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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
- 15,581 View
- 152 Download
- 11 Web of Science
- 11 Crossref
-
Abstract
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 - Clinical and Genetic Characteristics Associated With
Survival Outcome in Late-Onset Huntington’s Disease
in South Korea
Yun Su Hwang, Sungyang Jo, Gu-Hwan Kim, Jee-Young Lee, Ho-Sung Ryu, Eungseok Oh, Seung-Hwan Lee, Young Seo Kim, Sun Ju Chung
Journal of Clinical Neurology.2024;[Epub] 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
- Analysis of HTT CAG repeat expansion among healthy individuals and patients with chorea in Korea
Case Report
- Oromandibular Dyskinesia as the Initial Manifestation of Late-Onset Huntington Disease
- Dong-Seok Oh, Eun-Seon Park, Seong-Min Choi, Byeong-Chae Kim, Myeong-Kyu Kim, Ki-Hyun Cho
- J Mov Disord. 2011;4(2):75-77.
- DOI: https://doi.org/10.14802/jmd.11016
- 62,769 View
- 69 Download
- 4 Crossref
-
Abstract
PDF
Huntington’s disease (HD) is a neurodegenerative disorder characterized by a triad of choreoathetosis, dementia and dominant inheritance. The cause of HD is an expansion of CAG trinucleotide repeats in the HD gene. Typical age at onset of symptoms is in the 40s, but the disorder can manifest at any time. Late-onset (≥ 60 years) HD is clinically different from other adult or juvenile onset HD and characterized by mild motor problem as the initial symptoms, shorter disease duration, frequent lack of family history, and relatively low CAG repeats expansion. We report a case of an 80-year-old female with oromandibular dyskinesia as an initial manifestation of HD and 40 CAG repeats.
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Citations
Citations to this article as recorded by- The oral manifestations of Huntington's disease: A systematic review of prevalence
Luciana Munhoz, Ashjan Qasim Jabbar, William José e Silva Filho, Aline Yukari Nagai, Emiko Saito Arita
Oral Diseases.2023; 29(1): 62. CrossRef - Orofacial Dyskinesia and Intractable Hiccups in a Patient with Varicella-zoster Virus Encephalomyelitis
Akito Funatsu, Yohei Yamamoto, Midori Araki, Fumitoshi Aga, Hideki Mine
Internal Medicine.2023; 62(1): 119. CrossRef - Harmine prevents 3-nitropropionic acid-induced neurotoxicity in rats via enhancing NRF2-mediated signaling: Involvement of p21 and AMPK
Mohamed Z. Habib, Mariane G. Tadros, Hadwa A. Abd-Alkhalek, Magda I. Mohamad, Dalia M. Eid, Fatma E. Hassan, Hend Elhelaly, Yasser el Faramawy, Sawsan Aboul-Fotouh
European Journal of Pharmacology.2022; 927: 175046. CrossRef - Management of Traumatic Ulcerations of Lips in a Case of Huntington’s Disease: A Novel Application of Essix Retainer
Mohamed Iqbal J
Journal of Indian Orthodontic Society.2021; 55(4): 415. CrossRef
- The oral manifestations of Huntington's disease: A systematic review of prevalence
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