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.

View at publisher

Abstract

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:

12 citations in Scopus
Search Google Scholar™
12 citations in Web of Science®

Citation counts are sourced monthly from Scopus and Web of Science® citation databases.

These databases contain citations from different subsets of available publications and different time periods and thus the citation count from each is usually different. Some works are not in either database and no count is displayed. Scopus includes citations from articles published in 1996 onwards, and Web of Science® generally from 1980 onwards.

Citations counts from the Google Scholar™ indexing service can be viewed at the linked Google Scholar™ search.

ID Code: 53850
Item Type: Journal Article
Refereed: Yes
Additional Information: Export Date: 26 September 2012
Source: Scopus
CODEN: CNEUF
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
References: Ahmadi, M., Liu, J.X., Brannstrom, T., Andersen, P.M., Stal, P., Pedrosa-Domellof, F., Human extraocular muscles in ALS (2010) Invest Ophthalmol Vis Sci, 51, pp. 3494-3501; Ahn, S.W., Kim, S.H., Kim, J.E., Kim, S.M., Kim, S.H., Park, K.S., Reproducibility of the motor unit number index (MUNIX) in normal controls and amyotrophic lateral sclerosis patients (2010) Muscle Nerve, 42, pp. 808-813; Andres, P.L., Finison, L.J., Conlon, T., Thibodeau, L.M., Munsat, T.L., Use of composite scores (megascores) to measure deficit in amyotrophic lateral sclerosis (1988) Neurology, 38, pp. 405-408; Arasaki, K., Kato, Y., Hyodo, A., Ushijima, R., Tamaki, M., Longitudinal study of functional spinal alpha motor neuron loss in amyotrophic lateral sclerosis (2002) Muscle Nerve, 25, pp. 520-526; Armon, C., Brandstater, M.E., Motor unit number estimate-based rates of progression of ALS predict patient survival (1999) Muscle Nerve, 22, pp. 1571-1575; Bae, J.S., Sawai, S., Misawa, S., Kanai, K., Isose, S., Kuwabara, S., Differences in excitability properties of FDI and ADM motor axons (2009) Muscle Nerve, 39, pp. 350-354; Baumann, F., Henderson, R.D., Tremayne, F., Hutchinson, N., McCombe, P.A., Effects of prolonged repetitive stimulation of median, ulnar and peroneal nerves (2010) Muscle Nerve, 41, pp. 785-793; Baumann, F., Rose, S.E., Nicholson, G.A., Hutchinson, N., Pannek, K., Pettitt, A., Biomarkers of disease in a case of familial lower motor neuron ALS (2010) Amyotroph Lateral Scler, 11, pp. 486-489; Bland, J.M., Altman, D.G., Statistical methods for assessing agreement between two methods of clinical measurement (1986) Lancet, 1, pp. 307-310; Blok, J.H., Ruitenberg, A., Maathuis, E.M., Visser, G.H., The electrophysiological muscle scan (2007) Muscle Nerve, 36, pp. 436-446; Bromberg, M., Consensus (2003) Motor unit number estimation (MUNE), pp. 335-338. , Elsevier, Amsterdam, M.B. Bromberg (Ed.); Brooks, B.R., Miller, R.G., Swash, M., Munsat, T.L., El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis (2000) Amyotroph Lateral Scler Other Motor Neuron Disord, 1, pp. 293-299; Clarke, G., Collins, R.A., Leavitt, B.R., Andrews, D.F., Hayden, M.R., Lumsden, C.J., A one-hit model of cell death in inherited neuronal degenerations (2000) Nature, 406, pp. 195-199; Cox, D., Oaks, D., (1984) Analysis of survival data, , Chapman & Hall, London; Dadon-Nachum, M., Melamed, E., Offen, D., The "dying-back" phenomenon of motor neurons in ALS (2011) J Mol Neurosci, 43, pp. 470-477; Dantes, M., McComas, A., The extent and time course of motoneuron involvement in amyotrophic lateral sclerosis (1991) Muscle Nerve, 14, pp. 416-421; Dengler, R., Konstanzer, A., Kuther, G., Hesse, S., Wolf, W., Struppler, A., Amyotrophic lateral sclerosis: macro-EMG and twitch forces of single motor units (1990) Muscle Nerve, 13, pp. 545-550; Eisen, A., Kim, S., Pant, B., Amyotrophic lateral sclerosis (ALS): a phylogenetic disease of the corticomotoneuron? (1992) Muscle Nerve, 15, pp. 219-224; Frey, D., Schneider, C., Xu, L., Borg, J., Spooren, W., Caroni, P., Early and selective loss of neuromuscular synapse subtypes with low sprouting competence in motoneuron diseases (2000) J Neurosci, 20, pp. 2534-2542; Hegedus, J., Putman, C.T., Gordon, T., Time course of preferential motor unit loss in the SOD1 G93A mouse model of amyotrophic lateral sclerosis (2007) Neurobiol Dis, 28, pp. 154-164; Hegedus, J., Putman, C.T., Tyreman, N., Gordon, T., Preferential motor unit loss in the SOD1 G93A transgenic mouse model of amyotrophic lateral sclerosis (2008) J Physiol, 586, pp. 3337-3351; Henderson, R.D., Daube, J.R., Decrement in surface-recorded