PT -期刊文章盟Gustavo喇嘛盟辛西娅Bonilha Da Silva AU - Thiago Rezende盟Iscia Lopes-Cendes AU - Marcondes语言,Jr . TI -脑损伤SPG4-Hereditary痉挛性截瘫(HSP): VBM的一项研究(P7.077) DP - 2014 Apr 08年TA -神经病学PG - P7.077 VI - 82 IP - 10补充4099 - //www.ez-admanager.com/content/82/10_S首页upplement/P7.077.short 4100 - //www.ez-admanager.com/content/82/10_Supplement/P7.077.full所以Neurology2014 Apr 08年;82 AB -目的:研究脑损伤SPG4-HSP使用mri体积分析。背景:SPG4-HSP是最常见的autossomal显性遗传性痉挛性截瘫。大多数的体征和症状SPG4-HSP是由于脊髓功能障碍,而很少有研究调查脑损伤疾病。设计/方法:9名患者分子确认SPG4-HSP和19岁的准确性健康对照组进行了高分辨率磁共振成像在3 t Achieva-Intera飞利浦扫描仪。体积T1图像获得使用一个标准的8通道线圈:矢状方向,240 x240x180立体像素矩阵,体素的大小1 x1x1mm3 TR / TE 7/3.201毫秒,翻转角度8º。这些图片是用来运行基于体素的形态测量学SPM8 (VBM)分析。我们使用两个样本T测试搜索不同的白质(WM)和灰质(GM)卷之间的病人和控制。多重比较的结果不纠正和显著差异在p50体素被报道。结果:患者的平均年龄为47.3±16.3年,有6个人。 All patients had a pure HSP phenotype, and none had cognitive dysfunction. VBM analyses revealed GM atrophy in the SPG4 group at the right superior temporal gyrus (peak MNI coordinates X=22.5, Y=7.5, Z=-39), right posterior cerebellar lobe (X=-36, Y=-57, Z=-22.5), right fusiform gyrus (X=46.5, Y=-52.5, Z=-19.5) and right precentral gyrus (X=48, Y=-13.5, Z=52.5). SPG4-HSP patients also had WM volumetric reduction at right temporal lobe (X=40.5, Y=-48, Z=9), left insula (X=-34.5, Y=10.5, Z=-4.5), right precentral gyrus (X=42, Y=-9, Z=30), left frontal lobe (X=-34.5, Y=24, Z=34.5) and left cyngulate gyrus (X=-12, Y=-37.5, Z=33). CONCLUSIONS: Patients with SPG4-HSP have cerebral atrophy, not restricted to motor regions and including GM and WM matter. These atrophic regions might contribute both to motor and non-motor manifestations of the disease. Further studies are needed to determine the clinical relevance of these abnormalities. Study Supported by:Disclosure: Dr. Lamas has nothing to disclose. Dr. Silva has nothing to disclose. Dr. Rezende 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