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Intra-rater reliability of motor unit number estimation and quantitative motor unit analysis in subjects with amyotrophic lateral sclerosis
Institution:1. Health and Rehabilitation Sciences, Western University, London, Ontario, Canada;2. Department of Clinical Neurological Sciences, Western University, London, Ontario, Canada;3. Department of Physical Medicine and Rehabilitation, Western University, London, Ontario, Canada;1. Division of Pediatric Ophthalmology, Cincinnati Children''s Hospital Medical Center, Cincinnati, Ohio;2. Department of Ophthalmology, University of Cincinnati College of Medicine, Cincinnati, Ohio;3. Division of Biostatistics and Epidemiology, Cincinnati Children''s Hospital Medical Center, Cincinnati, Ohio;4. Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children''s Hospital Medical Center, Ohio;1. Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran;2. Pediatrics Center of Excellence, Children''s Medical Center, Tehran University of Medical Sciences, Tehran, Iran;3. Research Center for Immunodeficiencies, Children''s Medical Center, Tehran University of Medical Sciences, Tehran, Iran;4. Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran;5. Molecular Immunology Research Center, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran;6. Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
Abstract:ObjectivesTo assess the intra-rater reliability of decomposition-enhanced spike-triggered averaging (DE-STA) motor unit number estimation (MUNE) and quantitative motor unit potential analysis in the upper trapezius (UT) and biceps brachii (BB) of subjects with amyotrophic lateral sclerosis (ALS) and to compare the results from the UT to control data.MethodsPatients diagnosed with clinically probable or definite ALS completed the experimental protocol twice with the same evaluator for the UT (n = 10) and BB (n = 9).ResultsIntra-rater reliability for the UT was good for the maximum compound muscle action potential (CMAP) (ICC = 0.88), mean surface-detected motor unit potential (S-MUP) (ICC = 0.87) and MUNE (ICC = 0.88), and for the BB was moderate for maximum CMAP (ICC = 0.61), and excellent for mean S-MUP (ICC = 0.94) and MUNE (ICC = 0.93). A significant difference between tests was found for UT MUNE. Comparing subjects with ALS to control subjects, UT maximum CMAP (p < 0.01) and MUNE (p < 0.001) values were significantly lower, and mean S-MUP values significantly greater (p < 0.05) in subjects with ALS.ConclusionsThis study has demonstrated the ability of the DE-STA MUNE technique to collect highly reliable data from two separate muscle groups and to detect the underlying pathophysiology of the disease.SignificanceThis was the first study to examine the reliability of this technique in subjects with ALS, and demonstrates its potential for future use as an outcome measure in ALS clinical trials and studies of ALS disease severity and natural history.
Keywords:Decomposition-based quantitative electromyography (DQEMG)  Decomposition-enhanced spike-triggered averaging (DE-STA)  Motor unit number estimation (MUNE)  Reliability  Trapezius muscle  Amyotrophic lateral sclerosis (ALS)
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