TDP-43 pathology in subacute sclerosing panencephalitis
Albert Acewicz1, Tomasz Stępień1, Michał Grzegorczyk2
1Department of Neuropathology, Institute of Psychiatry and Neurology, Warsaw, Poland.
Journal of Neuropathology and Experimental Neurology
|March 8, 2024
Summary
Subacute sclerosing panencephalitis (SSPE), a fatal brain disorder, shows a link with TAR DNA-binding protein 43 (TDP-43) pathology. This finding suggests TDP-43 and tau protein aggregates contribute to neurodegeneration in SSPE.
Area of Science:
- Neurology
- Virology
- Pathology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a fatal neurological disorder caused by a mutated measles virus.
- Both inflammation and neurodegeneration are key in SSPE pathogenesis.
- TAR DNA-binding protein 43 (TDP-43) inclusions are observed in various neurodegenerative diseases.
Purpose of the Study:
- To investigate the presence of TDP-43 pathology in SSPE brain samples.
- To explore potential associations between TDP-43 pathology and tau pathology in SSPE.
Main Methods:
- Autopsied brain tissue from 16 SSPE patients was analyzed.
- Immunohistochemical staining was used to detect TDP-43 and tau pathologies.
Main Results:
- TDP-43 inclusions were identified in 31% of SSPE cases.
- TDP-43 pathology was prevalent in the cerebral cortex and associated with longer disease duration (>4 years).
- All cases with TDP-43 pathology also exhibited tau pathology (neurofibrillary tangles).
Conclusions:
- This study establishes a novel association between TDP-43 pathology and SSPE.
- The co-occurrence of TDP-43 and tau aggregates suggests their involvement in SSPE-induced neurodegeneration.
- Viral-induced inflammation may trigger pathological protein aggregation in SSPE.
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