Neuromyelitis optica

Neuromyelitis optica (NMO, Devic syndrome) is an autoimmune-mediated disorder of the central nervous system (CNS) with optic neuritis and longitudinally extensive transverse myelitis as key clinical features. The presence of highly specific serum autoantibodies, which target the water channel aquaporin-4 (AQP4), classifies NMO as a distinct entity and as the first inflammatory multiple sclerosis-like disorder of the CNS with a defined autoantigen. Recent observations strongly suggest that the AQP4 antibodies are pathophysiologically relevant and thus provide a rationale for the use of therapeutic strategies directed predominantly against the humoral effector arm of autoimmune responses such as plasma exchange or pharmacological B-cell depletion