There are 5 known human prion diseases, all of
which are exceedingly rare, untreatable, and fatal:
1) Kuru
2) Gerstmann-Str.ussler-Scheinker syndrome (GSS)
3) Fatal familial insomnia (FFI)
4) Creutzfeldt-Jakob disease (CJD)
5) Variant Creutzfeldt-Jakob disease (vCJD)
All of the prion diseases result in loss of neurons, glial cell proliferation, lack of an inflammatory response, and small vacuoles in the neuropil (dense neurofiber network). The pathogenesis is uncertain but is thought to involve accumulation of an abnormal protein called PrPSc, or Scrapie.
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