{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,7]],"date-time":"2026-11-07T18:21:51Z","timestamp":1794075711270,"version":"4.8.0"},"reference-count":18,"publisher":"SAGE Publications","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JHD"],"published-print":{"date-parts":[[2022,12,16]]},"abstract":"<jats:p>Background: The Unified Huntington\u2019s Disease Rating Scale (UHDRS) is a universal scale assessing disease severity of Huntington\u2019s disease (HD). However, the English version cannot be widely used in China, and the reliability and validity of the Chinese UHDRS have not yet been confirmed. Objective: To test the reliability and validity of Chinse UHDRS in patients with HD. Methods: Between August 2013 and August 2021, 159 HD patients, 40 premanifest HD, and 64 healthy controls were consecutively recruited from two medical centers in China and assessed by Chinese UHDRS. Internal consistency and interrater reliability of the scale were examined. Intercorrelation was performed to analyze the convergent and divergent validity of the scale. A receiver operating characteristic analysis was conducted to explore the optimal cutoff point of each cognitive test. Results: High internal consistency was found in Chinese UHDRS, and its Cronbach\u2019s alpha values of the motor, cognitive, behavioral and functional subscales were 0.954, 0.826, 0.804, and 0.954, respectively. The interrater reliability of the total motor score was 0.960. The convergent and divergent validity revealed that motor, cognitive and functional subscales strongly related to each other except for Problem Behavior Assessment. Furthermore, we not only provided the normal level of each cognitive test in controls, but also gave the optimal cutoff points of cognitive tests between controls and HD patients. Conclusion: We demonstrate for the first time that the translated version of UHDRS is reliable for assessing HD patients in China. This can promote the universal use of UHDRS in clinical practice.<\/jats:p>","DOI":"10.3233\/jhd-220542","type":"journal-article","created":{"date-parts":[[2022,9,13]],"date-time":"2022-09-13T11:45:37Z","timestamp":1663069537000},"page":"407-413","source":"Crossref","is-referenced-by-count":5,"title":["The Chinese Version of UHDRS in Huntington\u2019s Disease: Reliability and Validity Assessment"],"prefix":"10.1177","volume":"11","author":[{"given":"Xiao-Yan","family":"Li","sequence":"first","affiliation":[{"name":"Department of Neurology and Department of Medical Genetics in Second Affiliated Hospital and Key Laboratory of Medical Neurobiology of Zhejiang Province, Zhejiang University School of Medicine, Hangzhou, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu-Feng","family":"Bao","sequence":"additional","affiliation":[{"name":"Department of Neurology and Department of Medical Genetics in Second Affiliated Hospital and Key Laboratory of Medical Neurobiology of Zhejiang Province, Zhejiang University School of Medicine, Hangzhou, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan-Juan","family":"Xie","sequence":"additional","affiliation":[{"name":"Department of Neurology and Department of Medical Genetics in Second Affiliated Hospital and Key Laboratory of Medical Neurobiology of Zhejiang Province, Zhejiang University School of Medicine, Hangzhou, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shu-Xia","family":"Qian","sequence":"additional","affiliation":[{"name":"Department of Neurology and Department of Medical Genetics in Second Affiliated Hospital and Key Laboratory of Medical Neurobiology of Zhejiang Province, Zhejiang University School of Medicine, Hangzhou, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bin","family":"Gao","sequence":"additional","affiliation":[{"name":"Department of Neurology and Department of Medical Genetics in Second Affiliated Hospital and Key Laboratory of Medical Neurobiology of Zhejiang Province, Zhejiang University School of Medicine, Hangzhou, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miao","family":"Xu","sequence":"additional","affiliation":[{"name":"Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi","family":"Dong","sequence":"additional","affiliation":[{"name":"Department of Neurology and Department of Medical Genetics in Second Affiliated Hospital and Key Laboratory of Medical Neurobiology of Zhejiang Province, Zhejiang University School of Medicine, Hangzhou, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Marc","family":"Burgunder","sequence":"additional","affiliation":[{"name":"Swiss Huntington\u2019s Disease Centre, Siloah, G\u00fcmligen and, Department of Neurology, University of Bern, Bern, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhi-Ying","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Neurology and Department of Medical Genetics in Second Affiliated Hospital and Key Laboratory of Medical Neurobiology of Zhejiang Province, Zhejiang University School of Medicine, Hangzhou, China"},{"name":"NHC and CAMS Key Laboratory of Medical Neurobiology, MOE Frontier Science Center for Brain Research and Brain-Machine Integration, School of Brain Science and Brain Medicine, Zhejiang University, Hangzhou, China"},{"name":"CAS Center for Excellence in Brain Scienceand Intelligence Technology, Shanghai, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","reference":[{"issue":"3","key":"10.3233\/JHD-220542_ref1","doi-asserted-by":"publisher","first-page":"312","DOI":"10.1007\/s12264-016-0093-y","article-title":"Development of research on Huntington disease in China","volume":"33","author":"Li","year":"2017","journal-title":"Neurosci Bull"},{"issue":"1","key":"10.3233\/JHD-220542_ref2","doi-asserted-by":"publisher","first-page":"342","DOI":"10.1007\/s12035-015-9662-8","article-title":"Huntington\u2019s