The incidence of prion disease in humans is 1 to 2 per million per year worldwide.[2][19] The National Creutzfeldt-Jakob Disease Research and Surveillance Unit external link opens in a new window Taking into account that the mean and median age of onset is mid to late 60s, however, individual lifetime risk is about 1 in 30,000 to 1 in 60,000. Variant Creutzfeldt-Jakob disease tends to present in younger people than sporadic or genetic Creutzfeldt-Jakob disease, with an average age in the late 20s.[1][11] Approximately 85% to 90% of prion disease cases are sporadic, 10% to 15% are familial, and <1% are acquired.[5]

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