[Progressive multifocal leukoencephalopathy].

Abstract

PATHOGENESIS: Progressive multifocal leukoencephalopathy is a demyelinating disease of the central nervous system caused by infection and reactivation of JC-virus. About 5% of all HIV-infected patients develop this fatal disease. Although pathogenesis is not completely understood, progressive multifocal leukoencephalopathy is thought to be a persistent infection. The kidneys, bone marrow, peripheral blood lymphocytes and the brain itself are candidates for latency sites of JC-virus. Loss of T-helper-cells in the course of HIV-infection or other immunosuppressive states result in reactivation of JC-virus. DIAGNOSIS: Progressive multifocal leukoencephalopathy can be diagnosed by focal neurological symptoms, radiographic signs in magnetic resonance imaging and detection of JC-virus in brain tissue or cerebrospinal fluid. TREATMENT: A specific therapy is not yet available or established. Highly active antiretroviral therapy (HAART) and cidofovir are promising and may prove useful in the near future.