1.Validation of the Korean Version of the Huntington’s Disease Quality of Life Battery for Carers
Hee Jin CHANG ; Eungseok OH ; Won Tae YOON ; Chan Young LEE ; Kyum-Yil KWON ; Yun Su HWANG ; Chaewon SHIN ; Jee-Young LEE
Journal of Movement Disorders 2025;18(2):160-164
Objective:
The Huntington’s Disease Quality of Life Battery for Carers (HDQoL-C) is used to evaluate caregiver quality of life. This study aimed to develop and validate the Korean version of the HDQoL-C (K-HDQoL-C) to assess the burden on Korean caregivers of Huntington’s disease (HD) patients.
Methods:
A total of 19 HD caregivers (7 females, mean age 55.4±14.6 years) participated in this study. The K-HDQoL-C, a translation of the English version, consisted of demographic information, caring aspects, life satisfaction, and feelings about life. It was administered twice, 2 weeks apart. Internal consistency was evaluated using Cronbach’s α, and test-retest reliability was assessed with intraclass correlation coefficients. The relationship with the Zarit Burden Interview-12 (ZBI-12) was analyzed.
Results:
The internal consistencies of the K-HDQoL-C were 0.771 (part 2), 0.938 (part 3), and 0.891 (part 4). The test-retest reliability ranged from 0.908 to 0.936. Part 3 was negatively correlated with the ZBI-12, and part 4 was positively correlated with the ZBI-12 (r=-0.780, 0.923; p<0.001).
Conclusion
The K-HDQoL-C effectively evaluates the challenges faced by HD caregivers, particularly in terms of care aspects and life satisfaction.
2.A Practical Guide for Diagnostic Investigations and Special Considerations in Patients With Huntington’s Disease in Korea
Jangsup MOON ; Eungseok OH ; Minkyeong KIM ; Ryul KIM ; Dallah YOO ; Chaewon SHIN ; Jee-Young LEE ; Jong-Min KIM ; Seong-Beom KOH ; Manho KIM ; Beomseok JEON ;
Journal of Movement Disorders 2025;18(1):17-30
This review provides a comprehensive framework for the diagnostic approach and management of Huntington’s disease (HD) tailored to the Korean population. Key topics include genetic counseling, predictive testing, and reproductive options like preimplantation genetic testing. Strategies for assessing disease progression in premanifest HD through laboratory investigations, biofluid, and imaging biomarkers are highlighted. Special considerations for juvenile and late-onset HD, along with associated comorbidities like diabetes mellitus, hypertension, and cardiovascular abnormalities, are discussed. The guide emphasizes personalized symptom management, including pharmacotherapy, physical therapy, and nutritional support, while exploring emerging disease-modifying treatments. A multidisciplinary care model is advocated to improve outcomes for HD patients and caregivers in Korea.
3.Validation of the Korean Version of the Huntington’s Disease Quality of Life Battery for Carers
Hee Jin CHANG ; Eungseok OH ; Won Tae YOON ; Chan Young LEE ; Kyum-Yil KWON ; Yun Su HWANG ; Chaewon SHIN ; Jee-Young LEE
Journal of Movement Disorders 2025;18(2):160-164
Objective:
The Huntington’s Disease Quality of Life Battery for Carers (HDQoL-C) is used to evaluate caregiver quality of life. This study aimed to develop and validate the Korean version of the HDQoL-C (K-HDQoL-C) to assess the burden on Korean caregivers of Huntington’s disease (HD) patients.
Methods:
A total of 19 HD caregivers (7 females, mean age 55.4±14.6 years) participated in this study. The K-HDQoL-C, a translation of the English version, consisted of demographic information, caring aspects, life satisfaction, and feelings about life. It was administered twice, 2 weeks apart. Internal consistency was evaluated using Cronbach’s α, and test-retest reliability was assessed with intraclass correlation coefficients. The relationship with the Zarit Burden Interview-12 (ZBI-12) was analyzed.
Results:
The internal consistencies of the K-HDQoL-C were 0.771 (part 2), 0.938 (part 3), and 0.891 (part 4). The test-retest reliability ranged from 0.908 to 0.936. Part 3 was negatively correlated with the ZBI-12, and part 4 was positively correlated with the ZBI-12 (r=-0.780, 0.923; p<0.001).
Conclusion
The K-HDQoL-C effectively evaluates the challenges faced by HD caregivers, particularly in terms of care aspects and life satisfaction.
