Abstract
Florid plaques indistinguishable from those found in vCJD were identified at a postmortem examination in the brain of a 58‐year‐old clinical suspect case of Creutzfeldt‐Jakob disease (CJD). Western blotting of brain tissue revealed an unusual prion protein type. Since the patient had received a dura mater graft 20 years prior to death and florid plaques are not only found in new variant CJD, the findings argue in favor of an iatrogenic origin of the disease with the longest incubation time following a dura mater graft reported to date even though he may have been exposed to BSE. The peculiar pathological, clinical and biochemical features may define a new type of human prion disease.
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References
- 1. Asante EA, Linehan JM, Desbruslais M, Joiner S, Gowland I, Wood AL, Welch J, Hill AF, Lloyd SE, Wadsworth JDF, Collinge J (2002) BSE prions propagate as either variant CJD‐ like or sporadic CJD‐like prion strains in transgenic mice expressing human prion protein. EMBO J 21:6358–6366. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2. Brown P, Preece M, Brandel JP, Sato T, McShane L, Zerr I, Fletcher A, Will RG, Pocchiari M, Cashman NR, D'Aignaux JH et al (2000) Iatrogenic Creutzfeldt‐Jakob disease at the millennium. Neurology 55:075–1081. [DOI] [PubMed] [Google Scholar]
- 3. Collinge J, Sidle KCL, Meads J, Ironside J, Hill AF (1996) Molecular analysis of prion strain variation and the aetiology of ‘new variant’ CJD. Nature 383:685–690. [DOI] [PubMed] [Google Scholar]
- 4. Deslys JP, Lasmezas CI, Streichenberger N, Hill A, Collinge J, Dormont D, Kopp N (1997) New variant Creutzfeldt‐Jakob disease in France. Lancet 349:30–31. [DOI] [PubMed] [Google Scholar]
- 5. Katayama S, Watanabe K, Noda H, Tokinobu H, Yamamura Y, Jin C et al (2002) A case of sudden death six months after onset of Creutzfeldt‐Jakob disease with florid plaques after dural graft. Neuropathology 22:A17. [Google Scholar]
- 6. Kimura N, Kawai M (2002) Creutzfeldt‐Jakob disease with Kuru and florid plaques after cadaveric dura mater grafting. Neuropathology 22:A17. [Google Scholar]
- 7. Kopp N, Streichenberger N, Deslys JP, Laplanche JL, Chazot G (1996) Creutzfeldt‐Jakob disease in a 52‐year‐old woman with florid plaques. Lancet 348:1239–1240. [DOI] [PubMed] [Google Scholar]
- 8. Parchi P, Capellari S, Chen SG, Petersen RB, Gambetti P, Kopp N, Brown P, Kitamoto T, Tateishi J, Giese A, Kretzschmar HA (1997) Typing prion isoforms. Nature 386:232–233. [DOI] [PubMed] [Google Scholar]
- 9. Parchi P, Castellani R, Capellari S, Ghetti B, Young K, Chen SG, Farlow M, Dickson DW, Sima AAF, Trojanowski JQ, Petersen RB, Gambetti P (1996) Molecular basis of phenotypic variability in sporadic Creutzfeldt‐Jakob disease. Ann Neurol 39:767–778. [DOI] [PubMed] [Google Scholar]
- 10. Parchi P, Giese A, Capellari S, Brown P, Schulz‐Schaeffer W, Windl O, Zerr I, Budka H, Kopp N, Piccardo P, Poser S et al (1999) Classification of sporadic Creutzfeldt‐Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 46:224–233. [PubMed] [Google Scholar]
- 11. Shimizu S, Hoshi K, Muramoto T, Homma M, Ironside JW, Kuzuhara S, Sato T, Yamamoto T, Kitamoto T (1999) Creutzfeldt‐Jakob disease with florid‐type plaques after cadaveric dura mater grafting. Arch Neurol 56:357–362. [DOI] [PubMed] [Google Scholar]
- 12. Takashima S, Tateishi J, Taguchi Y, Inoue H (1997) Creutzfeldt‐Jakob disease with florid plaques after cadaveric dural graft in a Japanese woman. Lancet 350:865–866. [DOI] [PubMed] [Google Scholar]
- 13. Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alperovitch A, Poser S, Pocchiari M, Hofman A, Smith PG (2002) A new variant of Creutzfeldt‐Jakob disease in the UK. Lancet 347:921–925. [DOI] [PubMed] [Google Scholar]
- 14. Will RG, Zeidler M, Stewart GE, Macleod MA, Ironside JW, Cousens SN, Mackenzie J, Estibeiro K, Green AJE, Knight RSG (2000) Diagnosis of new variant Creutzfeldt‐Jakob disease. Ann Neurol 47:575–582. [PubMed] [Google Scholar]
- 15. Windl O, Giese A, Schulz‐Schaeffer W, Zerr I, Skworc K, Arendt S, Oberdieck C, Bodemer M, Poser S, Kretzschmar HA (1999) Molecular genetics of human prion diseases in Germany. Hum Genetics 105:244–252. [DOI] [PubMed] [Google Scholar]
