Neuromyelitis optica (NMO, Devic syndrome) is a rare demyelinating disease of the central nervous system which mostly follows a relapsing course. Key features of this disorder include unilateral or bilateral optic neuritis and longitudinally extensive myelitis (> or = three segments). Brain lesions are rarely present at onset. They may however evolve during the course of disease but usually remain asymptomatic. The histopathology of NMO is suggestive of an underlying humoral autoimmune pathomechanism and indicates that NMO is a distinct entity rather than a variant of multiple sclerosis. The recent detection of NMO-specific serum autoantibodies against the water channel aquaporin-4 (Aqp4) is of significant diagnostic relevance and classifies NMO as the first inflammatory demyelinating disorder of the CNS with a defined autoantigen. More recent therapeutic strategies such as plasma exchange or pharmacological B-cell depletion are expected to improve long-term prognosis of NMO.