title: Available treatment options for the management of Lambert-Eaton myasthenic syndrome.
abstract: Lambert-Eaton myasthenic syndrome is a rare, but reasonably well-understood, antibody-mediated autoimmune disease that is caused by serum auto-antibodies and results in muscle weakness and autonomic dysfunction. One half of the patients have an idiopathic form, the other half a tumour-associated form of the disease. Three randomised trials and a large number of smaller clinical studies have resulted in a number of drugs becoming available for the treatment of Lambert-Eaton myasthenic syndrome. Several drugs are available for the symptomatic treatment of the disease, including guanidine, aminopyridines or acetylcholinesterase inhibitors. Other therapies aim to deplete the serum autoantibodies or to suppress the immune system. For this purpose, immunomodulating strategies, such as intravenous immunoglobulins or plasmapheresis, or several immunosuppressive agents are available. Chemotherapy has successfully ameliorated the course of disease in Lambert-Eaton myasthenic syndrome patients with an underlying tumour.
authors.affiliation: Leiden University Medical Centre, Department of Neurology, PO Box 9600, 2300 RC Leiden, Leiden, The Netherlands. j.j.g.m.verschuuren@lumc.nl
authors.forename: Jan J G M
authors.initials: JJ
authors.lastname: Verschuuren
authors.forename: Paul W
authors.initials: PW
authors.lastname: Wirtz
authors.forename: Maarten J
authors.initials: MJ
authors.lastname: Titulaer
authors.forename: Luuk N A
authors.initials: LN
authors.lastname: Willems
authors.forename: Joop
authors.initials: J
authors.lastname: van Gerven
mesh_terms: D015761:4-Aminopyridine / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D018288:Carcinoma, Small Cell / Q000150:complications / Q000188:drug therapy*; D002800:Cholinesterase Inhibitors / Q000627:therapeutic use*; D002945:Cisplatin / Q000627:therapeutic use; D005047:Etoposide / Q000627:therapeutic use; D019791:Guanidine / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007167:Immunotherapy / Q000379:methods; D015624:Lambert-Eaton Myasthenic Syndrome / Q000150:complications / Q000188:drug therapy* / Q000628:therapy; D008175:Lung Neoplasms / Q000150:complications / Q000188:drug therapy*; D010277:Parasympathomimetics / Q000627:therapeutic use*; D010956:Plasmapheresis; D026902:Potassium Channel Blockers / Q000627:therapeutic use*; D011239:Prednisolone / Q000627:therapeutic use; D011729:Pyridostigmine Bromide / Q000627:therapeutic use; D016032:Randomized Controlled Trials as Topic
chemical_list: D000970:Antineoplastic Agents; D002800:Cholinesterase Inhibitors; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D010277:Parasympathomimetics; D026902:Potassium Channel Blockers; D005047:Etoposide; D011239:Prednisolone; D015761:4-Aminopyridine; D019791:Guanidine; D011729:Pyridostigmine Bromide; D002945:Cisplatin