Skip directly to site content Skip directly to page options Skip directly to A-Z link Skip directly to A-Z link Skip directly to A-Z link
Volume 12, Number 10—October 2006
Research

Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease

W. John Pape†, Jeri Forster*, C. Alan Anderson‡§, Patrick Bosque‡¶, Patrick Bosque‡¶, Michael Miller#, and Samantha MaWhinney
Author affiliations: *University of Colorado at Denver and Health Sciences Center, Denver, Colorado, USA; †Colorado Department of Public Health and Environment, Denver, Colorado, USA; ‡University of Colorado School of Medicine, Denver, Colorado, USA; §Denver Veteran's Affairs Medical Center, Denver, Colorado, USA; ¶Denver Health Medical Center, Denver, Colorado, USA; #Colorado Division of Wildlife, Fort Collins, Colorado, USA

Main Article

Figure 3

Annual age-standardized Creutzfeldt-Jakob (CJD) death rates per million population were calculated for chronic wasting disease (CWD)–endemic (ϒ) and non–CWD-endemic (∆) counties. Population rates were age-standardized to the 2001 age distribution for Colorado (34). We also display smoothed rates for the endemic (―) and non–CWD-endemic (∙ − ∙) counties.

Figure 3. Annual age-standardized Creutzfeldt-Jakob (CJD) death rates per million population were calculated for chronic wasting disease (CWD)–endemic (ϒ) and non–CWD-endemic (∆) counties. Population rates were age-standardized to the 2001 age distribution for Colorado (34). We also display smoothed rates for the endemic (―) and non–CWD-endemic (∙ − ∙) counties.

