Neuromyelitis optica (NMO) is a demyelinating and inflammatory disease essentially restricted to the spinal cord and the optic nerves. Emerging evidence indicates that serum antiaquaporin-4 (AQP4) antibodies have a critical role in its pathogenesis. NMO courses with multiple relapses, often leading to severe disability. Management of NMO focuses on the effective treatment of acute attacks and the prevention of relapses. The latter is currently attempted with immunosuppressive drugs. Although several factors have been associated with disease activity, especially serum levels of anti-AQP4 IgG, no single one of them has been proved clinically useful for guiding treatment. New drugs that target specifically AQP4 antibodies and complement activation are being developed; they may prove to be more efficient with fewer side effects.