[Lambert-Eaton myasthenic syndrome, an immune pathology of neuromuscular junctions].
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.
Jérôme
J
Honnorat
D000818:Animals; D001323:Autoantibodies / Q000502:physiology*; D001327:Autoimmune Diseases / Q000276:immunology; D003937:Diagnosis, Differential; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D009469:Neuromuscular Junction / Q000276:immunology*; D020361:Paraneoplastic Syndromes, Nervous System / Q000145:classification
D001323:Autoantibodies