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Neuromyelitis Optica: From Immunopathogenesis to Treatment Perspectives

Neuromyelitis optica is a severe, inflammatory, demyelinating disease of the central nervous system characterized by attacks of myelitis and optic neuritis. Once considered a malignant variant of multiple sclerosis, neuromyelitis optica recently was shown to have its own distinguishing features. Humoral immunity seems to have a prominent role in the pathogenesis of the disease. The definition of neuromyelitis optica has been bolstered by the identification of both a neuromyelitis optica/immunoglobulin G complex as a sensitive and specific in vivo diagnostic marker and by clinical, radiologic, and pathologic advances in our understanding of the disease. Treatment options for neuromyelitis optica are limited. Recent positive results reported with the use of the anti-CD20 monoclonal antibody rituximab are promising.

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