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Kim HS, Lyoo CH, Lee PH, et al. Current Status of Huntington's Disease in Korea: A Nationwide Survey and National Registry Analysis. J Mov Disord. 2015;8(1):14-20doi: 10.14802/jmd.14038.
Kim, H. S., Lyoo, C. H., Lee, P. H., Kim, S. J., Park, M. Y., Ma, H. I., Lee, J. H., Song, S. K., Baik, J. S., Kim, J. H., & Lee, M. S. (2015). Current Status of Huntington's Disease in Korea: A Nationwide Survey and National Registry Analysis. Journal of movement disorders, 8(1), 14-20. https://doi.org/10.14802/jmd.14038
Kim, Hyun Sook, et al. "Current Status of Huntington's Disease in Korea: A Nationwide Survey and National Registry Analysis." Journal of movement disorders vol. 8,1 (2015): 14-20. doi: https://doi.org/10.14802/jmd.14038
Kim HS, Lyoo CH, Lee PH, Kim SJ, Park MY, Ma HI, Lee JH, Song SK, Baik JS, Kim JH, Lee MS. Current Status of Huntington's Disease in Korea: A Nationwide Survey and National Registry Analysis. J Mov Disord. 2015 Jan;8(1):14-20. doi: 10.14802/jmd.14038. Epub 2015 Jan 13. PMID: 25614781; PMCID: PMC4298714.
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J Mov Disord. 2015 Jan;8(1):14-20. doi: 10.14802/jmd.14038. Epub 2015 Jan 13.

Current Status of Huntington's Disease in Korea: A Nationwide Survey and National Registry Analysis.

Journal of movement disorders

Hyun Sook Kim, Chul Hyoung Lyoo, Phil Hyu Lee, Sang Jin Kim, Mee Young Park, Hyeo-Il Ma, Jae Hyeok Lee, Sook Kun Song, Jong Sam Baik, Jin Ho Kim, Myung Sik Lee

Affiliations

  1. Department of Neurology, CHA Bundang Medical Center, CHA University, Seongnam, Korea.
  2. Department of Neurology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
  3. Department of Neurology and Brain Research Institute, Severance Biomedical Science Institute, Yonsei University College of Medicine, Seoul, Korea.
  4. Department of Neurology, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea.
  5. Department of Neurology, Yeungnam University Medical Center, Daegu, Korea.
  6. Department of Neurology, Hallym University Sacred Heart Hospital, Anyang, Korea.
  7. Department of Neurology, Pusan National University Yangsan Hospital, Yangsan, Korea.
  8. Department of Neurology, Jeju National University Hospital, Jeju, Korea.
  9. Department of Neurology, Sanggye Paik Hospital, Inje University College of Medicine, Seoul, Korea.
  10. Department of Neurology, Chosun University School of Medicine, Gwangju, Korea.

PMID: 25614781 PMCID: PMC4298714 DOI: 10.14802/jmd.14038

Abstract

OBJECTIVE: Huntington's disease (HD) is a rare neurological disorder, and its current status in Korea is not well investigated. This study aims to determine the prevalence and incidence of HD and to investigate the clinical features of HD patients in Korea.

METHODS: We estimated the crude prevalence and annual incidence of HD based on the databases of the Rare Diseases Registry (RDR) and the National Health Insurance (NHI). The clinical data of genetically confirmed HD patients was collected from 10 referral hospitals and analyzed.

RESULTS: The mean calculated annual incidence was 0.06 cases per 100,000 persons, and the mean calculated prevalence was 0.38 based on the NHI database. The estimated crude prevalence based on the RDR was 0.41. Of the sixty-eight HD patients recruited, the mean age of onset was 44.16 ± 14.08 years and chorea was most frequently reported as the initial symptom and chief complaint. The mean CAG repeat number of the expanded allele was 44.7 ± 4.8 and correlated inversely with the age of onset (p < 0.001). About two-thirds of the patients have a positive family history, and HD patients without positive family history showed a delay in onset of initial symptoms, a prolonged interval between initial symptom onset and genetic diagnosis and a delay in the age of genetic diagnosis.

CONCLUSIONS: To the best of our knowledge, this is the first study to estimate the prevalence and incidence of HD in Korea and the largest HD series in the Asian population. Our analyses might be useful for further studies and large-scale investigations in HD patients.

Keywords: Database; Huntington’s disease; Incidence; Phenotype; Prevalence

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