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. 2020 Sep 23;10(1):15509.
doi: 10.1038/s41598-020-72519-0.

A nationwide trend analysis in the incidence and mortality of Creutzfeldt-Jakob disease in Japan between 2005 and 2014

Yoshito Nishimura  1 , Ko Harada  2 , Toshihiro Koyama  3 , Hideharu Hagiya  2 , Fumio Otsuka  2
Affiliations

A nationwide trend analysis in the incidence and mortality of Creutzfeldt-Jakob disease in Japan between 2005 and 2014

Yoshito Nishimura et al. Sci Rep. .

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.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Figure 1
Figure 1
Total number of Creutzfeldt–Jakob Disease-associated death by sex, 2005–2014. The numbers of CJD-associated deaths by five-year age groups stratified by sex are shown. The number of CJD-associated deaths reached its peak in the 70–74 years age group in male (192 deaths over the 10-year period), and in the 70–74 and 75–79 years age groups in female (228 deaths over the 10-year period in both age groups).
Figure 2
Figure 2
Trends in crude mortality rates (per 1,000,000 population) of Creutzfeldt–Jakob Disease-associated death by age and sex, 2005–2014. The trends in crude mortality rates of CJD-associated deaths in patients over the age of 50 years by 10-year age groups and sex are described. The detailed numerical values for the age groups are available in Supplementary Table S1.
Figure 3
Figure 3
Trends in age-adjusted rates of Creutzfeldt–Jakob Disease-associated death by sex, 2005–2014. The trends of age-adjusted CJD mortality rates in the entire period by sex are shown. Average annual percentage change (%) [95% confidence interval] in total, male, and female were 3.2* [1.4, 5.1], 3.7* [1.3, 6.0], and 2.9 [− 0.1, 5.9], respectively. *Significantly different from zero (p < 0.05).
Figure 4
Figure 4
Numbers of Creutzfeldt–Jakob Disease incidence by sex, 2005–2014. The numbers of CJD incidence in each year stratified by sex are shown. The number of CJD incidence continuously increased in females over the entire study period, while it reached a peak in 2013 in males.
Figure 5
Figure 5
Trends in crude incidence rates (per 1,000,000 population) of Creutzfeldt–Jakob Disease, 2005–2014. The trends in crude incidence rates of CJD over the age of 50 years by 10-year age groups and sex are shown. The crude incidence rates per 1,000,000 population had continuous increases in the 60–69 years age groups or older.
Figure 6
Figure 6
Trends in age-adjusted rates (per 1,000,000) of Creutzfeldt–Jakob Disease incidence, 2005–2014. The trends of the overall age-adjusted CJD incidence rates in the entire period are shown. The overall age-adjusted CJD-associated incidence rates showed a significant increase over the period; average annual percentage change (%) [95% confidence interval]; 6.4* [4.7, 8.1]. *Significantly different from zero (p < 0.05).
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