66. Asymmetry of the motor unit number estimate and its rate of decline in patients with amyotrophic lateral sclerosis

2012 ◽  
Vol 123 (6) ◽  
pp. e35
Author(s):  
S. Ahn ◽  
Y. Hong ◽  
K. Lee ◽  
J. Sung ◽  
J. Kim ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Unit ◽  
Motor Unit Number Estimate ◽  
Rate Of Decline ◽  
Lateral Sclerosis

scholarly journals Motor unit number estimation versus compound muscle action potential in the evaluation of motor unit loss in amyotrophic lateral sclerosis

2021 ◽  
Vol 57 (1) ◽  
Author(s):  
Arwa Khalid Ibrahem ◽  
Akram Mohammed Al-Mahdawi ◽  
Farqad Bader Hamdan
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Unit ◽  
Rating Scale ◽  
Compound Muscle Action Potential ◽  
Motor Units ◽  
Case Detection ◽  
Muscle Action ◽  
Compound Muscle Action Potentials ◽  
Rate Of Decline ◽  
Lateral Sclerosis

Abstract Introduction Motor neuron loss is the primary pathologic feature of amyotrophic lateral sclerosis (ALS). An estimate of the number of surviving motor units (MUs) represents a direct measure of the disease state in ALS. The objective of the study is to compare MU number estimation (MUNE) using the multipoint stimulation method (MPS) and compound muscle action potentials (CMAP) amplitude in patients with ALS. Methods Twenty-eight patients with ALS with a disease duration of 3–48 months were studied. Nerve conduction study of the median, ulnar, tibial, common peroneal, and sural nerves were done. Besides, electromyography (EMG) of cranio-bulbar, cervical, thoracic and lumbosacral muscles, and MPS-MUNE. Results MUNE is decreased in patients with ALS. MUNE was positively correlated with CMAP amplitude, medical research council (MRC) scale, and ALS functional rating scale (ALS-FRS). On the contrary, MUNE was negatively correlated with MUAP duration. Case detection by the MUNE methods was high as compared to that of CMAP amplitude. Conclusions MUNE is highly specific and more sensitive than CMAP amplitude in detecting the neurophysiologic abnormalities in patients with ALS. Case detection by MUNE is three times more than CMAP amplitudes. The rate of decline of motor units using the MPS-MUNE is more sensitive than the MRC score and ALSFRS-R when expressed as the percentage change from baseline.

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.

Motor unit hyperexcitability in amyotrophic lateral sclerosis vs amino acids acting as neurotransmitters

2002 ◽  
Vol 106 (1) ◽  
pp. 34-38 ◽  
Author(s):  
A. Kostera-Pruszczyk ◽  
I. Niebroj-Dobosz ◽  
B. Emeryk-Szajewska ◽  
A. Karwańska ◽  
K. Rowińska-Marcińska
Keyword(s):  
Amino Acids ◽  
Amyotrophic Lateral Sclerosis ◽  
Motor Unit ◽  
Lateral Sclerosis

scholarly journals Plasma exchange with albumin replacement and disease progression in amyotrophic lateral sclerosis: a pilot study

2021 ◽  
Author(s):  
Mónica Povedano ◽  
Andrés Paipa ◽  
Miquel Barceló ◽  
Michael K. Woodward ◽  
Sandra Ortega ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Pilot Study ◽  
Disease Progression ◽  
Plasma Exchange ◽  
Rating Scale ◽  
Open Label ◽  
Primary Endpoints ◽  
Rate Of Decline ◽  
Post Hoc ◽  
Lateral Sclerosis

Abstract Background Plasma exchange (PE) is used to treat a range of neurological disorders. Based on results demonstrated in Alzheimer’s disease, we theorized that PE with albumin replacement (PE-A) might alter the metabolic profile of plasma and cerebrospinal fluid in patients with amyotrophic lateral sclerosis (ALS) by removing disease-inducing molecules. The aim of this study was to evaluate the effect of PE-A on disease progression in ALS. Methods In this open-label, non-controlled, single-arm, prospective pilot study, 13 adults with ALS had 6 months’ treatment with PE-A 5% and 6 months’ follow-up. Primary endpoints were changes from baseline in the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) score and forced vital capacity (FVC) through 48 weeks. A post hoc analysis compared individual patient data with the expected ALSFRS-R progression slope. Results The median ALSFRS-R score declined throughout the study, although the rate of decline was slower than expected in seven patients at treatment end and in five patients at study end. Six patients remained in the same baseline slope progression category, and four patients improved their slope category at treatment end. Median FVC decreased significantly during the study. Treatment was well tolerated. Of 330 PE-A procedures, 0.9% were associated with potentially related adverse events. Conclusion Although functional impairment progressed, about two-thirds of patients showed a slower than expected rate of decline at treatment end. Most patients had unaltered (54.5%) or reduced (36.4%) ALSFRS-R slope progression at treatment end. Further evaluation of PE-A in controlled studies involving more patients is warranted. EudraCT number 2013-004842-40. Trial registration ClinicalTrials.gov identifier: NCT02479802.

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