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Progressive Multifocal Leukoencephalopathy 

Progressive Multifocal Leukoencephalopathy
Progressive Multifocal Leukoencephalopathy

Maria G. Chiara

and Eugene O. Major

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date: 17 June 2019

Progressive multifocal leukoencephalopathy (PML), caused by the JC Virus (JCV), is a demyelinating disease of the white matter of the human brain. It is one of the principal opportunistic infections afflicting HIV-1-positive individuals and is an AIDS-defining illness. The role of the immune system in the pathogenesis of PML is complex. Interactions between the JC and HIV viruses further complicates the picture. Normally held in check by the immune system, JCV can become lethally active in AIDS patients either early in the progression of AIDS or in the months immediately following initiation of HAART in the form of immune reconstitution inflammatory syndrome (IRIS). This chapter provides an overview of many aspects of PML in AIDS, including reviews of the pathology, diagnosis, and incidence of PML, and of the association of PML with IRIS; a comparison of PML in AIDS vs. in other immunosuppressive disorders; a discussion of PML pathogenesis from viral latency through demyelination; and a discussion of PML in patients undergoing pharmacologic immunosuppression.

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