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Creutzfeldt-Jakob disease mortality in Canada, 1998 to 2013.
- Source :
- Canada Communicable Disease Report; 8/6/2015, Vol. 41 Issue 8, p182-191, 10p
- Publication Year :
- 2015
-
Abstract
- <bold>Background: </bold>Human prion diseases, known collectively as Creutzfeldt-Jakob disease (CJD), are fatal, infectious neurodegenerative disorders that occur in all human populations.<bold>Objective: </bold>To summarize national surveillance data for CJD in Canada between January 1, 1998, and December 31, 2013.<bold>Methods: </bold>Detailed investigations were conducted of individual suspected CJD cases, with collaboration between Canadian health professionals and investigators affiliated with a central CJD surveillance registry operated by the Public Health Agency of Canada. Data were collected on the clinical profile, family history, and results of paraclinical and laboratory investigations, including post-mortem neuropathological examination.<bold>Results: </bold>A total of 662 deaths from definite and probable CJD were identified in Canadian residents during the study period, comprising 613 cases of sporadic CJD (92.6%), 43 cases of genetic prion disease (6.5%), 4 cases of iatrogenic CJD (0.6%), and 2 cases of variant CJD disease (0.3%). The overall crude mortality rate for sporadic CJD was 1.18 per million per year [95% confidence interval (CI): 1.08,1.27]. Age-specific rates ranged from 0.05 [95% CI: 0.03,0.08] in persons under 50 years of age to 7.11 [95% CI: 6.20,8.11] in those aged 70 to 79. A significant net upward trend in age-adjusted rates was observed over the study period. Standardized mortality ratios, calculated for 10 individual Canadian provinces with reference to national average mortality rates, did not differ significantly from 1.0.<bold>Conclusion: </bold>Creutzfeldt-Jakob disease remains rare in Canada, although mortality rates vary by two orders of magnitude between older and younger age groups. The upward trend in age-standardized sporadic CJD mortality rate over the study period can be better accounted for by gradually improving case ascertainment than by a real increase in incidence. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 11884169
- Volume :
- 41
- Issue :
- 8
- Database :
- Complementary Index
- Journal :
- Canada Communicable Disease Report
- Publication Type :
- Academic Journal
- Accession number :
- 109211534
- Full Text :
- https://doi.org/10.14745/ccdr.v41i08a01