Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome

Continuum (Minneap Minn). 2016 Dec;22(6, Muscle and Neuromuscular Junction Disorders):1978-2005. doi: 10.1212/CON.0000000000000415.

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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adrenal Cortex Hormones / administration & dosage
  • Diagnosis, Differential
  • Female
  • Humans
  • Immunoglobulins, Intravenous / administration & dosage
  • Lambert-Eaton Myasthenic Syndrome / diagnosis*
  • Lambert-Eaton Myasthenic Syndrome / physiopathology*
  • Lambert-Eaton Myasthenic Syndrome / therapy
  • Middle Aged
  • Myasthenia Gravis / diagnosis*
  • Myasthenia Gravis / physiopathology*
  • Myasthenia Gravis / therapy

Substances

  • Adrenal Cortex Hormones
  • Immunoglobulins, Intravenous