Prion diseases are degenerative disorders of the central nervous system (CNS) caused by transmissible particles that contain a pathogenic isoform of the prion protein, a normal constituent of cell membranes. The most common human prion disease is Creutzfeldt–Jakob disease (CJD). Most cases are sporadic (sCJD), 10–15% of cases are inherited (genetic CJD, gCJD), and a small number may be transmitted by medical procedures (iatrogenic CJD) or diet as in new variant CJD [
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