van Vugt Jeroen P P, Piet Karlijne K E, Vink Liesbeth J, Siesling Sabine, Zwinderman Aeilko H, Middelkoop Huub A M, Roos Raymund A C
Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands.
Mov Disord. 2004 Mar;19(3):285-97. doi: 10.1002/mds.10718.
Functional disability of patients with Huntington's disease (HD) is determined by impairment of voluntary motor function rather than the presence of chorea. However, only few attempts have been made to quantify this motor impairment. By using a simple reaction time paradigm, we measured the time needed for movement initiation (akinesia) and execution (bradykinesia) in 76 HD patients and 127 controls. Akinesia and bradykinesia were already evident in early stages and increased linearly with increasing disease stage. Quantified motor slowness correlated with clinical impairment of voluntary movements but also with cognitive impairment and medication use. In patients without severe cognitive impairment, quantified motor slowness reflected clinical motor impairment more purely. During 1.9 years follow-up (range, 0.8-3.8 years), quantified akinesia and bradykinesia progressed concomitantly with progression of clinical impairment of voluntary movements, cognition, and functional capacity. However, rate of change in motor slowness did not discriminate between patients whose disease stage remained stable and those whose disease stage progressed. We conclude that the reaction time paradigm may be used to quantify akinesia and bradykinesia in HD, at least in patients without severe cognitive impairment. Although reaction and movement times increased in time, these measures failed to detect functionally important changes during our follow-up period.
亨廷顿舞蹈症（HD）患者的功能残疾是由自主运动功能受损决定的，而非舞蹈症的存在。然而，仅有少数研究尝试对这种运动障碍进行量化。通过使用简单反应时范式，我们测量了76例HD患者和127例对照者开始运动（运动不能）和执行运动（运动迟缓）所需的时间。运动不能和运动迟缓在疾病早期就已明显，并随疾病阶段的增加呈线性增加。量化的运动迟缓与自主运动的临床损伤相关，但也与认知损伤和药物使用有关。在没有严重认知损伤的患者中，量化的运动迟缓更纯粹地反映了临床运动损伤。在1.9年的随访期间（范围为0.8 - 3.8年），量化后的运动不能和运动迟缓与自主运动、认知和功能能力的临床损伤进展同步。然而，运动迟缓的变化率并不能区分疾病阶段保持稳定的患者和疾病阶段进展的患者。我们得出结论，反应时范式可用于量化HD患者的运动不能和运动迟缓，至少对于没有严重认知损伤的患者是如此。尽管反应时间和运动时间随时间增加，但在我们的随访期间，这些测量未能检测到功能上的重要变化。
