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@ARTICLE{DiLiberto:278073,
      author       = {Di Liberto, Giovanni and Egervari, Kristof and Vogrig,
                      Alberto and Spatola, Marianna and Piccinno, Margot and
                      Vincenti, Ilena and Wagner, Ingrid and Kreutzfeldt, Mario
                      and Endmayr, Verena and Ostertag, Karoline and Rahimi,
                      Jasmin and Vicino, Alex and Pröbstel, Anne-Katrin and
                      Meyronet, David and Frank, Stephan and Prinz, Marco and
                      Hewer, Ekkehard and Brouland, Jean-Philippe and de Leval,
                      Laurence and Parkkinen, Laura and Draganski, Bogdan and
                      Desestret, Virginie and Dubey, Divyanshu and Pittock, Sean J
                      and Roemer, Shanu F and Dickson, Dennis W and Höftberger,
                      Romana and Irani, Sarosh R and Honnorat, Jérôme and Du
                      Pasquier, Renaud and Merkler, Doron},
      title        = {{N}euronal p{STAT}1 hallmarks synaptic pathology in
                      autoimmune encephalitis against intracellular antigens.},
      journal      = {Acta neuropathologica},
      volume       = {149},
      number       = {1},
      issn         = {0001-6322},
      address      = {Heidelberg},
      publisher    = {Springer},
      reportid     = {DZNE-2025-00564},
      pages        = {35},
      year         = {2025},
      abstract     = {Autoimmune encephalitis (AE) is an inflammatory syndrome of
                      the central nervous system (CNS) triggered by aberrant
                      immune responses against neuronal intracellular (IC-AE) or
                      surface (NS-AE) autoantigens. The resulting neuronal
                      alterations and clinical trajectories differ, with IC-AE
                      often leading to fatal outcomes. Unfortunately, the scarce
                      availability of tissue from AE cases has hampered systematic
                      analyses that would allow an understanding of the
                      pathogenesis underlying neuronal alterations in T
                      cell-mediated AE syndromes. Here, we assembled a cohort
                      comprising both NS-AE (n = 8) and IC-AE (n = 12) from
                      multiple institutions to delineate key histopathological
                      features that distinguish neuronal pathology between IC-AE
                      and NS-AE. In contrast to NS-AE, IC-AE lesions present a
                      prominent neuronal pSTAT1 signature, accompanied by a high
                      proportion of brain-resident memory CD8 + T cells and
                      neurodegenerative GPNMB + phagocytes which show synaptic
                      engulfment with little C3-complement deposition. Our
                      findings highlight distinct histopathological features of
                      IC-AE compared to NS-AE, providing actionable biomarkers for
                      diagnostics and treatment strategies.},
      keywords     = {Humans / Encephalitis: pathology / Encephalitis: immunology
                      / Encephalitis: metabolism / Female / Male / Middle Aged /
                      Neurons: pathology / Neurons: immunology / Neurons:
                      metabolism / Hashimoto Disease: pathology / Hashimoto
                      Disease: immunology / Adult / STAT1 Transcription Factor:
                      metabolism / Synapses: pathology / Synapses: immunology /
                      Synapses: metabolism / Aged / Autoantigens: immunology /
                      Brain: pathology / Brain: immunology / CD8-Positive
                      T-Lymphocytes: immunology / CD8-Positive T-Lymphocytes:
                      pathology / Young Adult / Neurodegeneration (Other) /
                      Neuroinflammation (Other) / Phagocytes (Other) / Resident
                      memory T cells (Other) / Synapses (Other) / STAT1
                      Transcription Factor (NLM Chemicals) / Autoantigens (NLM
                      Chemicals)},
      cin          = {AG Pröbstel},
      ddc          = {610},
      cid          = {I:(DE-2719)1013045},
      pnm          = {353 - Clinical and Health Care Research (POF4-353)},
      pid          = {G:(DE-HGF)POF4-353},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:40278930},
      pmc          = {pmc:PMC12031792},
      doi          = {10.1007/s00401-025-02882-7},
      url          = {https://pub.dzne.de/record/278073},
}