Tan, SV;
Z'Graggen, WJ;
Boërio, D;
Turner, C;
Hanna, MG;
Bostock, H;
(2016)
In vivo assessment of muscle membrane properties in myotonic dystrophy.
Muscle & Nerve
, 54
(2)
pp. 249-257.
10.1002/mus.25025.
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Abstract
INTRODUCTION: Myotonia in myotonic dystrophy types 1 (DM1) and 2 (DM2) is generally attributed to reduced chloride channel conductance. We used muscle velocity recovery cycles (MVRCs) to investigate muscle membrane properties in DM1 and DM2, with comparisons with myotonia congenita (MC). METHODS: MVRCs and responses to repetitive stimulation were compared between patients with DM1 (n=18), DM2 (n=5), MC (n=18), and normal controls (n=20). RESULTS: Both DM1 and DM2 showed enhanced late supernormality after multiple conditioning stimuli, indicating delayed repolarization as in MC. Contrary to MC, however, DM1 showed reduced early supernormality after multiple conditioning stimuli, and weak DM1 patients also showed abnormally slow latency recovery after repetitive stimulation. DISCUSSION: These findings support impaired chloride conductance in both DM1 and DM2. The early supernormality changes indicate that sodium currents were reduced in DM1, while the weakness-associated slow recovery after repetitive stimulation may provide an indication of reduced Na(+) /K(+) -ATPase activation.
| Type: | Article |
|---|---|
| Title: | In vivo assessment of muscle membrane properties in myotonic dystrophy |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1002/mus.25025 |
| Publisher version: | http://dx.doi.org/10.1002/mus.25025 |
| Language: | English |
| Additional information: | Copyright © 2015 Wiley Periodicals, Inc. All rights reserved. This is the pre-peer reviewed version of the following article: [Tan, S. V., Z'graggen, W. J., Boërio, D., Turner, C., Hanna, M. G. and Bostock, H. (2016), In vivo assessment of muscle membrane properties in myotonic dystrophy. Muscle Nerve. doi: 10.1002/mus.25025], which has been published in final form at http://dx.doi.org/10.1002/mus.25025. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Self-Archiving. |
| Keywords: | Channelopathy, Chloride channel, Excitability, Membrane potential, Myotonic dystrophy, Sodium channel, Sodium-potassium pump, Velocity recovery cycle |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases |
| URI: | https://discovery-pp.ucl.ac.uk/id/eprint/1482282 |
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