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CD8(+) T-cell pathogenicity in Rasmussen encephalitis elucidated by large-scale T-cell receptor sequencing
Tilman Schneider-Hohendorf1, Hema Mohan1, Christian G Bien2
1Department of Neurology, University of Münster, 48149 Münster, Germany.
Nature Communications
|April 5, 2016
Summary
Rasmussen encephalitis involves T-cell attacks on the brain. Researchers found specific CD8(+) T-cell expansions in patients, suggesting an antigen-driven autoimmune response targeting central nervous system structures.
Area of Science:
- Neuroimmunology
- Epileptology
- Immunogenetics
Background:
- Rasmussen encephalitis (RE) is a rare pediatric epilepsy characterized by unilateral brain inflammation and progressive neurological decline.
- Understanding the immunopathogenesis of RE is crucial for developing targeted therapies.
Purpose of the Study:
- To investigate the T-cell receptor (TCR) repertoire in Rasmussen encephalitis patients.
- To elucidate the role of T-cells in the immunopathology of RE.
Main Methods:
- T-cell receptor (TCR) sequencing was performed on blood, cerebrospinal fluid, and brain biopsies from RE patients and pediatric controls.
- Analysis focused on T-cell expansions, clonal sharing, and TCR repertoire characteristics.
Main Results:
- RE patients exhibited significant peripheral CD8(+) T-cell expansions that correlated with disease severity.
- Prominent T-cell expansions within the central nervous system (CNS) were unique to RE among pediatric epilepsies.
- Common TCR clones were identified among RE patients, particularly those sharing MHC-I alleles, suggesting an antigen-specific response.
- Treatments like Rituximab did not alter TCR diversity, while stem cell transplantation led to a near-complete replacement of the TCR repertoire.
Conclusions:
- The findings support a hypothesis of an antigen-specific autoimmune attack mediated by peripherally expanded CD8(+) T-lymphocytes against CNS structures in RE.
- Restricting T-cell access to the CNS may represent a potential therapeutic strategy for Rasmussen encephalitis.