Main Article

References
  1. Williams  ES, Young  S. Chronic wasting disease of captive mule deer: A spongiform encephalopathy. J Wildl Dis. 1980;16:8998.PubMedGoogle Scholar
  2. Williams  ES, Miller  MW. Chronic wasting disease in North American deer and elk. Rev Sci Tech. 2002;21:30516.PubMedGoogle Scholar
  3. Williams  ES, Miller  MW. Transmissible spongiform encephalopathies in non-domestic animals: origin, transmission, and risk factors. Rev Sci Tech. 2003;22:14556.PubMedGoogle Scholar
  4. Williams  ES, Miller  MW, Kreeger  TJ, Kahn  RH, Thorne  ET. Chronic wasting disease of deer and elk: a review with recommendations for management. J Wildl Manage. 2002;66:55163. DOIGoogle Scholar
  5. Belay  ED, Maddox  RA, Williams  ES, Miller  MW, Gambetti  P, Schonberger  LB. Chronic wasting disease and potential transmission to humans. Emerg Infect Dis. 2004;10:97784.PubMedGoogle Scholar
  6. Prusiner  SB. Shattuck lecture–neurodegenerative diseases and prions. N Engl J Med. 2001;344:151626. DOIPubMedGoogle Scholar
  7. Miller  MW, Williams  ES, McCarty  CW, Spraker  TR, Kreeger  TJ, Larsen  CT, Epizootiology of chronic wasting disease in free-ranging cervids in Colorado and Wyoming. J Wildl Dis. 2000;36:67690.PubMedGoogle Scholar
  8. Spraker  TR, Miller  MW, Williams  ES, Getzy  DM, Adrian  WJ, Schoonveld  GG, Spongiform encephalopathy in free-ranging mule deer (Odocoileus hemionus), white-tailed deer (Odocoileus virginianus), and Rocky Mountain elk (Cervus elaphus nelsoni) in northcentral Colorado. J Wildl Dis. 1997;33:16.PubMedGoogle Scholar
  9. Alpers  MP. Epidemiology and clinical aspects of kuru. In: Prusiner SB, McKinley MP, editors. Prions: novel infectious pathogens causing scrapie and Creutzfeldt-Jakob disease. San Diego: Academic Press; 1987. p. 451−65.
  10. Anderson  RM, Donnelly  CA, Ferguson  NM, Woolhouse  ME, Watt  CJ, Udy  HJ, Transmission dynamics and epidemiology of BSE in British cattle. [Erratum, Nature 1997;386:302]. Nature. 1996;382:77988. DOIPubMedGoogle Scholar
  11. Raymond  GJ, Hope  J, Kocisko  DA, Priola  SA, Raymond  LD, Bossers  A, Molecular assessment of the potential transmissibilities of BSE and scrapie to humans. Nature. 1997;388:2858. DOIPubMedGoogle Scholar
  12. Raymond  GJ, Bossers  A, Raymond  LD, O'Rourke  KI, McHolland  LE, Bryant  PK III, Evidence of a molecular barrier limiting susceptibility of humans, cattle and sheep to chronic wasting disease. EMBO J. 2000;19:442530. DOIPubMedGoogle Scholar
  13. World Health Organization. WHO consultation on public health and animal transmissible spongiform encephalopathies: epidemiology, risk and research requirements. Geneva. Organization. 2000;:52.
  14. Belay  ED, Gambetti  P, Schonberger  LB, Parchi  P, Lyon  DR, Capellari  S, Creutzfeldt-Jakob disease in unusually young patients who consumed venison. Arch Neurol. 2001;58:16738. DOIPubMedGoogle Scholar
  15. Food and Drug Administration Transmissible Spongiform Encephalopathy Advisory Committee. Transcripts of open meeting, January 18, 2001, Bethesda, Maryland, USA [cited 20006 Aug 8]. Available from http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2_02.pdf
  16. Hamir  AN, Cutlip  RC, Miller  JM, Williams  ES, Stack  MJ, Miller  MW, Preliminary findings on the experimental transmission of chronic wasting disease agent of mule deer to cattle. J Vet Diagn Invest. 2001;13:916. DOIPubMedGoogle Scholar
  17. Gould  DH, Voss  JL, Miller  MW, Bachand  AM, Cummings  BA, Frank  AA. Survey of cattle in northeast Colorado for evidence of chronic wasting disease: geographical and high-risk targeted sample. J Vet Diagn Invest. 2003;15:2747. DOIPubMedGoogle Scholar
  18. Will  RG. Epidemiology of Creutzfeldt-Jakob disease. Br Med Bull. 1993;49:96070.PubMedGoogle Scholar
  19. Will  RG, Ironside  JW, Zeidler  M, Cousens  SN, Estibeiro  K, Alperovitch  A, A new variant of Creutzfeldt-Jakob disease in the UK. Lancet. 1996;347:9215. DOIPubMedGoogle Scholar
  20. Ghani  AC, Ferguson  NM, Donnelly  CA, Hagenaars  TJ, Anderson  RM. Epidemiological determinants of the pattern and magnitude of the vCJD epidemic in Great Britain. Proc Biol Sci. 1998;265:244352. DOIPubMedGoogle Scholar
  21. Ghani  AC, Ferguson  NM, Donnelly  CA, Anderson  RM. Predicted vCJD mortality in Great Britain. Nature. 2000;406:5834. DOIPubMedGoogle Scholar
  22. Ghani  AC, Ferguson  NM, Donnelly  CA, Anderson  RM. Updated projections of future vCJD deaths in the UK. BMC Infect Dis. 2003;3:4. DOIPubMedGoogle Scholar
  23. Ghani  AC, Ferguson  NM, Donnelly  CA, Anderson  RM. Factors determining the pattern of the variant Creutzfeldt-Jakob disease (vCJD) epidemic in Great Britain. Proc Biol Sci. 2003;270:68998. DOIPubMedGoogle Scholar
  24. National Creutzfeldt-Jakob Disease Surveillance Unit. CJD statistics (June 2, 2006) [cited 2006 Aug 8]. Available from http://www.cjd.ed.ac.uk/figures.htm
  25. Clarke  P, Ghani  AC. Projections of the future course of the primary vCJD epidemic in the UK: inclusion of subclinical infection and the possibility of wider genetic susceptibility. J R Soc Interface. 2005;2:1931. DOIPubMedGoogle Scholar
  26. Bruce  ME, Will  RG, Ironside  JW, McConnell  I, Drummond  D, Suttie  A, Transmission to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature. 1997;389:498501. DOIPubMedGoogle Scholar
  27. Bruce  M, Chree  A, Williams  ES, Fraser  H. Perivascular PrP amyloid in the brains of mice infected with chronic wasting disease. Brain Pathol. 2000;10:6623.
  28. Race  RE, Raines  A, Baron  TGM, Miller  MW, Jenny  A, Williams  ES. Comparison of abnormal prion protein glycoform patterns from transmissible spongiform encephalopathy agent-infected deer, elk, sheep, and cattle. J Virol. 2002;76:123658. DOIPubMedGoogle Scholar
  29. Hamir  AN, Kunkle  RA, Cutlip  RC, Miller  JM, O'Rourke  KI, Williams  ES, Experimental transmission of chronic wasting disease agent to cattle by the intracerebral route. J Vet Diagn Invest. 2005;17:27681. DOIPubMedGoogle Scholar
  30. Bosque  PJ. Bovine spongiform encephalopathy, chronic wasting disease, scrapie, and the threat to humans from prion disease epizootics. Curr Neurol Neurosci Rep. 2002;2:48895. DOIPubMedGoogle Scholar
  31. Majeed  A, Lehmann  P, Kirby  L, Coleman  MP. Mortality from dementias and neurodegenerative disorders in people aged 15–64 in England and Wales in 1979–96. BMJ. 1998;317:3201. DOIPubMedGoogle Scholar
  32. Collinge  J, Rossor  M. A new variant of prion disease. Lancet. 1996;347:9167. DOIPubMedGoogle Scholar
  33. National Creutzfeldt-Jakob Disease Surveillance Unit. Incidence of variant Creutzfeldt-Jakob disease onsets and deaths in the UK, January 1994–December 2004 [cited 2006 Aug 8]. Available from http://www.cjd.ed.ac.uk/vcjdq.htm
  34. Colorado Demography Office. Population totals [cited 2006 Aug 8]. Available from http://dola.colorado.gov/demog
  35. National Creutzfeldt-Jakob Disease Surveillance Unit. Creutzfeldt-Jakob disease surveillance in the UK, twelfth annual report, 2003 [cited 2006 Aug 8]. Available from http://www.cjd.ed.ac.uk/twelfth/rep2003.htm
  36. Belay  ED, Maddox  RA, Gambetti  P, Schonberger  LB. Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States. Neurology. 2003;60:17681.PubMedGoogle Scholar
  37. Ladogana  A, Puopolo  M, Croes  EA, Budka  H, Jarius  C, Collins  S, Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Neurology. 2005;64:158691. DOIPubMedGoogle Scholar
  38. Colorado Demography Office. Population by age and gender [cited 2006 Aug 8]. Available from http://dola.colorado.gov/demog/CreateTable1.cfm

Main Article

Page created: December 05, 2011
Page updated: December 05, 2011
Page reviewed: December 05, 2011
The conclusions, findings, and opinions expressed by authors contributing to this journal do not necessarily reflect the official position of the U.S. Department of Health and Human Services, the Public Health Service, the Centers for Disease Control and Prevention, or the authors' affiliated institutions. Use of trade names is for identification only and does not imply endorsement by any of the groups named above.
file_external