Favorable response to rituximab in a patient with anti-VGCC-positive Lambert-Eaton myasthenic syndrome and cerebellar dysfunction.
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.
Pellkofer
Hannah L
HL
Institute of Clinical Neuroimmunology, Ludwig Maximilians University, Campus Grosshadern, Marchioninistr. 15, D-81377 Munich, Germany. hannah.pellkofer@med.uni-muenchen.de
Voltz
Raymond
R
Kuempfel
Tania
T
D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D015220:Calcium Channels / Q000276:immunology; D002526:Cerebellar Diseases / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D015624:Lambert-Eaton Myasthenic Syndrome / Q000150:complications / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange / Q000379:methods; D000069283:Rituximab
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D015220:Calcium Channels; D007155:Immunologic Factors; D000069283:Rituximab