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KMID : 1143420200130251796
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Public Health Weekly Report
2020 Volume.13 No. 25 p.1796 ~ p.1807
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Surveillance of Creutzfeldt-Jakob Disease (CJD) Patients in the Republic of Korea, 2016-2019
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Lee Shin-Young
Lee Ji-Yeon
Park Sook-Kyoung
Kim Mi-Young
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Abstract
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Creutzfeldt-Jacob Disease (CJD) is a neurodegenerative disorder believed to be caused by an abnormal isoform of cellular glycoprotein known as the prion protein. This report is the result of an analysis of the epidemiological and clinical characteristics of CJD patients, which were notified to the Korea Centers for Disease Control and Prevention (KCDC) from 2016 to 2019. From 2016 to 2019, a total of 188 CJD patients were reported. Of that total, 64.9% (n=122) were between 60 to 70 years of age and 58% (n=109) were female. Cases were reported in each province with 25% (n=45) reported in Gyeonggi Province and 22.9% (n=43) reported in Seoul Metropolitan City. Across all province, 1.6% (n=3) were confirmed by autopsy, 95.2% (n=179) were probable cases, and 3.2% (n=6) were possible cases. By types of CJD, sporadic CJD accounted for 87.8% (n=165) of the cases; genetic CJD accounted for 12.2% (n=23) with 0 cases of both iatrogenic and variant CJD. The median age of onset for sporadic CJD was 67 years (ages 35 to 93), and the average duration of the disease until death was approximately 4 months (2 to 11 months). About 66.5% (n=125) of those with CJD had cerebellar dysfunction.
CJD is a fatal and incurable disease. This reported concluded that CJD requires active and continuous surveillance for early detection, and infection control to prevent its transmission through surgical equipment contaminated with prion.
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KEYWORD
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Creutzfeldt-Jakob Disease (CJD), Variant Creutzfeldt-Jakob Disease (vCJD), Prion, Transmissible Spongiform Encephalopathy
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