Lambert-Eaton Myasthenic Syndrome (LES)

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Amifampridine, CAS# 54-96-6 is the first and only approved drug thus far for the symptomatic treatment of Lambert-Eaton Myasthenic Syndrome in adults. As a rare autoimmune disease, Lambert-Eaton Myasthenic Syndrome, also called LEMS, is typically caused by  auto antibodies  to voltage gated calcium channels, leading to a reduction in the amount of acetylcholine released from nerve terminals in patients. The predominant symptom of LEMS is muscle weakness, with this weakness usually concentrated in the proximal muscles of the legs and trunk. Additional, devastating symptoms of this rare disease include extreme fatigue, reduced reflexes, drooping of the eyelids, problems with swallowing,

Update on treatment options for Lambert–Eaton myasthenic syndrome: focus on use of amifampridine.   Published online 2011 May 30  Abstract The mainstay of treatment for LEMS is symptomatic but immunotherapy is needed in more severely affected patients. 3, thereby prolonging the action potential and increasing presynaptic calcium concentrations 4-DAP blocks presynaptic potassium channels. 4-DAP for increasing muscle strength and resting compound muscle action potentials has been demonstrated by four placebo-controlled trials the efficacy of 3. More than half of the affected patients have associated small cell lung cancer, and thorough screening for an underlying malignancy is crucial. 4-diaminopyridine (3, amifampridine) has been shown to be more effective 4-DAP, 3, while acetylcholinesterase inhibitors were the first drugs to be used for the amelioration of symptoms. The most common serious adverse events are epileptic seizures. 3, 4-DAP is currently the treatment of choice in p