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 PrP^c (for Common or Cellular), whereas the misfolded protein is PrP^sc (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].