Epidemiology
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 Opens in 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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