肝豆状核变性脑部磁敏感加权成像研究

发布时间：2018-06-08 06:06

本文选题：肝豆状核变性 + 磁共振成像　；参考：《安徽医科大学》2014年硕士论文

【摘要】：背景肝豆状核变性于1912年由Kinnier Wilson首次详细描述而得名，故又称Wilson病(Wilson’s disease，WD)，是一种常染色体隐性遗传性代谢障碍疾病。肝豆状核变性患者体内铜代谢异常已被大量临床试验及病理学研究所证实，铜代谢异常成为目前诊断肝豆状核变性的主要标准之一。同时WD是目前少数可以用药物控制的神经系统遗传性疾病。临床治疗以金属螯合剂驱铜治疗为主，，青霉胺为其特效药物。但是部分患者经青霉胺治疗后，疗效欠佳，甚至出现病情加重的情况。进一步研究发现铜代谢与铁代谢密切相关。临床实验及活体组织学标本证实WD患者体内铁代谢异常。磁敏感加权成像(SWI)是一种利用组织间磁敏感性不同而成像的新技术，能够测量脑组织铁含量的改变。目的探讨SWI在WD患者脑部的应用价值及WD患者相位值改变与血清铜、铜蓝蛋白、铜氧化酶之间的关系。方法对20例确诊WD患者及20名年龄性别相匹配的健康志愿者进行常规MRI平扫及SWI序列扫描，在校正相位图上手动绘制感兴趣区（ROI），分别测量双侧尾状核头、苍白球、壳核、红核、黑质、丘脑的相位值。WD组与正常对照组各核团相位值比较采用两样本t检验行统计学分析。WD组各核团相位值与患者血清铜、铜蓝蛋白、铜氧化酶进行Spearman相关分析。结果WD组双侧尾状核头、苍白球、壳核、红核、黑质、丘脑可见异常低信号。与正常对照组比较，WD组各核团信号减低更显著，相位值明显降低，差异有统计学意义（P0.05），且两组间比较双侧壳核差异最显著。 WD组双侧尾状核头、苍白球、壳核、红核、黑质、丘脑相位值与患者血清铜、铜蓝蛋白、铜氧化酶活力均无显著相关关系。结论WD患者脑内灰质核团有异常顺磁性物质沉积，SWI相位值可作为评估WD患者脑内顺磁性物质的有效手段。
[Abstract]:Background Hepatolenticular degeneration was first described in detail by Kinnier Wilson in 1912. It is also called Wilson disease Wilsonian disease (WDN), which is an autosomal recessive hereditary metabolic disorder. The abnormality of copper metabolism in patients with hepatolenticular degeneration has been confirmed by a large number of clinical trials and pathological studies. The abnormality of copper metabolism has become one of the main criteria for the diagnosis of hepatolenticular degeneration. Meanwhile WD is one of the few genetic diseases of nervous system that can be controlled by drugs. In clinical treatment, metal chelating agent was used to drive copper mainly, penicillamine was its special drug. However, some patients were treated with penicillamine, the curative effect was not good, even the condition became worse. Further studies show that copper metabolism is closely related to iron metabolism. Clinical trials and in vivo histological specimens confirmed abnormal iron metabolism in WD patients. Magnetic sensitivity weighted imaging (SWI) is a new imaging technique which can measure the changes of iron content in brain tissue by using different magnetic sensitivity among tissues. Objective to investigate the application value of SWI in WD patients' brain and the changes of phase value and serum copper in WD patients. Methods the relationship between ceruloplasmin and copper oxidase was studied in 20 patients with WD and 20 healthy volunteers matched by age and sex. The region of interest was manually drawn on the corrected phase map. The bilateral caudate head, globus pallidus, putamen, red nucleus, substantia nigra were measured. Phase value of thalamus. WD group was compared with normal control group. Two samples t test were used to analyze the phase value of thalamus group and serum copper and ceruloplasmin. Results abnormal low signals were observed in both caudate nucleus head, globus pallidus, putamen nucleus, red nucleus, substantia nigra and thalamus in WD group. Compared with the control group, the signal intensity and phase value of all nuclei in WD group were significantly decreased, the difference was statistically significant (P 0.05), and the difference between the two groups was the most significant, and the bilateral caudate nucleus head, globus pallidus, putamen nucleus, red nucleus, substantia nigra were most significant in WD group. Phase value of thalamus and serum copper, ceruloplasmin, Conclusion the phase value of abnormal paramagnetic substance deposition in gray nucleus of WD patients can be used as an effective method to evaluate the paramagnetic substance in WD patients.
【学位授予单位】：安徽医科大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R445.2;R742.4

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