Kostera-Pruszczyk A, Ryniewicz B, Rowinska-Marcinska K, Dutkiewicz M, Kamińska A
Lambert-Eaton myasthenic syndrome in childhood. [JOURNAL ARTICLE]
Eur J Paediatr Neurol 2008 Jun 26.
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.
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