@article {RezendeP2.124作者={蒂亚戈Rezende辛西娅·席尔瓦和阿尔贝托·马丁内斯Anelyssa D {\ textquoteright}阿伯和Iscia Lopes-Cendes和费尔南多Cendes Marcondes语言,Jr .)}, title ={进步锥体和胼胝体的损害Friedreich {\ textquoteright}年代共济失调(P2.124)},体积={84}={14}补充数量,elocation-id = {P2.124} ={2015},出版商= {Wolters Kluwer健康,公司代表美国神经病学学会},文摘={目的:评估白质损伤患者(WM) Friedreich {\ textquoteright}年代共济失调(FRDA)及其进展。首页背景: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 {\ textpm} 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Friedreich {\ textquoteright}年代共济失调评定量表(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:意思是法尔斯的得分和疾病持续时间78.5 {\ textpm} 27.1和12.4 {\ textpm} 8.6 y,分别。 Mean GAA expansions were longGAA=1001{\textpm}291 and shortGAA=843{\textpm}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{\textquoteright}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}, issn = {0028-3878}, URL = {//www.ez-admanager.com/content/84/14_Supplement/P2.124}, eprint = {//www.ez-admanager.com/content}, journal = {Neurology} }