Lambert-Eaton Myasthenic Syndrome

Abstract

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.

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