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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

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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, Fusun; Henderson, Robert; Ridall, Gareth et al.
In: Clinical Neurophysiology, Vol. 123, No. 10, 10.2012, p. 2092–2098.

Research output: Contribution to Journal/Magazine › Journal article › peer-review

Harvard

Baumann, F, Henderson, R, Ridall, G, Pettitt, A & McCombe, P 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, vol. 123, no. 10, pp. 2092–2098. https://doi.org/10.1016/j.clinph.2012.03.007

APA

Baumann, F., Henderson, R., Ridall, G., Pettitt, A., & McCombe, P. (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), 2092–2098. https://doi.org/10.1016/j.clinph.2012.03.007

Vancouver

Baumann F, Henderson R, Ridall G, Pettitt A, McCombe P. 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. 2012 Oct;123(10):2092–2098. doi: 10.1016/j.clinph.2012.03.007

Author

Baumann, Fusun ; Henderson, Robert ; Ridall, Gareth et al. / 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. In: Clinical Neurophysiology. 2012 ; Vol. 123, No. 10. pp. 2092–2098.

Bibtex

@article{78034aad68bf418ca9563db52ba59e7e,
title = "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",
abstract = "ObjectiveTo use our Bayesian method of motor unit number estimation (MUNE) to evaluate lower motor neuron degeneration in ALS.MethodsIn 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.ResultsThe 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.ConclusionsThe 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.SignificanceThis shows that the Bayesian MUNE method is useful in following the course and exploring the clinical features of ALS.",
keywords = "ALS, Mathematical models , Motor unit , MUNE",
author = "Fusun Baumann and Robert Henderson and Gareth Ridall and Anthony Pettitt and Pam McCombe",
year = "2012",
month = oct,
doi = "10.1016/j.clinph.2012.03.007",
language = "English",
volume = "123",
pages = "2092–2098",
journal = "Clinical Neurophysiology",
issn = "1388-2457",
publisher = "Elsevier Ireland Ltd",
number = "10",

}

RIS

TY - JOUR

T1 - Quantitative studies of lower motor neuron degeneration in amyotrophic lateral sclerosis

T2 - evidence for exponential decay of motor unit numbers and greatest rate of loss at the site of onset

AU - Baumann, Fusun

AU - Henderson, Robert

AU - Ridall, Gareth

AU - Pettitt, Anthony

AU - McCombe, Pam

PY - 2012/10

Y1 - 2012/10

N2 - ObjectiveTo use our Bayesian method of motor unit number estimation (MUNE) to evaluate lower motor neuron degeneration in ALS.MethodsIn 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.ResultsThe 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.ConclusionsThe 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.SignificanceThis shows that the Bayesian MUNE method is useful in following the course and exploring the clinical features of ALS.

AB - ObjectiveTo use our Bayesian method of motor unit number estimation (MUNE) to evaluate lower motor neuron degeneration in ALS.MethodsIn 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.ResultsThe 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.ConclusionsThe 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.SignificanceThis shows that the Bayesian MUNE method is useful in following the course and exploring the clinical features of ALS.

KW - ALS

KW - Mathematical models

KW - Motor unit

KW - MUNE

U2 - 10.1016/j.clinph.2012.03.007

DO - 10.1016/j.clinph.2012.03.007

M3 - Journal article

VL - 123

SP - 2092

EP - 2098

JO - Clinical Neurophysiology

JF - Clinical Neurophysiology

SN - 1388-2457

IS - 10

ER -