Introduction: A descriptive epidemiological analysis to update trends of Creutzfeldt-Jakob disease (CJD) deaths, from 1979-2001, was undertaken.
Methods: Cases with CJD as underlying cause were extracted. Age-adjusted death rates by age, sex, and province were calculated. Information on birthplace, autopsy indications and type of work were examined for death certificates from 1979 to 1997.
Results: 462 cases were identified between 1979 and 1997. The average annual age-standardized mortality rate was 0.93 deaths per million persons during this period and 1.03 for 1998-2001. Persons 60 years or older demonstrated the highest average annual mortality rate. Rates were slightly higher among males and increased with age. Persons born in Canada accounted for 72% of deaths. Cause of death was verified by autopsy for 9.1% of patients while 21% of deaths indicated that additional information relating to underlying cause was expected. The service industry occupation represented the largest mortality (Quebec does not capture these data).
Conclusions: Canadian rates are consistent with those of the United States and slightly higher than those of certain European countries. Approximately 44% of CJD cases had an autopsy record, though many were incomplete. We are unable to determine a relation with occupation. We recommend annual analysis of CJD death registrations for updated surveillance of trends, as mortality data are an efficient tool for monitoring incidence.