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Lambert-Eaton myasthenic syndrome in a young girl

Abstract

Lambert-Eaton Myasthenic Syndrome (LEMS) is a neuromuscular junction defect that results in weakness. It is rarely seen in children and most commonly is associated with small-cell lung cancer. Diagnosis of LEMS requires characteristic electromyogram (EMG) findings and the detection of antibodies directed against voltage-gated calcium channels. We report the case of an 8-year-old female child who presented with profound weakness found to have EMG evidence of a neuromuscular junction defect and detectable antivoltage-gated calcium channel antibodies. She received plasma exchange electrophoresis, intravenous immunoglobulin, oral steroids and amifampridine with some clinical improvement. We present a case of a child with a demonstrated autoimmune propensity and no evidence of malignancy, thereby adding to the 12 previously reported cases of LEMS in children.

  • neuromuscular disease
  • paediatrics

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