Neuromyelitis optica in childhood; [Optički neuromijelitis u dječjoj dobi]

Abstract

Neuromyelitis optica is a rare and severe autoimmune demyelinating disease of the central nervous system, clinicaly presented as optic neuritis and transverse myelitis that affects more than 3 spinal cord segments. Its initial course usually doesn't affect the brain. This feature differentiates it from the multiple sclerosis. It has a usual course of form with frequent relapses and rarely has a monophasic form. Frequent relapses often lead to a severe neurological deterioration (blindness, para/tetraplegia). The onset ranges from early childhood to late adulthood with the mean age in the forthies. Discovery of the autoantibodies directed toward aquaporine-4 receptor has significantnly revealed its pathogenesis. These antibodies are highly specific (99%) and sensitive (48- 72%) for the diagnosis of optic neuromyelitis. Current therapy strategy is directed toward intensive treatment of the acute relapse with i.v. methylprednisolone and plasmapheresis and remision prevention with imunosupressive drugs like azathioprine, mycophenolate mofetil and rituximab.