Abstract
Creutzfeldt–Jakob disease (CJD) is transmitted by pathogenic substances called prions and can rapidly lead to death. However, no specialized treatment (medicine) has been developed to date. The route of infection is classified into sporadic (sCJD), iatrogenic, genetic forms (gCJD), and variant (vCJD). sCJD accounts for 85–90%, while gCJD accounts for 10–15%. CJD was managed through infectious disease surveillance based on the Infectious Disease Prevention and Control Act. In 2011, the monitoring method was changed to a total surveillance system. From 2017 to 2021, 276 patients had probable CJD in the Republic of Korea. Of the total cases, CJD was diagnosed in 35%, and the incidence was higher in patients aged 60–70 years. Of 276 patients, 150 were women (54.3%) and 126 were men (45.7%), with more women than men by 8.6%. For sCJD, the incidence was 92.6% higher in those aged ≥50 years than that in those <50 years old, and for gCJD, the incidence was 54.2% higher in those aged ≥50 years. Upon analysis of the incidence of cases by CJD type and age groups above 50 and under, a significant difference (p<0.05) was observed. From 2017 to 2021, the incidence of CJD by type was 87.0% for sCJD and 13.0% for gCJD, with an incidence of 0.1 per 100,000 people. There was no domestic occurrence of vCJD.