open access

Vol 53, No 4 (2019)
Review Article
Submitted: 2018-12-05
Accepted: 2019-06-11
Published online: 2019-06-17
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Electrodiagnostics: MUNE and MUNIX as methods of estimating the number of motor units – biomarkers in lower motor neurone disease

Małgorzata Gaweł1
·
Pubmed: 31206166
·
Neurol Neurochir Pol 2019;53(4):251-257.
Affiliations
  1. Faculty of Medicine, Medical University of Warsaw, Poland

open access

Vol 53, No 4 (2019)
Review articles
Submitted: 2018-12-05
Accepted: 2019-06-11
Published online: 2019-06-17

Abstract

Routine quantitative electromyography is used for the assessment of the presence of lower motor neurone involvement and its consequences, including primary denervation and compensatory reinnervation of muscle fibres. However, it is not useful for the assessment of the motor unit number reserve. The need for a valid biomarker to evaluate lower motor neurone disease progression in such diseases as amyotrophic lateral sclerosis, and for use in clinical trials, has led to a number of studies of the methods that allow assessment of the number of motor units. In this review, motor unit number estimation (MUNE) methods with incremental stimulation and the recently developed motor unit number index (MUNIX) method, along with their technical and clinical aspects, are presented as methods which reflect motor unit loss in neurogenic processes. These electrodiagnostic tests may allow a valuable assessment of disease progression and the efficacy of new therapeutic methods in clinical trials in diseases with lower motor neurone degeneration.

Abstract

Routine quantitative electromyography is used for the assessment of the presence of lower motor neurone involvement and its consequences, including primary denervation and compensatory reinnervation of muscle fibres. However, it is not useful for the assessment of the motor unit number reserve. The need for a valid biomarker to evaluate lower motor neurone disease progression in such diseases as amyotrophic lateral sclerosis, and for use in clinical trials, has led to a number of studies of the methods that allow assessment of the number of motor units. In this review, motor unit number estimation (MUNE) methods with incremental stimulation and the recently developed motor unit number index (MUNIX) method, along with their technical and clinical aspects, are presented as methods which reflect motor unit loss in neurogenic processes. These electrodiagnostic tests may allow a valuable assessment of disease progression and the efficacy of new therapeutic methods in clinical trials in diseases with lower motor neurone degeneration.

Get Citation

Keywords

motor unit, motor unit number estimation, MUNE, motor unit number index, MUNIX, amyotrophic lateral sclerosis

About this article
Title

Electrodiagnostics: MUNE and MUNIX as methods of estimating the number of motor units – biomarkers in lower motor neurone disease

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 53, No 4 (2019)

Article type

Review Article

Pages

251-257

Published online

2019-06-17

Page views

1785

Article views/downloads

708

DOI

10.5603/PJNNS.a2019.0026

Pubmed

31206166

Bibliographic record

Neurol Neurochir Pol 2019;53(4):251-257.

Keywords

motor unit
motor unit number estimation
MUNE
motor unit number index
MUNIX
amyotrophic lateral sclerosis

