Use of Bayesian MUNE to show differing rate of loss of motor units in subgroups of ALS

Baumann, F., Henderson, R.D., Ridall, G., Pettitt, A.N., & McCombe, P.A. (2012) Use of Bayesian MUNE to show differing rate of loss of motor units in subgroups of ALS. Clinical Neurophysiology, 123(12), pp. 2446-2453.

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To evaluate differences among patients with different clinical features of ALS, we used our Bayesian method of motor unit number estimation (MUNE).


We performed serial MUNE studies on 42 subjects who fulfilled the diagnostic criteria for ALS during the course of their illness. Subjects were classified into three subgroups according to whether they had typical ALS (with upper and lower motor neurone signs) or had predominantly upper motor neurone weakness with only minor LMN signs, or predominantly lower motor neurone weakness with only minor UMN signs. In all subjects we calculated the half life of MUs, defined as the expected time for the number of MUs to halve, in one or more of the abductor digiti minimi (ADM), abductor pollicis brevis (APB) and extensor digitorum brevis (EDB) muscles.


The mean half life of MUs was less in subjects who had typical ALS with both upper and lower motor neurone signs than in those with predominantly upper motor neurone weakness or predominantly lower motor neurone weakness. In 18 subjects we analysed the estimated size of the MUs and demonstrated the appearance of large MUs in subjects with upper or lower motor neurone predominant weakness. We found that the appearance of large MUs was correlated with the half life of MUs.


Patients with different clinical features of ALS have different rates of loss and different sizes of MUs. Significance: These findings could indicate differences in disease pathogenesis.

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5 citations in Scopus
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6 citations in Web of Science®

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ID Code: 50638
Item Type: Journal Article
Refereed: Yes
Additional Information: Export Date: 28 May 2012 Source: Scopus Article in Press CODEN: CNEUF Language of Original Document: English Correspondence Address: McCombe, P.A.; Department of Neurology, Royal Brisbane and Women's Hospital, Queensland, Australiaemail:
Keywords: ALS, Disease phenotypes, Exponential decay, Motor unit, MUNE
DOI: 10.1016/j.clinph.2012.04.022
ISSN: 1872-8952
Divisions: Current > Schools > School of Mathematical Sciences
Current > QUT Faculties and Divisions > Science & Engineering Faculty
Copyright Owner: Copyright 2012 International Federation of Clinical Neurophysiology and Elsevier Ireland Ltd.
Copyright Statement: This is the author’s version of a work that was accepted for publication in Clinical Neurophysiology. Changes resulting from the publishing process, such as peer review, editing, corrections, structural formatting, and other quality control mechanisms may not be reflected in this document. Changes may have been made to this work since it was submitted for publication. A definitive version was subsequently published in Clinical Neurophysiology, [VOL 123, ISSUE 12, (2012)] DOI: 10.1016/j.clinph.2012.04.022
Deposited On: 28 May 2012 22:41
Last Modified: 27 Nov 2013 03:39

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