Iatrogenic Creutzfeldt-Jakob disease, final assessment

Abstract

The era of iatrogenic Creutzfeldt-Jakob disease (CJD) has nearly closed; only occasional cases with exceptionally long incubation periods are still appearing. The principal sources of these outbreaks are contaminated growth hormone (226 cases) and dura mater grafts (228 cases) derived from human cadavers with undiagnosed CJD infections; a small number of additional cases are caused by neurosurgical instrument contamination, corneal grafts, gonadotrophic hormone, and secondary infection with variant CJD transmitted by transfusion of blood products. No new sources of disease have been identified, and current practices, which combine improved recognition of potentially infected persons with new disinfection methods for fragile surgical instruments and biological products, should continue to minimize the risk for iatrogenic disease until a blood screening test for the detection of preclinical infection is validated for human use.

Figure

Annual incidence of variant Creutzfeldt-Jakob disease (vCJD) caused by ingestion of meat products contaminated with bovine spongiform encephalopathy agent (A) and iatrogenic CJD caused by contaminated dura mater (B) and cadaveric human growth hormone (C), 1982–2011. White bars in panel A represent cases from outside the United Kingdom, which were delayed in parallel with the later appearance of bovine spongiform encephalopathy outside the United Kingdom (not a second wave resulting from codon 129 genotype differences). Two patients are excluded: 1 presymptomatic patient from the United States who received human growth hormone and died of an intercurrent illness and 1 dura mater recipient from the United Kingdom with disease onset in 1978.