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

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.

• Authors: Hannah L Pellkofer, Raymond Voltz, Tania Kuempfel
• Journal: Muscle & nerve (Muscle Nerve) Vol. 40 Issue 2 Pg. 305-8 (Aug 2009) ISSN: 0148-639X [Print] United States
• PMID: 19609921 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

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

Topics

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