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

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 pathogen...

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Autor principal: Gilhus, Nils Erik
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE-Hindawi Access to Research 2011
Materias:
Review Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3182560/
https://www.ncbi.nlm.nih.gov/pubmed/21969911
http://dx.doi.org/10.4061/2011/973808
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author Gilhus, Nils Erik
author_facet Gilhus, Nils Erik
author_sort Gilhus, Nils Erik
collection PubMed
description 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.
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spelling pubmed-31825602011-10-03 Lambert-Eaton Myasthenic Syndrome; Pathogenesis, Diagnosis, and Therapy Gilhus, Nils Erik Autoimmune Dis Review Article 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. SAGE-Hindawi Access to Research 2011 2011-09-29 /pmc/articles/PMC3182560/ /pubmed/21969911 http://dx.doi.org/10.4061/2011/973808 Text en Copyright © 2011 Nils Erik Gilhus. https://creativecommons.org/licenses/by/3.0/ 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.
spellingShingle Review Article
Gilhus, Nils Erik
Lambert-Eaton Myasthenic Syndrome; Pathogenesis, Diagnosis, and Therapy
title Lambert-Eaton Myasthenic Syndrome; Pathogenesis, Diagnosis, and Therapy
title_full Lambert-Eaton Myasthenic Syndrome; Pathogenesis, Diagnosis, and Therapy
title_fullStr Lambert-Eaton Myasthenic Syndrome; Pathogenesis, Diagnosis, and Therapy
title_full_unstemmed Lambert-Eaton Myasthenic Syndrome; Pathogenesis, Diagnosis, and Therapy
title_short Lambert-Eaton Myasthenic Syndrome; Pathogenesis, Diagnosis, and Therapy
title_sort lambert-eaton myasthenic syndrome; pathogenesis, diagnosis, and therapy
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3182560/
https://www.ncbi.nlm.nih.gov/pubmed/21969911
http://dx.doi.org/10.4061/2011/973808
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