Delval Arnaud, Krystkowiak Pierre, Blatt Jean-Louis, Labyt Etienne, Dujardin Kathy, Destée Alain, Derambure Philippe, Defebvre Luc
Department of Neurology and Movement, Disorders, EA 2683, Hospital R. Salengro, Regional University Hospital, 59037, Lille cedex, France.
J Neurol. 2006 Jan;253(1):73-80. doi: 10.1007/s00415-005-0929-2. Epub 2005 Aug 17.
To evaluate specific patterns of locomotion in Huntington's disease (HD) and notably the respective roles of hypokinesia (i. e. a decrease in the amplitude of movement) and bradykinesia (i. e. difficulty in executing a movement, slowness) in gait disturbance.
Kinematic, spatial (stride length, speed), temporal (cadence, speed, and stride time) and angular gait parameters (joint ankle range) were recorded in 15 early-stage HD patients by means of a video motion analysis system and then compared with 15 controls and 15 Parkinson's disease (PD) patients. Hypokinesia was studied in terms of both spatial (decrease in stride length) and angular gait parameters (decrease in joint ankle range), whereas hyperkinesia was characterized by an increase in joint ankle range. Bradykinesia (defined by a decrease in gait velocity) was also assessed in terms of temporal parameters (cadence, stride time). We studied the influence of clinical symptoms (motor dysfunction, chorea, overall disability and cognitive impairment) and the CAG repeat number on gait abnormalities.
we observed a clear decrease in gait speed, a decrease in cadence and an increase in stride time (i. e. bradykinesia) for HD, with significant intra-individual variability. Cadence remained normal in PD. In HD, there was no evidence for a clear decrease in stride length, although the latter is a characteristic feature of hypokinetic gait (such as that observed in PD). Angle analysis revealed the coexistence of hyperkinesia and hypokinesia in HD, which thus participate in gait abnormalities. Gait speed in HD was correlated to the motor part of the UHDRS.
Gait in HD is mainly characterized by a timing disorder: bradykinesia was present, with severe intra-individual variability in temporal gait parameters.
评估亨廷顿舞蹈症（HD）患者的特定运动模式，尤其是运动减退（即运动幅度减小）和运动迟缓（即执行运动困难、动作缓慢）在步态障碍中各自所起的作用。
通过视频运动分析系统记录15例早期HD患者的运动学、空间（步幅长度、速度）、时间（步频、速度和步幅时间）及角度步态参数（踝关节关节活动范围），然后与15名对照者及15例帕金森病（PD）患者进行比较。从空间（步幅长度减小）和角度步态参数（踝关节关节活动范围减小）两方面研究运动减退，而运动亢进则以踝关节关节活动范围增加为特征。还根据时间参数（步频、步幅时间）评估运动迟缓（由步态速度降低定义）。我们研究了临床症状（运动功能障碍、舞蹈症、整体残疾和认知障碍）及CAG重复次数对步态异常的影响。
我们观察到HD患者步态速度明显降低、步频降低及步幅时间增加（即运动迟缓），且个体内差异显著。PD患者的步频保持正常。在HD患者中，虽然步幅长度减小是运动减退性步态的一个特征性表现（如在PD中观察到的那样），但没有证据表明其明显减小。角度分析显示HD患者存在运动亢进和运动减退并存的情况，因此二者均参与了步态异常。HD患者的步态速度与统一亨廷顿舞蹈症评定量表（UHDRS）的运动部分相关。
HD患者的步态主要特征为时间紊乱：存在运动迟缓，且步态时间参数存在严重的个体内差异。
