Prion diseases are fatal, progressive, neurodegenerative diseases caused by accumulation of an abnormal isoform of the cellular prion protein in and around neurons leading to synaptic dysfunction and eventual neuronal loss.1 The most common type is sporadic Creutzfeldt-Jakob disease (sCJD) which accounts for ∼85% of the annual incidence of prion disease. This evidence concerns the gene PRNP and prion disease.