The Chinese Version of UHDRS in Huntington’s Disease: Reliability and Validity Assessment

Article type: Research Article

Authors: Li, Xiao-Yan^{a; 1} | Bao, Yu-Feng^{a; 1} | Xie, Juan-Juan^a | Qian, Shu-Xia^a | Gao, Bin^a | Xu, Miao^b | Dong, Yi^a | Burgunder, Jean-Marc^c | Wu, Zhi-Ying^{a; d; e; *}

Affiliations: [a] 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 | [b] Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai, China | [c] Swiss Huntington’s Disease Centre, Siloah, Gümligen and, Department of Neurology, University of Bern, Bern, Switzerland | [d] 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 | [e] CAS Center for Excellence in Brain Scienceand Intelligence Technology, Shanghai, China

Correspondence: [*] Correspondence to: Zhi-Ying Wu, MD, PhD, 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, 88 Jiefang Road, Hangzhou, 310009, China. Tel./Fax: +86 571 87783569; E-mail: [email protected].

Note: [1] These authors contributed equally to this work.

Abstract: Background:The Unified Huntington’s Disease Rating Scale (UHDRS) is a universal scale assessing disease severity of Huntington’s 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’s 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.

Keywords: Huntington’s disease, UHDRS, Chinese, reliability assessment

DOI: 10.3233/JHD-220542

Journal: Journal of Huntington's Disease, vol. 11, no. 4, pp. 407-413, 2022