AN EXPLORATION OF THE EVOLUTION AND SPATIAL DISTRIBUTION OF MORTALITY FROM CREUTZFELDT-JAKOB DISEASE IN BRAZIL (1996– 2024)

Abstract

Introduction: Creutzfeldt-Jakob Disease (CJD) is a rare, rapidly progressive, and highly fatal transmissible spongiform encephalopathy. It results from the conversion of the normal prion protein into its pathogenic form, leading to neurodegeneration. CJD is classified into sporadic (most common), familial, and acquired forms. Objectives: To analyze the temporal trends and spatial distribution of mortality from Creutzfeldt-Jakob Disease in Brazil from 1996 to 2024, assessing regional patterns and demographic characteristics. Methods: Ecological time-series study using data from the Integrated Health Surveillance Platform. Mortality rates were standardized per one million inhabitants and analyzed using Prais-Winsten regression. Annual percent change was calculated, and the chi-square test was applied to assess sociodemographic associations. Results: A total of 1,009 deaths were recorded. Most occurred among women (54.2%) and individuals aged 60–69 years. The Southeast region concentrated the highest number of cases. White individuals accounted for 75.3% of deaths, although non-white individuals had a higher proportion of early mortality, defined as those occurring before the age of 60. Conclusion: CJD mortality has increased, reflecting population aging and improvements in surveillance and diagnosis. Regional and racial disparities suggest underreporting and limited access to diagnosis in less developed areas.