title: Treatment in Lambert-Eaton myasthenic syndrome.
abstract: Besides antitumor therapy for patients with the paraneoplastic form of Lambert-Eaton myasthenic syndrome (LEMS), the mainstay of symptomatic treatment in LEMS is 3,4-diaminopyridine (3,4-DAP). Data from four randomized, placebo-controlled trials have revealed that muscle strength scores increased significantly with 3,4-DAP. A limited meta-analysis performed on two trials using the Quantitative Myasthenia Gravis score indicated that the clinical benefits seen were modest. Meta-analysis of the mean change in compound muscle action potential amplitude following 3,4-DAP treatment revealed a significant improvement compared to placebo. However, most patients with noncancer LEMS require long-term immunosuppression, usually with prednisolone and azathioprine. A single crossover study has previously shown significant short-term benefit in limb strength following intravenous immunoglobulin, and there are isolated case reports of medium term benefit from rituximab. Overall, a combination of symptomatic treatment with 3,4-DAP and immunosuppression, with or without antitumor therapy, is often successful for most LEMS patients, with other more aggressive regimens rarely needed.
authors.affiliation: Department of Clinical Neurology, Queen's Medical Center, Nottingham, United Kingdom. paul.maddison@nhs.net
authors.forename: Paul
authors.initials: P
authors.lastname: Maddison
mesh_terms: D015761:4-Aminopyridine / Q000031:analogs & derivatives / Q000627:therapeutic use; D000077770:Amifampridine; D000970:Antineoplastic Agents / Q000627:therapeutic use; D019791:Guanidine / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D015624:Lambert-Eaton Myasthenic Syndrome / Q000188:drug therapy*; D010919:Placebos; D026902:Potassium Channel Blockers / Q000627:therapeutic use; D011729:Pyridostigmine Bromide / Q000627:therapeutic use; D016032:Randomized Controlled Trials as Topic
chemical_list: D000970:Antineoplastic Agents; D016756:Immunoglobulins, Intravenous; D010919:Placebos; D026902:Potassium Channel Blockers; D015761:4-Aminopyridine; D019791:Guanidine; D011729:Pyridostigmine Bromide; D000077770:Amifampridine