TY -的T1 -脊髓萎缩引起的遗传性痉挛性截瘫< em > SPG4 < / em >突变(P7.076) JF -神经学乔-神经学六世- 82 - 10补充SP P7.076 AU -卢卡斯布兰科盟喇嘛Gustavo AU -菲首页利普Bergo盟Iscia Lopes-Cendes AU - Marcondes语言,Jr . Y1 - 2014/04/08 UR - //www.ez-admanager.com/content/82/10_Supplement/P7.076.abstract N2 -目的:探讨脊髓(SC)萎缩SPG4突变引起的遗传性痉挛性截瘫患者(SPG4-HSP)。背景:HSP的神经退行性疾病是一个异质群体。常染色体显性HSP SPG4-HSP是最普遍,特别是成年人。其核心功能是痉挛性截瘫和进步。虽然皮质脊髓束在SPG4-HSP受损,很少有专门调查了SC的mri研究疾病。设计/方法:分子确认SPG4-HSP患者11和22 age-and-gender-matched健康对照组进行了MRI在3 t阿奇沃飞利浦扫描仪。我们使用t1 3 d图像覆盖整个大脑和颈部SC估计颈SC面积和偏心C2和C3水平基于半自动图像分割协议使用一个软件(Spineseg)进行验证。采集参数:TE = 3.2毫秒,TR = 7.1毫秒,翻转角度= 8 o,体素的大小= 1.0 x 1.0 x 1.0 mm3 FOV = 240 x240。SC地区和古怪的病人和对照组相比使用Mann-Whitney测试。P值& lt; 0.05被认为是重要的。 RESULTS: Mean age of patients was 49 years (range 15-68) and there were 7 men. The two groups were significantly different regarding SC areas (67.5±7.8 mm² vs 54.7±5.3 mm², p<0.001). However, eccentricity values were similar in both groups (p=0.917). SC areas did not correlate with age of the patients (p=0.336). CONCLUSIONS: Patients with SPG4-HSP have SC atrophy, but no flattening. Further studies are needed to determine the clinical relevance of these abnormalities. Study Supported by: Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Disclosure: Dr. Branco has nothing to disclose. Dr. Gustavo has nothing to disclose. Dr. Bergo has nothing to disclose. Dr. Lopes-Cendes has nothing to disclose. Dr. Franca, Jr. has nothing to disclose.Thursday, May 1 2014, 3:00 pm-6:30 pm ER -