PMID- 31191548
OWN - NLM
STAT- MEDLINE
DCOM- 20200616
LR  - 20200616
IS  - 1664-3224 (Electronic)
IS  - 1664-3224 (Linking)
VI  - 10
DP  - 2019
TI  - Severe Progressive Multifocal Leukoencephalopathy (PML) and Spontaneous Immune 
      Reconstitution Inflammatory Syndrome (IRIS) in an Immunocompetent Patient.
PG  - 1188
LID - 10.3389/fimmu.2019.01188 [doi]
LID - 1188
AB  - Background: Progressive multifocal leukoencephalopathy (PML) is an opportunistic 
      infection with JC-virus (JCV), a papova-virus, affecting mostly oligodendrocytes 
      and the white matter of the central nervous system. Progressive Multifocal 
      Leukoencephalopathy (PML) almost exclusively occurs in immunocompromised patients 
      based on different underlying conditions of severe cellular immunodeficiency such 
      as HIV/AIDS, secondary to neoplastic and autoimmune diseases, or during 
      immunosuppressive therapy. Case presentation: We present the case of an otherwise 
      healthy and immunocompetent patient without immunosuppressive therapy who was 
      admitted with hemianopsia to the right side, sensory aphasia and changes of 
      behavior. Magnet resonance imaging (MRI) and laboratory testing confirmed the 
      diagnosis of PML, although functional tests did not show any evidence for 
      cellular immunodeficiency. Extensive immunological tests did not reveal an 
      apparent immunodeficiency. During symptomatic therapy the patient developed 
      seizures which were assumed to be caused by a spontaneous immune reconstitution 
      inflammatory syndrome (IRIS) demonstrated by MRI. We added a high dose of 
      intravenous corticosteroids to the antiepileptic treatment and seizures ended 
      shortly thereafter. However, the impairments of vision, behavior and language 
      persisted. Conclusions: Our case report highlights that an apparently 
      immunocompetent patient can develop PML and IRIS spontaneously. Therefore, MRI 
      should be applied immediately whenever a rapid progression of PML symptoms occurs 
      as treatment of IRIS with corticosteroids can result in a marked clinical 
      improvement.
FAU - Krey, Lea
AU  - Krey L
AD  - Department of Neurology, Hannover Medical School, Hanover, Germany.
FAU - Raab, Peter
AU  - Raab P
AD  - Hannover Medical School, Institute of Neuroradiology, Hanover, Germany.
FAU - Sherzay, Romilda
AU  - Sherzay R
AD  - Department of Nuclear Medicine, Hannover Medical School, Hanover, Germany.
FAU - Berding, Georg
AU  - Berding G
AD  - Department of Nuclear Medicine, Hannover Medical School, Hanover, Germany.
FAU - Stoll, Matthias
AU  - Stoll M
AD  - Clinic for Immunology and Rheumatology, Unit for Infectious Diseases, Hannover 
      Medical School, Hanover, Germany.
FAU - Stangel, Martin
AU  - Stangel M
AD  - Department of Neurology, Hannover Medical School, Hanover, Germany.
FAU - Wegner, Florian
AU  - Wegner F
AD  - Department of Neurology, Hannover Medical School, Hanover, Germany.
LA  - eng
PT  - Case Reports
DEP - 20190528
PL  - Switzerland
TA  - Front Immunol
JT  - Frontiers in immunology
JID - 101560960
SB  - IM
MH  - Aged
MH  - Brain/diagnostic imaging/pathology
MH  - Humans
MH  - Immune Reconstitution Inflammatory Syndrome/*diagnosis/etiology/therapy
MH  - *Immunocompromised Host
MH  - JC Virus/genetics
MH  - Leukoencephalopathy, Progressive Multifocal/*diagnosis/etiology/therapy
MH  - Magnetic Resonance Imaging
MH  - Male
MH  - Polymerase Chain Reaction
MH  - Positron-Emission Tomography
MH  - Serologic Tests
PMC - PMC6546850
OTO - NOTNLM
OT  - JC-virus
OT  - corticosteroid
OT  - immunocompetence
OT  - progressive multifocal leukoencephalopathy (PML)
OT  - spontaneous immune reconstitution inflammatory syndrome (IRIS)
EDAT- 2019/06/14 06:00
MHDA- 2020/06/17 06:00
PMCR- 2019/01/01
CRDT- 2019/06/14 06:00
PHST- 2019/03/08 00:00 [received]
PHST- 2019/05/10 00:00 [accepted]
PHST- 2019/06/14 06:00 [entrez]
PHST- 2019/06/14 06:00 [pubmed]
PHST- 2020/06/17 06:00 [medline]
PHST- 2019/01/01 00:00 [pmc-release]
AID - 10.3389/fimmu.2019.01188 [doi]
PST - epublish
SO  - Front Immunol. 2019 May 28;10:1188. doi: 10.3389/fimmu.2019.01188. eCollection 
      2019.