disease: relationship between phenotype and genotype","volume":"54","author":"Sun","year":"2017","journal-title":"Mol Neurobiol"},{"key":"10.3233\/JHD-220542_ref3","doi-asserted-by":"publisher","first-page":"342","DOI":"10.1038\/nrd2015.5","article-title":"Huntington disease","volume":"1","author":"Bates","year":"2015","journal-title":"Nat Rev Dis Primers.5"},{"issue":"2","key":"10.3233\/JHD-220542_ref4","doi-asserted-by":"publisher","first-page":"273","DOI":"10.1111\/ene.14072","article-title":"Haplotype analysis, encompassing HTT gene in Chinese patients with Huntington\u2019s disease","volume":"27","author":"Li","year":"2020","journal-title":"Eur J Neurol"},{"issue":"10","key":"10.3233\/JHD-220542_ref5","doi-asserted-by":"publisher","first-page":"1156","DOI":"10.1136\/jnnp-2012-304636","article-title":"Prevalence of adult Huntington\u2019s disease in the UK based on diagnoses recorded in general practice records","volume":"84","author":"Evans","year":"2013","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"2","key":"10.3233\/JHD-220542_ref6","doi-asserted-by":"publisher","first-page":"136","DOI":"10.1002\/mds.870110204","article-title":"Huntington Study Grou","volume":"11","author":"Unified Huntington\u2019s Disease Rating Scale: reliability and consistency","year":"1996","journal-title":"Mov Disord"},{"issue":"6","key":"10.3233\/JHD-220542_ref7","doi-asserted-by":"publisher","first-page":"502","DOI":"10.1016\/S1474-4422(20)30143-5","article-title":"Biological and clinical characteristics of gene carriers far from predicted onset in the Huntington\u2019s disease Young Adult Study (HD-YAS): a cross-sectional analysis","volume":"19","author":"Scahill","year":"2020","journal-title":"Lancet Neurol"},{"key":"10.3233\/JHD-220542_ref8","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.aat7108"},{"issue":"1","key":"10.3233\/JHD-220542_ref9","doi-asserted-by":"publisher","first-page":"42","DOI":"10.1016\/S1474-4422(11)70263-0","article-title":"Potential endpoints for clinical trials in premanifest and early Huntington\u2019s disease in the TRACK-HD study: analysis of 24 month observational data","volume":"11","author":"Tabrizi","year":"2012","journal-title":"Lancet Neurol"},{"issue":"4","key":"10.3233\/JHD-220542_ref10","doi-asserted-by":"publisher","first-page":"380","DOI":"10.1111\/j.1399-0004.2012.01927.x","article-title":"Chinese patients with Huntington\u2019s disease initially presenting with spinocerebellar ataxia","volume":"83","author":"Dong","year":"2013","journal-title":"Clin Genet"},{"issue":"4","key":"10.3233\/JHD-220542_ref11","doi-asserted-by":"publisher","first-page":"556","DOI":"10.1212\/wnl.62.4.556","article-title":"Diagnostic utility of abbreviated fluency measures in Alzheimer disease and vascular dementia","volume":"62","author":"Canning","year":"2004","journal-title":"Neurology"},{"issue":"1","key":"10.3233\/JHD-220542_ref12","doi-asserted-by":"publisher","first-page":"105","DOI":"10.1093\/arclin\/acz020","article-title":"Symbol Digit Modalities Test:regression-based normative data and clinical utility","volume":"35","author":"Fellows","year":"2019","journal-title":"Arch ClinNeuropsychol"},{"key":"10.3233\/JHD-220542_ref13","doi-asserted-by":"publisher","first-page":"12557","DOI":"10.3389\/fpsyg.2017.00557","article-title":"The Stroop Color and Word Test","volume":"8","author":"Scarpina","year":"2017","journal-title":"Front Psychol"},{"issue":"2","key":"10.3233\/JHD-220542_ref14","doi-asserted-by":"publisher","first-page":"203","DOI":"10.1016\/S0887-6177(03)00039-8","article-title":"Trail Making Test A and B: normative data stratified by age and education","volume":"19","author":"Tombaugh","year":"2004","journal-title":"Arch Clin Neuropsychol"},{"key":"10.3233\/JHD-220542_ref15","doi-asserted-by":"publisher","first-page":"336","DOI":"10.1016\/j.ijge.2018.06.003","article-title":"Reliability of the Chinese version of the Trail Making Test and Stroop Color and Word Test among older adults","volume":"12","author":"Wang","year":"2018","journal-title":"Int J Gerontol"},{"issue":"3","key":"10.3233\/JHD-220542_ref16","doi-asserted-by":"publisher","first-page":"287","DOI":"10.1176\/jn2010.22.3.287","article-title":"Correlates of apathy in Huntington\u2019s disease","volume":"22","author":"van Duijn","year":"2010","journal-title":"J Neuropsychiatry Clin Neurosci"},{"key":"10.3233\/JHD-220542_ref17","doi-asserted-by":"publisher","first-page":"333","DOI":"10.1016\/j.jpsychires.2021.07.024","article-title":"The clinical, imaging and","volume":"141","author":"Li","year":"2021","journal-title":"biological features of psychosis in Han Chinese patients with Huntington\u2019s disease. J Psychiatr Res"},{"issue":"1","key":"10.3233\/JHD-220542_ref18","doi-asserted-by":"publisher","first-page":"1","DOI":"10.3233\/JHD-180335","article-title":"Obsessive-compulsive and perseverative behaviors in Huntington\u2019s Disease","volume":"8","author":"Oosterloo","year":"2019","journal-title":"J Huntingtons Dis"}],"container-title":["Journal of Huntington's Disease"],"original-title":[],"link":[{"URL":"https:\/\/content.iospress.com\/download?id=10.3233\/JHD-220542","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T08:56:11Z","timestamp":1777452971000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/full\/10.3233\/JHD-220542"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12,16]]},"references-count":18,"journal-issue":{"issue":"4"},"URL":"https:\/\/doi.org\/10.3233\/jhd-220542","relation":{},"ISSN":["1879-6397","1879-6400"],"issn-type":[{"value":"1879-6397","type":"print"},{"value":"1879-6400","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,12,16]]}}}