J. Capel ◽  
Patrick C. Wheeler ◽  
Mark P. Lewis
Keyword(s):  
Neuromuscular Junction ◽  
Motor Neuron ◽  
Motor Unit ◽  
Motor Nerve ◽  
Motor Units ◽  
Extracellular Matrices ◽  
Synaptic Membrane ◽  
Type I ◽  
Dependent Manner ◽  
Human Motor

AbstractInvestigations of the human neuromuscular junction (NMJ) have predominately utilised experimental animals, model organisms, or monolayer cell cultures that fail to represent the physiological complexity of the synapse. Consequently, there remains a paucity of data regarding the development of the human NMJ and a lack of systems that enable investigation of the motor unit. This work addresses this need, providing the methodologies to bioengineer 3D models of the human motor unit. Spheroid culture of iPSC derived motor neuron progenitors augmented the transcription of OLIG2, ISLET1 and SMI32 motor neuron mRNAs ~ 400, ~ 150 and ~ 200-fold respectively compared to monolayer equivalents. Axon projections of adhered spheroids exceeded 1000 μm in monolayer, with transcription of SMI32 and VACHT mRNAs further enhanced by addition to 3D extracellular matrices in a type I collagen concentration dependent manner. Bioengineered skeletal muscles produced functional tetanic and twitch profiles, demonstrated increased acetylcholine receptor (AChR) clustering and transcription of MUSK and LRP4 mRNAs, indicating enhanced organisation of the post-synaptic membrane. The number of motor neuron spheroids, or motor pool, required to functionally innervate 3D muscle tissues was then determined, generating functional human NMJs that evidence pre- and post-synaptic membrane and motor nerve axon co-localisation. Spontaneous firing was significantly elevated in 3D motor units, confirmed to be driven by the motor nerve via antagonistic inhibition of the AChR. Functional analysis outlined decreased time to peak twitch and half relaxation times, indicating enhanced physiology of excitation contraction coupling in innervated motor units. Our findings provide the methods to maximise the maturity of both iPSC motor neurons and primary human skeletal muscle, utilising cell type specific extracellular matrices and developmental timelines to bioengineer the human motor unit for the study of neuromuscular junction physiology.

scholarly journals Motor Unit Number Estimation in Neuromuscular Disease

2008 ◽  
Vol 35 (2) ◽  
pp. 153-159 ◽  
Author(s):  
Omid Rashidipour ◽  
K. Ming Chan
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Unit ◽  
Neuromuscular Disease ◽  
Muscular Atrophy ◽  
Neuromuscular Diseases ◽  
Neuronal Loss ◽  
Response To Treatment ◽  
Motor Unit Number Estimation ◽  
Electrophysiological Method ◽  
Lateral Sclerosis

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.

F37. Motor unit number estimation methods for quantification of motor unit loss in Guillain Barre Syndrome

2018 ◽  
Vol 129 ◽  
pp. e80
Author(s):  
Christina S. Nielsen ◽  
Michael Vaeggemose ◽  
Anna B. Jacobsen ◽  
Anders Fuglsang-Frederiksen ◽  
Henning Andersen ◽  
...  
Keyword(s):  
Motor Unit ◽  
Guillain Barre Syndrome ◽  
Estimation Methods ◽  
Motor Unit Number Estimation ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

32. Contribution of far-field potentials on motor unit number estimation (MUNE)

2012 ◽  
Vol 123 (9) ◽  
pp. e94
Author(s):  
Mana Higashihara ◽  
Masahiro Sonoo ◽  
Tomotaka Yamamoto ◽  
Yu Nagashima ◽  
Yasuo Terao ◽  
...  
Keyword(s):  
Motor Unit ◽  
Far Field ◽  
Field Potentials ◽  
Motor Unit Number Estimation

WS-22-2 Validity of EMG and tension as means of motor unit number estimation (MUNE)

1995 ◽  
Vol 97 (4) ◽  
pp. S46 ◽  
Author(s):  
Keisuke Arasaki ◽  
Mitsuyuki Tamaki ◽  
Yasuhiko Hosoya ◽  
Norio Kudo
Keyword(s):  
Motor Unit ◽  
Motor Unit Number Estimation
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