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

Abstract

Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder that, according to the most well accepted hypothesis (1), is caused by replicating, transmissible, abnormal forms of a host-encoded prion protein (prions). Most CJD cases occur spontaneously (sporadic CJD) or are inherited (genetic CJD). Iatrogenic CJD can occur after exposure to prion-contaminated instruments or products in medical/surgical settings. Cadaveric dura mater graft-associated CJD (dCJD) accounts for a common form of iatrogenic CJD. This report summarizes the epidemiologic features of 154 cases of dCJD identified in Japan during 1975-2017; these cases account for >60% of dCJD cases reported worldwide (1,2). The unusually high prevalence of dCJD in Japan was first reported in 1997 (3). In 2008, a single brand of graft (Lyodura [B. Braun Melsungen AG, Melsungen, Germany]), frequently used as a patch in neurosurgical procedures, was identified as the probable vehicle of transmission (4). No international recall of the implicated Lyodura occurred, the product had a relatively long shelf life, and the grafts were used frequently in Japanese patients with non-life-threatening conditions (4,5). Since 2008, additional cases have been ascertained, reflecting the identification of previously missed cases and the occurrence of new cases with longer latency periods (interval from exposure to symptom onset) for dCJD (up to 30 years), underscoring the importance of maintaining surveillance for dCJD.

Figure 1

Number of cases (N = 154) of dura mater graft–associated Creutzfeldt-Jakob disease (dCJD), by year of neurosurgical procedure and year of symptom onset — Japan, 1975–2017

Figure 2

Number of surgical procedures linked to cases of dura mater graft-associated Creutzfeldt-Jakob disease (dCJD),* by year of surgical procedure — Japan, 1975–1993^† * Among 154 dura mater graft procedures, the brand was documented as Lyodura in 140 (91%). ^† The manufacturer of Lyodura reported that it revised its collection and processing procedures in May 1987 to reduce the risk for CJD contamination; the recommended shelf life for Lyodura was 5 years.

Figure 3

Interval from surgical procedure to illness onset* in 154 cases of dura mater graft–associated Creutzfeldt-Jakob disease — Japan, 1975–2017 * Median = 13 years; range = 1–30 years.