脑型肝豆状核变性脑皮层及皮层下核团体积的磁共振研究  

Impairments of cerebral cortex and subcortical nucleus in cerebral hepatolenticular degeneration: magnetic resonance imaging study

作　　者：康泰山[1] 杨天和[1] 林建忠[1] 蔡聪波 张家兴[3] KANG Taishan;YANG Tianhe;LIN Jianzhong;CAI Congbo;ZHANG Jiaxing(Department of MRI, Zhongshan Affiliated Hospital of Xiamen University, Xiamen 361004, China;College of information science and technology, Xiamen University, Xiamen 361005, China;Institute of Brain Diseases and Cognition, Medical College of Xiamen University, Xiamen 361102, China)

机构地区：[1]厦门大学附属中山医院磁共振科,厦门361004 [2]厦门大学信息科学与技术学院,厦门361005 [3]厦门大学医学院脑疾病与认知研究所,厦门361102

出　　处：《磁共振成像》2019年第5期337-341,共5页Chinese Journal of Magnetic Resonance Imaging

基　　金：国家自然科学基金项目(编号:81671674;81871519)~~

摘　　要：目的探索脑型肝豆状核变性患者脑灰质的损害。材料与方法采集30例初发且未经治疗的脑型肝豆状核变性患者脑3D T1WI图像,利用FSL软件,运用基于体素的形态学测量(voxel_based morphometry,VBM)对脑皮层及深部核团的体积进行分析。30名健康志愿者作为对照组,采用单因素方差分析对双侧脑叶灰质及深部核团统计分析。结果脑型肝豆状核变性患者与对照组相比,各脑区皮质萎缩率及两侧脑叶比较如下,小脑:左侧(16.48%)<右侧(16.54%),P>0.05;额叶:左侧(23.6%)>右侧(19.5%),P<0.001;颞叶:左侧(16.9%)<右侧(25.4%),P<0.001;顶叶:左侧(15.5%)<右侧(16.1%),P<0.05;枕叶:左侧(21.0%)<右侧(27.2%),P<0.001;岛叶:左侧(58.7%)>右侧(49.2%),P<0.001。脑干和深部核团(杏仁核除外)体积均显著萎缩,萎缩率依次为伏隔核>壳核>苍白球>丘脑>尾状核>脑干,但左、右两侧各核团萎缩率无显著性差异。结论脑型肝豆状核变性可引起脑皮层广泛而非对称性损害及深部核团对称性损害。Objective: To explore the damages of brain gray matter in patients with cerebral hepatolenticular degeneration. Materials and Methods: Brain 3D T1 weighted images were obtained from 30 patients with untreated cerebral hepatolenticular degeneration. One-way ANOVA was used to analyze group difference. Cortical gray matter and deep nucli volumes were analyzed using FMRIB Software Library (FSL) software and voxel based morphometry (VBM). Thirty healthy volunteers were enrolled as controls. Results: Compared with controls, the percentages of each cortical lobe atrophy and the comparison of bilateral cortical lobes were as follows: cerebellum (left 16.48%< right 16.54%, P>0.05), frontal cortex (left 23.6%> right 19.5%, P<0.001), temporal cortex (left 16.9%< right 25.4%, P<0.001), parietal cortex (left 15.5%< right 16.1%, P<0.001), occipital cortex (left 21.0%< right 27.2%, P<0.001), and insular cortex (left 58.7%> right 49.2%, P<0.001). The volumes of the brainstem and deep gray matter nuclei except the amygdale were significant atrophy. Atrophy percentage arranged in descending order was the accumbens nuclei, putamen nuclei, globus pallidus, thalamus, caudate nuclus, and brain-stem. There were no significant difference in the percentage of atrophy in each nucleus between the hemispheres. Conclusions: Cerebral hepatolenticular degeneration was characterized by bilaterally non-symmetrical cortical atrophy and symmetrical deep gray matter atrophy.

关 键 词：肝豆状核变性 脑 磁共振成像 

分 类 号：R445.2[医药卫生—影像医学与核医学] R742.4[医药卫生—诊断学]