4.A Practical Guide for Diagnostic Investigations and Special Considerations in Patients With Huntington’s Disease in Korea
Jangsup MOON ; Eungseok OH ; Minkyeong KIM ; Ryul KIM ; Dallah YOO ; Chaewon SHIN ; Jee-Young LEE ; Jong-Min KIM ; Seong-Beom KOH ; Manho KIM ; Beomseok JEON ;
Journal of Movement Disorders 2025;18(1):17-30
This review provides a comprehensive framework for the diagnostic approach and management of Huntington’s disease (HD) tailored to the Korean population. Key topics include genetic counseling, predictive testing, and reproductive options like preimplantation genetic testing. Strategies for assessing disease progression in premanifest HD through laboratory investigations, biofluid, and imaging biomarkers are highlighted. Special considerations for juvenile and late-onset HD, along with associated comorbidities like diabetes mellitus, hypertension, and cardiovascular abnormalities, are discussed. The guide emphasizes personalized symptom management, including pharmacotherapy, physical therapy, and nutritional support, while exploring emerging disease-modifying treatments. A multidisciplinary care model is advocated to improve outcomes for HD patients and caregivers in Korea.
5.Validation of the Korean Version of the Huntington’s Disease Quality of Life Battery for Carers
Hee Jin CHANG ; Eungseok OH ; Won Tae YOON ; Chan Young LEE ; Kyum-Yil KWON ; Yun Su HWANG ; Chaewon SHIN ; Jee-Young LEE
Journal of Movement Disorders 2025;18(2):160-164
Objective:
The Huntington’s Disease Quality of Life Battery for Carers (HDQoL-C) is used to evaluate caregiver quality of life. This study aimed to develop and validate the Korean version of the HDQoL-C (K-HDQoL-C) to assess the burden on Korean caregivers of Huntington’s disease (HD) patients.
Methods:
A total of 19 HD caregivers (7 females, mean age 55.4±14.6 years) participated in this study. The K-HDQoL-C, a translation of the English version, consisted of demographic information, caring aspects, life satisfaction, and feelings about life. It was administered twice, 2 weeks apart. Internal consistency was evaluated using Cronbach’s α, and test-retest reliability was assessed with intraclass correlation coefficients. The relationship with the Zarit Burden Interview-12 (ZBI-12) was analyzed.
Results:
The internal consistencies of the K-HDQoL-C were 0.771 (part 2), 0.938 (part 3), and 0.891 (part 4). The test-retest reliability ranged from 0.908 to 0.936. Part 3 was negatively correlated with the ZBI-12, and part 4 was positively correlated with the ZBI-12 (r=-0.780, 0.923; p<0.001).
Conclusion
The K-HDQoL-C effectively evaluates the challenges faced by HD caregivers, particularly in terms of care aspects and life satisfaction.
6.A Practical Guide for Diagnostic Investigations and Special Considerations in Patients With Huntington’s Disease in Korea
Jangsup MOON ; Eungseok OH ; Minkyeong KIM ; Ryul KIM ; Dallah YOO ; Chaewon SHIN ; Jee-Young LEE ; Jong-Min KIM ; Seong-Beom KOH ; Manho KIM ; Beomseok JEON ;
Journal of Movement Disorders 2025;18(1):17-30
This review provides a comprehensive framework for the diagnostic approach and management of Huntington’s disease (HD) tailored to the Korean population. Key topics include genetic counseling, predictive testing, and reproductive options like preimplantation genetic testing. Strategies for assessing disease progression in premanifest HD through laboratory investigations, biofluid, and imaging biomarkers are highlighted. Special considerations for juvenile and late-onset HD, along with associated comorbidities like diabetes mellitus, hypertension, and cardiovascular abnormalities, are discussed. The guide emphasizes personalized symptom management, including pharmacotherapy, physical therapy, and nutritional support, while exploring emerging disease-modifying treatments. A multidisciplinary care model is advocated to improve outcomes for HD patients and caregivers in Korea.
8.Caregiver Burden of Patients With Huntington’s Disease in South Korea
Chan Young LEE ; Chaewon SHIN ; Yun Su HWANG ; Eungseok OH ; Manho KIM ; Hyun Sook KIM ; Sun Ju CHUNG ; Young Hee SUNG ; Won Tae YOON ; Jin Whan CHO ; Jae-Hyeok LEE ; Han-Joon KIM ; Hee Jin CHANG ; Beomseok JEON ; Kyung Ah WOO ; Seong-Beom KOH ; Kyum-Yil KWON ; Jangsup MOON ; Young Eun KIM ; Jee-Young LEE
Journal of Movement Disorders 2024;17(1):30-37
Objective:
This is the first prospective cohort study of Huntington’s disease (HD) in Korea. This study aimed to investigate the caregiver burden in relation to the characteristics of patients and caregivers.
