PMID- 26323992
OWN - NLM
STAT- MEDLINE
DCOM- 20160824
LR  - 20220317
IS  - 1432-0533 (Electronic)
IS  - 0001-6322 (Linking)
VI  - 130
IP  - 6
DP  - 2015 Dec
TI  - Progressive multifocal leukoencephalopathy and immune reconstitution inflammatory 
      syndrome (IRIS).
PG  - 751-64
LID - 10.1007/s00401-015-1471-7 [doi]
AB  - 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.
FAU - Bauer, Jan
AU  - Bauer J
AD  - Department of Neuroimmunology, Center for Brain Research, Medical University of 
      Vienna, Spitalgasse 4, 1090, Vienna, Austria. jan.bauer@meduniwien.ac.at.
FAU - Gold, Ralf
AU  - Gold R
AD  - Department of Neurology, St. Josef-Hospital, Ruhr-University, Bochum, Germany. 
      ralf.gold@rub.de.
FAU - Adams, Ortwin
AU  - Adams O
AD  - Institute of Virology, University of Dusseldorf, Dusseldorf, Germany. 
      ortwin.adams@uni-duesseldorf.de.
FAU - Lassmann, Hans
AU  - Lassmann H
AD  - Department of Neuroimmunology, Center for Brain Research, Medical University of 
      Vienna, Spitalgasse 4, 1090, Vienna, Austria. hans.lassmann@meduniwien.ac.at.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20150901
PL  - Germany
TA  - Acta Neuropathol
JT  - Acta neuropathologica
JID - 0412041
SB  - IM
MH  - Humans
MH  - Immune Reconstitution Inflammatory 
      Syndrome/*complications/*immunology/therapy/virology
MH  - JC Virus
MH  - Leukoencephalopathy, Progressive 
      Multifocal/*complications/*immunology/therapy/virology
MH  - Polyomavirus Infections/complications/immunology/pathology/therapy
MH  - Tumor Virus Infections/complications/immunology/pathology/therapy
OTO - NOTNLM
OT  - Immune reconstitution inflammatory syndrome
OT  - JC virus
OT  - Neuropathology
OT  - Progressive multifocal leukoencephalopathy
OT  - T cell cytotoxicity
EDAT- 2015/09/02 06:00
MHDA- 2016/08/25 06:00
CRDT- 2015/09/02 06:00
PHST- 2015/07/24 00:00 [received]
PHST- 2015/08/25 00:00 [accepted]
PHST- 2015/08/25 00:00 [revised]
PHST- 2015/09/02 06:00 [entrez]
PHST- 2015/09/02 06:00 [pubmed]
PHST- 2016/08/25 06:00 [medline]
AID - 10.1007/s00401-015-1471-7 [pii]
AID - 10.1007/s00401-015-1471-7 [doi]
PST - ppublish
SO  - Acta Neuropathol. 2015 Dec;130(6):751-64. doi: 10.1007/s00401-015-1471-7. Epub 
      2015 Sep 1.