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1 "Chae-Won Shin"
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Original Article
Survival of Korean Huntington’s Disease Patients
Han-Joon Kim, Chae-Won Shin, Beomseok Jeon, Hyeyoung Park
J Mov Disord. 2016;9(3):166-170.   Published online September 21, 2016
DOI: https://doi.org/10.14802/jmd.16022
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  • 11 Web of Science
  • 10 Crossref
AbstractAbstract PDF
Objective
The survival of Huntington’s disease (HD) patients is reported to be 15–20 years. However, most studies on the survival of HD have been conducted in patients without genetic confirmation with the possible inclusion of non-HD patients, and all studies have been conducted in Western countries. The survival of patients with HD in East Asia, where its prevalence is 10–50-fold lower compared with Western populations, has not yet been reported.
Methods
Forty-seven genetically confirmed Korean HD patients from independent families were included in this retrospective medical record review study.
Results
The mean age at onset among the 47 patients was 46.1 ± 14.0 years. At the time of data collection, 25 patients had died, and these patients had a mean age at death of 57.8 ± 13.7 years. The Kaplan-Meier estimate of the median survival from onset in the 47 patients was 14.5 years (95% confidence interval: 12.3–16.6). None of the following factors were associated with the survival time in the univariate Cox regression analysis: gender, age at onset, normal CAG repeat size, mutant CAG repeat size, and the absence or presence of non-motor symptoms at onset.
Conclusion
This is the first Asian study on survival in HD patients. Survival in Korean HD patients may be shorter than that reported for Western populations, or at least is in the lower range of expected survival. A larger longitudinal observation study is needed to confirm the results found in this study.

Citations

Citations to this article as recorded by  
  • Analysis of HTT CAG repeat expansion among healthy individuals and patients with chorea in Korea
    Ryul Kim, Moon-Woo Seong, Bumjo Oh, Ho Seop Shin, Jee-Soo Lee, Sangmin Park, Mihee Jang, Beomseok Jeon, Han-Joon Kim, Jee-Young Lee
    Parkinsonism & Related Disorders.2024; 118: 105930.     CrossRef
  • Increased 10-Year Prevalence of Huntington’s Disease in South Korea: An Analysis of Medical Expenditure Through the National Healthcare System
    Chan Young Lee, Jun-soo Ro, Hyemin Jung, Manho Kim, Beomseok Jeon, Jee-Young Lee
    Journal of Clinical Neurology.2023; 19(2): 147.     CrossRef
  • Clustering and prediction of disease progression trajectories in Huntington's disease: An analysis of Enroll-HD data using a machine learning approach
    Jinnie Ko, Hannah Furby, Xiaoye Ma, Jeffrey D. Long, Xiao-Yu Lu, Diana Slowiejko, Rita Gandhy
    Frontiers in Neurology.2023;[Epub]     CrossRef
  • Survival in Huntington’s disease and other young‐onset dementias
    Samantha M. Loi, Paraskevi Tsoukra, Emily Sun, Zhibin Chen, Pierre Wibawa, Maria di Biase, Sarah Farrand, Dhamidhu Eratne, Wendy Kelso, Andrew Evans, Mark Walterfang, Dennis Velakoulis
    International Journal of Geriatric Psychiatry.2023;[Epub]     CrossRef
  • Functional Intercellular Transmission of miHTT via Extracellular Vesicles: An In Vitro Proof-of-Mechanism Study
    Roberto D. V. S. Morais, Marina Sogorb-González, Citlali Bar, Nikki C. Timmer, M. Leontien Van der Bent, Morgane Wartel, Astrid Vallès
    Cells.2022; 11(17): 2748.     CrossRef
  • Huntington's disease: Mortality and risk factors in an Australian cohort
    Emily Sun, Matthew Kang, Pierre Wibawa, Vivian Tsoukra, Zhibin Chen, Sarah Farrand, Dhamidhu Eratne, Wendy Kelso, Andrew Evans, Mark Walterfang, Dennis Velakoulis, Samantha M. Loi
    Journal of the Neurological Sciences.2022; 442: 120437.     CrossRef
  • Huntington’s disease in Turkey: genetic counseling, clinical features, and outcome
    Yesim Sucullu Karadag, Busranur Erozan Cavdarli, Rabia Nazik Yuksel
    Neurological Research.2021; 43(5): 381.     CrossRef
  • Validation of diagnostic codes and epidemiologic trends of Huntington disease: a population-based study in Navarre, Spain
    Esther Vicente, Ainara Ruiz de Sabando, Fermín García, Itziar Gastón, Eva Ardanaz, María A. Ramos-Arroyo
    Orphanet Journal of Rare Diseases.2021;[Epub]     CrossRef
  • Epidemiology of Huntington disease in Cyprus: A 20‐year retrospective study
    C.A. Demetriou, A. Heraclides, C. Salafori, G.A. Tanteles, K. Christodoulou, Y. Christou, E. Zamba‐Papanicolaou
    Clinical Genetics.2018; 93(3): 656.     CrossRef
  • Population-specific genetic modification of Huntington's disease in Venezuela
    Michael J. Chao, Kyung-Hee Kim, Jun Wan Shin, Diane Lucente, Vanessa C. Wheeler, Hong Li, Jared C. Roach, Leroy Hood, Nancy S. Wexler, Laura B. Jardim, Peter Holmans, Lesley Jones, Michael Orth, Seung Kwak, Marcy E. MacDonald, James F. Gusella, Jong-Min L
    PLOS Genetics.2018; 14(5): e1007274.     CrossRef

JMD : Journal of Movement Disorders