Skip to main content
SpringerLink
Log in

Incidence of Creutzfeldt-Jakob disease in Taiwan: a prospective 10-year surveillance

  • Neuro-Epidemiology
  • Published:
European Journal of Epidemiology Aims and scope Submit manuscript

Abstract

This study was performed to estimate the incidence of Creutzfeldt-Jakob Disease (CJD) in Taiwan from 1998 to 2007. Suspected cases of CJD were reported to the Taiwan Creutzfeldt-Jakob Disease Surveillance Unit, a nationwide, hospital-based case report system initiated since 1996 to prospectively conduct a CJD epidemiological study. Consecutive patients who met the diagnostic criteria recommended by the World Health Organization were enrolled. The clinical information of each suspected case was collected and case ascertainment was performed by an expert committee. A total of 123 sporadic CJD were identified without any iatrogenic or new variant CJD cases. The overall annual incidence rate (95% CI) was 0.55 (0.46–0.65) cases per million person-year. There was no statistically significant difference between the calendar year of disease onset (P = 0.97). The incidence rates were not significantly different between women and men (P = 0.63). Age was the main factor for the risk of CJD (P < 0.0001). Age-specific incidence rate increased after the age of 40 years with the peak being in the 70–79 years age group. Our data showed low annual incidence rate and high frequency of methionine homozygous prion protein genotype of sCJD in Taiwan. This report provided important epidemiological data on ethnic Chinese.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Caramelli M, Ru G, Acutis P, Forloni G. Prion diseases: current understanding of epidemiology and pathogenesis, and therapeutic advances. CNS Drugs. 2006;20(1):15–28.

    Article  CAS  PubMed  Google Scholar 

  2. Andrews NJ, Farrington CP, Ward HJ, Cousens SN, Smith PG, Molesworth AM, et al. Deaths from variant Creutzfeldt-Jakob disease in the UK. Lancet. 2003;361(9359):751–2.

    Article  CAS  PubMed  Google Scholar 

  3. Capek I, Vaillant V. Creutzfeldt-Jakob disease and related diseases in France from 1998 to 2000. Euro Surveill. 2003;8(1):14–8.

    CAS  PubMed  Google Scholar 

  4. Collins S, Boyd A, Lee JS, Lewis V, Fletcher A, McLean CA, et al. Creutzfeldt-Jakob disease in Australia 1970–1999. Neurology. 2002;59(9):1365–71.

    CAS  PubMed  Google Scholar 

  5. D’Aignaux JH, Cousens SN, Delasnerie-Laupretre N, Brandel JP, Salomon D, Laplanche JL, et al. Analysis of the geographical distribution of sporadic Creutzfeldt-Jakob disease in France between 1992 and 1998. Int J Epidemiol. 2002;31(2):490–5.

    Article  PubMed  Google Scholar 

  6. Doi Y, Yokoyama T, Sakai M, Nakamura Y. Creutzfeldt-Jakob disease mortality in Japan, 1979–2004: analysis of national death certificate data. J Epidemiol. 2007;17(4):133–9.

    Article  PubMed  Google Scholar 

  7. Elsaadany S, Semenciw R, Ricketts M, Mao Y, Giulivi A. Epidemiological study of Creutzfeldt-Jakob disease death certificates in Canada, 1979–2001. Neuroepidemiology. 2005;24(1–2):15–21.

    Article  PubMed  Google Scholar 

  8. Glatzel M, Rogivue C, Ghani A, Streffer JR, Amsler L, Aguzzi A. Incidence of Creutzfeldt-Jakob disease in Switzerland. Lancet. 2002;360(9327):139–41.

    Article  PubMed  Google Scholar 

  9. Heinemann U, Krasnianski A, Meissner B, Varges D, Kallenberg K, Schulz-Schaeffer WJ, et al. Creutzfeldt-Jakob disease in Germany: a prospective 12-year surveillance. Brain. 2007;130(5):1350–9.

    Article  CAS  PubMed  Google Scholar 

  10. Horan G, Keohane C, Molloy S, Howley R, Harney M, Heffernan J, et al. Creutzfeldt-Jakob disease in Ireland: epidemiological aspects 1980–2002. Eur Neurol. 2004;51(3):132–7.

    Article  PubMed  Google Scholar 

  11. Ladogana A, Puopolo M, Croes EA, Budka H, Jarius C, Collins S, et al. Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Neurology. 2005;64(9):1586–91.

