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Favorable response to rituximab in a patient with anti‐VGCC‐positive Lambert‐Eaton myasthenic syndrome and cerebellar dysfunction
Author(s) -
Pellkofer Hannah L.,
Voltz Raymond,
Kuempfel Tania
Publication year - 2009
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.21315
Subject(s) - lambert eaton myasthenic syndrome , medicine , rituximab , myasthenia gravis , neuromuscular transmission , pyridostigmine , autoantibody , weakness , muscle weakness , dysautonomia , immunology , surgery , disease , antibody
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