TY - T1的进行性锥体和胼胝体的损害弗里德希氏共济失调(P2.124) JF -神经学乔-神经学六世- 84 - 14补充SP - P2.124盟蒂亚戈Rezende盟辛西娅·席尔瓦A首页U -阿尔贝托·马丁内斯盟Anelyssa D 'Abreu AU - Iscia Lopes-Cendes盟——费尔南多Cendes盟Marcondes语言,Jr . Y1 - 2015/04/06 UR - //www.ez-admanager.com/content/84/14_Supplement/P2.124.abstract N2 -目的:评估白质损伤患者(WM)弗里德希氏共济失调(FRDA)及其进展。背景:FDRA是最常见的常染色体隐性共济失调,但是精确分布的神经退化疾病还不清楚。特别是,一些神经成像研究解决脑白质损伤及其临床效果。设计/方法:我们为27例(平均年龄26.8±10.4年,18人)匹配27健康对照组,进行评估在基线和随访一年以后。我们使用了弗里德希氏共济失调评定量表(FARS)量化共济失调的严重程度,其他临床变量如棉酚扩张、发病年龄、疾病持续时间和临床亚型也测量。所有的受试者都接受核磁共振成像和扩散张量成像(DTI)序列被用来评估WM异常。DTI图像通过tract-based空间统计(tbs)进行评估。组分析在横向研究中使用两个示例进行t检验和纵向研究评估使用配对t检验,所有结果修正多重比较和约翰霍普金斯白质DTI-based地图集被用来确定纤维束。我们也采用一般线性模型研究横向扩散和临床参数之间的相关性研究。结果:平均法尔斯得分和疾病持续时间分别为78.5±27.1,12.4±8.6 y,分别。 Mean GAA expansions were longGAA=1001±291 and shortGAA=843±212. Transversal group analyses showed extensive microstructural damage including superior cerebellar peduncles, corpus callosum(CC) and pyramidal tracts. Longitudinal TBSS revealed progressive microstructural abnormalities at the corpus callosum, pyramidal tracts and superior cerebellar peduncle after one year of follow-up. FA at pyramidal tracts correlated with FARS score and disease duration. We also found that FA at the posterior part of CC correlated with FARS score and disease duration. CONCLUSIONS:Patients with FDRA present widespread WM damage and have progressive microstructural abnormalities, which are detectable in a short-term of follow-up. Study Supported by:FAPESP and Capes.Disclosure: Dr. Rezende has nothing to disclose. Dr. Silva has nothing to disclose. Dr. Martinez has nothing to disclose. Dr. D'Abreu has nothing to disclose. Dr. Lopes-Cendes has nothing to disclose. Dr. Cendes has nothing to disclose. Dr. Franca, Jr. has nothing to disclose.Tuesday, April 21 2015, 7:30 am-12:00 pm ER -