title: Lambert-Eaton myasthenic syndrome in childhood.
abstract: Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder of the neuromuscular junction. LEMS can be associated with a variety of neoplasms. Patients present with proximal muscle weakness and fatigability, often combined with areflexia. Only 5% of reported cases are children. We report a case of 11-year old boy with non-neoplastic Lambert-Eaton myasthenic syndrome. Repetitive nerve stimulation test showed 83% increment after maximal voluntary contraction, presence of antibodies against voltage-gated calcium channels confirmed the diagnosis. The boy responded well to immunosuppressive treatment with prednisone and azathioprine and remains cancer-free for 4 years.
authors.affiliation: Department of Neurology, Medical University of Warsaw, Poland. akostera@amwaw.edu.pl
authors.forename: A
authors.initials: A
authors.lastname: Kostera-Pruszczyk
authors.forename: B
authors.initials: B
authors.lastname: Ryniewicz
authors.forename: K
authors.initials: K
authors.lastname: Rowinska-Marcinska
authors.forename: M
authors.initials: M
authors.lastname: Dutkiewicz
authors.forename: A
authors.initials: A
authors.lastname: Kamińska
mesh_terms: D001323:Autoantibodies / Q000378:metabolism; D001379:Azathioprine / Q000627:therapeutic use; D020865:Calcium Channels, P-Type / Q000276:immunology*; D002648:Child; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015624:Lambert-Eaton Myasthenic Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology* / Q000503:physiopathology; D008297:Male; D018908:Muscle Weakness / Q000209:etiology / Q000503:physiopathology; D011241:Prednisone / Q000627:therapeutic use; D011863:Radioimmunoassay
chemical_list: D001323:Autoantibodies; D020865:Calcium Channels, P-Type; D007166:Immunosuppressive Agents; D001379:Azathioprine; D011241:Prednisone