TY -的T1 -定量肌核磁共振成像和功能措施一群晚发型GSDII病人(P07.027) JF -神经学乔-神经病学SP - P07.027 LP - P07.027六世- 80 - 7补充AU -埃巴萨盟Musumeci盟奥林匹亚-米歇首页尔·加埃塔盟阿喀琉斯Mileto AU -保罗最近AU -朱塞佩·维塔AU -安东尼奥Toscano Y1 - 2013/02/12 UR - //www.ez-admanager.com/content/80/7_Supplement/P07.027.abstract N2 -目的:我们使用定量核磁共振技术与肌肉脂肪分数(MFF)计算探索不同肌肉的参与模式和分析可能的相关性与12晚发型患者不同临床方面GSDII。背景:成人发病GSDII带来了极大的变异性临床特征和发展速度。三个主要临床表现已被观察到;与肢带肌参与肌病(LGMD),无症状hyperCKemia (HCK)和呼吸肌肉参与前肢体肌肉无力。一些关于核磁共振数据是可用的模式和跟进在这样的病人。设计/方法:12例晚期GSDII患者招募开始前酶替代治疗和基于临床表现分为3组(肢带参与,孤立hyperckemia或呼吸衰竭)。临床评估包括沃顿规模6 mwt GSGC, FVC %及MRC得分。肌肉核磁共振成像协议都使用了dual-echo dual-flip角被宠坏的梯度召回它允许精确量化和显示肌肉脂肪分数(MFF)。我们评估低腰的MFF paraspinal,腹部的肌肉和呼吸12 ambulant晚发型筛疾病患者。统计相关性是由ρ斯皮尔曼测试。结果:MFF的低腰的肌肉与肌肉功能措施6 mwt, GSGC, MRC。 In particular we found a positive correlation with ileopsoas and gluteus muscles. Moreover, we found a statistically significant correlation between degree of diaphragm atrophy and pulmonary dysfunction especially in upright position.CONCLUSIONS: In the present study we demonstrate that MFF, obtained with this MRI technique, presents a good correlation with different clinical data that are currently used as functional measures. Our data suggested that muscle MRI study could be a useful tool to evaluate the muscle impairment even the early stages.Disclosure: Dr. Barca has nothing to disclose. Dr. Musumeci has nothing to disclose. Dr. Gaeta has nothing to disclose. Dr. Mileto has nothing to disclose. Dr. Ruggeri has nothing to disclose. Dr. Vita has nothing to disclose. Dr. Toscano has nothing to disclose.Thursday, March 21 2013, 2:00 pm-7:00 pm ER -