Spectrum of gait impairments in presymptomatic and symptomatic Huntington's disease
Spectrum of gait impairments in presymptomatic and symptomatic Huntington's disease
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DOI：
10.1002/mds.21987
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发表时间：
2008-06-15
影响因子：
8.6
通讯作者：
Marder, Karen S.
中科院分区：
文献类型：
--
作者：
Rao, Ashwini K.;Muratori, Lisa;Marder, Karen S.
本研究的目的是量化症状前和症状性亨廷顿舞蹈病（HD）受试者的步态障碍，并检查步态测量的敏感性。我们的样本（n = 65）包括症状前突变携带者（PMC） （n = 15）、症状性HD受试者（SHD） （n = 30）和健康对照（n = 20）。参与者被要求以自己喜欢的速度在记录时空变量的计算机化人行道上行走。我们对PMC和SHD进行统一HD评定量表（UHDRS）。PMC降低了步态速度（P < 0.01）、步长（P < 0.008），增加了双支撑时间（P < 0.001）；步幅长度（P < 0.01）和步幅时间（P < 0.004）的变异性高于对照组。这些损伤随着SHD疾病严重程度的增加而恶化。步态障碍与PMC的预测发病年数相关（速度= -0.65；步频= -0.70，步长= 0.71），并且在区分对照组和突变携带者方面表现出高度的敏感性和特异性。相比之下，UHDRS评分并未显示步态和平衡方面的损伤。步态运动迟缓和动态平衡障碍始于HD的症状前阶段，并在症状期继续恶化。即使在临床神经学检查未检测到损伤的情况下，步态测量在区分突变阳性和阴性个体方面也很敏感。(C) 2008运动障碍学会。
The purpose of this study was to quantify gait impairments in presymptomatic and symptomatic Huntington's disease (HD) subjects, and examine sensitivity of gait measures. Our sample (n = 65) included presymptomatic mutation carriers (PMC) (n = 15), symptomatic HD subjects (SHD) (n = 30) and healthy controls (n = 20). Participants were requested to walk at their preferred speed on a computerized walkway that recorded spatiotemporal variables. We administered the Unified HD Rating Scale (UHDRS) for PMC and SHD. PMC demonstrated decreased gait velocity (P < 0.01), stride length (P < 0.008), and increased time in double support (P < 0.001); and demonstrated higher variability in stride length (P < 0.01) and step time (P < 0.004) compared with controls. These impairments worsened with increasing disease severity for SHD. Gait impairments were con-elated with predicted years to onset in PMC (velocity = -0.65; cadence = -0.70, step time = 0.71) and demonstrated high sensitivity and specificity in distinguishing between controls and mutation carriers. In contrast, UHDRS scores did not reveal impairments in gait and balance. Gait bradykinesia and dynamic balance impairments begin in the presymptomatic stage of HD and continue to worsen in the symptomatic stages. Gait measures are sensitive in differentiating between mutation positive and negative individuals even when impairments were not detected by clinical neurological examination. (C) 2008 Movement Disorder Society.