Prion Diseases
All prion diseases cause fatal, progressive spongiform encephalopathies — neurodegenerative disorders characterised by vacuolation of brain tissue:
PRION DISEASES
- Definition: Rapidly progressive neurodegenerative disorders caused by aggregation and intercellular spread of misfolded prion protein (PrP)
- Forms: Sporadic · Familial · Transmitted
- Human examples: CJD · Gerstmann-Sträussler-Scheinker (GSS) · Fatal familial insomnia · Kuru
- Animal examples: Scrapie (sheep/goats) · BSE/mad cow (cattle) · Chronic wasting disease (deer/elk) · Mink-transmissible encephalopathy
- Morphologic → spongiform change (intracellular vacuoles in neurons & glia)
Read this in Neurodegenerative Prion Diseases: CJD, Pathology & Rapid Dementia
What our notes say
- All prion diseases cause fatal, progressive spongiform encephalopathies — neurodegenerative disorders characterised by vacuolation of brain tissue: (Medical Mycology Q&A Section B)
- Slow infection – long silent incubation then progressive fatal disease (prion diseases, SSPE from measles) (Essential Medical Virology: Structure, Classification & Replication)
- Transmissible Spongiform Encephalopathies (TSEs) — Prion Diseases (EXAM: Basic Virology, Viral Replication, Prions & Slow Viruses, Poxviruses & VZV)
- Originally Called Slow Virus — Now Known to be Prion Diseases (EXAM: Basic Virology, Viral Replication, Prions & Slow Viruses, Poxviruses & VZV)
- Examples: HIV (years before AIDS), prion diseases (decades) (EXAM: Influenza, Viral Receptors & Spikes, Interferons, and Viral Pathogenesis)
- | Prion diseases | Rapidly progressive dementia | Kuru plaques · diffuse PrPsc | PrP (PRNP) | (Neurodegenerative Prion Diseases: CJD, Pathology & Rapid Dementia)
Study material on Prion Diseases
- Neurodegenerative Prion Diseases: CJD, Pathology & Rapid Dementia
- EXAM: Basic Virology, Viral Replication, Prions & Slow Viruses, Poxviruses & VZV