Abstract
In the era of hyper-ageing, Creutzfeldt–Jakob disease (CJD) can become more prevalent as an important cause of dementia. This study aimed to evaluate the trends in crude and age-adjusted CJD-associated mortality and incidence rates in Japan using national vital statistics data on CJD-associated deaths among individuals aged over 50 years, as well as the government-funded nationwide CJD surveillance data (pertaining to the years 2005–2014) in Japan. The data were analysed using the Joinpoint Regression Program to estimate the long-term trends and average annual percentage changes (AAPCs). Overall, the AAPCs of age-adjusted CJD-associated mortality rates rose significantly over the study period (3.2%; 95% confidence interval [CI] 1.4–5.1%). The AAPC of the age-adjusted incidence rates also increased (overall 6.4%; 95% CI 4.7–8.1%). The CJD-associated increases in the mortality and incidence rates were especially prominent among adults over the age of 70 years. Given this trend in aging of population, the disease burden of CJD will continue to increase in severity. Our findings thus recommend that policymakers be aware of the importance of CJD and focus on preparing to address the increasing prevalence of dementia.
Highlights
In the era of hyper-ageing, Creutzfeldt–Jakob disease (CJD) can become more prevalent as an important cause of dementia
The annual percentage changes (AAPCs) of the crude mortality rates period remained stable in the age groups of 50–59 and 60–69 years across the entire study period, while significant increases were observed in the age groups of 70–79 and 80–89 years
Our study revealed an increase in the absolute number of deaths, mortality rates, and incidence rates associated with CJD—even after age-adjustment—in Japan between 2005 and 2014
Summary
In the era of hyper-ageing, Creutzfeldt–Jakob disease (CJD) can become more prevalent as an important cause of dementia. The CJD-associated increases in the mortality and incidence rates were especially prominent among adults over the age of 70 years. Given this trend in aging of population, the disease burden of CJD will continue to increase in severity. Characterized by spongiform encephalopathy that causes rapidly-progressing dementia and a rate of high mortality within a year of the onset of clinical symptoms, Creutzfeldt–Jakob disease (CJD) is the most common human prion d isease[1]. By 2030, the global population of individuals aged 60 years or older will increase by 34%9 This rise will shift the demographic and epidemiological landscapes of disease. Vulnerability to progressive dementia, it is imperative to evaluate longitudinal national trends of CJD-associated mortality rates and thereby help to inform robust dementia response action and future strategic policy planning
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