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001     278073
005     20250504001227.0
024 7 _ |a 10.1007/s00401-025-02882-7
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024 7 _ |a pmid:40278930
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024 7 _ |a pmc:PMC12031792
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024 7 _ |a 0001-6322
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024 7 _ |a 1432-0533
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037 _ _ |a DZNE-2025-00564
041 _ _ |a English
082 _ _ |a 610
100 1 _ |a Di Liberto, Giovanni
|b 0
245 _ _ |a Neuronal pSTAT1 hallmarks synaptic pathology in autoimmune encephalitis against intracellular antigens.
260 _ _ |a Heidelberg
|c 2025
|b Springer
336 7 _ |a article
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336 7 _ |a ARTICLE
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520 _ _ |a 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.
536 _ _ |a 353 - Clinical and Health Care Research (POF4-353)
|0 G:(DE-HGF)POF4-353
|c POF4-353
|f POF IV
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588 _ _ |a Dataset connected to CrossRef, PubMed, , Journals: pub.dzne.de
650 _ 7 |a Neurodegeneration
|2 Other
650 _ 7 |a Neuroinflammation
|2 Other
650 _ 7 |a Phagocytes
|2 Other
650 _ 7 |a Resident memory T cells
|2 Other
650 _ 7 |a Synapses
|2 Other
650 _ 7 |a STAT1 Transcription Factor
|2 NLM Chemicals
650 _ 7 |a Autoantigens
|2 NLM Chemicals
650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a Encephalitis: pathology
|2 MeSH
650 _ 2 |a Encephalitis: immunology
|2 MeSH
650 _ 2 |a Encephalitis: metabolism
|2 MeSH
650 _ 2 |a Female
|2 MeSH
650 _ 2 |a Male
|2 MeSH
650 _ 2 |a Middle Aged
|2 MeSH
650 _ 2 |a Neurons: pathology
|2 MeSH
650 _ 2 |a Neurons: immunology
|2 MeSH
650 _ 2 |a Neurons: metabolism
|2 MeSH
650 _ 2 |a Hashimoto Disease: pathology
|2 MeSH
650 _ 2 |a Hashimoto Disease: immunology
|2 MeSH
650 _ 2 |a Adult
|2 MeSH
650 _ 2 |a STAT1 Transcription Factor: metabolism
|2 MeSH
650 _ 2 |a Synapses: pathology
|2 MeSH
650 _ 2 |a Synapses: immunology
|2 MeSH
650 _ 2 |a Synapses: metabolism
|2 MeSH
650 _ 2 |a Aged
|2 MeSH
650 _ 2 |a Autoantigens: immunology
|2 MeSH
650 _ 2 |a Brain: pathology
|2 MeSH
650 _ 2 |a Brain: immunology
|2 MeSH
650 _ 2 |a CD8-Positive T-Lymphocytes: immunology
|2 MeSH
650 _ 2 |a CD8-Positive T-Lymphocytes: pathology
|2 MeSH
650 _ 2 |a Young Adult
|2 MeSH
700 1 _ |a Egervari, Kristof
|b 1
700 1 _ |a Vogrig, Alberto
|b 2
700 1 _ |a Spatola, Marianna
|b 3
700 1 _ |a Piccinno, Margot
|b 4
700 1 _ |a Vincenti, Ilena
|b 5
700 1 _ |a Wagner, Ingrid
|b 6
700 1 _ |a Kreutzfeldt, Mario
|b 7
700 1 _ |a Endmayr, Verena
|b 8
700 1 _ |a Ostertag, Karoline
|b 9
700 1 _ |a Rahimi, Jasmin
|b 10
700 1 _ |a Vicino, Alex
|b 11
700 1 _ |a Pröbstel, Anne-Katrin
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700 1 _ |a Meyronet, David
|b 13
700 1 _ |a Frank, Stephan
|b 14
700 1 _ |a Prinz, Marco
|b 15
700 1 _ |a Hewer, Ekkehard
|b 16
700 1 _ |a Brouland, Jean-Philippe
|b 17
700 1 _ |a de Leval, Laurence
|b 18
700 1 _ |a Parkkinen, Laura
|b 19
700 1 _ |a Draganski, Bogdan
|b 20
700 1 _ |a Desestret, Virginie
|b 21
700 1 _ |a Dubey, Divyanshu
|b 22
700 1 _ |a Pittock, Sean J
|b 23
700 1 _ |a Roemer, Shanu F
|b 24
700 1 _ |a Dickson, Dennis W
|b 25
700 1 _ |a Höftberger, Romana
|b 26
700 1 _ |a Irani, Sarosh R
|b 27
700 1 _ |a Honnorat, Jérôme
|b 28
700 1 _ |a Du Pasquier, Renaud
|b 29
700 1 _ |a Merkler, Doron
|b 30
773 _ _ |a 10.1007/s00401-025-02882-7
|g Vol. 149, no. 1, p. 35
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|t Acta neuropathologica
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910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
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