Update: Dura Mater Graft-Associated Creutzfeldt-Jakob Disease - Japan, 1975-2017

What is already known about this topic? During 1975-2008, a total of 132 cases of dura mater graft-associated Creutzfeldt-Jakob disease (dCJD), a fatal neurodegenerative disease caused by replicating, transmissible prion proteins, had been identified in Japan and accounted for >60% of patients worldwide with dCJD. This relatively high number of cases was most likely related to the increased use in Japan of the primary vehicle of transmission, Lyodura brand cadaveric grafts produced before May 1987, when the manufacturer changed its production process to reduce the risk for prion transmission. What is added by this report? During 2008-2017, an additional 22 dCJD patients, with onset from 1985 through 2016, were identified in Japan, resulting in 154 dCJD patients in Japan. No new dCJD patient whose surgery occurred after 1993 has been identified. However, the latency period is now known to be at least 30 years and because of the known potential for even longer latency periods for prion diseases, this outbreak is likely to continue. What are the implications for public health practice? The dCJD outbreak underscores the importance of strict screening of donors, appropriate record keeping, avoidance of comingling of grafts, and ideally, the use of validated sterilization procedures whenever dura mater grafts are manufactured. The long latency (decades) of human prion diseases can pose challenges to the detection of new sources of infection and highlights the need to recognize prion disease outbreaks and implement preventive measures as early as possible. © 2018, Massachusetts Medical Society. All rights reserved.