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Progressive multifocal leukoencephalopathy or immune reconstitution inflammatory syndrome after fingolimod cessation?
D Mickeviciene1,2, A Baltusiene3,4,5, B Afanasjeva1,2,6
1Department of Neurology, Hospital of Lithuanian University of Health Sciences Kauno Klinikos, Kaunas, Lithuania.
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.
Insights
Discontinuing fingolimod can cause rare but serious conditions like PML-IRIS or MS-IRIS. Careful monitoring is crucial for patients after stopping fingolimod treatment to manage these potential risks.
Area of Science:
- Neurology
- Immunology
- Pharmacology
Background:
- Fingolimod use increases the risk of progressive multifocal leukoencephalopathy (PML).
- Discontinuation of fingolimod may trigger severe immune reconstitution inflammatory syndrome (IRIS).
- Differentiating between PML-IRIS and MS-IRIS can be diagnostically challenging due to rarity.
Observation:
- A patient experienced severe clinical decline (EDSS 3.5 to 7.5) with new brain MRI lesions after fingolimod withdrawal.
- Diagnostic considerations included fingolimod withdrawal-induced IRIS versus PML-IRIS.
- Positive JC virus (JCV) antibody index but negative CSF JCV-PCR; brain biopsy was not performed.
Findings:
- Aggressive treatment including methylprednisolone, plasmapheresis, dexamethasone, and mirtazapine led to gradual recovery (EDSS 2.5).
- Brain MRI lesions decreased following treatment.
- The case highlights diagnostic challenges in distinguishing PML-IRIS from MS-IRIS.
Implications:
- Emphasizes the need for vigilant patient monitoring after fingolimod discontinuation.
- Highlights the potential for both PML-IRIS and rebound MS with IRIS to present similarly.
- Informs clinical practice regarding the management of neurological complications post-fingolimod therapy.
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