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Published on: May 18, 2010
[Progressive multifocal leukoencephalopathy]
C Warnke1, M P Wattjes2, O Adams3
1Klinik für Neurologie, Medizinische Fakultät, Universitätsklinikum Düsseldorf, Heinrich-Heine-Universität Düsseldorf, Moorenstraße 5, 40225, Düsseldorf, Deutschland. warnke@uni-duesseldorf.de.
Abstract:
Progressive multifocal leukoencephalopathy (PML) is a disease of immunosuppressed patients caused by the JC polyomavirus (JCPyV). Due to the elevated risk in patients treated with natalizumab for multiple sclerosis (MS) and also treatment with other biologicals for different indications, the relevance of PML has increased in recent years. This article summarizes the published knowledge on the biology and pathogenesis of PML with a focus on the role of cerebrospinal fluid diagnostics in the work-up for PML and the current PML case definition. Current recommendations regarding risk management are discussed, as are possible therapies and prevention.
Insights
Progressive multifocal leukoencephalopathy (PML), a brain disease caused by the JC polyomavirus (JCPyV), is increasingly relevant in immunosuppressed patients. This review covers PML biology, diagnostics, and management strategies.
Area of Science:
- Neurovirology
- Immunology
- Neurology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare but serious opportunistic infection of the brain.
- It is caused by the JC polyomavirus (JCPyV), typically in immunocompromised individuals.
- The incidence of PML has risen due to its association with certain biologic therapies, including natalizumab for multiple sclerosis.
Purpose of the Study:
- To provide a comprehensive overview of the biology and pathogenesis of PML.
- To highlight the importance of cerebrospinal fluid (CSF) diagnostics in PML detection.
- To discuss current PML case definitions, risk management, and therapeutic options.
Main Methods:
- Review of published literature on JC polyomavirus and PML.
- Analysis of diagnostic approaches, particularly CSF analysis.
- Synthesis of current recommendations for risk management, therapy, and prevention.
Main Results:
- JCPyV infects oligodendrocytes, leading to demyelination in PML.
- CSF analysis is crucial for accurate and timely PML diagnosis.
- Risk stratification and monitoring are essential for patients on immunosuppressive therapies.
Conclusions:
- Understanding PML pathogenesis is key to developing effective treatments.
- Early diagnosis via CSF testing improves patient outcomes.
- Multidisciplinary management strategies are necessary for PML prevention and treatment.
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