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Published in:

01-12-2015 | Review

Progressive multifocal leukoencephalopathy and immune reconstitution inflammatory syndrome (IRIS)

Authors: Jan Bauer, Ralf Gold, Ortwin Adams, Hans Lassmann

Published in: Acta Neuropathologica | Issue 6/2015

Abstract

Progressive multifocal leukoencephalopathy is a viral encephalitis induced by the John Cunningham (JC) virus, an ubiquitous neurotropic papovavirus of the genus polyomavirus that in healthy people in latency resides in kidney and bone marrow cells. Activation and entry into the CNS were first seen in patients with malignancies of the hematopoietic system and an impaired immune system. During the 1980 and the 1990s with the appearance of human immunodeficiency virus infection in humans, PML was found to be the most important opportunistic infection of the central nervous system. As a result of highly efficient immunosuppressive and immunomodulatory treatments, in recent years, the number of PML cases again increased. PML is prevented by an intact cellular immune response and accordingly immune reconstitution can terminate established disease in the CNS. However, forced immune reconstitution can lead to massive destruction of virus-infected cells. This may result in clinical exacerbation associated with high morbidity and mortality and referred to as PML with immune reconstitution inflammatory syndrome (PML–IRIS). In the present review, we discuss virological properties and routes of infection in the CNS, but mostly focus on the pathology of PML and PML–IRIS and on the role of the immune system in these disorders. We show that PML and PML–IRIS result from predominant JC virus infection of oligodendrocytes and, to a lesser extent, of infected neurons. Inflammation in these encephalitides seems to be driven by a dominant cytotoxic T cell response which is massively exaggerated during IRIS.

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Title: Progressive multifocal leukoencephalopathy and immune reconstitution inflammatory syndrome (IRIS)
Authors: Jan Bauer
Ralf Gold
Ortwin Adams
Hans Lassmann
Publication date: 01-12-2015
Publisher: Springer Berlin Heidelberg
Published in: Acta Neuropathologica / Issue 6/2015
Print ISSN: 0001-6322
Electronic ISSN: 1432-0533
DOI: https://doi.org/10.1007/s00401-015-1471-7

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Progressive multifocal leukoencephalopathy and immune reconstitution inflammatory syndrome (IRIS)

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

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