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Progressive multifocal leukoencephalopathy

2014; Elsevier BV; Linguagem: Inglês

10.1016/b978-0-444-53488-0.00017-1

2212-4152

Joseph R. Berger,

Antenna Design and Analysis

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disorder due to an ubiquitous polyomavirus, referred to as JC virus. Prior to the human immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS) pandemic, it was a disorder seen predominantly in individuals with underlying lymphomas and leukemias, in particular, B-cell malignancies. In the pre-antiretroviral therapy era of the HIV/AIDS pandemic, it ultimately developed in approximately 5% of all infected individuals. In 2005, following the recognition that it may complicate the use of natalizumab, an α4β1 integrin inhibitor, used in the treatment of multiple sclerosis and inflammatory bowel disorders, it achieved even greater notoriety. PML is characterized by a triad of histopathologic findings, demyelination, bizarre astrocytes, and enlarged oligodendroglial nuclei. JC virus leads to demyelination by establishing a lytic infection of the oligodendrocytes. The clinical features are diverse and depend on the region of the brain affected. The disease is often heralded by cognitive and behavioral changes, language disturbances, weakness, or visual deficits. The diagnosis can often be established by cranial magnetic resonance imaging findings coupled with positive polymerase chain reaction for JC virus in the cerebrospinal fluid. To date, no treatment has been convincingly effective.