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. 2012 Dec 12;12(12):CD005081.
doi: 10.1002/14651858.CD005081.pub3.

Medical and surgical treatment for ocular myasthenia

Michael Benatar  1 , Henry Kaminski
Affiliations

Medical and surgical treatment for ocular myasthenia

Michael Benatar et al. Cochrane Database Syst Rev. .

Abstract

Background: Approximately 50% of people with myasthenia gravis present with purely ocular symptoms, so called ocular myasthenia. Of these between 50% to 60% develop generalized disease, most within two years. Their management is controversial. This is an update of a review first published in 2006 and previously updated in 2008 and 2010.

Objectives: To assess the effects of treatments for ocular myasthenia and to answer three specific questions. Are there any treatments that impact the progression from ocular to generalized disease? Are there any treatments that improve symptoms of diplopia or ptosis? What is the frequency of adverse effects associated with treatments used?

Search methods: In this updated review, we searched the Cochrane Neuromuscular Disease Group Specialized Register (3 August 2012), CENTRAL (2012, Issue 7), MEDLINE (January 1996 to July 2012) and EMBASE (January 1974 to July 2012) for randomized controlled trials (RCTs) as well as case-control and cohort studies. The titles and abstracts of all articles were read by both authors and the full texts of possibly relevant articles were reviewed. The references of all manuscripts included in the review were scanned to identify additional articles of relevance and experts in the field were contacted to identify additional published and unpublished data. Where necessary, we contacted authors for further information.

Selection criteria: Inclusion required meeting three criteria: (a) randomized (or quasi-randomized) controlled study design; (b) active treatment compared to placebo, no treatment or some other treatment; and (c) results reported separately for patients with ocular myasthenia (grade 1) as defined by the Myasthenia Gravis Foundation of America.

Data collection and analysis: We collected data regarding the risk of progression to generalized myasthenia gravis, improvement in ocular symptoms, and the frequency of treatment-related side effects.

Main results: In the original review, we identified two RCTs relevant to the treatment of ocular myasthenia, only one of which reported results in terms of the pre-specified outcome measures used in this review. This study included only three participants and was of limited methodological quality. There were no new RCTs in searches conducted for this or previous updates. In the absence of data from RCTs, we present a review of the available observational data.

Authors' conclusions: The available randomized controlled literature does not permit any meaningful conclusions about the efficacy of any form of treatment for ocular myasthenia. Data from several reasonably good quality observational studies suggest that corticosteroids and azathioprine may be beneficial in reducing the risk of progression to generalized myasthenia gravis.

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Conflict of interest statement

Dr Michael Benatar is the principal investigator on a RCT designed to compare the efficacy of prednisone plus pyridostigmine versus placebo plus pyridostigmine for the treatment of ocular myasthenia. He receives research funding from both the US National Institutes of Health (NIH) and the Food and Drug Administration for clinical trials in the field of MG.

Henry Kaminski is co‐investigator of the NIH‐sponsored trial of thymectomy for treatment of non‐thymomatous MG. He serves as consultant for Varleigh Limited, which is developing a drug for treatment of MG. He has served as a consultant for GlaxoSmithKline and serves on the Data and Safety Monitoring Board for a clinical trial sponsored by Cytokinetics. He receives royalties from Springer Publishing. He has performed legal consultations in cases related to MG.

Figures

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1
Methodological quality summary: review authors' judgements about each methodological quality item for each included study.
See this image and copyright information in PMC

Update of

References

References to studies included in this review

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Benatar 2016 {published data only}
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References to other published versions of this review

Benatar 2006
    1. Benatar M, Kaminski H. Medical and surgical treatment for ocular myasthenia. Cochrane Database of Systematic Reviews 2005, Issue 1. [DOI: 10.1002/14651858.CD005081] - DOI - PMC - PubMed
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