Skip to main content
Top
Published in: The Egyptian Journal of Neurology, Psychiatry and Neurosurgery 1/2021

Open Access 01-12-2021 | Amyotrophic Lateral Sclerosis | Research

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

Authors: Arwa Khalid Ibrahem, Akram Mohammed Al-Mahdawi, Farqad Bader Hamdan

Published in: The Egyptian Journal of Neurology, Psychiatry and Neurosurgery | Issue 1/2021

Login to get access

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.
Literature
10.
go back to reference Jacobsen AB, Bostock H, Tankisi H. CMAP scan MUNE (MScan) - a novel motor unit number estimation (MUNE) method. J Vis Exp. 2018;136:e56805. Jacobsen AB, Bostock H, Tankisi H. CMAP scan MUNE (MScan) - a novel motor unit number estimation (MUNE) method. J Vis Exp. 2018;136:e56805.
11.
go back to reference Onesti E, Gori MC, Ceccanti M, Tartaglia G, Petrucci A, Frasca V, et al. The compound muscle action potential as neurophysiological marker for amyotrophic lateral sclerosis. EC Neurol. 2016;3:509–19. Onesti E, Gori MC, Ceccanti M, Tartaglia G, Petrucci A, Frasca V, et al. The compound muscle action potential as neurophysiological marker for amyotrophic lateral sclerosis. EC Neurol. 2016;3:509–19.
13.
go back to reference Ludolph A, Drory V, Hardiman O, Nakano I, Ravits J, Robberecht W, et al. A revision of the El Escorial criteria – 2015. Amyotroph Lateral Scler Frontotemporal Degener. 2015;16(5-6):291–2.CrossRef Ludolph A, Drory V, Hardiman O, Nakano I, Ravits J, Robberecht W, et al. A revision of the El Escorial criteria – 2015. Amyotroph Lateral Scler Frontotemporal Degener. 2015;16(5-6):291–2.CrossRef
15.
go back to reference Ciesla N, Dinglas V, Fan E, Kho M, Kuramoto J, Needham D. Manual muscle testing: a method of measuring extremity muscle strength applied to critically ill patients. J Vis Exp. 2011; (50): pii 3632. Ciesla N, Dinglas V, Fan E, Kho M, Kuramoto J, Needham D. Manual muscle testing: a method of measuring extremity muscle strength applied to critically ill patients. J Vis Exp. 2011; (50): pii 3632.
16.
go back to reference Kimura J. Electrodiagnosis in diseases of nerve and muscle. In: Principles and Practice. 4th (ed.) ed. New York: Oxford University Press; 2013. Kimura J. Electrodiagnosis in diseases of nerve and muscle. In: Principles and Practice. 4th (ed.) ed. New York: Oxford University Press; 2013.
20.
go back to reference Neuwirth C, Barkhaus PE, Burkhardt C, Castro J, Czell D, de Carvalho M, et al. Motor Unit Number Index (MUNIX) detects motor neuron loss in pre-symptomatic muscles in Amyotrophic Lateral Sclerosis. Clin Neurophysiol. 2017;128(3):495–500.CrossRef Neuwirth C, Barkhaus PE, Burkhardt C, Castro J, Czell D, de Carvalho M, et al. Motor Unit Number Index (MUNIX) detects motor neuron loss in pre-symptomatic muscles in Amyotrophic Lateral Sclerosis. Clin Neurophysiol. 2017;128(3):495–500.CrossRef
30.
go back to reference Boekestein WA, Schelhaas HJ, van Putten MJ, Stegeman DF, Zwarts MJ, Van Dijk JP. Motor unit number index (MUNIX) versus motor unit number estimation (MUNE): a direct comparison in a longitudinal study of ALS patients. Clin Neurophysiol. 2012;123(8):1644–9.CrossRef Boekestein WA, Schelhaas HJ, van Putten MJ, Stegeman DF, Zwarts MJ, Van Dijk JP. Motor unit number index (MUNIX) versus motor unit number estimation (MUNE): a direct comparison in a longitudinal study of ALS patients. Clin Neurophysiol. 2012;123(8):1644–9.CrossRef
Metadata
Title
Motor unit number estimation versus compound muscle action potential in the evaluation of motor unit loss in amyotrophic lateral sclerosis
Authors
Arwa Khalid Ibrahem
Akram Mohammed Al-Mahdawi
Farqad Bader Hamdan
Publication date
01-12-2021
Publisher
Springer Berlin Heidelberg
DOI
https://doi.org/10.1186/s41983-021-00301-8

Other articles of this Issue 1/2021

The Egyptian Journal of Neurology, Psychiatry and Neurosurgery 1/2021 Go to the issue