Quantitative studies of lower motor neuron degeneration in amyotrophic lateral sclerosis : Evidence for exponential decay of motor unit numbers and greatest rate of loss at the site of onset

Baumann, F., Henderson, R. D., Gareth Ridall, P., Pettitt, Anthony N., & McCombe, P. A. (2012) Quantitative studies of lower motor neuron degeneration in amyotrophic lateral sclerosis : Evidence for exponential decay of motor unit numbers and greatest rate of loss at the site of onset. Clinical Neurophysiology, 123(10), pp. 2092-2098.

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Objective: To use our Bayesian method of motor unit number estimation (MUNE) to evaluate lower motor neuron degeneration in ALS.

Methods: In subjects with ALS we performed serial MUNE studies. We examined the repeatability of the test and then determined whether the loss of MUs was fitted by an exponential or Weibull distribution.

Results: The decline in motor unit (MU) numbers was well-fitted by an exponential decay curve. We calculated the half life of MUs in the abductor digiti minimi (ADM), abductor pollicis brevis (APB) and/or extensor digitorum brevis (EDB) muscles. The mean half life of the MUs of ADM muscle was greater than those of the APB or EDB muscles. The half-life of MUs was less in the ADM muscle of subjects with upper limb than in those with lower limb onset.

Conclusions: The rate of loss of lower motor neurons in ALS is exponential, the motor units of the APB decay more quickly than those of the ADM muscle and the rate of loss of motor units is greater at the site of onset of disease.

Significance: This shows that the Bayesian MUNE method is useful in following the course and exploring the clinical features of ALS. 2012 International Federation of Clinical Neurophysiology.

Impact and interest:

13 citations in Scopus
13 citations in Web of Science®
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ID Code: 53850
Item Type: Journal Article
Refereed: Yes
Additional Information: Export Date: 26 September 2012
Source: Scopus
Language of Original Document: English
Correspondence Address: McCombe, P.A.; University of Queensland Centre for Clinical Research, Queensland, Herston Campus, 4029 QLD, Australia; email: pamela.mccombe@uq.edu.au
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Keywords: ALS, Mathematical models, Motor unit, MUNE
DOI: 10.1016/j.clinph.2012.03.007
ISSN: 1872-8952 (online) 1388-2457 (print)
Divisions: Current > QUT Faculties and Divisions > Science & Engineering Faculty
Copyright Owner: Copyright 2012 Elsevier Ireland Ltd. All rights reserved.
Deposited On: 27 Sep 2012 05:29
Last Modified: 16 Jul 2017 19:01

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