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亨廷顿病的运动障碍始于错误反控制中的功能障碍
M A Smith1, J Brandt, R Shadmehr
1Department of Biomedical Engineering, Behavioral Sciences, Johns Hopkins University, Baltimore, Maryland 21205-2195, USA. msmith@bme.jhu.edu
Nature
|February 17, 2000
概括
亨廷顿病 (HD) 导致运动功能障碍. 早期症状包括动和错误校正不良,在基因载体出现临床症状多年前就可检测到.
科学领域:
- 神经科学是一个神经科学.
- 发动机控制器的控制器
- 人类运动分析 人类运动分析
背景情况:
- 亨廷顿氏病 (HD) 的特征是逐渐发展的运动功能障碍.
- 在HD中,运动控制障碍的确切来源尚不清楚.
- 了解早期运动缺陷对于及时干预至关重要.
研究的目的:
- 调查无症状的HD基因携带者 (AGCs) 和明显的HD患者的运动控制缺陷.
- 为了识别HD进展的早期指标在达到运动.
- 阐明错误纠正在与HD相关的运动功能障碍中的作用.
主要方法:
- 在AGC,表现为HD患者和健康对照中分析到达运动.
- 运动震动,变化和纠正反应的量化.
- 对自我产生和外部生成的运动错误的敏感度的评估.
主要成果:
- 运动功能障碍的标志物 - - 运动动的增加 - - 在很大比例的AGC中被观察到,甚至在预测疾病发作前几年.
- 在HD患者中,运动终止比启动更受损和变化.
- 患有HD的人对早期运动错误的敏感性增加,对外部干扰的纠正反应受到干扰.
结论:
- 运动错误纠正的功能障碍是早期亨廷顿病的一个关键特征,可以在症状前检测到.
- 这种错误校正缺陷有助于观察到的HD中抽和运动终止受损.
- 在HD中,运动控制异常可以在临床诊断前几年被发现,并且随着疾病的进展而恶化.
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