    Article  CAS  PubMed  Google Scholar 

  12. Puopolo M, Ladogana A, Almonti S, Daude N, Bevivino S, Petraroli R, et al. Mortality trend from sporadic Creutzfeldt-Jakob disease (CJD) in Italy, 1993–2000. J Clin Epidemiol. 2003;56(5):494–9.

    Article  PubMed  Google Scholar 

  13. Shi Q, Gao C, Zhou W, Zhang BY, Chen JM, Tian C, et al. Surveillance for Creutzfeldt-Jakob disease in China from 2006 to 2007. BMC Public Health. 2008;8:360.

    Article  PubMed  Google Scholar 

  14. Stratton E, Ricketts MN, Gully PR. The epidemiology of Creutzfeldt-Jakob disease in Canada: a review of mortality data. Emerg Infect Dis. 1997;3(1):63–4.

    Article  CAS  PubMed  Google Scholar 

  15. Van Everbroeck B, Michotte A, Sciot R, Godfraind C, Deprez M, Quoilin S, et al. Increased incidence of sporadic Creutzfeldt-Jakob disease in the age groups between 70 and 90 years in Belgium. Eur J Epidemiol. 2006;21(6):443–7.

    Article  PubMed  Google Scholar 

  16. Velasquez-Perez L, Rembao-Bojorquez D, Guevara J, Guadarrama-Torres RM, Trejo-Contreras A. Creutzfeldt-Jakob disease in Mexico. Neuropathology. 2007;27(5):419–28.

    Article  PubMed  Google Scholar 

  17. Will RG, Alperovitch A, Poser S, Pocchiari M, Hofman A, Mitrova E, et al. Descriptive epidemiology of Creutzfeldt-Jakob disease in six European countries, 1993–1995. EU Collaborative Study Group for CJD. Ann Neurol. 1998;43(6):763–7.

    Article  CAS  PubMed  Google Scholar 

  18. Cousens SN, Linsell L, Smith PG, Chandrakumar M, Wilesmith JW, Knight RS, et al. Geographical distribution of variant CJD in the UK (excluding Northern Ireland). Lancet. 1999;353(9146):18–21. (see comment).

    Article  CAS  PubMed  Google Scholar 

  19. Unit NCS. Incidence of variant Creutzfeldt-Jakob disease diagnoses and deaths in the UK. January 1994–December 2008 (online). http://wwwcjdedacuk/cjdq60pdf. 2009, yearly report.

  20. Brown P, Cathala F, Raubertas RF, Gajdusek DC, Castaigne P. The epidemiology of Creutzfeldt-Jakob disease: conclusion of a 15-year investigation in France and review of the world literature. Neurology. 1987;37(6):895–904.

    CAS  PubMed  Google Scholar 

  21. Cousens SN, Zeidler M, Esmonde TF, De Silva R, Wilesmith JW, Smith PG, et al. Sporadic Creutzfeldt-Jakob disease in the United Kingdom: analysis of epidemiological surveillance data for 1970–96. BMJ. 1997;315(7105):389–95.

    CAS  PubMed  Google Scholar 

  22. Satishchandra P, Shankar SK. Creutzfeldt-Jakob disease in India (1971–1990). Neuroepidemiology. 1991;10(1):27–32.

    Article  CAS  PubMed  Google Scholar 

  23. Prusiner SB. Prion biology and diseases. 2nd ed. New York: Cold Spring Harbor Laboratory Press; 2004.

    Google Scholar 

  24. WHO. Human transmissible spongiform encephalopathies. Wkly Epidemiol Rec. 1998;73(47):361–5.

    Google Scholar 

  25. Cambier DM, Kantarci K, Worrell GA, Westmoreland BF, Aksamit AJ. Lateralized and focal clinical, EEG, and FLAIR MRI abnormalities in Creutzfeldt-Jakob disease. Clin Neurophysiol. 2003;114(9):1724–8.

    Article  PubMed  Google Scholar 

  26. Tsuji S, Kuroiwa Y. Creutzfeldt-Jakob disease in Japan. Neurology. 1983;33(11):1503–6.

    CAS  PubMed  Google Scholar 

  27. Will RG, Matthews WB, Smith PG, Hudson C. A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970–1979. II: Epidemiology. J Neurol Neurosurg Psychiatry. 1986;49(7):749–55.

    Article  CAS  PubMed  Google Scholar 

  28. Masullo C, Pocchiari M, Neri G, Casaccia P, Iavarone A, Ladogana A, et al. A retrospective study of Creutzfeldt-Jakob disease in Italy (1972–1986). Eur J Epidemiol. 1988;4(4):482–7.

