Which medications are used in the treatment of Lambert-Eaton myasthenic syndrome (LEMS)?

Updated: May 23, 2019
  • Author: David E Stickler, MD; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Answer

Limited data from randomized controlled trials examining different interventions for LEMS are available. A Cochrane review identified only 4 controlled trials of 3,4-diaminopyridine (DAP) and a single cross-over study that examined the use of IV immunoglobulin (IVIg) and concluded that there was limited but moderate-to-high-quality evidence to suggest improved muscle strength with these interventions. [7] All other potential interventions have not been examined in controlled trials.

Recent studies have introduced a Ca2+ channel agonist (GV-58) as a potential therapeutic alternative for LEMS. In addition, in a mouse model, GV-58 and 3,4-DAP were shown to interact in a supra-additive manner to restore the magnitude of neurotransmitter release at the neuromuscular junctions. [2, 11]

The initial pharmacotherapy for LEMS is with agents that increase the transmission of acetylcholine (ACh) across the neuromuscular junction, either by increasing the release of ACh (eg, DAP [12] ) or by decreasing the action of acetylcholinesterase (eg, pyridostigmine). Treatment of the associated cancer may also decrease the weakness and other symptoms.


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