1.Characteristics of Genetic Creutzfeldt-Jakob Disease in Korea: 2017-2023
InSook SHIN ; JongHee KIM ; SuGwon ROH ; ByoungChul GILL ; YoungSoon YANG
Journal of the Korean Neurological Association 2025;43(1):12-20
Background:
Creutzfeldt-Jakob disease (CJD) is a rare and a rapidly progressive, invariably fatal neurodegenerative disorder believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein. The disease develops in very different ways, such as sporadic, genetic, and iatrogenic. We aim to identify the incidence of CJD genotypes registered in the statutory infectious disease surveillance system from 2017 to 2023 and analyze the types and characteristics of domestic pathogenic CJD genotypes to serve as a basis for preventing transmission.
Methods:
We analyzed the characteristics of hereditary CDJ among subjects reported through the Korean Centers for Disease Control and Prevention's statutory infectious disease surveillance system from 2017 to 2023.
Results:
In total, six pathogenic genotypes were identified in Korean patients, with P102L belonging to Gerstmann-Sträussler-Scheinker syndrome being the most common (16, 30.2%), followed by V180I (13, 24.5%), M232R (10, 18.9%), and E200K (seven, 18.9%), which are highly prevalent in Asia, and D178N (six, 11.3%) and V203I (one, 1.9%), which belong to fatal familial insomnia.
Conclusions
The significance of this study lies in the identification of P102L as the predominant genotype observed among individuals in their 30s and 40s in Korea. Moreover, it highlights that the occurrence of visual symptoms at an early stage is more prevalent in this age group compared to individuals over 50. Therefore, if a young individual reports a subjective decline in visual acuity not adequately accounted for by structural abnormalities of the eye, it becomes imperative to confirm the presence of CJD before undergoing ophthalmic procedures (such as corneal or retinal surgery), as these procedures involve high-risk organs for CJD transmission.
2.Characteristics of Genetic Creutzfeldt-Jakob Disease in Korea: 2017-2023
InSook SHIN ; JongHee KIM ; SuGwon ROH ; ByoungChul GILL ; YoungSoon YANG
Journal of the Korean Neurological Association 2025;43(1):12-20
Background:
Creutzfeldt-Jakob disease (CJD) is a rare and a rapidly progressive, invariably fatal neurodegenerative disorder believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein. The disease develops in very different ways, such as sporadic, genetic, and iatrogenic. We aim to identify the incidence of CJD genotypes registered in the statutory infectious disease surveillance system from 2017 to 2023 and analyze the types and characteristics of domestic pathogenic CJD genotypes to serve as a basis for preventing transmission.
Methods:
We analyzed the characteristics of hereditary CDJ among subjects reported through the Korean Centers for Disease Control and Prevention's statutory infectious disease surveillance system from 2017 to 2023.
Results:
In total, six pathogenic genotypes were identified in Korean patients, with P102L belonging to Gerstmann-Sträussler-Scheinker syndrome being the most common (16, 30.2%), followed by V180I (13, 24.5%), M232R (10, 18.9%), and E200K (seven, 18.9%), which are highly prevalent in Asia, and D178N (six, 11.3%) and V203I (one, 1.9%), which belong to fatal familial insomnia.
Conclusions
The significance of this study lies in the identification of P102L as the predominant genotype observed among individuals in their 30s and 40s in Korea. Moreover, it highlights that the occurrence of visual symptoms at an early stage is more prevalent in this age group compared to individuals over 50. Therefore, if a young individual reports a subjective decline in visual acuity not adequately accounted for by structural abnormalities of the eye, it becomes imperative to confirm the presence of CJD before undergoing ophthalmic procedures (such as corneal or retinal surgery), as these procedures involve high-risk organs for CJD transmission.
3.Characteristics of Genetic Creutzfeldt-Jakob Disease in Korea: 2017-2023
InSook SHIN ; JongHee KIM ; SuGwon ROH ; ByoungChul GILL ; YoungSoon YANG
Journal of the Korean Neurological Association 2025;43(1):12-20
Background:
Creutzfeldt-Jakob disease (CJD) is a rare and a rapidly progressive, invariably fatal neurodegenerative disorder believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein. The disease develops in very different ways, such as sporadic, genetic, and iatrogenic. We aim to identify the incidence of CJD genotypes registered in the statutory infectious disease surveillance system from 2017 to 2023 and analyze the types and characteristics of domestic pathogenic CJD genotypes to serve as a basis for preventing transmission.
Methods:
We analyzed the characteristics of hereditary CDJ among subjects reported through the Korean Centers for Disease Control and Prevention's statutory infectious disease surveillance system from 2017 to 2023.
