Neuromyelitis optica

Abstract

Neuromyelitis optica (NMO), an inflammatory demyelinating disease of the central nervous system, is characterized by relapsing optic neuritis and transverse myelitis. The detection of pathogenic anti-aquaporin-4 (AQP4) antibody distinguishes NMO from other demyelinating disorders, such as multiple sclerosis. Although early administration of high-dose intravenous methyl-prednisolone may improve symptoms that occur during relapses, the application of plasmapheresis is often required for sufficient recovery from each attack. The management of relapses is done with early steroid treatment, typically 1 g intravenous methyl-prednisolone for 3 to 5 days followed by oral prednisone. Relapses that do not respond to intravenous steroids could benefit from plasmapheresis. Treatments for the prevention that have been used to date for NMO have generally been immunosuppressive drugs rather than immunomodulatory agents that are commonly used for multiple sclerosis (MS). Although low dose oral prednisolone is reported to be effective for prevention in Japanese NMO, non-steroidal immunosuppressive agents are recommended in other countries to be changed from prednisolone within six months to avoid the side effects of steroids.