PT -期刊文章AU -米歇尔Blumenschine盟杰奎琳·蒙特斯AU - Ashwini Rao盟阿里扎改变非盟-克里斯汀Engelstad盟Darryl De Vivo TI - 6分钟步行试验抓住弱点和步态异常过剩1缺陷综合症(P2.333) DP - 2014 Apr 08年TA -神经病学PG - P2.333 VI - 82 IP - 10补充4099 - //www.ez-admanager.com/content/82/10_Supplement/P2.333.short 4100 - //www.ez-admanager.com/c首页ontent/82/10_Supplement/P2.333.full所以Neurology2014 Apr 08年;82 AB -目的:评估6分钟步行试验(6 mwt)与步态分析作为一个结果测量过剩1缺陷综合症(Glut1DS)。背景:临床结果的措施需要Glut1DS评估潜在疗法的效果。6 mwt是一个被广泛接受的步行能力在不同神经疾病与出版规范标准。定量步态分析加上6 mwt可以识别疲劳和步态异常。设计/方法:21患者和21个控件完成了6 mwt步态分析;平均年龄分别为16.4和18.7年。Percent-predicted距离从规范计算值确定的弱点。疲劳被定义为距离衰减的第一个六分钟。步态参数包括步幅、速度、节奏、基础支持和percent-time上双的支持。 Paired sample t-tests compared differences between Glut1DS and controls. Repeated measures ANOVA evaluated 6MWT and gait parameters over three time points in the two groups. RESULTS: Glut1DS patients (61.6%) walked shorter distances than controls (85.8%) (p<0.05). There was no measurable fatigue in either group. Glut1DS had lower velocities (p<0.0001), shorter stride lengths (p<0.0001), and spent more time in double support (p<0.001) than controls. Base of support for Glut1DS and controls was similar (p=0.11). Gait variables did not change significantly in controls, but variability in base of support increased during the six minutes in the Glut1DS group (p=0.009). CONCLUSIONS: Our studies show that Glut1DS patients are weaker than healthy controls. Fatigue is negligible. Glut1DS patients have impaired balance, spend more time in double support, and show increasing variability in base of support during the 6MWT. These disturbances reflect poor balance and weakness, and result in a decomposition of gait. Our findings indicate that the 6MWT with gait analysis captures key motor abnormalities in Glut1DS and may serve as an informative outcome measure in future clinical trials.Disclosure: Dr. Blumenschine has nothing to disclose. Dr. Montes has received personal compensation for activities with Isis Pharmeceuticals as a consultant. Dr. Rao has nothing to disclose. Dr. Alter has nothing to disclose. Dr. Engelstad has nothing to disclose. Dr. De Vivo has received personal compensation for activities with Isis Pharmaceuticals and Ultragenyx Pharmaceuticals.Tuesday, April 29 2014, 7:30 am-11:00 am