Les formes aiguës de maladies inflammatoires démyélinisantes du système nerveux central

Acute demyelinated diseases of the central nervous system are rare disorders dominated by Marburg's forms of multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM). Several other borderlines MS forms such as Balò's, Schilder's and Devic's are seldom described. These diseases share a fulminate evolution and are often characterised by a fatal outcome. Beside neuropathological examination in cases of fatal outcome, MRI and CSF studies help to separate these entities that involve distinct immunological mechanisms. Treatments must be effective, aggressive and introduced early. They encompass high doses of steroids, intravenous immunoglobulins and/or plasma exchanges, and immunosuppressive drugs (mitoxantrone or cyclophosphamide) in the more severe cases.