Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome

Donald B. Sanders, MD, FAAN; Jeffrey T. Guptill, MD, MA, MHS Peripheral Nervous System Disorders p. 1413-1425 October 2014, Vol.20, No.5 doi: 10.1212/01.CON.0000455873.30438.9b
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Purpose of Review:: This article reviews the clinical presentations, diagnostic findings, and treatment options for autoimmune myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome.

Recent Findings:: Immunologic research is unraveling the immunopathology of MG and identifying targets for novel immune-based therapy of this condition. MG patients with antibodies to muscle-specific tyrosine kinase (MuSK) frequently present with symptoms and clinical findings that suggest nerve or muscle disease.

Summary:: Early diagnosis and treatment have a marked effect on outcome in these diseases. In most cases, the diagnosis of MG or Lambert-Eaton myasthenic syndrome can be made from the history, supplemented with directed questions, and a physical examination designed to demonstrate variable weakness in affected muscle groups. Appropriate confirmatory tests almost always establish the diagnosis. Although several novel treatment modalities for MG are under investigation, currently available therapies produce substantial improvement in function and quality of life in most patients with this condition. Knowledge about the dosing, adverse effects, and costs of immunomodulatory therapies is essential for the effective management of patients with MG and Lambert-Eaton myasthenic syndrome.

Address correspondence to Dr Donald B. Sanders, Box 3403, Duke University Medical Center, Durham, NC 27710, Donald.Sanders@Duke.edu.

Relationship Disclosure: Dr Sanders has served as a speaker for Athena Diagnostics, Inc; as a consultant for Accordant Health Services, GlaxoSmithKline, Jacobus Pharmaceutical Company, Inc, and UCB, Inc; and has participated in review activities with Alexion and Cytokinetics, Inc. Dr Guptill serves as a consultant for Grifols and UCB, Inc, and receives research support from the Myasthenia Gravis Foundation of America. Dr Guptill’s institution receives grants from Grifols and UCB, Inc.

Unlabeled Use of Products/Investigational Use Disclosure: Drs Sanders and Guptill discuss the unlabeled use of amifampridine and rituximab for the treatment of Lambert-Eaton myasthenic syndrome and the unlabeled use of azathioprine, cyclosporine A, mycophenolate mofetil, and rituximab for the treatment of myasthenia gravis.

Supplemental digital content: Videos accompanying this article are cited in the text as Supplemental Digital Content. Videos may be accessed by clicking on links provided in the HTML, PDF, and iPad versions of this article; the URLs are provided in the print version. Video legends begin on page 963.

© 2014 American Academy of Neurology