Lambert-Eaton Myasthenic Syndrome - 13/04/18

Doi : 10.1016/j.ncl.2018.01.008

Vita G. Kesner, MD, PhD ^a,^⁎ , Shin J. Oh, MD ^b, Mazen M. Dimachkie, MD ^c, Richard J. Barohn, MD ^c
^a Neurology Department, 12 Executive Park Drive NE, Atlanta, GA 30329, USA
^b University of Alabama at Birmingham, Department of Neurology, SC 350, 1720 2nd Ave South, Birmingham, AL 35294, USA
^c Department of Neurology, University of Kansas Medical Center, 3901 Rainbow Boulevard, Mail Stop 2012, Kansas City, KS 66160, USA

^∗Corresponding author.

Résumé

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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Keywords : Lambert-Eaton myasthenic syndrome, Neuromuscular transmission disorder, Paraneoplastic syndrome, P/Q-type voltage-gated calcium channels, 3,4-Diaminopyridine

Plan

Tumor association and immunopathophysiology

Clinical features

Diagnosis

Voltage-Gated Calcium Channels and SOX1 Antibodies

Electrodiagnostic Studies

Screening for small cell lung cancer carcinoma

Treatment

Summary

Disclosures: Dr. M.M. Dimachkie is on the speaker’s bureau or is a consultant for Alnylam, Baxalta, Catalyst, CSLBehring, Mallinckrodt, Novartis, NuFactor, and Terumo. He has also received grants from Alexion, Biomarin, Catalyst, CSL Behring, FDA/OPD, GSK, Grifols, MDA, NIH, Novartis, Orphazyme, Sanofi, and TMA. Dr R.J. Barohn is a consultant for NuFactor and is on the advisory board for Novartis. He has received an honorarium from Option Care and PlatformQ Health Education. He has received research grants from NIH, FDA/OOPD, NINDS, Novartis, Sanofi/Genzyme, Biomarin, IONIS, Teva, Cytokinetics, Eli Lilly, PCORI, ALSA, and PTC. This work was supported by a CTSA grant from NCATS awarded to the University of Kansas for Frontiers: University of Kansas Clinical and Translational Science Institute (# UL1TR002366) The contents are solely the responsibility of the authors and do not necessarily represent the official views of the NIH or NCATS.