Via practica 10/2008

TRANSMISSIVE DEMENTIAS – PRION DISEASES

Prion diseases (PD) – transmissible spongioform encephalopathies (TSE) – are a relatively new (old-new) group of degenerative CNS disorders with unusual chronic interaction between immune system and the presumed etiologic PD (prion diseases) factor – prions (proteinaceous infectious particles), which after peripheral (extraneural) infection replicates in the reticulo-endothelial system, with subsequent spreading into brain and spinal cord structures, where, by the mechanism(-s) at present still unknown, causes neurocyte and glial cell vacuolisation and spongiomatous neuropil restructuralisation, neurocyte restriction, and astrogliosis without a marked presence of inflammation reaction.

Keywords: prion diseases, cognition disorders, dementia, transmissive spongioform encephalopathies, point gene mutation, recombinant synthetic prion.