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Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome

Nicolle, Michael W. MD

doi: 10.1212/CON.0000000000000415
Review Articles

ABSTRACT: Purpose of Review: This article discusses the pathogenesis, diagnosis, and management of autoimmune myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS).

Recent Findings: Recognition of new antigenic targets and improved diagnostic methods promise to improve the diagnosis of MG, although the clinical phenotypes associated with newer antibodies have not yet been defined. Future therapies might specifically target the aberrant immune response. The apparent increase in the prevalence of MG is not fully explained. Results of a long-awaited trial of thymectomy support the practice of performing a thymectomy under specific conditions.

Summary: The current treatment options are so effective in most patients with MG or LEMS that in patients with refractory disease the diagnosis should be reconsidered. The management of MG is individualized, and familiarity with mechanisms, adverse effects, and strategies to manage these commonly used treatments improves outcome. Patient education is important. LEMS, frequently associated with an underlying small cell lung cancer, is uncommon, and the mainstay of treatment is symptomatic in most patients.

Address correspondence to Dr Michael W. Nicolle, London Health Sciences Centre, University Hospital, 339 Windermere Rd, London, ON N6A 5A5, Canada, mnicolle@uwo.ca.

Relationship Disclosure: Dr Nicolle has given expert medical testimony in court cases for the Canadian Medical Protective Association.

Unlabeled Use of Products/Investigational Use Disclosure: Dr Nicolle discusses the unlabeled/investigational use of azathioprine, mycophenolate, and rituximab for the treatment of myasthenia gravis and Lambert-Eaton myasthenic syndrome and the use of 3,4-diaminopyridine for the treatment of Lambert-Eaton myasthenic syndrome.

© 2016 American Academy of Neurology