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- 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).