[Prion diseases].

PRION DISEASES. Prion diseases or subacute transmissible spongiform encephalopathies (TSE) are neurodegenerative diseases. The agent responsible for the disease and its transmission is a protein with an abnormal conformation, derived from a normal protein: the PrP protein. TSE exist in several forms: sporadic Creutzfeldt-Jakob disease (CJD) (the most common), genetic and iatrogenic forms. Variant CJD is the only form linked to bovine spongiform encephalopathy. Despite therapeutic trials carried out since the early 2000s, no treatment has yet demonstrated its effectiveness.