TDP-43 pathology in subacute sclerosing panencephalitis.

Autor:	Acewicz A; Department of Neuropathology, Institute of Psychiatry and Neurology, Warsaw, Poland., Stępień T; Department of Neuropathology, Institute of Psychiatry and Neurology, Warsaw, Poland., Grzegorczyk M; Department of Descriptive and Clinical Anatomy, Medical University of Warsaw, Warsaw, Poland., Ostrowski RP; Department of Neurooncology, Mossakowski Medical Research Institute, Polish Academy of Sciences, Warsaw, Poland., Tarka S; Department of Forensic Medicine, Medical University of Warsaw, Warsaw, Poland., Felczak P; Department of Neuropathology, Institute of Psychiatry and Neurology, Warsaw, Poland., Wierzba-Bobrowicz T; Department of Neuropathology, Institute of Psychiatry and Neurology, Warsaw, Poland.
Jazyk:	angličtina
Zdroj:	Journal of neuropathology and experimental neurology [J Neuropathol Exp Neurol] 2024 Mar 20; Vol. 83 (4), pp. 251-257.
DOI:	10.1093/jnen/nlae017
Abstrakt:	Subacute sclerosing panencephalitis (SSPE) is a fatal, slowly progressive brain disorder caused by a mutated measles virus. Both subacute inflammatory and neurodegenerative mechanisms appear to play significant roles in the pathogenesis. TAR DNA-binding protein 43 (TDP-43) inclusions are a common co-pathology in several neurodegenerative disorders with diverse pathogenesis. In the present study, we examined brains of 16 autopsied SSPE patients for the presence of TDP-43 pathology and possible associations with tau pathology. Immunohistochemical staining identified TDP-43 inclusions in 31% of SSPE cases. TDP-43 pathology was widely distributed in the brains, most severely in the atrophied cerebral cortex (temporal and parietal), and most frequently as tangle- and thread-like neuronal cytoplasmic inclusions. It was associated with longer disease duration (>4 years) and tau pathology (all TDP-43-positive cases had tau-positive neurofibrillary tangles). This study demonstrates for the first time an association between TDP-43 pathology and SSPE. The co-occurrence of TDP-43 and tau aggregates and correlation with the disease duration suggest that both pathological proteins are involved in the neurodegenerative process induced by viral inflammation. (© The Author(s) 2024. Published by Oxford University Press on behalf of American Association of Neuropathologists, Inc. All rights reserved. For permissions, please email: journals.permissions@oup.com.)
Databáze:	MEDLINE
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