- Current Status of Huntington’s Disease in Korea: A Nationwide Survey and National Registry Analysis
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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
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J Mov Disord. 2015;8(1):14-20. Published online January 31, 2015
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DOI: https://doi.org/10.14802/jmd.14038
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- 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.
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- Suppression of Myoclonus in Corticobasal Degeneration by Levetiracetam
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Jae Wook Cho, Jae Hyeok Lee
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J Mov Disord. 2014;7(1):28-30. Published online April 30, 2014
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DOI: https://doi.org/10.14802/jmd.14007
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10,587
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- Myoclonus in corticobasal degeneration (CBD) has often been associated with severe and difficult to treat disabilities. Levetiracetam is a new antiepileptic agent with antimyoclonic effects. Herein, we present a 72-year-old woman with clinically probable CBD and with spontaneous rhythmic myoclonus in the right foot, which was markedly ameliorated through treatment with levetiracetam. The effect of levetiracetam was associated with the decreased amplitude of enlarged cortical somatosensory evoked potentials. This result suggests that the antimyoclonic effect of levetiracetam might be mediated through the suppression of increased cortical excitability.
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Lawren VandeVrede, Peter A. Ljubenkov, Julio C. Rojas, Ariane E. Welch, Adam L. Boxer
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Ashley B. Pena, John N. Caviness
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Current Treatment Options in Neurology.2016;[Epub] CrossRef - An Update and Review of the Treatment of Myoclonus
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- Oculogyric Crisis Associated with Disulfiram-Induced Pallidonigral Lesion
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Jae Hyeok Lee, Chul Hyoung Lyoo, Jin Goo Lee, Myung Sik Lee
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J Mov Disord. 2009;2(1):48-49.
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DOI: https://doi.org/10.14802/jmd.09013
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9,453
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We report a man who developed oculogyric crisis one month after disulfiram intoxication. Brain MRI showed lesions involving bilateral globus pallidus and left substantia nigra. In our patient, neuronal discharges from pathologically reorganized basal ganglia circuit to the mid-brain ocular motor center might lead to tonic deviation of the eyes. -
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Ping Jin, Xiao‐Ming Fu, Yu Wang, Xin‐Feng Ma, Wen‐Long Ai, Ya‐Yun Xu, Bo Li, Qun‐Rong Ye, Gong‐Qiang Wang
Movement Disorders Clinical Practice.2024; 11(3): 315. CrossRef - Delayed onset of impaired consciousness complicated with ketoacidosis after disulfiram overdose
Kohei Kakimoto, Mayu Hikone, Kazuhiro Sugiyama
Acute Medicine & Surgery.2023;[Epub] CrossRef
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