Via practica 12/2007

PRION DISEASES OF MAN (WITH EMPHASIS ON THE CREUTZFELDT-JAKOB DISEASE)

Prion diseases – transmissible spongiform encephalopathies form a group of lethal neurodegenerative ailments of man and some animal species. Prion diseases are transmissible (but not contagious) and simultaneously controlled by genetic predisposition. The prion gene mutations play an important role in the development of the genetic form of the disease. In the target system, the central nervous system emerges and replicates the prion, the pathologic izoform of the prion protein, physiologically present in the membrane of nervous cells. Prions are extraordinary resistant against the exogennous influences, including the conventional sterilization. The most important prion disease is the Creutzfeldt-Jakob disease, known to occur in three forms: sporadic (idiopathic), genetic and iatrogennic. During a large epizootic the newly described bovinne spongioform encephalopathy, were observed rare cases of the disease transmissions from cows to man. Its clinical picture is described as the variant Creutzfeldt-Jakob disease.

Keywords: prions, diseases, man, genetics, iatrogennic transmission.