TY - T1的双边尾状萎缩区分矛盾的Vs Non-Dystonic Machado-Joseph疾病患者(P2.133) JF -神经学乔-神经学六世- 84 - 14补充SP - P2.133盟蒂亚戈Rezende AU -阿尔贝托·马丁内斯盟马塞洛Nune首页s AU -瑞秋吉马良斯盟Anelyssa D 'Abreu盟Iscia Lopes-Cendes AU - Marcondes语言,Jr . Y1 - 2015/04/06 UR - //www.ez-admanager.com/content/84/14_Supplement/P2.133.abstract N2 -目的:比较Machado-Joseph患者脑萎缩疾病的模式(dMJD / SCA3)和没有使用VBM分析(cMJD / SCA3)肌张力障碍。背景:在SCA3 / MJD肌张力障碍是常见的,但对其解剖衬底所知甚少。设计/方法:我们招募了52 SCA3 / MJD患者和52匹配控制。我们使用萨拉(共济失调的规模评估和评级)和Marsden-Fahn评定量表(MFR-S)分别量化共济失调的严重程度和肌张力障碍。体积T1W图像获得3 t设备上使用1毫米片,TE = 3.2毫秒,TR = 7.1毫秒,翻转8°角,各向同性体素的1毫米³,FOV = 240 x240。我们与VBM SPM软件使用工具箱来评估不同的灰质体积控制和cMJD / SCA3之间,同时,控制和dMJD / SCA3。我们进行了一般线性模型使用年龄和性别不评估小组。我们采用t检验进行组分析和最后两组统计地图覆盖MNI152模板来比较不同的萎缩模式cMJD / SCA3和dMJD / SCA3。结果:我们包括33 cMJD / SCA3(平均年龄52.7±9.2 y, 16人)和19 dMJD / SCA3(平均年龄38.7±14.1 y, 11人)。dMJD / SCA3患者较长(CAG)扩张(75±3.9 vs 70±2.9,术中;0.001)和早期发病(29.6±13.3 vs 42.8±9.2 y, p = 0.001)相比,病人在cMJD / SCA3组。 Dystonic patients also had higher SARA scores (18.2±6.9 vs 12.9±6.9, p=0.017). Ten out of 19 dMJD/SCA3 had generalized dystonia, 2 blepharospasm and 7 focal limb dystonia; mean MFR-S score was 16.5±12.6. VBM analyses demonstrated significant volumetric reduction for both groups in the cerebellum and occipital lobes when compared to controls. However, bilateral caudate nuclei and left precentral gyri atrophy were only found in the dMJD/SCA3 group. CONCLUSIONS: Caudate nuclei and precentral damage underlie dystonia in MJD/SCA3. These structures are potential therapeutic targets to relieve dystonia through neuromodulation. Study Supported by: FAPESP and CapesDisclosure: Dr. Rezende has nothing to disclose. Dr. Martinez has nothing to disclose. Dr. Nunes has nothing to disclose. Dr. Guimaraes has nothing to disclose. Dr. D'Abreu has nothing to disclose. Dr. Lopes-Cendes has nothing to disclose. Dr. Franca, Jr. has nothing to disclose.Tuesday, April 21 2015, 7:30 am-12:00 pm ER -