人朊病毒病的发病率是全世界每年 1-2/100 万人。[2]Will RG, Alperovitch A, Poser S, et al. Descriptive epidemiology of Creutzfeldt-Jakob disease in six European countries, 1993-1995. EU Collaborative Study Group for CJD. Ann Neurol. 1998 Jun;43(6):763-7.http://www.ncbi.nlm.nih.gov/pubmed/9629846?tool=bestpractice.com[19]Belay ED, Holman RC, Schonberger LB. Creutzfeldt-Jakob disease surveillance and diagnosis. Clin Infect Dis. 2005 Sep 15;41(6):834-6.http://www.ncbi.nlm.nih.gov/pubmed/16107982?tool=bestpractice.comThe National Creutzfeldt-Jakob Disease Research and Surveillance Unit 然而,考虑发病的平均年龄为 65 到 69 岁,个人终身风险约为 1/30,000 到 1/60,000。相比散发性或遗传性克雅氏病,变异型克雅氏病通常出现在较年轻的人中,平均年龄近 30 岁。[1]Mastrianni JA, Roos RP. The prion diseases. Semin Neurol. 2000;20(3):337-52.http://www.ncbi.nlm.nih.gov/pubmed/11051298?tool=bestpractice.com[11]Will RG, Zeidler M, Stewart GE, et al. Diagnosis of new variant Creutzfeldt-Jakob disease. Ann Neurol. 2000 May;47(5):575-82.http://www.ncbi.nlm.nih.gov/pubmed/10805327?tool=bestpractice.com 大约 85%-90% 的朊蛋白病例为散发性,10%-15% 为遗传性,<1% 为获得性。[5]Prusiner SB. Prions. Proc Natl Acad Sci U S A. 1998 Nov 10;95(23):13363-83.http://www.pnas.org/content/95/23/13363.fullhttp://www.ncbi.nlm.nih.gov/pubmed/9811807?tool=bestpractice.com