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Projection of Creutzfeldt-Jakob Disease Frequency Based on Cadaveric Dura Transplantation in Japan

Background: Projections of the size of the Creutzfeldt-Jakob disease (CJD) epidemic are of great importance for future health care and understanding of the natural history of this disease. Objective: We have estimated the incubation period and have projected the number of patients with iatrogenic CJD in Japan, using 75 patients who were transplanted with cadaveric dura mater grafts between 1979 and 1991 and developed CJD by the end of 2000. Methods: Several plausible scenarios, combinations of incubation period distribution forms, competing risks and exposure distributions were evaluated for the projections using data from the Japanese Ministry of Health, Labor and Welfare. Results: The range of median incubation periods from the time of dura mater transplantation to the onset of the disease was 10.8–34.3 years, and the range of the number of new CJD patients between 2001 and 2020 was 21–84. Conclusions: Since the potential incubation period was estimated to be considerably long, as few as 21 and as many as 84 new patients may develop CJD by 2020.