motor unit potentials in amyotrophic lateral sclerosis (2004) Neurology, 63, pp. 1670-1674; Henderson, R.D., Ridall, P.G., Pettitt, A.N., McCombe, P.A., Daube, J.R., The stimulus-response curve and motor unit variability in normal subjects and subjects with amyotrophic lateral sclerosis (2006) Muscle Nerve, 34, pp. 34-43; Henderson, R.D., Ridall, P.G., Hutchinson, N.M., Pettitt, A.N., McCombe, P.A., Bayesian statistical MUNE method (2007) Muscle Nerve, 36, pp. 206-213; Kuether, G., Lipinski, H.G., The dynamics of motor neuron degeneration in motor neuron disease. A theoretical approach (1991) Handbook of amyotrophic lateral sclerosis, pp. 391-432. , Marcel Dekker, New York, R.A. Smith (Ed.); Kuwabara, S., Mizobuchi, K., Ogawara, K., Hattori, T., Dissociated small hand muscle involvement in amyotrophic lateral sclerosis detected by motor unit number estimates (1999) Muscle Nerve, 22, pp. 870-873; McCombe, P.A., Henderson, R.D., Ridall, P.G., Pettitt, A.N., Biological basis for motor unit number estimation through Bayesian statistical analysis of the stimulus-response curve (2009) Suppl Clin Neurophysiol, 60, pp. 39-45; Miller, J., Arrasate, M., Shaby, B.A., Mitra, S., Masliah, E., Finkbeiner, S., Quantitative relationships between huntingtin levels, polyglutamine length, inclusion body formation, and neuronal death provide novel insight into huntington's disease molecular pathogenesis (2010) J Neurosci, 30, pp. 10541-10550; Munsat, T.L., Andres, P.L., Finison, L., Conlon, T., Thibodeau, L., The natural history of motoneuron loss in amyotrophic lateral sclerosis (1988) Neurology, 38, pp. 409-413; Nandedkar, S.D., Barkhaus, P.E., Stalberg, E.V., Motor unit number index (MUNIX): principle, method, and findings in healthy subjects and in patients with motor neuron disease (2010) Muscle Nerve, 42, pp. 798-807; Ngo, S., Baumann, F., Ridall, P.G., Pettitt, A., Henderson, R., Bellingham, M., The relationship between Bayesian motor unit number estimation and histological measurements of motor neurons in wild-type and SOD1G93A mice (2012) Clin Neurophysiol; Polgar, J., Johnson, M.A., Weightman, D., Appleton, D., Data on fibre size in thirty-six human muscles. An autopsy study (1973) J Neurol Sci, 19, pp. 307-318; Pun, S., Santos, A.F., Saxena, S., Xu, L., Caroni, P., Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF (2006) Nat Neurosci, 9, pp. 408-419; Ravits, J., Laurie, P., Fan, Y., Moore, D.H., Implications of ALS focality: rostral-caudal distribution of lower motor neuron loss postmortem (2007) Neurology, 68, pp. 1576-1582; Ridall, P.G., Pettitt, A.N., Friel, N., McCombe, P.A., Henderson, R.D., Motor unit number estimation using reversible jump Markov chain Monte Carlo (2007) J Roy Stat Soc C-App, 56, pp. 235-269; Shefner, J.M., Cudkowicz, M.E., Brown, R.H., Comparison of incremental with multipoint MUNE methods in transgenic ALS mice (2002) Muscle Nerve, 25, pp. 39-42; Shefner, J.M., Cudkowicz, M.E., Zhang, H., Schoenfeld, D., Jillapalli, D., The use of statistical MUNE in a multicenter clinical trial (2004) Muscle Nerve, 30, pp. 463-469; Shefner, J.M., Gooch, C.L., Motor unit number estimation in neurologic disease (2002) Adv Neurol, 88, pp. 33-52; Shefner, J.M., Reaume, A.G., Flood, D.G., Scott, R.W., Kowall, N.W., Ferrante, R.J., Mice lacking cytosolic copper/zinc superoxide dismutase display a distinctive motor axonopathy (1999) Neurology, 53, pp. 1239-1246; Van den Berg-Vos, R., Visser, J., Kalmijn, S., Fischer, K., de Visser, M., de Jong, V., A long-term prospective study of the natural course of sporadic adult-onset lower motor neuron syndromes (2009) Arch Neurol, 66, pp. 751-757; van Dijk, J.P., Schelhaas, H.J., Van Schaik, I.N., Janssen, H.M., Stegeman, D.F., Zwarts, M.J., Monitoring disease progression using high-density motor unit number estimation in amyotrophic lateral sclerosis (2010) Muscle Nerve, 42, pp. 239-244; Weber, M., Eisen, A., Stewart, H., Hirota, N., The split hand in ALS has a cortical basis (2000) J Neurol Sci, 180, pp. 66-70; Wilbourn, A.J., The "split hand syndrome" (2000) Muscle Nerve, 23, p. 138; Yamanaka, K., Chun, S.J., Boillee, S., Fujimori-Tonou, N., Yamashita, H., Gutmann, D.H., Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis (2008) Nat Neurosci, 11, pp. 251-253
Keywords: ALS, Mathematical models, Motor unit, MUNE
DOI: 10.1016/j.clinph.2012.03.007
ISSN: 1872-8952 (online) 1388-2457 (print)
Divisions: Current > Schools > School of Mathematical Sciences
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: 03 Oct 2012 01:43

Export: EndNote | Dublin Core | BibTeX

Repository Staff Only: item control page