PMID: 29769950Aug 6, 2015Paper

Creutzfeldt-Jakob disease mortality in Canada, 1998 to 2013

Canada Communicable Disease Report = Relevé Des Maladies Transmissibles Au Canada
M B CoulthartN R Cashman

Abstract

Human prion diseases, known collectively as Creutzfeldt-Jakob disease (CJD), are fatal, infectious neurodegenerative disorders that occur in all human populations. To summarize national surveillance data for CJD in Canada between January 1, 1998, and December 31, 2013. 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. 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 signific...Continue Reading

Citations

Sep 28, 2016·Brain : a Journal of Neurology·Michael B CoulthartLawrence B Schonberger
Mar 12, 2019·Journal of Neuropathology and Experimental Neurology·Colleen E D'ArcyGerard H Jansen
Feb 10, 2021·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Yanyuan DaiLi Cui

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