THE ROLE OF THE IMMUNE-SYSTEM IN THE PRION DISEASES

The transmissible spongiform encephalopathies appear as neurodegenerat ive diseases of the central nervous system, without associated inflamm atory syndrome, with a double genetic and infectious determinism. Neve rtheless recent observations emphasise the importance of the immune sy stem in the pathophysiology of these diseases, both in the initial rep lication of the agent at the peripheral level and in the appearance of cerebral lesions. These data which run contrary to a direct role for the PrPSc in the development of brain damage provide new information c oncerning the transmission of these disease.