title: Lambert-Eaton myasthenic syndrome: from clinical characteristics to therapeutic strategies.
abstract: Lambert-Eaton myasthenic syndrome (LEMS) is a neuromuscular autoimmune disease that has served as a model for autoimmunity and tumour immunology. In LEMS, the characteristic muscle weakness is thought to be caused by pathogenic autoantibodies directed against voltage-gated calcium channels (VGCC) present on the presynaptic nerve terminal. Half of patients with LEMS have an associated tumour, small-cell lung carcinoma (SCLC), which also expresses functional VGCC. Knowledge of this association led to the discovery of a wide range of paraneoplastic and non-tumour-related neurological disorders of the peripheral and central nervous systems. Detailed clinical studies have improved our diagnostic skills and knowledge of the pathophysiological mechanisms and association of LEMS with SCLC, and have helped with the development of a protocol for early tumour detection.
authors.affiliation: Department of Neurology, Leiden University Medical Center, Netherlands. m.j.titulaer@lumc.nl
authors.forename: Maarten J
authors.initials: MJ
authors.lastname: Titulaer
authors.forename: Bethan
authors.initials: B
authors.lastname: Lang
authors.forename: Jan Jgm
authors.initials: JJ
authors.lastname: Verschuuren
mesh_terms: D015761:4-Aminopyridine / Q000031:analogs & derivatives / Q000627:therapeutic use; D000077770:Amifampridine; D001323:Autoantibodies / Q000276:immunology; D015220:Calcium Channels / Q000276:immunology; D018288:Carcinoma, Small Cell / Q000150:complications / Q000276:immunology; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D008175:Lung Neoplasms / Q000150:complications / Q000276:immunology
chemical_list: D001323:Autoantibodies; D015220:Calcium Channels; D015761:4-Aminopyridine; D000077770:Amifampridine