4.1 Article

Lambert-Eaton Myasthenic Syndrome

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

Funding

  1. Alexion
  2. Biomarin
  3. Catalyst
  4. CSL Behring
  5. FDA/OPD
  6. GSK
  7. Grifols
  8. MDA
  9. NIH
  10. Novartis
  11. Orphazyme
  12. Sanofi
  13. TMA
  14. FDA/OOPD
  15. NINDS
  16. Sanofi/Genzyme
  17. IONIS
  18. Teva
  19. Cytokinetics
  20. Eli Lilly
  21. PCORI
  22. ALSA
  23. PTC
  24. 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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