Update on treatment options for Lambert-Eaton myasthenic syndrome: focus on use of amifampridine.
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Lindquist S, Stangel M
Update on treatment options for Lambert-Eaton myasthenic syndrome: focus on use of amifampridine.
Neuropsychiatr Dis Treat. 2011;7:341-9. doi: 10.2147/NDT.S10464. Epub 2011 May 30.
- PubMed ID
- 21822385 [ View in PubMed]
- Abstract
In Lambert-Eaton myasthenic syndrome (LEMS), antibodies against presynaptic voltage-gated calcium channels reduce the quantal release of acetylcholine, causing muscle weakness and autonomic dysfunction. More than half of the affected patients have associated small cell lung cancer, and thorough screening for an underlying malignancy is crucial. The mainstay of treatment for LEMS is symptomatic but immunotherapy is needed in more severely affected patients. Symptomatic therapies aim at increasing the concentration of acetylcholine at the muscle endplate. While acetylcholinesterase inhibitors were the first drugs to be used for the amelioration of symptoms, 3,4-diaminopyridine (3,4-DAP, amifampridine) has been shown to be more effective. 3,4-DAP blocks presynaptic potassium channels, thereby prolonging the action potential and increasing presynaptic calcium concentrations. This then results in increased quantal release of acetylcholine. The efficacy of 3,4-DAP for increasing muscle strength and resting compound muscle action potentials has been demonstrated by four placebo-controlled trials. Side effects are usually mild, and the most frequently reported are paresthesias. The most common serious adverse events are epileptic seizures. 3,4-DAP is currently the treatment of choice in patients with Lambert-Eaton myasthenic syndrome.
DrugBank Data that Cites this Article
- Drugs
- Drug Targets
Drug Target Kind Organism Pharmacological Action Actions Amifampridine Potassium voltage-gated channel subfamily A member 1 Protein Humans YesBlockerDetails - Drug Reactions
Reaction Details - Drug Interactions
Drugs Interaction Integrate drug-drug
interactions in your softwareAmifampridineCyclosporine The risk or severity of seizure can be increased when Cyclosporine is combined with Amifampridine. AmifampridineAmphetamine The risk or severity of seizure can be increased when Amphetamine is combined with Amifampridine. AmifampridineTramadol The risk or severity of seizure can be increased when Tramadol is combined with Amifampridine. AmifampridineEnflurane The risk or severity of seizure can be increased when Enflurane is combined with Amifampridine. AmifampridineTheophylline The risk or severity of seizure can be increased when Theophylline is combined with Amifampridine.