[Creutzfeldt-Jakob disease--the past or the future]

Abstract

Some recent views on ethiopathogenesis and epidemiology of four main forms of CJD, based on up to-day experiences and expectations for the future, are presented. The sporadic form of the disease (sCJD) displays a stable morbidity--ca. 1 case/1 million population yearly. The reasons of its so constant appearance remain still unknown. The familial forms of CJD (fCJD) depending upon more than 40 mutations in PRNP gene known today are inherited as autosomal dominant train. The clinical and neuropathological phenotype of patients belonging to various families are usually very different. The best epidemiological prognosis is attributed to iatrogenic form of CJD (iCJD), since both the medical errors causative of the disease and methods of avoiding of them are now very good recognized. The serious fears in many countries raises variant CJD (vCJD), connected etiologically with BSE, because of unknown duration of its incubation period and regular chronic involvement by prions reticulo-lymphatic tissue in infected persons.