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Iatrogenic Creutzfeldt-Jakob disease at the millennium
P Brown1, M Preece, J P Brandel
1Laboratory of CNS Studies, NINDS, NIH, Bethesda, MD 20892, USA. brownp@ninds.nih.gov
Abstract:
The causes and geographic distribution of 267 cases of iatrogenic Creutzfeldt-Jakob disease (CJD) are here updated at the millennium. Small numbers of still-occurring cases result from disease onsets after longer and longer incubation periods following infection by cadaveric human growth hormone or dura mater grafts manufactured and distributed before the mid-1980s. The proportion of recipients acquiring CJD from growth hormone varies from 0.3 to 4.4% in different countries, and acquisition from dura mater varies between 0.02 and 0.05% in Japan (where most cases occurred). Incubation periods can extend up to 30 years, and cerebellar onsets predominate in both hormone and graft recipients (in whom the site of graft placement had no effect on the clinical presentation). Homozygosity at codon 129 of the PRNP gene is over-represented in both forms of disease; it has no effect on the incubation period of graft recipients, but may promote shorter incubation periods in hormone cases. Knowledge about potential high-risk sources of contamination gained during the last quarter century, and the implementation of methods to circumvent them, should minimize the potential for iatrogenic contributions to the current spectrum of CJD.
Insights
Iatrogenic Creutzfeldt-Jakob disease (CJD) cases, though rare, still emerge due to long incubation periods from prior human growth hormone and dura mater treatments. Understanding contamination risks has helped minimize future iatrogenic CJD occurrences.
Area of Science:
- Neurology
- Epidemiology
- Genetics
Background:
- Iatrogenic Creutzfeldt-Jakob disease (CJD) arises from medical procedures.
- Cases linked to cadaver-derived human growth hormone and dura mater grafts predate the mid-1980s.
Purpose of the Study:
- To update the causes and geographic distribution of 267 iatrogenic CJD cases.
- To analyze risk factors, incubation periods, and genetic influences.
Main Methods:
- Review of 267 iatrogenic CJD cases.
- Analysis of treatment sources (human growth hormone, dura mater grafts).
- Assessment of PRNP gene codon 129 polymorphism.
Main Results:
- Long incubation periods (up to 30 years) are observed.
- Cerebellar onset is common in both treatment groups.
- Homozygosity at PRNP codon 129 is over-represented and may shorten incubation in hormone-related cases.
Conclusions:
- Knowledge of contamination sources and mitigation strategies should reduce future iatrogenic CJD.
- Continued vigilance is necessary for rare, late-onset cases.