Authors

Małgorzata Gaweł

References (30)
  1. Sherrington CS. Flexion-reflex of the limb, crossed extension-reflex, and reflex stepping and standing. J Physiol. 1910; 40(1-2): 28–121.
  2. McComas AJ, Fawcett PR, Campbell MJ, et al. Electrophysiological estimation of the number of motor units within a human muscle. J Neurol Neurosurg Psychiatry. 1971; 34(2): 121–131.
  3. Brown WF. A method for estimating the number of motor units in thenar muscles and the changes in motor unit count with ageing. J Neurol Neurosurg Psychiatry. 1972; 35(6): 845–852.
  4. Sica RE, McComas AJ, Upton AR, et al. Motor unit estimations in small muscles of the hand. J Neurol Neurosurg Psychiatry. 1974; 37(1): 55–67.
  5. Defaria CR, Toyonaga K. Motor unit estimation in a muscle supplied by the radial nerve. J Neurol Neurosurg Psychiatry. 1978; 41(9): 794–797.
  6. Shefner J, Gooch C. Motor unit number estimation. Physical Medicine and Rehabilitation Clinics of North America. 2003; 14(2): 243–260.
  7. Shefner JM, Cudkowicz ME, Zhang H, et al. Northeast ALS Consortium. The use of statistical MUNE in a multicenter clinical trial. Muscle Nerve. 2004; 30(4): 463–469.
  8. Cudkowicz ME, Shefner JM, Schoenfeld DA, et al. Trial of celecoxib in amyotrophic lateral sclerosis. Ann Neurol. 2006; 60(1): 22–31.
  9. Shefner JM, Watson ML, Simionescu L, et al. Multipoint incremental motor unit number estimation as an outcome measure in ALS. Neurology. 2011; 77(3): 235–241.
  10. Gawel M, Kostera-Pruszczyk A. Effect of age and gender on the number of motor units in healthy subjects estimated by the multipoint incremental MUNE method. J Clin Neurophysiol. 2014; 31(3): 272–278.
  11. Gawel M, Kostera-Pruszczyk A, Lusakowska A, et al. Motor unit loss estimation by the multipoint incremental MUNE method in children with spinal muscular atrophy--a preliminary study. Neuromuscul Disord. 2015; 25(3): 216–221.
  12. Shefner JM. Statistical motor unit number estimation and ALS trials: the effect of motor unit instability. Suppl Clin Neurophysiol. 2009; 60: 135–141.
  13. Vucic S. Motor Unit Number Index (MUNIX): A novel biomarker for ALS? Clin Neurophysiol. 2016; 127(4): 1938–1939.
  14. Nandedkar SD, Barkhaus PE, Stålberg EV. Motor unit number index (MUNIX): principle, method, and findings in healthy subjects and in patients with motor neuron disease. Muscle Nerve. 2010; 42(5): 798–807.
  15. Swash M. MUNIX in the clinic in ALS: MUNE comes of age. Clin Neurophysiol. 2017; 128(3): 482–483.
  16. Neuwirth C, Nandedkar S, Stålberg E, et al. Motor Unit Number Index (MUNIX): a novel neurophysiological marker for neuromuscular disorders; test-retest reliability in healthy volunteers. Clin Neurophysiol. 2011; 122(9): 1867–1872.
  17. Nandedkar SD, Nandedkar DS, Barkhaus PE, et al. Motor unit number index (MUNIX). IEEE Trans Biomed Eng. 2004; 51(12): 2209–2211.
  18. Nandedkar SD, Barkhaus PE, Stålberg EV, et al. Motor unit number index: Guidelines for recording signals and their analysis. Muscle Nerve. 2018; 58(3): 374–380.
  19. Gawel M, Kuzma-Kozakiewicz M. Does the MUNIX Method Reflect Clinical Dysfunction in Amyotrophic Lateral Sclerosis: A Practical Experience. Medicine (Baltimore). 2016; 95(19): e3647.
  20. Brooks BR, Miller RG, Swash M, et al. World Federation of Neurology Research Group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000; 1(5): 293–299.
  21. de Carvalho M, Dengler R, Eisen A, et al. Electrodiagnostic criteria for diagnosis of ALS. Clin Neurophysiol. 2008; 119(3): 497–503.
  22. Boekestein WA, Schelhaas HJ, van Putten MJ, et al. 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): 1644–1649.
  23. Fathi D, Mohammadi B, Dengler R, et al. Lower motor neuron involvement in ALS assessed by motor unit number index (MUNIX): Long-term changes and reproducibility. Clin Neurophysiol. 2016; 127(4): 1984–1988.
  24. Neuwirth C, Braun N, Claeys KG, et al. Implementing Motor Unit Number Index (MUNIX) in a large clinical trial: Real world experience from 27 centres. Clin Neurophysiol. 2018; 129(8): 1756–1762.
  25. Neuwirth C, Barkhaus P, Burkhardt C, et al. Motor Unit Number Index (MUNIX) detects motor neuron loss in pre-symptomatic muscles in Amyotrophic Lateral Sclerosis. Clinical Neurophysiology. 2017; 128(3): 495–500.
  26. Kuzma-Kozakiewicz M, Marchel A, Kaminska A, et al. Intraspinal Transplantation of the Adipose Tissue-Derived Regenerative Cells in Amyotrophic Lateral Sclerosis in Accordance with the Current Experts' Recommendations: Choosing Optimal Monitoring Tools. Stem Cells Int. 2018; 2018: 4392017.
  27. Günther R, Neuwirth C, Koch JC, et al. Motor Unit Number Index (MUNIX) of hand muscles is a disease biomarker for adult spinal muscular atrophy. Clin Neurophysiol. 2019; 130(2): 315–319.
  28. Delmont E, Benvenutto A, Grimaldi S, et al. Motor unit number index (MUNIX): Is it relevant in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)? Clin Neurophysiol. 2016; 127(3): 1891–1894.
  29. Philibert M, Grapperon AM, Delmont E, et al. Monitoring the short-term effect of intravenous immunoglobulins in multifocal motor neuropathy using motor unit number index. Clin Neurophysiol. 2017; 128(1): 235–240.
  30. Higashihara M, Menon P, van den Bos M, et al. Reproducibility of motor unit number index and multiple point stimulation motor unit number estimation in controls. Muscle Nerve. 2018; 58(5): 660–664.

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