Neuropathology of prion diseases
Abstract
In prion diseases, neuropathology has remained the most important tool to give a definite diagnosis, and neuropathological research has contributed significantly to our current pathogenetic understanding. Immunohistochemistry for the disease-associated prion protein (PrP(Sc)) is indispensable for the neuropathological confirmation of prion diseases. The amount and distribution of PrP(Sc) deposits do not always correlate with type and severity of local tissue damage. PrP(Sc) deposition occurs only where neuronal parenchyma is present; in scarred infarctions with prominent gliosis, PrP(Sc) does not accumulate. Early, severe and selective loss affects a subset of inhibitory GABAergic neurons both in human and experimental prion diseases. The central pathogenetic cascade includes oxidative stress to neurons and their apoptosis. New patterns of PrP(Sc) immunoreactivity include granular ganglionic and tiny adaxonal PrP(Sc) deposits in peripheral nervous tissue, and dendritic cells and macrophages in vessel walls, suggesting that mobile haematogenous cells may be involved in spread of prions.
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The heterogeneity of PrPSc deposition patterns is higher in human prion diseases and comprises synaptic, perivacuolar, plaque-like, kuru-plaque, florid-plaque, punctuate, perineuronal, and intraneuronal patterns, which might occur side-by-side or even overlapping in the same patient.
“Although the deposition patterns of misfolded PrPSc are diverse in murine models of prion disease, the heterogeneity of PrPSc deposition patterns is higher in human prion diseases and comprises synaptic, perivacuolar, plaque-like, kuru-plaque, florid-plaque, punctuate, perineuronal, and intraneuronal patterns, which might occur side-by-side or even overlapping in the same patient [119,120].”
From Loss of Homeostatic Microglia Signature in Prion Diseases (2022) · cites this paper as [119]
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- Authors
- Budka, H.
- Affiliations
- University of Vienna (AT)
- Corresponding author
- Budka, Herbert
- ORCID
- Budka, Herbert
- Venue
- British Medical Bulletin
- Publisher
- Oxford University Press (OUP)
- Published
- 2003-06-01
- Volume
- 66
- Issue
- 1
- Pages
- 121-130
- ISSN
- 0007-1420 (ISSN-L)
- 1471-8391
- OpenAlex
- W2150554290
- Cited by
- 196
- References
- 56
- Citations by year
- 2026: 3 · 2025: 9 · 2024: 1 · 2023: 5 · 2022: 9 · 2021: 4 · 2020: 9 · 2019: 7 · 2018: 4 · 2017: 8 · 2016: 10 · 2015: 10 · 2014: 9 · 2013: 9 · 2012: 10
- FWCI
- 0.98
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- British Medical Bulletin (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Bundesministerium für Gesundheit und Frauen
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
- Keywords
- prion disease, neuropathology, oxidative stress, neuronal apoptosis, dendritic cells, macrophages, pathogenesis
- MeSH terms
- Animals, Brain, Humans, Immunohistochemistry, Neurons, Prions, Species Specificity, Prion Diseases, Peripheral Nervous System
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