中国南方地区亨廷顿患者不同运动分型的临床特征  

Clinical Characteristics of Different Motor Phenotypes in Patients with Huntington’s Disease in Southern China

作　　者：林丽山 苏凤娟[1] 吴腾腾 曾译萱 尤桦菁 陈定邦[1] 李洵桦[1] 裴中[1] LIN Li-shan;SU Feng-juan;WU Teng-teng;ZENG Yi-xuan;YOU Hua-jing;CHEN Ding-bang;LI Xun-hua;PEI Zhong(Department of Neurology,The First Affiliated Hospital of Sun Yat-sen University,Guangzhou 510080,China;De-partment of Neurology,Zhongshan Hospital of Fudan University,Shanghai 200032,China;Department of Neurology,Shenzhen Second People's Hospital,Shenzhen 518035,China)

机构地区：[1]中山大学附属第一医院神经科,广东广州510080 [2]复旦大学附属中山医院神经内科,上海200032 [3]深圳市第二人民医院神经内科,广东深圳518035

出　　处：《中山大学学报（医学科学版）》2021年第6期944-949,共6页Journal of Sun Yat-Sen University:Medical Sciences

基　　金：国家重点研发计划项目(2017YFA0105104);广州市民生科技攻关计划项目(201803010092)。

摘　　要：【目的】探讨中国南方地区亨廷顿病患者不同运动分型的临床特征,便于个体化精准治疗。【方法】纳入自2014年3月至2021年5月在中国亨廷顿病协作网广州中心登记的亨廷顿病患者58例,利用亨廷顿舞蹈症整体评估量表对患者进行运动分型,精神状态、认知功能以及全面生活能力测试,最后利用Kruskal-Wallis H检验方法对不同分型患者各组临床特征进行分析。【结果】中国南方地区亨廷顿患者运动分型以混合型为主,共42例,占比72.41%。不同分型临床特征不同,运动机能减退-僵硬为主型患者全面生活能力低于舞蹈症状为主型患者[8.00(4.00~11.00)vs 13.00(11.00~13.00);P=0.037]。【结论】中国南方地区亨廷顿患者运动症状复杂。精准的运动分型,对药物治疗与预后评估有一定的意义。【Objective】To explore clinical characteristics of different motor phenotypes in patients with Huntington’s disease in southern China for individualized precise treatment.【Method】A total of 58 Huntington’s disease(HD)patients were enrolled from Guangzhou Center of Chinese Huntington’s Disease Network(CHDN)from March 2014 to May 2021.United Huntington’s Disease Rating Scale(UHDRS)was used to assess patients’motor function,mental state,cognition and total functional capacity(TFC).The differences of clinical characteristics among different motor phenotypes were analyzed by Kruskal-Wallis test.【Results】In southern China,HD patients showed a predominant mixed-motor phenotype,with 72.41%(42/58)of all cases.The clinical characteristics among different motor phenotypes were different,and the TFC score in hypokinetic-rigidity phenotype was lower than that in choreatic motor phenotype[8.00(4.00~11.00)vs 13.00(11.00~13.00),P=0.037].【Conclusion】The symptoms of movement disorders in HD patients in southern China are complex.The precise classification of motor phenotype is helpful for the treatment and prognosis of HD.

关 键 词：亨廷顿病 运动分型 药物治疗 预后评估 

分 类 号：R441[医药卫生—诊断学]