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

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Abstract

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.

Original languageEnglish
JournalMuscle & Nerve
Volume59
Issue1
Pages (from-to)82-87
Number of pages6
ISSN0148-639X
DOIs
Publication statusPublished - Jan 2019

Keywords

  • Aged
  • Disease Progression
  • Electromyography
  • Evoked Potentials, Motor/physiology
  • Female
  • Humans
  • Male
  • Middle Aged
  • Motor Neuron Disease/physiopathology
  • Motor Neurons/physiology
  • Muscle, Skeletal/physiopathology
  • Retrospective Studies

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