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Prion disease

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Prion diseases, also known as transmissible spongiform encephalopathies, are any diseases believed to be caused by prions, or small protein-like infectious particles. These prions change other cellular proteins, breaking them down to form holes referred to as 'spongiform change' — this change results in a disruption of the function of neurons. They can be transmitted in many ways including through inheritance, biological warfare, organ transplants and even the eating of the flesh of infected animals.

All prion diseases begin with a very long incubation period followed immediately by a steep decline into dementia and death.

Examples of prion diseases that affect humans are Creutzfeldt-Jakob disease (CJD), kuru, fatal familial insomnia (FFI) and Gerstmann-Sträussler-Scheinker syndrome (GSS).

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