Although infectiousness is a feature of Creutzfeldt–Jakob disease (CJD), only a small proportion of cases are linked to transmission through healthcare provision.
As of January 2003, over 120 cases of CJD associated with use of human cadaveric dura mater had been recognised worldwide; almost all were associated with the commercial product Lyodura.
Most cases (97) have occurred in Japan, giving an overall risk estimate of around 1 per 2268 patients treated with Lyodura (0.04%) in that country.
In Australia, five cases of CJD have so far been linked to Lyodura, but, given the protracted tails of previous epidemics of transmissible spongiform encephalopathies, further cases are possible.
Results of surveys of Lyodura use in Australia are incomplete, but information from the manufacturer suggests that 2208–2478 sheets of Lyodura may have been used here.
This use translates to a relatively high incidence of Lyodura-associated CJD, with current overall rates appearing around five times higher than those reported in Japan; reasons for this difference are unclear.
- 1. Brown P, Preece M, Brandel JP, et al. Iatrogenic Creutzfeldt-Jakob disease at the millennium. Neurology 2000; 55: 1075-1081.
- 2. Update: Creutzfeldt-Jakob disease in a patient receiving a cadaveric dura mater graft. MMWR Morb Mortal Wkly Rep 1987; 36: 324-325.
- 3. Food and Drug Administration. FDA safety alert: possibly contaminated dura mater transplant material. Rockville, Md: US Department of Health and Human Services, Public Health Service, Apr 28 1987.
- 4. Update: Creutzfeldt-Jakob disease in a second patient who received a cadaveric dura mater graft. MMWR Morb Mortal Wkly Rep 1989; 38: 37-38.
- 5. Nakamura Y, Watanabe M, Nagoshi K, et al. Update: Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts — Japan, 1979–2003. MMWR Morb Mortal Wkly Rep 2003; 52: 1179-1181.
- 6. Hannah EL, Belay ED, Gambetti P, et al. Creutzfeldt-Jakob disease after receipt of a previously unimplicated brand of dura mater graft. Neurology 2001; 56: 1080-1083.
- 7. Defebvre L, Destee A, Caron J, et al. Creutzfeldt-Jakob disease after an embolization of intercostal arteries with cadaveric dura mater suggesting a systemic transmission of the prion agent. Neurology 1997; 48: 1470-1471.
- 8. Dobbins JG, Belay ED, Malecki J, et al. Creutzfeldt-Jakob disease in a recipient of a dura mater graft processed in the US: cause or coincidence? Neuroepidemiology 2000; 19: 62-66.
- 9. Nakamura Y, Aso E, Yanagawa H. Relative risk of Creutzfeldt-Jakob disease with cadaveric dura transplantation in Japan. Neurology 1999; 53: 218-220.
- 10. Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts — Japan, January 1979–May 1996. MMWR Morb Mortal Wkly Rep 1997; 46: 1066-1069.
- 11. Hoshi K, Yoshino H, Urata J, et al. Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts in Japan. Neurology 2000; 55: 718-721.
- 12. Hamada C, Sadaike T, Fukushima M. Projection of Creutzfeldt-Jakob Disease frequency based on cadaveric dura transplantation in Japan. Neuroepidemiology 2003; 22: 57-64.
- 13. Boyd A, Fletcher A, Lee JS, et al. Transmissible spongiform encephalopathies in Australia. Commun Dis Intell 2001; 25: 248-252.
- 14. Collins S, Boyd A, Lee JS, et al. Creutzfeldt-Jakob disease in Australia 1970-1999. Neurology 2002; 59: 1365-1371.
- 15. Simpson D, Masters CL, Ohlrich G, et al. Iatrogenic Creutzfeldt-Jakob disease and its neurosurgical implications. J Clin Neurosci 1996; 3: 118-123.
- 16. Newcombe RL. Neurosurgery and iatrogenic transmission of Creutzfeldt-Jakob disease. Med J Aust 1996; 164: 603-604.
- 17. Huillard d’Aignaux J, Costagliola D, et al. Incubation period of Creutzfeldt-Jakob disease in human growth hormone recipients in France. Neurology 1999; 53: 1197-1201.
- 18. Brandel J-P, Preece M, Brown P, et al. Distribution of codon 129 genotype in human growth hormone-treated CJD patients in France and the UK. Lancet 2003; 362: 128-130.
- 19. Doh-ura K, Kitamoto T, Sakaki Y, Tateishi J. CJD discrepancy. Nature 1991; 353: 801-802.
- 20. Antoine JC, Michel D, Bertholon P, et al. Creutzfeldt-Jakob disease after extracranial dura mater embolization for a nasopharyngeal angiofibroma. Neurology 1997; 48: 1451-1453.
- 21. Dickinson AG, Fraser H, Outram GW. Scrapie incubation time can exceed natural lifespan. Nature 1975; 256: 732-733.
- 22. Hill AF, Joiner S, Linehan J, et al. Species-barrier-independent prion replication in apparently resistant species. Proc Natl Acad Sci USA 2000; 97: 10248-10253.
- 23. Frigg R, Klein MA, Hegyi I, et al. Scrapie pathogenesis in subclinically infected B-cell-deficient mice. J Virol 1999; 73: 9584-9588.
- 24. Race R, Chesebro B. Scrapie infectivity found in resistant species. Nature 1998; 392: 770.
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