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Autoimmune Diseases
Volume 2011 (2011), Article ID 973808, 5 pages
http://dx.doi.org/10.4061/2011/973808
Review Article

Lambert-Eaton Myasthenic Syndrome; Pathogenesis, Diagnosis, and Therapy

1Department of Clinical Medicine, University of Bergen, 5020 Bergen, Norway
2Department of Neurology, Haukeland University Hospital, 5021 Bergen, Norway

Received 26 May 2011; Accepted 4 August 2011

Academic Editor: Johan A. Aarli

Copyright © 2011 Nils Erik Gilhus. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare disease with a well-characterized pathogenesis. In 50% of the patients, LEMS is a paraneoplastic manifestation and caused by a small cell lung carcinoma (SCLC). Both LEMS patients with SCLC and those without this tumour have in 85% of cases pathogenetic antibodies of very high LEMS specificity against voltage-gated calcium channels (VGCCs) in the cell membrane of the presynaptic motor nerve terminal. Better understanding of LEMS pathogenesis has lead to targeted symptomatic therapy aimed at the neuromuscular junction and to semispecific immuno-suppression. For SCLC LEMS, tumour therapy is essential.