    Article  CAS  PubMed  Google Scholar 

  29. Radbauer C, Hainfellner JA, Jellinger K, Pilz P, Maier H, Kleinert R, et al. Epidemiology of transmissible spongiform encephalopathies (prion diseases) in Austria. Wien Med Wochenschr. 1998;148(4):101–6.

    CAS  PubMed  Google Scholar 

  30. Brandel JP, Delasnerie-Laupretre N, Laplanche JL, Hauw JJ, Alperovitch A. Diagnosis of Creutzfeldt-Jakob disease: effect of clinical criteria on incidence estimates. Neurology. 2000;54(5):1095–9.

    CAS  PubMed  Google Scholar 

  31. Masters CL, Harris JO, Gajdusek DC, Gibbs CJ Jr, Bernoulli C, Asher DM. Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann Neurol. 1979;5(2):177–88.

    Article  CAS  PubMed  Google Scholar 

  32. Hsich G, Kenney K, Gibbs CJ, Lee KH, Harrington MG. The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies. N Engl J Med. 1996;335(13):924–30.

    Article  CAS  PubMed  Google Scholar 

  33. Hsiao K, Baker HF, Crow TJ, Poulter M, Owen F, Terwilliger JD, et al. Linkage of a prion protein missense variant to Gerstmann-Straussler syndrome. Nature. 1989;338(6213):342–5.

    Article  CAS  PubMed  Google Scholar 

  34. Salvatore M, Genuardi M, Petraroli R, Masullo C, D’Alessandro M, Pocchiari M. Polymorphisms of the prion protein gene in Italian patients with Creutzfeldt-Jakob disease. Hum Genet. 1994;94(4):375–9.

    Article  CAS  PubMed  Google Scholar 

  35. Pocchiari M. Prions and related neurological diseases. Mol Aspects Med. 1994;15(3):195–291.

    Article  CAS  PubMed  Google Scholar 

  36. Finkenstaedt M, Szudra A, Zerr I, Poser S, Hise JH, Stoebner JM, et al. MR imaging of Creutzfeldt-Jakob disease. Radiology. 1996;199(3):793–8.

    CAS  PubMed  Google Scholar 

  37. Kretzschmar HA, Ironside JW, DeArmond SJ, Tateishi J. Diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Arch Neurol. 1996;53(9):913–20.

    CAS  PubMed  Google Scholar 

  38. Reinwald S, Westner IM, Niedermaier N. Rapidly progressive Alzheimer’s disease mimicking Creutzfeldt-Jakob disease. J Neurol. 2004;251(8):1020–2.

    Article  PubMed  Google Scholar 

  39. Brown P, Rodgers-Johnson P, Cathala F, Gibbs CJ Jr, Gajdusek DC. Creutzfeldt-Jakob disease of long duration: clinicopathological characteristics, transmissibility, and differential diagnosis. Ann Neurol. 1984;16(3):295–304.

    Article  CAS  PubMed  Google Scholar 

  40. Burns A, O’Brien J, Ames D. Dementia. 3rd ed. London: Hodder Arnold; 2005.

    Google Scholar 

  41. Rabenau HF, Cinatl J, Doerr HW. Prions: a challenge for science, medicine, and public health system. S. Karger; 2004.

  42. Parchi P, Giese A, Capellari S, Brown P, Schulz-Schaeffer W, Windl O, et al. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol. 1999;46(2):224–33.

    Article  CAS  PubMed  Google Scholar 

  43. Brown P, Gibbs CJ Jr, Rodgers-Johnson P, Asher DM, Sulima MP, Bacote A, et al. Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol. 1994;35(5):513–29.

    Article  CAS  PubMed  Google Scholar 

  44. Zeidler M, Green A. Advances in diagnosing Creutzfeldt-Jakob disease with MRI and CSF 14-3-3 protein analysis. Neurology. 2004;63(3):410–1.

    PubMed  Google Scholar 

  45. Cohen CH. Does improvement in case ascertainment explain the increase in sporadic Creutzfeldt-Jakob disease since 1970 in the United Kingdom? Am J Epidemiol. 2000;152(5):474–9.

    Article  CAS  PubMed  Google Scholar 

  46. Dyrbye H, Broholm H, Dziegiel MH, Laursen H. The M129V polymorphism of codon 129 in the prion gene (PRNP) in the Danish population. Eur J Epidemiol. 2008;23(1):23–7.

