Journal
MUSCLE & NERVE
Volume 59, Issue 1, Pages 82-87Publisher
WILEY
DOI: 10.1002/mus.26304
Keywords
ALS Functional Rating Scale; amyotrophic lateral sclerosis; follow-up study; MScan, motor unit loss; MUNE, motor unit number estimation; MUNIX, multiple-point stimulation
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Funding
- Lundbeck Foundation
- Knud and Edith Eriksens Mindefond
- Verner Lipperts Fond
- Fonden til Laegevidenskabens Fremme
- Johanne Louis Hansens Fond
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Introduction The objective of this study was to evaluate a recently developed motor unit number estimation (MUNE) method, MScanFit MUNE (MScan), as a measure of disease progression in amyotrophic lateral sclerosis (ALS) compared with compound muscle action potential (CMAP) amplitude and 2 traditional MUNE methods. Methods ALS patients were evaluated clinically using the ALS Functional Rating Scale-Revised (ALSFRS-R). MScan, multiple-point stimulation MUNE (MPS), and motor unit number index (MUNIX) were performed in the abductor pollicis brevis (APB) muscle at baseline (27 patients), 4 months (23 patients), and 8 months (16 patients). Results Of the 5 measures, MScan registered the largest decline (8.7% per month), compared with MPS (3.4%), MUNIX (4.8%), CMAP amplitude (2.0%), and ALSFRS-R (1.9%). Only MScan and ALSFRS-R registered significant decrements over 4 and 8 months. Discussion MScan may be useful as a sensitive, objective tool for quantifying motor unit loss in ALS. Muscle Nerve 59:82-87, 2019
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