PT -期刊文章盟n柏克德AU -罗德Scahill盟收听距离玫瑰花AU - t . Acharya盟S.J.A. van den Bogaard AU - c . Jauffret盟M.J.说,非盟- a Sturrock AU - h·约翰逊AU -长石球奥诺拉托盟d.h礼拜盟——a·杜尔盟开国元勋之一B.R.安贝德卡对莱维特AU - R.A.C.鲁斯盟G.B. Landwehrmeyer AU -湄janice盟J.C.胖胖盟中华民国Tabrizi AU - r . Reilmann TI开发相关功能和结构premanifest和亨廷顿病症状援助- 10.1212 / WNL。0 b013e3182020123 DP - 2010年12月14日TA -神首页经病学第六PG - 2150 - 2160 - 75 IP - 24 4099 - //www.ez-admanager.com/content/75/24/2150.short 4100 - //www.ez-admanager.com/content/75/24/2150.full所以Neurology2010 12月14日;75 AB -目的:电动机亨廷顿疾病的迹象在功能上禁用功能。运动信号定义疾病表现特征。评估他们的严重程度和发作的电动机总分统一亨廷顿氏舞蹈症评定量表,绝对规模有限,评分者间信premanifest科目的可变性和不敏感。更客观、可靠和精确的措施需要允许premanifest人群的临床试验。我们假设电动机赤字可以客观地量化force-transducer-based攻与疾病负担和脑萎缩。方法:总共有123控制,120 premanifest和123早期症状基因携带者进行了加速和一个节拍器在多中心研究TRACK-HD开发任务。电动机总得分,CAG重复长度和核磁共振成像。premanifest组细分为A和B,基于距离估计疾病发作,清单组分为几个阶段1和2,根据他们的总功能能力的分数。分析集中和失明。 Results: Tapping variability distinguished between all groups and subgroups in both tasks and correlated with 1) disease burden, 2) clinical motor phenotype, 3) gray and white matter atrophy, and 4) cortical thinning. Speeded tapping was more sensitive to the detection of early changes. Conclusion: Tapping deficits are evident throughout manifest and premanifest stages. Deficits are more pronounced in later stages and correlate with clinical scores as well as regional brain atrophy, which implies a link between structure and function. The ability to track motor phenotype progression with force-transducer-based tapping measures will be tested prospectively in the TRACK-HD study.