Epidemiological study of Creutzfeldt-Jakob disease death certificates in Canada, 1979-2001.

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.