Gueguen, A;
Jardel, C;
Polivka, M;
Tan, SV;
Gray, F;
Vignal, C;
Lombes, A;
... Bostock, H; + view all
(2017)
Nerve excitability changes related to muscle weakness in chronic progressive external ophthalmoplegia.
Clinical Neurophysiology
, 128
(7)
pp. 1258-1263.
10.1016/j.clinph.2017.04.013.
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Abstract
Objective: To explore potential spreading to peripheral nerves of the mitochondrial dysfunction in chronic progressive external ophthalmoplegia (CPEO) by assessing axonal excitability. Methods: CPEO patients (n = 13) with large size deletion of mitochondrial DNA and matching healthy controls (n = 22) were included in a case-control study. Muscle strength was quantified using MRC sum-score and used to define two groups of patients: CPEO-weak and CPEO-normal (normal strength). Nerve excitability properties of median motor axons were assessed with the TROND protocol and changes interpreted with the aid of a model. Results: Alterations of nerve excitability strongly correlated with scores of muscle strength. CPEO-weak displayed abnormal nerve excitability compared to CPEO-normal and healthy controls, with increased superexcitability and responses to hyperpolarizing current. Modeling indicated that the CPEO-weak recordings were best explained by an increase in the ‘Barrett-Barrett’ conductance across the myelin sheath. Conclusion: CPEO patients with skeletal weakness presented sub-clinical nerve excitability changes, which were not consistent with axonal membrane depolarization, but suggested Schwann cell involvement. Significance: This study provides new insights into the spreading of large size deletion of mitochondrial DNA to Schwann cells in CPEO patients.
| Type: | Article |
|---|---|
| Title: | Nerve excitability changes related to muscle weakness in chronic progressive external ophthalmoplegia |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1016/j.clinph.2017.04.013 |
| Publisher version: | http://doi.org/10.1016/j.clinph.2017.04.013 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | CPEO, Mitochondrial disease, Nerve excitability, Myelin sheath, Barrett-Barrett conductance |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices 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 |
| URI: | https://discovery-pp.ucl.ac.uk/id/eprint/1558521 |
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