3,4-diaminopyridine for the treatment of Lambert-Eaton myasthenic syndrome

Author(s):
Wirtz, P.W.; Titulaer, M.J.; Gerven, J.M.A. van (0000-0002-1444-7415); Verschuuren, J.J.
Published
2010
Publication type
Article / Letter to editor

Abstract

The Lambert Eaton myasthenic syndrome (LEMS) is an autoimmune disease in which antibodies against voltage-gated calcium channels inhibit cholinergic neurotransmission LEMS is clinically characterized by muscle weakness and autonomic dysfunction 3 4-diaminopyridine (3 4-DAP) blocks potassium channels in nerve terminals resulting in an increase in acetylcholine release This article describes the four randomized placebo-controlled trials of 3 4-DAP in patients with LEMS All trials demonstrated a significant effect on muscle strength and compound muscle action potential amplitude Furthermore the safety and tolerability of 3 4-DAP are reviewed The side effects of 3 4-DAP are generally mild and most frequently consist of paresthesias but epileptic seizures and arrhythmias have been described in patients using high doses Given the efficacy and safety of 3 4-DAP in LEMS, this drug is the mainstay for symptomatic treatment of LEMS

3 4 diaminopyridine clinical trials Lambert-Eaton myasthenic syndrome neuromuscular diseases cell lung-carcinoma neuromuscular-transmission double-blind transmitter release multiple-sclerosis syndrome lems 4-aminopyridine cancer placebo trial
Date issued
2010-11-30
Language
English
Is part of
Expert Review of Clinical Immunology (2010), Vol. 6, no. 6, p. 867-874
hdl
1887/100743
doi
10.1586/eci.10.57