EPIDEMIOLOGY OF CREUTZFELDT-JAKOB-DISEASE IN THE UNITED-STATES, 1979-1990 - ANALYSIS OF NATIONAL MORTALITY DATA

The trends and current incidence of Creutzfeldt-Jakob disease (CJD) wa s examined by using a unique and potentially highly sensitive source f or case ascertainment. We analyzed death certificate information for 1 979-1990 from US multiple-cause-of-death mortality data, compiled by t he National Center for Health Statistics, Centers for Disease Control and Prevention. We evaluated death certificate data for US residents f or whom CJD was listed as one of the multiple causes of death on the d eath certificate (046.1) from the International Statistical Classifica tion of Diseases, Injuries, and Causes of Death (9th revision). Age-ad justed and age-specific CJD death rates by gender, race, and region we re calculated to measure the disease incidence because of the rapidly fatal course of the disease for most patients with CJD. We identified 2,614 deaths with CJD listed on the death certificates. The average an nual age-adjusted mortality rate was 0.9 deaths per million persons (r ange 0.8-1.1). The mean age at death was 67 years. CJD-related deaths were uncommon among persons younger than 50 years of age (4.3% of all deaths). The highest average annual mortality rate was for those perso ns aged 70-74 years (5.9 deaths per million persons). A slight majorit y (53.0%) of the deaths was in females, but the age-adjusted mortality rate was 1.2 times higher for males. Most deaths (94.8%) were in whit es; the mortality rate for blacks was only 40% of that for whites. The age-adjusted CJD mortality rate in the United States is similar to pu blished estimates of the crude incidence of CJD worldwide. Annual revi ew of national multiple-cause-of-death data may provide an efficient a nd cost-effective method to monitor the incidence of CJD in the United States. The relative paucity of cases among blacks requires further s tudy to rule out detection biases, but may reflect, in part, differenc es in genetic and/or environmental factors.