Prion Disease
DEFINITION
- Prion diseases are rare and invariably fatal neurodegenerative disorders caused by transmissible misfolded prion protein, or "PrP." The endogenous, properly folded form is PrPc (for Common or Cellular), whereas the misfolded protein is PrPsc (for SCrapie).
- Collectively these are known as transmissible spongiform encephalopathies. They primarily affect the central nervous system of humans and other mammals.
- The conditions may be hereditary, idiopathic, or acquired through exposure to affected tissues or contaminated surgical instruments.
- Discussed here are Creutzfeldt-Jakob disease, variant Creutzfeldt-Jakob disease, kuru, Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia, and variably protease-sensitive prionopathy.
- Cognitive impairment due to prion disease is classified under the neurocognitive disorders (NCDs) section of the Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (DSM-5)[1].
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Last updated: September 27, 2025
Citation
Gary, Joseph, et al. "Prion Disease." Johns Hopkins Psychiatry Guide, The Johns Hopkins University, 2025. Johns Hopkins Guides, www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787113/all/Prion_Disease.
Gary J, Yin O, Onyike C. Prion Disease. Johns Hopkins Psychiatry Guide. The Johns Hopkins University; 2025. https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787113/all/Prion_Disease. Accessed October 16, 2025.
Gary, J., Yin, O., & Onyike, C. (2025). Prion Disease. In Johns Hopkins Psychiatry Guide. The Johns Hopkins University. https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787113/all/Prion_Disease
Gary J, Yin O, Onyike C. Prion Disease [Internet]. In: Johns Hopkins Psychiatry Guide. The Johns Hopkins University; 2025. [cited 2025 October 16]. Available from: https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787113/all/Prion_Disease.
* Article titles in AMA citation format should be in sentence-case
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T1 - Prion Disease
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AU - Yin,Ophelia,M.D.
AU - Onyike,Chiadi,M.D.
Y1 - 2025/09/27/
BT - Johns Hopkins Psychiatry Guide
UR - https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787113/all/Prion_Disease
PB - The Johns Hopkins University
DB - Johns Hopkins Guides
DP - Unbound Medicine
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