Optic-spinal multiple sclerosis : clarification of pathogenesis, establishment of new disease entity and treatment

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Optic-spinal multiple sclerosis (OSMS) is an intractable neurologic disease characterized by recurrent severe optic neuritis and transverse myelitis, and is essentially identical to neuromyelitis optica (NMO). Based on the discovery of aquaporin 4 (AQP4) antibody, an OSMS/NMO-specific autoantibody, the present study clarified its clinical features including longitudinally extended myelitis (>3 vertebral segments), established pathogenicity of AQP4 antibody and the new clinical entity as astrocytopathy, and proved therapeutic efficacy of plasma exchange and immunosuppression in the disease.