NEURO-OPHTHALMOLOGY: Edited by Heather E. MossOcular myasthenia gravis: updates on an elusive targetMelson, Andrew T.a; McClelland, Collin M.b; Lee, Michael S.bAuthor Information aDepartment of Ophthalmology, University of Oklahoma, Oklahoma City, Oklahoma bDepartment of Ophthalmology and Visual Neurosciences, University of Minnesota, Minneapolis, Minnesota, USA Correspondence to Michael S. Lee, Department of Ophthalmology & Visual Neurosciences, University of Minnesota, 420 Delaware St. SE, MMC 493, Minneapolis, MN 55455-0501, USA. Tel: +1 612 625 3553; fax: +1 612 626 3119; e-mail: [email protected] Current Opinion in Neurology: February 2020 - Volume 33 - Issue 1 - p 55-61 doi: 10.1097/WCO.0000000000000775 Buy Metrics Abstract Purpose of review Ocular myasthenia gravis (OMG) is a complex condition with heterogenous phenotypes and ill-defined diagnostic criteria. Understanding concomitant risk factors and autoimmune serology can help inform prognosis for generalization and guide treatment. Recent findings Although antibodies to acetylcholine receptors or muscle-specific kinase likely increase risk of generalization, they are less frequent in OMG. Patients without either antibody tend to have a milder disease process and often have variable antibodies to other end-plate proteins such as LRP4, agrin, or cortactin. The treatment of OMG begins with pyridostigmine and is supplemented by oral prednisone if treatment-resistant or high risk for generalization. Variable oral prednisone regimens have been used with success and further immunosuppression may be best achieved with mycophenolate mofetil and azathioprine. Checkpoint inhibitor-induced myasthenia gravis is increasingly recognized and likely has high rates of mortality associated with myocarditis. Summary Our understanding of OMG and its variable phenotypes continues to evolve. Autoantibody testing increasingly provides valuable diagnostic and prognostic information. Despite these improvements, a lack of quality treatment trials creates significant challenges for evidence-based management guidelines. Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.