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From the Laboratory of CNS Studies (Dr. Brown), NINDS, Biometric Research Branch (Dr. McShane), NCI, and Division of Diabetes, Endocrinology, and Metabolic Diseases (Dr. Fradkin), NIDDKD, NIH, Bethesda, MD; Institute of Child Health (M. Preece), University College London, UK; U 360 INSERM (J.-P. Brandel and J.H. d’Aignaux), Centre National de Référence de la Maladie de CJ, Hôpital de la Salpêtrière, Paris, France; Khonodai Hospital (T. Sato), Ichikawa, Chiba, Japan; Klinik und Poliklinik für Neurologie (I. Zerr), Georg-August-Universität Göttingen, Germany; Department of Pathology (A. Fletcher and S.J. Collins), University of Melbourne, Australia; CJD Surveillance Unit (Dr. Will), Western General Hospital, Edinburgh, UK; Istituto Superiore de Sanità (M. Pocchiari), Laboratory of Virology, Rome, Italy; Canadian CJD Surveillance System (N.R. Cashman), Ottawa, and University of Toronto, Canada; Jerome H. Holland Laboratory (L. Cervenáková), American Red Cross, Rockville, MD; and Division of Viral and Rickettsial Diseases (Dr. Schonberger), National Center for Infectious Diseases, CDC, Atlanta, GA.
Address correspondence and reprint requests to Dr. Paul Brown, Building 36, Room 4A-05, National Institutes of Health, 36 Convent Drive, MSC 4122, Bethesda, MD 20892; e-mail: brownp{at}ninds.nih.gov
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.
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 A. F Hill and J. Collinge Subclinical prion infection in humans and animals Br. Med. Bull., June 1, 2003; 66(1): 161 - 170. [Abstract] [Full Text] [PDF]
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R Lim, B Dhillon, K M Kurian, P A Aspinall, K Fernie, and J W Ironside Retention of corneal epithelial cells following Goldmann tonometry: implications for CJD risk Br J Ophthalmol, May 1, 2003; 87(5): 583 - 586. [Abstract] [Full Text] [PDF]
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J. Mastrianni and R. P. Roos "Out, damned spot! out, I say!. . .": Issues related to prion decontamination Neurology, August 27, 2002; 59(4): 488 - 488. [Full Text] [PDF]
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E A Croes, G Roks, G H Jansen, P C G Nijssen, and C M van Duijn Creutzfeldt-Jakob disease 38 years after diagnostic use of human growth hormone J. Neurol. Neurosurg. Psychiatry, June 1, 2002; 72(6): 792 - 793. [Abstract] [Full Text] [PDF]
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A. A. Rabinstein, M. L. Whiteman, and R. T. Shebert Abnormal Diffusion-Weighted Magnetic Resonance Imaging in Creutzfeldt-Jakob Disease Following Corneal Transplantations Arch Neurol, April 1, 2002; 59(4): 637 - 639. [Abstract] [Full Text] [PDF]
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E. D. Belay, P. Gambetti, L. B. Schonberger, P. Parchi, D. R. Lyon, S. Capellari, J. H. McQuiston, K. Bradley, G. Dowdle, J. M. Crutcher, et al. Creutzfeldt-Jakob Disease in Unusually Young Patients Who Consumed Venison Arch Neurol, October 1, 2001; 58(10): 1673 - 1678. [Abstract] [Full Text] [PDF]
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R. P. Roos Controlling New Prion Diseases N. Engl. J. Med., May 17, 2001; 344(20): 1548 - 1551. [Full Text] [PDF]
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 R. H. Kennedy, R. N. Hogan, P. Brown, E. Holland, R. T. Johnson, W. Stark, and J. Sugar Eye Banking and Screening for Creutzfeldt-Jakob Disease Arch Ophthalmol, May 1, 2001; 119(5): 721 - 726. [Abstract] [Full Text] [PDF]
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E. L. Hannah, E. D. Belay, P. Gambetti, G. Krause, P. Parchi, S. Capellari, R. E. Hoffman, and L. B. Schonberger Creutzfeldt-Jakob disease after receipt of a previously unimplicated brand of dura mater graft Neurology, April 24, 2001; 56(8): 1080 - 1083. [Abstract] [Full Text] [PDF]
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A. Hamad, A. Hamad, T. E. O. Sokrab, S. Momeni, and P. Brown Iatrogenic Creutzfeldt-Jakob disease at the millennium Neurology, April 10, 2001; 56(7): 987 - 987. [Full Text] [PDF]
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