Favorable response to rituximab in a patient with anti-VGCC-positive Lambert-Eaton myasthenic syndrome and cerebellar dysfunction

Muscle Nerve. 2009 Aug;40(2):305-8. doi: 10.1002/mus.21315.

Abstract

Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disease that is characterized by impaired transmission across the neuromuscular junction due to autoantibodies directed against the presynaptic voltage-gated calcium channels (VGCC-ab). Clinical symptoms are usually characterized by proximal muscle weakness and mild dysautonomia. In some patients there are signs of cerebellar dysfunction as well, usually associated with cancer. Here we report the long-term follow-up of a patient with VGCC-ab-positive LEMS and a severe cerebellar syndrome but without evidence of cancer over 5 years. While conventional immunosuppressive therapy (steroids, azathioprine) failed, he improved with plasma exchange and consecutive treatment with rituximab. Muscle Nerve 40: 305-308, 2009.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antibodies, Monoclonal / therapeutic use*
  • Antibodies, Monoclonal, Murine-Derived
  • Calcium Channels / immunology
  • Cerebellar Diseases / complications
  • Cerebellar Diseases / drug therapy*
  • Humans
  • Immunologic Factors / therapeutic use*
  • Lambert-Eaton Myasthenic Syndrome / complications
  • Lambert-Eaton Myasthenic Syndrome / drug therapy*
  • Male
  • Middle Aged
  • Plasma Exchange / methods
  • Rituximab

Substances

  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Calcium Channels
  • Immunologic Factors
  • Rituximab