[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.

Der Nervenarzt
|October 13, 2016
PubMed

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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