Neuronal pSTAT1 hallmarks synaptic pathology in autoimmune encephalitis against intracellular antigens DOI Creative Commons
Giovanni Di Liberto, Kristóf Égervári, Alberto Vogrig

et al.

Acta Neuropathologica, Journal Year: 2025, Volume and Issue: 149(1)

Published: April 25, 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 alterations and clinical trajectories differ, with IC-AE often leading to fatal outcomes. Unfortunately, scarce availability tissue from AE cases has hampered systematic analyses that would allow understanding pathogenesis underlying in T cell-mediated syndromes. Here, we assembled a cohort comprising both NS-AE (n = 8) 12) multiple institutions delineate key histopathological features distinguish pathology between NS-AE. In contrast NS-AE, lesions present prominent pSTAT1 signature, accompanied high proportion brain-resident memory CD8 + cells neurodegenerative GPNMB phagocytes which show synaptic engulfment little C3-complement deposition. Our findings highlight distinct compared providing actionable biomarkers for diagnostics treatment strategies.

Language: Английский

Neuronal pSTAT1 hallmarks synaptic pathology in autoimmune encephalitis against intracellular antigens DOI Creative Commons
Giovanni Di Liberto, Kristóf Égervári, Alberto Vogrig

et al.

Acta Neuropathologica, Journal Year: 2025, Volume and Issue: 149(1)

Published: April 25, 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 alterations and clinical trajectories differ, with IC-AE often leading to fatal outcomes. Unfortunately, scarce availability tissue from AE cases has hampered systematic analyses that would allow understanding pathogenesis underlying in T cell-mediated syndromes. Here, we assembled a cohort comprising both NS-AE (n = 8) 12) multiple institutions delineate key histopathological features distinguish pathology between NS-AE. In contrast NS-AE, lesions present prominent pSTAT1 signature, accompanied high proportion brain-resident memory CD8 + cells neurodegenerative GPNMB phagocytes which show synaptic engulfment little C3-complement deposition. Our findings highlight distinct compared providing actionable biomarkers for diagnostics treatment strategies.

Language: Английский

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