Progressive multifocal leukoencephalopathy after orthotopic liver transplantation

Progressive multifocal leukoencephalopathy (PML) is a rare, subacute, demyelinating disease of the central nervous system. It was first reported in association with chronic lymphatocytic leukemia and Hodgkin's disease in 1958. On the basis of intranuclear inclusion bodies, absence of minimal inflammatory response, and the presence of immunosuppressive condition, a viral infection of oligodendrocytes was postulated. PML is caused by a Papova virus, usually JC virus, which is named according to the patient in whom the virus was isolated for the first time. Based on mortality data from 1979 to 1987 reported to the Centers for Disease Control and Prevention, deaths related to PML increased fourfold from 1.5 per 10 million persons in 1979 to 6.1 per 10 million persons in 1987. This progression is mainly due to the steady increase of acquired immunodeficiency syndrome, but it also occurs in persons under immunosuppressive medication for organ transplants.