Methods:
From August 2020 to February 2022, we enrolled patients with HD from 13 university hospitals in Korea. We used the 12-item Zarit Burden Interview (ZBI-12) to evaluate the caregiver burden. We evaluated the clinical associations of the ZBI-12 scores by linear regression analysis and investigated the differences between the low- and high-burden groups.
Results:
Sixty-five patients with HD and 45 caregivers were enrolled in this cohort study. The average age at onset of motor symptoms was 49.3 ± 12.3 years, with an average cytosine-adenine-guanine (CAG)n of 42.9 ± 4.0 (38–65). The median ZBI-12 score among our caregivers was 17.6 ± 14.2. A higher caregiver burden was associated with a more severe Shoulson–Fahn stage (p = 0.038) of the patients. A higher ZBI-12 score was also associated with lower independence scale (B = -0.154, p = 0.006) and functional capacity (B = -1.082, p = 0.002) scores of patients. The caregiving duration was longer in the high- than in the low-burden group. Caregivers’ demographics, blood relation, and marital and social status did not affect the burden significantly.
Conclusion
HD patients’ neurological status exerts an enormous impact on the caregiver burden regardless of the demographic or social status of the caregiver. This study emphasizes the need to establish an optimal support system for families dealing with HD in Korea. A future longitudinal analysis could help us understand how disease progression aggravates the caregiver burden throughout the entire disease course.
9.Evaluating the Validity and Reliability of the Korean Version of the Scales for Outcomes in Parkinson’s Disease–Cognition
Jinse PARK ; Eungseok OH ; Seong-Beom KOH ; In-Uk SONG ; Tae-Beom AHN ; Sang Jin KIM ; Sang-Myung CHEON ; Yoon-Joong KIM ; Jin Whan CHO ; Hyeo-Il MA ; Mee Young PARK ; Jong Sam BAIK ; Phil Hyu LEE ; Sun Ju CHUNG ; Jong-Min KIM ; Han-Joon KIM ; Young-Hee SUNG ; Do Young KWON ; Jae-Hyeok LEE ; Jee-Young LEE ; Ji Seon KIM ; Ji Young YUN ; Hee Jin KIM ; Jin Yong HONG ; Mi-Jung KIM ; Jinyoung YOUN ; Hui-Jun YANG ; Won Tae YOON ; Sooyeoun YOU ; Kyum-Yil KWON ; Su-Yun LEE ; Younsoo KIM ; Hee-Tae KIM ; Joong-Seok KIM ; Ji-Young KIM
Journal of Movement Disorders 2024;17(3):328-332
Objective:
The Scales for Outcomes in Parkinson’s Disease–Cognition (SCOPA-Cog) was developed to assess cognition in patients with Parkinson’s disease (PD). In this study, we aimed to evaluate the validity and reliability of the Korean version of the SCOPACog (K-SCOPA-Cog).
Methods:
We enrolled 129 PD patients with movement disorders from 31 clinics in South Korea. The original version of the SCOPA-Cog was translated into Korean using the translation-retranslation method. The test–retest method with an intraclass correlation coefficient (ICC) and Cronbach’s alpha coefficient were used to assess reliability. Spearman’s rank correlation analysis with the Montreal Cognitive Assessment-Korean version (MOCA-K) and the Korean Mini-Mental State Examination (K-MMSE) were used to assess concurrent validity.
Results:
The Cronbach’s alpha coefficient was 0.797, and the ICC was 0.887. Spearman’s rank correlation analysis revealed a significant correlation with the K-MMSE and MOCA-K scores (r = 0.546 and r = 0.683, respectively).
Conclusion
Our results demonstrate that the K-SCOPA-Cog has good reliability and validity.
10.Delayed Post-hypoxic Leukoencephalopathy after Carbon Monoxide Intoxication Improved by High-dose Steroid Treatment
Journal of the Korean Neurological Association 2024;42(3):259-261
Delayed post-hypoxic leukoencephalopathy (DHPL) is a demyelinating syndrome characterized by acute onset of neuropsychiatric symptoms within days to weeks following apparent recovery from coma after a period of prolonged cerebral hypo-oxygenation. The majority of DPHL cases are associated with carbon monoxide intoxication. There is no specific treatment, and the prognosis is usually poor. However, we present a case of DHPL patient with akinetic mutism, parkinsonism, stool and urine incontinence, and severe cognitive impairment improved after treatment with high-dose steroid therapy.

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