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Natalizumab-Related Progressive Multifocal Leukoencephalopathy-Immune Reconstitution Inflammatory Syndrome: A Case
Bela Purohit1, Eranga Ganewatte2, Spyros S Kollias2
1Institute for Neuroradiology, University Hospital of Zurich, Frauenklinikstrasse 10, 8091 Zurich, Switzerland; Dept. of Neuroradiology, National Neuroscience Institute, 11 Jalan Tan Tock Seng, Singapore-308433.
Abstract:
Multiple sclerosis (MS) patients treated with natalizumab often face the uncommon but severe complication of developing progressive multifocal leukoencephalopathy (PML). PML may be further complicated by immune reconstitution inflammatory syndrome (IRIS) after the removal of the drug. Since both PML and IRIS are associated with high morbidity and mortality rates, early clinical and radiological diagnosis of these complications is of paramount importance. Here, we report a case of an adult male patient who was diagnosed with PML after receiving natalizumab therapy for 6 years for the treatment of MS. Upon cessation of natalizumab, he presented with a paradoxical worsening of clinical and radiological findings consistent with an inflammatory brain injury due to IRIS. He was treated with high dose corticosteroid therapy followed by a gradual improvement in clinical and imaging findings. This article illustrates the magnetic resonance imaging (MRI) features of natalizumab-associated PML-IRIS, along with a brief overview of its clinical features, complications and management strategies.
Insights
Patients on natalizumab for multiple sclerosis (MS) can develop progressive multifocal leukoencephalopathy (PML) and immune reconstitution inflammatory syndrome (IRIS). Early diagnosis and corticosteroid treatment are crucial for managing these severe natalizumab complications.
Area of Science:
- Neurology
- Immunology
- Radiology
Background:
- Natalizumab is a treatment for multiple sclerosis (MS).
- Progressive multifocal leukoencephalopathy (PML) is a rare but serious complication of natalizumab therapy.
- Immune reconstitution inflammatory syndrome (IRIS) can occur after natalizumab cessation in PML patients.
Observation:
- A case of an adult male MS patient on natalizumab for 6 years who developed PML.
- The patient experienced a paradoxical worsening of PML with IRIS upon natalizumab withdrawal.
- Magnetic resonance imaging (MRI) revealed inflammatory brain injury consistent with IRIS.
Findings:
- High-dose corticosteroid therapy led to gradual clinical and radiological improvement.
- This case highlights the MRI features of natalizumab-associated PML-IRIS.
- PML and IRIS present significant morbidity and mortality risks.
Implications:
- Early diagnosis of PML-IRIS is critical for MS patients on natalizumab.
- Understanding PML-IRIS pathogenesis aids in developing management strategies.
- Corticosteroids may be effective in managing natalizumab-associated PML-IRIS.
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