PT -期刊文章盟蒂亚戈Rezende AU - Anelyssa D 'Abreu盟瑞秋吉马良斯盟Iscia Lopes-Cendes AU - Tatila Lopes盟加芙可以见到效果非盟- Marcondes语言,Jr . TI -广泛的脑皮质损伤Machado-Joseph疾病(P6.050) DP - 2014 Apr 08年TA -神经病学PG - P6.050 VI - 82 IP - 10补充4099 - //www.ez-admanager.com/content/82/10_Supplement/P6.050.short 41首页00 - //www.ez-admanager.com/content/82/10_Supplement/P6.050.full所以Neurology2014 Apr 08年;82 AB -目的:研究大脑皮层损伤SCA3 / MJD和与临床相关受影响地区,遗传学和神经心理数据。背景:Machado-Joseph疾病(SCA3 / MJD)是最常见的脊髓小脑的共济失调,表现为脑干、基底神经节和小脑损伤。很少有mri研究大脑皮层损伤SCA3 / MJD调查。设计/方法:我们包括49 SCA3 / MJD患者(平均年龄47.7±13.0,27人)和49健康对照组(平均年龄47.5±12.7,27人)。共济失调的规模评估和评级(SARA)是用来量化共济失调严重。电池我们也进行了全面的神经心理学评估认知缺陷。T1 MRI-Acquisition: 3 t设备,和3 d图像被用于分析(片1毫米,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)。皮质厚度和体积进行测量使用v.5.3 FreeSurfer软件。我们执行ANCOVA使用主题´s年龄、性别和颅内体积作为协变量比较漠视组织和执行回归评估与临床相关性,遗传学和神经心理数据。 RESULTS: Mean CAG expansion, SARA score and age-at-onset were 72.1±4.2, 14.7±7.2 and 37.5±12.5, respectively. Patients had atrophy at precentral and paracentral cortices as well as the hippocampi, temporal and occipital lobes. We also found volumetric reduction of the cerebellum, thalamus, caudate, putamen, pallidum, brainstem and ventral diencephalon. SARA scores correlated with left precentral gyrus thickness (r=-0.302, p=0.035) and brainstem volume (r=-0.414, p=0.003). Right angular gyrus thickness correlated with disease duration (r=0.587, p=0.001), but not (CAG) expansion. Similarity subscore of WAIS III presented a correlation with thickness of Left central sulcus (r=0.752, p=0.004) and Right superior occipital gyrus (r=0.704, p=0.016). CONCLUSIONS:There is cerebral cortical damage in SCA3/MJD, including motor regions and both hippocampi. These abnormalities help to explain motor and cognitive symptoms in the disease. Furthermore, severity of ataxia correlates with brainstem and precentral gyrus thickness. Study Supported by:FAPESP, CNPQ and CAPESDisclosure: Dr. Rezende has nothing to disclose. Dr. D'Abreu has nothing to disclose. Dr. Guimarães has nothing to disclose. Dr. Lopes-Cendes has nothing to disclose. Dr. Lopes has nothing to disclose. Dr. Castellano has nothing to disclose. Dr. Franca, Jr. has nothing to disclose.Thursday, May 1 2014, 7:30 am-11:00 am