Mestre Tiago A, Forjaz Maria João, Mahlknecht Philipp, Cardoso Francisco, Ferreira Joaquim J, Reilmann Ralf, Sampaio Cristina, Goetz Christopher G, Cubo Esther, Martinez-Martin Pablo, Stebbins Glenn T
Division of Neurology Department of Medicine Parkinson's Disease and Movement Disorders Center The Ottawa Hospital Research Institute University of Ottawa Brain and Mind Institute Ottawa Canada.
Department of Epidemiology and Biostatistics National School of Public Health Carlos III Institute of Health and La Red de Investigación en Servicios de Salud en Enfermedades Crónicas (REDISSEC) Madrid Spain.
Mov Disord Clin Pract. 2018 Jan 3;5(2):111-117. doi: 10.1002/mdc3.12571. eCollection 2018 Mar-Apr.
Motor symptoms are a major feature of Huntington's disease (HD). The International Parkinson and Movement Disorder Society (MDS) commissioned the assessment of the clinimetric properties of motor rating scales in HD to make recommendations regarding their use, following previously established standardized criteria. After a systematic literature search, a total of 6 rating scales assessing motor symptoms and signs in HD were included for review. Performance testing (reviewed elsewhere) and quantitative motor rating methods were excluded. Only the Unified Huntington's Disease Rating Scale-Total Motor Score (UHDRS-TMS) was classified as "recommended" for assessing the severity of motor signs in HD. The following scales were classified as "suggested": Abnormal Involuntary Movement Scale, the UHDRS-TMS4, the Quantified Neurological Examination, and the Marsden and Quinn Chorea Severity Scale. The committee also concluded that further assessment of existing rating scales, including the UHDRS-TMS, is necessary to determine sensitivity to change and to screening for the presence of motor signs specific to HD. There is also a need to develop a motor rating scale to be used in positive gene carriers with subtle but not definite motor signs.
运动症状是亨廷顿舞蹈病（HD）的主要特征。国际帕金森和运动障碍协会（MDS）委托对HD运动评定量表的临床测量特性进行评估，以便按照先前制定的标准化标准就其使用提出建议。经过系统的文献检索，共纳入6个评估HD运动症状和体征的评定量表进行综述。性能测试（在其他地方进行综述）和定量运动评定方法被排除在外。只有统一亨廷顿舞蹈病评定量表-总运动评分（UHDRS-TMS）被归类为“推荐”用于评估HD运动体征的严重程度。以下量表被归类为“建议使用”：异常不自主运动量表、UHDRS-TMS4、定量神经学检查以及马斯登和奎因舞蹈症严重程度量表。委员会还得出结论，有必要对包括UHDRS-TMS在内的现有评定量表进行进一步评估，以确定对变化的敏感性以及筛查HD特有的运动体征。还需要开发一种运动评定量表，用于有轻微但不明确运动体征的阳性基因携带者。