    Article  CAS  PubMed  Google Scholar 

  47. Nurmi MH, Bishop M, Strain L, Brett F, McGuigan C, Hutchison M, et al. The normal population distribution of PRNP codon 129 polymorphism. Acta Neurol Scand. 2003;108(5):374–8.

    Article  CAS  PubMed  Google Scholar 

  48. Windl O, Dempster M, Estibeiro JP, Lathe R, de Silva R, Esmonde T, et al. Genetic basis of Creutzfeldt-Jakob disease in the United Kingdom: a systematic analysis of predisposing mutations and allelic variation in the PRNP gene. Hum Genet. 1996;98(3):259–64.

    Article  CAS  PubMed  Google Scholar 

  49. Palmer MS, Dryden AJ, Hughes JT, Collinge J. Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease. Nature. 1991;352(6333):340–2.

    Article  CAS  PubMed  Google Scholar 

  50. Yu SL, Jin L, Sy MS, Mei FH, Kang SL, Sun GH, et al. Polymorphisms of the PRNP gene in Chinese populations and the identification of a novel insertion mutation. Eur J Hum Genet. 2004;12(10):867–70.

    Article  CAS  PubMed  Google Scholar 

  51. Parchi P, Castellani R, Capellari S, Ghetti B, Young K, Chen SG, et al. Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol. 1996;39(6):767–78.

    Article  CAS  PubMed  Google Scholar 

  52. Lo RY, Shyu WC, Li H. Long-duration sCJD with PRNP codon 129 methionine homozygosity and cerebral cortical plaques. Neurology. 2006;66(12):1944–5.

    Article  PubMed  Google Scholar 

  53. Gelpi E, Heinzl H, Hoftberger R, Unterberger U, Strobel T, Voigtlander T, et al. Creutzfeldt-Jakob disease in Austria: an autopsy-controlled study. Neuroepidemiology. 2008;30(4):215–21.

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

The authors thank the members of the Taiwan Creutzfeldt-Jakob Disease Surveillance Unit for their assistance and participation. The names of these members are listed in the “Appendix”. This study was supported by the scientific grants from Center for Disease Control (Taiwan): DOH91-DC-1036 (in 2002), DOH92-DC-1029 (in 2003), DOH93-DC-1018 (in 2004), DOH94-DC-1035 (in 2005), DOH95-DC-1103 (in 2006), DOH96-DC-1013 (in 2007). There was no any other support from other industry. There was no potential conflict of interest relevant to this article and all the authors.

Author information

Authors and Affiliations

  1. Department of Neurology, En Chu Kong Hospital, No. 399, Fuhsin Road, San-shia Town, Taipei, 23702, Taiwan

    Chien-Jung Lu, Yu Sun & Shun-Sheng Chen

  2. Department of Neurology, Chang Gung Memorial Hospital, Kaohsiung Medical Center, School of Medicine, Chang Gung University, Kaohsiung County, Taiwan

    Shun-Sheng Chen

Authors
  1. Chien-Jung Lu
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Yu Sun
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Shun-Sheng Chen
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding authors

Correspondence to Yu Sun or Shun-Sheng Chen.

Appendix

Appendix

This study is based on the data of Taiwan Creutzfeldt-Jakob Disease Surveillance Unit. The following are the director and members of expert panel of this unit.

Director

Prof. Shun-Sheng Chen.

Members of expert panel

Chien-Jung Lu, M.D., Ching-Kuan Liu, M.D., Yang-Chyuan Chang, M.D., Rong-Chi Chen, M.D., Zin-An Wu, M.D., Hsiu-Chih Liu, M.D., Hou-Chang Chiu, M.D., Wen-Long Tsao, M.D., Ching-Chang Hung, M.D., Woei-Cherng Shyu, M.D., Chin-Cheng Lee, M.D., Ming-Chyi Pai, M.D., Jien-Wei Liu, M.D., Lok-Ming Tang, M.D., Shung-Lon Lai, M.D., Yi-Fen Kao, M.D., Ling-Ling Hsieh, Shu-Hwae Lee, M.D., Chun-Chung Lui, M.D., Cheng-Hsien Lu, M.D., and Ming Chu Chiang. M.D.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Lu, CJ., Sun, Y. & Chen, SS. Incidence of Creutzfeldt-Jakob disease in Taiwan: a prospective 10-year surveillance. Eur J Epidemiol 25, 341–347 (2010). https://doi.org/10.1007/s10654-010-9446-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10654-010-9446-4

Keywords

Search

Navigation