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Spongiform encephalopathies are the group of neurodegenerative infectious diseases recognised in humans and animals. One of these - Creutzfeld-Jakob disease (CJD) is caused by unconventional infectious factors defined as a prion proteins, infectious protein molecules devoid of nucleic acid. In recent years epidemic of bovine spongiform encephalopathy, reported in UK created fear against bovine-human transmission of the disease. First described causes of human encephalopathy, probably due to the bovine-human transmission of bovine spongiform encephalopathy was defined as a new variant of the Creutzfeld-Jakob disease (nvCJD). Our research presents chosen matters according to the prion diseases with taking into consideration the Creutzfeld-Jakob disease and its new variant (nvCJD).