Lambert-Eaton myasthenic syndrome in childhood

Eur J Paediatr Neurol. 2009 Mar;13(2):194-6. doi: 10.1016/j.ejpn.2008.03.006. Epub 2008 Jun 30.


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.

Publication types

  • Case Reports

MeSH terms

  • Autoantibodies / metabolism
  • Azathioprine / therapeutic use
  • Calcium Channels, P-Type / immunology*
  • Child
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lambert-Eaton Myasthenic Syndrome / diagnosis*
  • Lambert-Eaton Myasthenic Syndrome / drug therapy
  • Lambert-Eaton Myasthenic Syndrome / immunology*
  • Lambert-Eaton Myasthenic Syndrome / physiopathology
  • Male
  • Muscle Weakness / etiology
  • Muscle Weakness / physiopathology
  • Prednisone / therapeutic use
  • Radioimmunoassay


  • Autoantibodies
  • Calcium Channels, P-Type
  • Immunosuppressive Agents
  • Azathioprine
  • Prednisone