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CD8+ T-cell pathogenicity in Rasmussen encephalitis elucidated by large-scale T-cell receptor sequencing

Author

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  • Tilman Schneider-Hohendorf

    (University of Münster)

  • Hema Mohan

    (University of Münster)

  • Christian G. Bien

    (Epilepsy Center Bethel, Krankenhaus Mara)

  • Johanna Breuer

    (University of Münster)

  • Albert Becker

    (Institute of Neuropathology, University Hospital Bonn)

  • Dennis Görlich

    (Institute of Biostatistics and Clinical Research, University of Münster)

  • Tanja Kuhlmann

    (University of Münster)

  • Guido Widman

    (University of Bonn)

  • Sebastian Herich

    (University of Münster)

  • Christiane Elpers

    (Children’s Hospital of the University Medical Center, University of Münster)

  • Nico Melzer

    (University of Münster)

  • Klaus Dornmair

    (Institute for Clinical Neuroimmunology, Ludwig-Maximilians-University Munich
    Biomedical Center, Ludwig-Maximilians-University Munich
    Munich Cluster for Systems Neurology (SyNergy), Ludwig-Maximilians-University Munich)

  • Gerhard Kurlemann

    (Children’s Hospital of the University Medical Center, University of Münster)

  • Heinz Wiendl

    (University of Münster)

  • Nicholas Schwab

    (University of Münster)

Abstract

Rasmussen encephalitis (RE) is a rare paediatric epilepsy with uni-hemispheric inflammation and progressive neurological deficits. To elucidate RE immunopathology, we applied T-cell receptor (TCR) sequencing to blood (n=23), cerebrospinal fluid (n=2) and brain biopsies (n=5) of RE patients, and paediatric controls. RE patients present with peripheral CD8+ T-cell expansion and its strength correlates with disease severity. In addition, RE is the only paediatric epilepsy with prominent T-cell expansions in the CNS. Consistently, common clones are shared between RE patients, who also share MHC-I alleles. Public RE clones share Vβ genes and length of the CDR3. Rituximab/natalizumab/basiliximab treatment does not change TCR diversity, stem cell transplantation replaces the TCR repertoire with minimal overlap between donor and recipient, as observed in individual cases. Our study supports the hypothesis of an antigen-specific attack of peripherally expanded CD8+ lymphocytes against CNS structures in RE, which might be ameliorated by restricting access to the CNS.

Suggested Citation

  • Tilman Schneider-Hohendorf & Hema Mohan & Christian G. Bien & Johanna Breuer & Albert Becker & Dennis Görlich & Tanja Kuhlmann & Guido Widman & Sebastian Herich & Christiane Elpers & Nico Melzer & Kla, 2016. "CD8+ T-cell pathogenicity in Rasmussen encephalitis elucidated by large-scale T-cell receptor sequencing," Nature Communications, Nature, vol. 7(1), pages 1-14, September.
    • Handle: RePEc:nat:natcom:v:7:y:2016:i:1:d:10.1038_ncomms11153
    DOI: 10.1038/ncomms11153
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