PMID- 35986243
OWN - NLM
STAT- MEDLINE
DCOM- 20220823
LR  - 20220826
IS  - 1471-2377 (Electronic)
IS  - 1471-2377 (Linking)
VI  - 22
IP  - 1
DP  - 2022 Aug 19
TI  - Progressive multifocal leukoencephalopathy or immune reconstitution inflammatory 
      syndrome after fingolimod cessation? A case report.
PG  - 306
LID - 10.1186/s12883-022-02839-3 [doi]
LID - 306
AB  - BACKGROUND: Fingolimod is associated with an increased risk of developing 
      progressive multifocal leukoencephalopathy (PML); however, its discontinuation 
      may cause severe immune reconstitution inflammatory syndrome (IRIS). As both of 
      these conditions (especially fingolimod induced PML) are rarely described in 
      medical case reports distinguishing between PML-IRIS and MS-IRIS may be 
      diagnostically challenging. CASE PRESENTATION: We report a patient with severe 
      clinical decline (Expanded Disability Status Scale (EDSS) increasing from 3.5 to 
      7.5) and multiple, large, contrast-enhancing lesions on brain magnetic resonance 
      imaging (MRI) a few months after fingolimod withdrawal. The diagnostic 
      possibilities included IRIS due to fingolimod withdrawal versus PML-IRIS. The JC 
      virus (JCV) antibody index was positive (2.56); however, cerebrospinal fluid 
      (CSF) JCV real-time polymerase chain reaction (JCV-PCR) was negative and brain 
      biopsy was not performed. After a long course of aggressive treatment (several 
      pulsed methylprednisolone infusions, plasmapheresis, intravenous dexamethasone, 
      oral mirtazapine) the patient gradually recovered (EDSS 2.5) and MRI lesions 
      decreased. CONCLUSIONS: This case report demonstrates the importance of 
      monitoring patients carefully after the discontinuation of fingolimod for 
      PML-IRIS and rebound MS with IRIS as these conditions may manifest similarly.
CI  - (c) 2022. The Author(s).
FAU - Mickeviciene, D
AU  - Mickeviciene D
AD  - Department of Neurology, Hospital of Lithuanian University of Health Sciences 
      Kauno Klinikos, Kaunas, Lithuania.
AD  - Department of Neurology, Lithuanian University of Health Sciences Clinical, 
      Kaunas, Lithuania.
FAU - Baltusiene, A
AU  - Baltusiene A
AUID- ORCID: 0000-0003-3953-597X
AD  - Department of Neurology, Hospital of Lithuanian University of Health Sciences 
      Kauno Klinikos, Kaunas, Lithuania. auguste.august@gmail.com.
AD  - Department of Neurology, Lithuanian University of Health Sciences Clinical, 
      Kaunas, Lithuania. auguste.august@gmail.com.
AD  - Faculty of Medicine, Lithuanian University of Health Sciences, Kaunas, Lithuania. 
      auguste.august@gmail.com.
FAU - Afanasjeva, B
AU  - Afanasjeva B
AD  - Department of Neurology, Hospital of Lithuanian University of Health Sciences 
      Kauno Klinikos, Kaunas, Lithuania.
AD  - Department of Neurology, Lithuanian University of Health Sciences Clinical, 
      Kaunas, Lithuania.
AD  - Faculty of Medicine, Lithuanian University of Health Sciences, Kaunas, Lithuania.
FAU - Afanasjevas, D
AU  - Afanasjevas D
AD  - Faculty of Medicine, Lithuanian University of Health Sciences, Kaunas, Lithuania.
FAU - Gleizniene, R
AU  - Gleizniene R
AD  - Department of Radiology, Lithuanian University of Health Sciences Clinical, 
      Kaunas, Lithuania.
FAU - Rastenyte, D
AU  - Rastenyte D
AD  - Department of Neurology, Hospital of Lithuanian University of Health Sciences 
      Kauno Klinikos, Kaunas, Lithuania.
AD  - Department of Neurology, Lithuanian University of Health Sciences Clinical, 
      Kaunas, Lithuania.
FAU - Berger, J R
AU  - Berger JR
AD  - Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20220819
PL  - England
TA  - BMC Neurol
JT  - BMC neurology
JID - 100968555
RN  - 0 (Natalizumab)
RN  - G926EC510T (Fingolimod Hydrochloride)
SB  - IM
MH  - Brain/diagnostic imaging/pathology
MH  - Fingolimod Hydrochloride/adverse effects
MH  - Humans
MH  - *Immune Reconstitution Inflammatory Syndrome
MH  - *JC Virus
MH  - *Leukoencephalopathy, Progressive Multifocal/chemically induced/diagnostic 
      imaging
MH  - Magnetic Resonance Imaging
MH  - Natalizumab/adverse effects
PMC - PMC9392231
OTO - NOTNLM
OT  - Case report
OT  - Fingolimod
OT  - Immune reconstitution inflammatory syndrome
OT  - Multiple sclerosis
OT  - Progressive multifocal leukoencephalopathy
COIS- Dr. Joseph R. Berger has received grant support from Biogen and Genentech/Roche. 
      He has received honoraria as a consultant for Amgen, Biogen, Bristol Myers 
      Squibb/Celgene, Dr. Reddy, EMD Serono, Encycle, Excision-Bio, Genentech/Roche, 
      Genzyme, MAPI, Merck, Millennium/Takeda, Morphic, and Shire. D. Mickeviciene 
      reports grants and consulting fees from Merck/EMD Serono, Novartis, Roche, and 
      Sanofi Genzyme, outside the submitted work. A. Baltusiene has no conflict of 
      interest. B. Afanasjeva has no conflict of interest. D. Afanasjevas has no 
      conflict of interest. Prof. R. Gleizniene has no conflict of interest. Prof. D. 
      Rastenyte has no conflict of interest.
EDAT- 2022/08/20 06:00
MHDA- 2022/08/24 06:00
PMCR- 2022/08/19
CRDT- 2022/08/19 23:31
PHST- 2021/10/13 00:00 [received]
PHST- 2022/08/14 00:00 [accepted]
PHST- 2022/08/19 23:31 [entrez]
PHST- 2022/08/20 06:00 [pubmed]
PHST- 2022/08/24 06:00 [medline]
PHST- 2022/08/19 00:00 [pmc-release]
AID - 10.1186/s12883-022-02839-3 [pii]
AID - 2839 [pii]
AID - 10.1186/s12883-022-02839-3 [doi]
PST - epublish
SO  - BMC Neurol. 2022 Aug 19;22(1):306. doi: 10.1186/s12883-022-02839-3.