These two authors contributed equally to this paper.
EFNS/ENS GUIDELINES/CME Article
EFNS/ENS Guidelines for the treatment of ocular myasthenia
Article first published online: 28 JAN 2014
© 2014 The Author(s) European Journal of Neurology © 2014 EFNS
European Journal of Neurology
Volume 21, Issue 5, pages 687–693, May 2014
How to Cite
Kerty, E., Elsais, A., Argov, Z., Evoli, A. and Gilhus, N. E. (2014), EFNS/ENS Guidelines for the treatment of ocular myasthenia. European Journal of Neurology, 21: 687–693. doi: 10.1111/ene.12359
This is a Continuing Medical Education article, and can be found with corresponding questions on the Internet at http://www.efns.org/EFNS Continuing-Medical-Education-online.301.0.html. Certificates for correctly answering the questions will be issued by the EFNS.
- Issue published online: 9 APR 2014
- Article first published online: 28 JAN 2014
- Manuscript Accepted: 17 DEC 2013
- Manuscript Received: 21 NOV 2013
- myasthenia gravis;
- ocular myasthenia;
Background and purpose
The symptoms of acquired autoimmune ocular myasthenia are restricted to the extrinsic eye muscles, causing double vision and drooping eyelids. These guidelines are designed to provide advice about best clinical practice based on the current state of clinical and scientific knowledge and the consensus of an expert panel.
Evidence for these guidelines was collected by searches in the MEDLINE and Cochrane databases. The task force working group reviewed evidence from original articles and systematic reviews. The evidence was classified (I, II, III, IV) and consensus recommendation graded (A, B or C) according to the EFNS guidance. Where there was a lack of evidence but clear consensus, good practice points are provided.
The treatment of ocular myasthenia should initially be started with pyridostigmine (good practice point). If this is not successful in relieving symptoms, oral corticosteroids should be used on an alternate-day regimen (recommendation level C). If steroid treatment does not result in good control of the symptoms or if it is necessary to use high steroid doses, steroid-sparing treatment with azathioprine should be started (recommendation level C). If ocular myasthenia gravis is associated with thymoma, thymectomy is indicated. Otherwise, the role of thymectomy in ocular myasthenia is controversial. Steroids and thymectomy may modify the course of ocular myasthenia and prevent myasthenia gravis generalization (good practice point).