Results:
In total, six pathogenic genotypes were identified in Korean patients, with P102L belonging to Gerstmann-Sträussler-Scheinker syndrome being the most common (16, 30.2%), followed by V180I (13, 24.5%), M232R (10, 18.9%), and E200K (seven, 18.9%), which are highly prevalent in Asia, and D178N (six, 11.3%) and V203I (one, 1.9%), which belong to fatal familial insomnia.
Conclusions
The significance of this study lies in the identification of P102L as the predominant genotype observed among individuals in their 30s and 40s in Korea. Moreover, it highlights that the occurrence of visual symptoms at an early stage is more prevalent in this age group compared to individuals over 50. Therefore, if a young individual reports a subjective decline in visual acuity not adequately accounted for by structural abnormalities of the eye, it becomes imperative to confirm the presence of CJD before undergoing ophthalmic procedures (such as corneal or retinal surgery), as these procedures involve high-risk organs for CJD transmission.
4.Annual Fluctuation in Chigger Mite Populations and Orientia Tsutsugamushi Infections in Scrub Typhus Endemic Regions of South Korea
Seong Yoon KIM ; Byoungchul GILL ; Bong Gu SONG ; Hyuk CHU ; Won Il PARK ; Hee Il LEE ; E hyun SHIN ; Shin Hyeong CHO ; Jong Yul ROH
Osong Public Health and Research Perspectives 2019;10(6):351-358
OBJECTIVES: Chigger mites are vectors for scrub typhus. This study evaluated the annual fluctuations in chigger mite populations and Orientia tsutsugamushi infections in South Korea.METHODS: During 2006 and 2007, chigger mites were collected monthly from wild rodents in 4 scrub typhus endemic regions of South Korea. The chigger mites were classified based on morphological characteristics, and analyzed using nested PCR for the detection of Orientia tsutsugamushi.RESULTS: During the surveillance period, the overall trapping rate for wild rodents was 10.8%. In total, 17,457 chigger mites (representing 5 genera and 15 species) were collected, and the average chigger index (representing the number of chigger mites per rodent), was 31.7. The monthly chigger index was consistently high (> 30) in Spring (March to April) and Autumn (October to November). The mite species included Leptotrombidium pallidum (43.5%), L. orientale (18.9%), L. scutellare (18.1%), L. palpale (10.6%), and L. zetum (3.6%). L. scutellare and L. palpale populations, were relatively higher in Autumn. Monthly O. tsutsugamushi infection rates in wild rodents (average: 4.8%) and chigger mites (average: 0.7%) peaked in Spring and Autumn.CONCLUSION: The findings demonstrated a bimodal pattern of the incidence of O. tsutsugamushi infections. Higher infection rates were observed in both wild rodents and chigger mites, in Spring and Autumn. However, this did not reflect the unimodal incidence of scrub typhus in Autumn. Further studies are needed to identify factors, such as human behavior and harvesting in Autumn that may explain this discordance.
Globus Pallidus
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Humans
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Incidence
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Korea
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Mites
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Orientia tsutsugamushi
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Polymerase Chain Reaction
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Rodentia
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Scrub Typhus
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Trombiculidae
5.Intranasal Vaccination with OuterMembrane Protein of Orientia tsutsugamushi induces Protective Immunity Against Scrub Typhus
Sung-Moo PARK ; Min Jeong GU ; Young-Jun JU ; In Su CHEON ; Kyu-Jam HWANG ; Byoungchul GILL ; Byoung-Shik SHIM ; Hang-Jin JEONG ; Young Min SON ; Sangho CHOI ; Woonhee JEUNG ; Seung Hyun HAN ; Hyuk CHU ; Cheol-Heui YUN
Immune Network 2021;21(2):e14-
Scrub typhus develops after the individual is bitten by a trombiculid mite infected with Orientia tsutsugamushi. Since it has been reported that pneumonia is frequently observed in patients with scrub typhus, we investigated whether intranasal (i.n.) vaccination with the outer membrane protein of O. tsutsugamushi (OMPOT) would induce a protective immunity against O. tsutsugamushi infection. It was particular interest that when mice were infected with O. tsutsugamushi, the bacteria disseminated into the lungs, causing pneumonia. The i.n. vaccination with OMPOT induced IgG responses in serum and bronchoalveolar lavage (BAL) fluid. The anti-O. tsutsugamushi IgA Abs in BAL fluid after the vaccination showed a high correlation of the protection against O. tsutsugamushi. The vaccination induced strong Ag-specific Th1 and Th17 responses in the both spleen and lungs. In conclusion, the current study demonstrated that i.n. vaccination with OMPOT elicited protective immunity against scrub typhus in mouse with O. tsutsugamushi infection causing subsequent pneumonia.