Journal
NEUROLOGIC CLINICS
Volume 36, Issue 2, Pages 379-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.ncl.2018.01.008
Keywords
Lambert-Eaton myasthenic syndrome; Neuromuscular transmission disorder; Paraneoplastic syndrome; P/Q-type voltage-gated calcium channels; 3,4-Diaminopyridine
Categories
Funding
- Alexion
- Biomarin
- Catalyst
- CSL Behring
- FDA/OPD
- GSK
- Grifols
- MDA
- NIH
- Novartis
- Orphazyme
- Sanofi
- TMA
- FDA/OOPD
- NINDS
- Sanofi/Genzyme
- IONIS
- Teva
- Cytokinetics
- Eli Lilly
- PCORI
- ALSA
- PTC
- CTSA grant from NCATS [UL1TR002366]
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Lambert-Eaton myasthenic syndrome is a paraneoplastic or primary autoimmune neuromuscular junction disorder characterized by proximal weakness, autonomic dysfunction and ariflexia. The characteristic symptoms are thought to be caused by antibodies generated against the P/Q-type voltage-gated calcium channels present on presynaptic nerve terminals and by diminished release of acetylcholine. More than half of Lambert-Eaton myasthenic syndrome cases are associated with small cell lung carcinoma. Diagnosis is confirmed by serologic testing and electrophysiologic studies. 3,4-diaminopyridine is effective symptomatic treatment of LEMS.
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