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000278073 1001_ $$aDi Liberto, Giovanni$$b0
000278073 245__ $$aNeuronal pSTAT1 hallmarks synaptic pathology in autoimmune encephalitis against intracellular antigens.
000278073 260__ $$aHeidelberg$$bSpringer$$c2025
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000278073 520__ $$aAutoimmune encephalitis (AE) is an inflammatory syndrome of the central nervous system (CNS) triggered by aberrant immune responses against neuronal intracellular (IC-AE) or surface (NS-AE) autoantigens. The resulting neuronal alterations and clinical trajectories differ, with IC-AE often leading to fatal outcomes. Unfortunately, the scarce availability of tissue from AE cases has hampered systematic analyses that would allow an understanding of the pathogenesis underlying neuronal alterations in T cell-mediated AE syndromes. Here, we assembled a cohort comprising both NS-AE (n = 8) and IC-AE (n = 12) from multiple institutions to delineate key histopathological features that distinguish neuronal pathology between IC-AE and NS-AE. In contrast to NS-AE, IC-AE lesions present a prominent neuronal pSTAT1 signature, accompanied by a high proportion of brain-resident memory CD8 + T cells and neurodegenerative GPNMB + phagocytes which show synaptic engulfment with little C3-complement deposition. Our findings highlight distinct histopathological features of IC-AE compared to NS-AE, providing actionable biomarkers for diagnostics and treatment strategies.
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000278073 650_7 $$2Other$$aNeurodegeneration
000278073 650_7 $$2Other$$aNeuroinflammation
000278073 650_7 $$2Other$$aPhagocytes
000278073 650_7 $$2Other$$aResident memory T cells
000278073 650_7 $$2Other$$aSynapses
000278073 650_7 $$2NLM Chemicals$$aSTAT1 Transcription Factor
000278073 650_7 $$2NLM Chemicals$$aAutoantigens
000278073 650_2 $$2MeSH$$aHumans
000278073 650_2 $$2MeSH$$aEncephalitis: pathology
000278073 650_2 $$2MeSH$$aEncephalitis: immunology
000278073 650_2 $$2MeSH$$aEncephalitis: metabolism
000278073 650_2 $$2MeSH$$aFemale
000278073 650_2 $$2MeSH$$aMale
000278073 650_2 $$2MeSH$$aMiddle Aged
000278073 650_2 $$2MeSH$$aNeurons: pathology
000278073 650_2 $$2MeSH$$aNeurons: immunology
000278073 650_2 $$2MeSH$$aNeurons: metabolism
000278073 650_2 $$2MeSH$$aHashimoto Disease: pathology
000278073 650_2 $$2MeSH$$aHashimoto Disease: immunology
000278073 650_2 $$2MeSH$$aAdult
000278073 650_2 $$2MeSH$$aSTAT1 Transcription Factor: metabolism
000278073 650_2 $$2MeSH$$aSynapses: pathology
000278073 650_2 $$2MeSH$$aSynapses: immunology
000278073 650_2 $$2MeSH$$aSynapses: metabolism
000278073 650_2 $$2MeSH$$aAged
000278073 650_2 $$2MeSH$$aAutoantigens: immunology
000278073 650_2 $$2MeSH$$aBrain: pathology
000278073 650_2 $$2MeSH$$aBrain: immunology
000278073 650_2 $$2MeSH$$aCD8-Positive T-Lymphocytes: immunology
000278073 650_2 $$2MeSH$$aCD8-Positive T-Lymphocytes: pathology
000278073 650_2 $$2MeSH$$aYoung Adult
000278073 7001_ $$aEgervari, Kristof$$b1
000278073 7001_ $$aVogrig, Alberto$$b2
000278073 7001_ $$aSpatola, Marianna$$b3
000278073 7001_ $$aPiccinno, Margot$$b4
000278073 7001_ $$aVincenti, Ilena$$b5
000278073 7001_ $$aWagner, Ingrid$$b6
000278073 7001_ $$aKreutzfeldt, Mario$$b7
000278073 7001_ $$aEndmayr, Verena$$b8
000278073 7001_ $$aOstertag, Karoline$$b9
000278073 7001_ $$aRahimi, Jasmin$$b10
000278073 7001_ $$aVicino, Alex$$b11
000278073 7001_ $$0P:(DE-2719)9003515$$aPröbstel, Anne-Katrin$$b12$$udzne
000278073 7001_ $$aMeyronet, David$$b13
000278073 7001_ $$aFrank, Stephan$$b14
000278073 7001_ $$aPrinz, Marco$$b15
000278073 7001_ $$aHewer, Ekkehard$$b16
000278073 7001_ $$aBrouland, Jean-Philippe$$b17
000278073 7001_ $$ade Leval, Laurence$$b18
000278073 7001_ $$aParkkinen, Laura$$b19
000278073 7001_ $$aDraganski, Bogdan$$b20
000278073 7001_ $$aDesestret, Virginie$$b21
000278073 7001_ $$aDubey, Divyanshu$$b22
000278073 7001_ $$aPittock, Sean J$$b23
000278073 7001_ $$aRoemer, Shanu F$$b24
000278073 7001_ $$aDickson, Dennis W$$b25
000278073 7001_ $$aHöftberger, Romana$$b26
000278073 7001_ $$aIrani, Sarosh R$$b27
000278073 7001_ $$aHonnorat, Jérôme$$b28
000278073 7001_ $$aDu Pasquier, Renaud$$b29
000278073 7001_ $$aMerkler, Doron$$b30
000278073 773__ $$0PERI:(DE-600)1458410-4$$a10.1007/s00401-025-02882-7$$gVol. 149, no. 1, p. 35$$n1$$p35$$tActa neuropathologica$$v149$$x0001-6322$$y2025
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