PMID- 24752885
OWN - NLM
STAT- MEDLINE
DCOM- 20140811
LR  - 20211021
IS  - 1531-8249 (Electronic)
IS  - 0364-5134 (Print)
IS  - 0364-5134 (Linking)
VI  - 75
IP  - 5
DP  - 2014 May
TI  - Hyperintense cortical signal on magnetic resonance imaging reflects focal 
      leukocortical encephalitis and seizure risk in progressive multifocal 
      leukoencephalopathy.
PG  - 659-69
LID - 10.1002/ana.24144 [doi]
AB  - OBJECTIVE: To determine the frequency of hyperintense cortical signal (HCS) on 
      T1-weighted precontrast magnetic resonance (MR) images in progressive multifocal 
      leukoencephalopathy (PML) patients, its association with seizure risk and immune 
      reconstitution inflammatory syndrome (IRIS), and its pathologic correlate. 
      METHODS: We reviewed clinical data including seizure history, presence of IRIS, 
      and MR imaging scans from PML patients evaluated at our institution between 2003 
      and 2012. Cases that were diagnosed either using cerebrospinal fluid JC virus 
      (JCV) polymerase chain reaction, brain biopsy, or autopsy, and who had MR images 
      available were included in the analysis (n=49). We characterized pathologic 
      findings in areas of the brain that displayed HCS in 2 patients and compared them 
      with isointense cortex in the same individuals. RESULTS: Of 49 patients, 17 
      (34.7%) had seizures and 30 (61.2%) had HCS adjacent to subcortical PML lesions 
      on MR images. Of the 17 PML patients with seizures, 15 (88.2%) had HCS compared 
      with 15 of 32 (46.9%) patients without seizures (p=0.006). HCS was associated 
      with seizure development with a relative risk of 4.75 (95% confidence 
      interval=1.2-18.5, p=0.006). Of the 20 patients with IRIS, 16 (80.0%) had HCS 
      compared with 14 of 29 (49.3%) patients without IRIS (p=0.04). On histological 
      examination, HCS areas were associated with striking JCV-associated demyelination 
      of cortical and subcortical U fibers, significant macrophage infiltration, and a 
      pronounced reactive gliosis in the deep cortical layers. INTERPRETATION: Seizures 
      are a frequent complication in PML. HCS is associated with seizures and IRIS, and 
      correlates histologically with JCV focal leukocortical encephalitis.
CI  - (c) 2014 American Neurological Association.
FAU - Khoury, Michael N
AU  - Khoury MN
AD  - Division of Neurovirology, Department of Neurology, Beth Israel Deaconess Medical 
      Center, Boston, MA; Center for Virology and Vaccine Research, Beth Israel 
      Deaconess Medical Center, Boston, MA.
FAU - Alsop, David C
AU  - Alsop DC
FAU - Agnihotri, Shruti P
AU  - Agnihotri SP
FAU - Pfannl, Rolf
AU  - Pfannl R
FAU - Wuthrich, Christian
AU  - Wuthrich C
FAU - Ho, Mai-Lan
AU  - Ho ML
FAU - Hackney, David
AU  - Hackney D
FAU - Ngo, Long
AU  - Ngo L
FAU - Anderson, Matthew P
AU  - Anderson MP
FAU - Koralnik, Igor J
AU  - Koralnik IJ
LA  - eng
GR  - K24 NS 060950/NS/NINDS NIH HHS/United States
GR  - R01 NS081916/NS/NINDS NIH HHS/United States
GR  - T32 AI052074/AI/NIAID NIH HHS/United States
GR  - R21 MH100868/MH/NIMH NIH HHS/United States
GR  - R01 NS 047029/NS/NINDS NIH HHS/United States
GR  - R01 NS074995/NS/NINDS NIH HHS/United States
GR  - R01 NS 074995/NS/NINDS NIH HHS/United States
GR  - R01 NS047029/NS/NINDS NIH HHS/United States
GR  - K24 NS060950/NS/NINDS NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
DEP - 20140428
PL  - United States
TA  - Ann Neurol
JT  - Annals of neurology
JID - 7707449
SB  - IM
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Cerebral Cortex/*pathology/physiopathology
MH  - Encephalitis/epidemiology/pathology/physiopathology
MH  - Female
MH  - Humans
MH  - Leukoencephalopathy, Progressive 
      Multifocal/*epidemiology/pathology/physiopathology
MH  - *Magnetic Resonance Imaging/methods
MH  - Male
MH  - Middle Aged
MH  - Retrospective Studies
MH  - Risk Factors
MH  - Seizures/*epidemiology/*pathology/physiopathology
MH  - Young Adult
PMC - PMC4426496
MID - NIHMS685573
EDAT- 2014/04/23 06:00
MHDA- 2014/08/12 06:00
PMCR- 2015/05/11
CRDT- 2014/04/23 06:00
PHST- 2013/11/14 00:00 [received]
PHST- 2014/02/10 00:00 [revised]
PHST- 2014/03/16 00:00 [accepted]
PHST- 2014/04/23 06:00 [entrez]
PHST- 2014/04/23 06:00 [pubmed]
PHST- 2014/08/12 06:00 [medline]
PHST- 2015/05/11 00:00 [pmc-release]
AID - 10.1002/ana.24144 [doi]
PST - ppublish
SO  - Ann Neurol. 2014 May;75(5):659-69. doi: 10.1002/ana.24144. Epub 2014 Apr 28.