Clarification of aquaporin-4 related molecular and cellular pathomechanism in neuromyelitis optica (Devic's disease)

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Neuromyelitis optica (NMO)is an intractable neurologic disease characterized by recurrent severe optic neuritis and transverse myelitis. The present study showed that the titers of aquaporin 4 (AQP4)antibody, an autoantibody unique to NMO, reflect disease activity. We also demonstrated that AQP4 antibody has a pathogenic potential in vitro and in vivo, and that NMO is a new clinical entity (immune-mediated astrocytopathy). Concerning therapy of NMO, we found therapeutic efficacy of long-term gradual steroid tapering in preventing early relapse of this disease.