Department of Neurology, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea
Neuromyelitis optica (NMO) is a relapsing inflammatory disorder of the central nervous system that closely resembles multiple sclerosis (MS). It is originally described as a monophasic disorder, but most of patients with NMO experience recurrent attacks with severe attack-related disability. The recently identified serum immunoglobulin marker, NMO-IgG binds selectively to the aquaporin-4 (AQP4) water channel and appears to be specific for NMO. Anti-AQP4 antibody has a pathogenetic role in the development of NMO and serves as a useful diagnostic and prognostic marker. Detection of this autoantibody has led to the categorization of NMO as an autoimmune channelopathy.
Because this highly specific autoantibody marker is also found in patients with a limited form of NMO such as recurrent optic neuritis, longitudinally extensive transverse myelitis, and optic neuritis or myelitis associated with brain lesions typical of NMO, the concept of NMO has been broadened to NMO spectrum disorders. Acute attacks usually result in moderate to severe functional impairment without the absence of a secondary progressive course. Prevention of relapse is of crucial
importance in the management of the disease.
Journal of Multiple Sclerosis 1(2):38-43, 2010
