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Progressive multifokale Leukenzephalopathie

Progressive multifocal leukoencephalopathy

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Zusammenfassung

Die progressive multifokale Leukenzephalopathie (PML) wird durch das JC-Polyomavirus (JCPyV) verursacht und ist eine schwere Komplikation bei immunsupprimierten Patienten. Die klinische Bedeutung der PML hat in den letzten Jahren durch ihr gehäuftes Auftreten in der Behandlung der Multiplen Sklerose mit Natalizumab, aber auch bei der Therapie mit anderen Biologika zugenommen. Im Folgenden soll eine Übersicht über das JCPyV und das gegenwärtige Verständnis der PML-Pathogenese gegeben werden. Dies dient als Ausgangspunkt, um die aktuelle Falldefinition der PML unter Einbezug von Liquorbefunden zu erläutern. Zudem werden aktuelle Maßnahmen zur verbesserten PML-Risikominimierung diskutiert.

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.

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Author information

Authors and Affiliations

  1. Klinik für Neurologie, Medizinische Fakultät, Universitätsklinikum Düsseldorf, Heinrich-Heine-Universität Düsseldorf, Moorenstraße 5, 40225, Düsseldorf, Deutschland

    C. Warnke & H.‑P. Hartung

  2. Abteilung für Radiologie und Nuklearmedizin, VU University Medical Center, Amsterdam, Niederlande

    M. P. Wattjes

  3. Institut für Virologie, Medizinische Fakultät, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Deutschland

    O. Adams

  4. Klinik für Neurologie, Universitätsspital Zürich, Zürich, Schweiz

    R. Martin

  5. Klinik für Neurologie, Kath. Marienkrankenhaus, Hamburg, Deutschland

    T. Weber

  6. Klinik für Neurologie, Medizinische Hochschule Hannover, Hannover, Deutschland

    M. Stangel

Authors
  1. C. Warnke
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  2. M. P. Wattjes
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  3. O. Adams
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  4. H.‑P. Hartung
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  5. R. Martin
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  6. T. Weber
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  7. M. Stangel
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Corresponding author

Correspondence to C. Warnke.

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Interessenkonflikt

C. Warnke: Referentenhonorare, Reisekostenzuschüsse und/oder Beraterhonorare von Bayer, Biogen, Novartis, TEVA. M. P. Wattjes: Referentenhonorare und Forschungsunterstützung: Biogen, Novartis. O. Adams: Referentenhonorare und Forschungsunterstützung: Biogen. H.-P. Prof. Hartung: Referentenhonorare, Reisekostenzuschüsse und/oder Beraterhonorare: Bayer, Biogen Idec, Genzyme, Merck Serono, Novartis Pharma, Roche und Teva Sanofi-Aventis. R. Martin: nicht zweckgebundene Zuwendung von Biogen und Gehalt von Vortragstätigkeiten bzw. Beratung von Biogen, Neuway Pharma. Desweiteren ist R. Martin Mitentwickler von Patenten auf die aktive und passive Immunisierung gegen PML. T. Weber: Reisekostenzuschüsse von Biogen; Beraterhonorare von Biogen, Novartis, PML Consortium; Referentenhonorare von Novartis, PML Consortium, Roche, Drittmittelförderung von Biogen M. Stangel: Referentenhonorare, Reisekostenzuschüsse und Beraterhonorare von Biogen, Baxter/Baxalta, Bayer Vital, CSL Behring, Euroimmune, Genzyme, Grifols, Merck-Serono, Roche, Novartis, Sanofi Aventis und Teva.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Warnke, C., Wattjes, M.P., Adams, O. et al. Progressive multifokale Leukenzephalopathie. Nervenarzt 87, 1300–1304 (2016). https://doi.org/10.1007/s00115-016-0225-7

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  • DOI: https://doi.org/10.1007/s00115-016-0225-7

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