Clinical Epidemiology (Oct 2020)

Incidence of and Mortality Due to Human Prion Diseases in Taiwan: A Prospective 20-Year Nationwide Surveillance Study from 1998 to 2017

  • Sun Y,
  • Liu CC,
  • Fan LY,
  • Huang CT,
  • Chen TF,
  • Lu CJ,
  • Guo WY,
  • Chang YC,
  • Chiu MJ

Journal volume & issue
Vol. Volume 12
pp. 1073 – 1081

Abstract

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Yu Sun,1,2 Chih-Ching Liu,3,* Ling-Yun Fan,4,* Chung-Te Huang,5 Ta-Fu Chen,2 Chien-Jung Lu,1,2 Wan-Yuo Guo,6,7 Yang-Chyuan Chang,2,8 Ming-Jang Chiu2,9– 11 1Department of Neurology, En Chu Kong Hospital, New Taipei City, Taiwan; 2Department of Neurology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan; 3Department of Healthcare Administration, College of Medical and Health Science, Asia University, Taichung, Taiwan; 4Queensland Brain Institute, University of Queensland, St. Lucia, Brisbane, QLD, Australia; 5Center for Research, Diagnostics and Vaccine Development, Taiwan Centers for Disease Control, Taipei, Taiwan; 6Department of Radiology, Taipei Veterans General Hospital, Taipei, Taiwan; 7School of Medicine, National Yang-Ming University, Taipei, Taiwan; 8Department of Neurology, Min-Sheng General Hospital, Taoyuan, Taiwan; 9Graduate Institute of Brain and Mind Sciences, College of Medicine, National Taiwan University, Taipei, Taiwan; 10Graduate Institute of Psychology, College of Science, National Taiwan University, Taipei, Taiwan; 11Graduate Institute of Biomedical Electronics and Bioinformatics, National Taiwan University, Taipei, Taiwan*These authors contributed equally to this workCorrespondence: Ming-Jang Chiu Tel +886-2-23123456 ext 65339Fax +886-2-23418395Email [email protected]: Epidemiologic studies of Creutzfeldt-Jakob disease (CJD) have been undertaken worldwide since the new variant CJD outbreak in 1996 in the United Kingdom. A nationwide report system, the Creutzfeldt-Jakob Disease Surveillance Unit (CJDSU), directed by the Centers for Disease Control of Taiwan, was established in 1997 to identify human prion diseases.Methods: From 1998 to 2017, 647 cases were referred to the committee for confirmation. The report to CJDSU included a structured questionnaire recording the clinical, demographic data, and potential iatrogenic exposure, and the results of the clinical and laboratory examination, including tests of blood and cerebrospinal fluid, electroencephalography, and brain magnetic resonance imaging.Results: In total, 356 cases (women, n=178) were ascertained to be human prion diseases, and 97.4% (n=347) were sporadic CJD, including three definite, 314 probable, and 30 possible cases; one probable variant CJD and 8 cases of the genetic form human prion diseases. The age- and gender-specific average annual incidence were also significantly higher in the second decade (0.95/1,000,000) than in the first decade (0.63/1,000,000), with an incidence rate ratio of 1.51. The incidences increased with increasing age, reaching a peak at the age of 70– 79 years. The 10-year survival curve for sCJD patients showed that the 1-, 5-, and 10-year cumulative survival rate were 52%, 5%, and 1%, respectively. PRNP polymorphisms in 170 patients showed that 98.8% were M129M and 97.6% E219E.Discussion: The significant increase in incidence after 2008 suggests the increase in the awareness of this rare disease among physicians. The longer disease duration in patients with sCJD in Taiwan than in other countries indicates that the comprehensive support of the health care system, as well as the end-of-life care culture in Taiwan, may prolong survival time in patients with such a progressive and fatal disease.Keywords: human prion diseases, spongiform encephalopathy, incidence, mortality, disease duration

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