Neuromyelitis Optica-AQP4: an update.Curr Rheumatol Rep 2011; 13(6):496-505CR
Devic disease (neuromyelitis optica [NMO]) is an idiopathic inflammatory demyelinating and necrotizing disease characterized by optic neuritis and transverse myelitis, either simultaneously or in isolation. NMO is often idiopathic but may also be associated with systemic autoimmune disease. The prognosis of NMO is severe, especially in those with early and recurrent relapses. MRI studies have revealed that most frequently, there is a long spinal cord lesion that extends through three or more vertebral segments in length. NMO-IgG is the first antibody marker for any inflammatory central nervous system disorder and is both sensitive and specific for NMO. The identification of NMO-IgG in patients with recurrent optic neuritis or longitudinally extensive myelitis and its ability to predict subsequent relapse support the concept of a spectrum of NMO disorders. Treatment in the acute phase includes intravenous steroids and plasma exchange therapy. Immunosuppressive agents are recommended for the prophylaxis of relapses.