- Prion diseases are rare and invariably fatal neurodegenerative disorders caused by transmissible misfolded prion proteins.
- The "prion protein" that causes all known mammalian prion diseases is a misfolded form of protease-resistant protein (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.
- Discussed here are Creutzfeldt-Jakob disease, variant Creutzfeldt-Jakob disease, kuru, Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia.
- The condition may be hereditary, idiopathic, or acquired through exposure to affected tissues or contaminated surgical instruments.
- 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).
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Last updated: February 26, 2017
Yin, Ophelia, and Chiadi Onyike. "Prion Disease." Johns Hopkins Psychiatry Guide, 2017. Johns Hopkins Guides, www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787113/all/Prion_Disease.
Yin O, Onyike C. Prion Disease. Johns Hopkins Psychiatry Guide. 2017. https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787113/all/Prion_Disease. Accessed June 1, 2023.
Yin, O., & Onyike, C. (2017). Prion Disease. In Johns Hopkins Psychiatry Guide https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787113/all/Prion_Disease
Yin O, Onyike C. Prion Disease [Internet]. In: Johns Hopkins Psychiatry Guide. ; 2017. [cited 2023 June 01]. Available from: https://www.hopkinsguides.com/hopkins/view/Johns_Hopkins_Psychiatry_Guide/787113/all/Prion_Disease.
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