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[Lambert-Eaton myasthenic syndrome, an immune pathology of neuromuscular junctions].

Authors
  • Honnorat, Jérôme
Type
Published Article
Journal
Bulletin de l'Académie nationale de médecine
Publication Date
Feb 01, 2014
Volume
198
Issue
2
Identifiers
PMID: 26263702
Source
Medline
License
Unknown

Abstract

Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder affecting neuromuscular junctions. LEMS has served as a model of paraneoplastic neurological syndromes and antitumoral immunity, shedding light on the pathological role of autoantibodies directed against synaptic targets. Autoantibodies associated with LEMS are directed against voltage-gated calcium channels (VGCC) present on nerve terminals of neuromuscular synapses. Anti-VGGC antibodies play a direct pathological role in LEMS by blocking VGCC and calcium entry during depolarisation. Nearly half of patients with LEMS have small-cell lung cancer (SCLC), which also expresses VGCC. Diagnosis of LEMS frequently permits early detection and treatment of SCLC Knowledge of this syndrome has led to the discovery of a broad range of cancerous and non cancerous antibody-mediated neurological syndromes, and led to the concept of autoimmune synaptopathies.

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