Prion Disease

Ophelia Yin, Chiadi Onyike‎, M.D.
Prion Disease is a topic covered in the Johns Hopkins Psychiatry Guide.

To view the entire topic, please or .

Official website of the Johns Hopkins Antibiotic (ABX), HIV, Diabetes, and Psychiatry Guides, powered by Unbound Medicine. Johns Hopkins Guide App for iOS, iPhone, iPad, and Android included. Explore these free sample topics:

-- The first section of this topic is shown below --

DEFINITION

  • 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)[1].

-- To view the remaining sections of this topic, please or --

DEFINITION

  • 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)[1].

There's more to see -- the rest of this entry is available only to subscribers.

Last updated: February 26, 2017