M B Coulthart1, G H Jansen1,2, T Connolly1, R D'Amour1, J Kruse1, J Lynch1, S Sabourin1, Z Wang1,3, A Giulivi2, M N Ricketts4, N R Cashman5. 1. Canadian Creutzfeldt-Jakob Disease Surveillance System, Centre for Foodborne, Environmental and Zoonotic Infectious Diseases, Public Health Agency of Canada, Ottawa, ON. 2. The Ottawa Hospital, University of Ottawa and Eastern Ontario Regional Laboratory Association, Ottawa, ON. 3. Deceased November 2014. 4. University of Ottawa, Ottawa, ON. 5. Department of Medicine (Neurology), Brain Research Centre, University of British Columbia, Vancouver, BC.
Abstract
BACKGROUND: Human prion diseases, known collectively as Creutzfeldt-Jakob disease (CJD), are fatal, infectious neurodegenerative disorders that occur in all human populations. OBJECTIVE: To summarize national surveillance data for CJD in Canada between January 1, 1998, and December 31, 2013. METHODS: 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. RESULTS: 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. CONCLUSION: 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.
BACKGROUND: Human prion diseases, known collectively as Creutzfeldt-Jakob disease (CJD), are fatal, infectious neurodegenerative disorders that occur in all human populations. OBJECTIVE: To summarize national surveillance data for CJD in Canada between January 1, 1998, and December 31, 2013. METHODS: 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. RESULTS: 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. CONCLUSION: 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.
Authors: O Noel Gill; Yvonne Spencer; Angela Richard-Loendt; Carole Kelly; Reza Dabaghian; Lynnette Boyes; Jacqueline Linehan; Marion Simmons; Paul Webb; Peter Bellerby; Nick Andrews; David A Hilton; James W Ironside; Jon Beck; Mark Poulter; Simon Mead; Sebastian Brandner Journal: BMJ Date: 2013-10-15
Authors: Jesús de Pedro-Cuesta; Ignacio Mahillo-Fernandez; Miguel Calero; Alberto Rábano; Mabel Cruz; Åke Siden; Pablo Martínez-Martín; Henning Laursen; María Ruiz-Tovar; Kåre Mølbak Journal: PLoS One Date: 2014-10-03 Impact factor: 3.240