Prion diseases

The definition of prion diseases is spongiform encephalopathies whic are rare, invariably lethal and generally with a rapid development, caused by the amass in the nerve cells of a protein that normally is in the brain called “prion” (PRP), but in this case we find it transformed. The mutation, called prion scrapie from the sheep disease (PRPsc), originates the so called apoptosi of the neurons (inducement to suicide).

Classification of man prion diseases
Genetic forms

  1. CJD – Rapidly evolutive dementia, myoclonus, ataxia.
  2. FFI – Total insomnia, serious upset of the autonomic system.
  3. GSS – Progressive ataxia and late dementia.

Assimilated forms

  1. Kuru – Prion disease of historical interest, today it is passed away.
  2. iCJD – Transmission from man to man.
  3. vCJD – Transmission from animal to man (BSE)

Sporadic forms

  1. CJD
  2. FFI