肝豆状核变性患者脑MRI特征与临床表现的相关性研究  

Correlation between the findings by MRI in brain of Wilson disease and clinical manifestations

作　　者：黄丽[1] 李洵桦[2] 粱秀龄 

机构地区：[1]广州开发区医院ICU,广东广州510730 [2]中山大学附属第一医院神经科,广东广州510080

出　　处：《中风与神经疾病杂志》2011年第3期216-219,共4页Journal of Apoplexy and Nervous Diseases

基　　金：国家自然科学基金资助项目(编号:30671154)

摘　　要：目的探讨肝豆状核变性(wilson disease WD)患者头部MRI特征与临床表现的相关性。方法对79例确诊WD患者的头部MRI特征与临床症状及其它主要辅助检查资料进行分析。结果根据首次头部MRI检查结果,将患者分为A组(14例):头部MRI检查无异常发现;B组(65例):头部MRI检查有异常发现。病变集中于豆状核、脑干、尾状核和丘脑,多呈长T1W长T2W信号,但8例患者呈现短T2W其中2例为长T2W短T2W混杂信号。A、B两组年龄有显著性差异,A组患者神经系统检查全部正常,B组中有9例患者神经系统检查正常。WD患者脑部病变部位与病程之间无统计学意义(P>0.05)。构音障碍与尾状核关系较为密切,肌张力障碍与中脑的关系较为密切,震颤与丘脑的关系较为密切。结论 MRI检查是诊断WD的有效方法,头部MRI异常信号可先于临床表现而出现,病变较集中于豆状核、脑干、尾状核和丘脑,短T2W信号为本病具特征性的病理改变。WD患者脑部病变部位与病程之间无很好相关性,构音障碍与尾状核关系较为密切,肌张力障碍与中脑的关系较为密切,震颤与丘脑的关系较为密切。Objective To analyze the correlation between the findings by MRI in brain of Wilson disease and clinical manifestations. Methods The data of 79 patients with Wilson disease as the first diagnosis were retrospectively analyzed. The correlation between the findings by MRI and clinical manifestations were analyzed. Results The patients were divided into 2 groups according to initial findings by MRI. Group A（n = 14）showed normal findings by MRI,and group B （n = 65 ）showed abnormal findings. The abnormal findings focused on lentiform nucleus, brainstem, caudate nucleus and thalamus nuclei. Most images showed long T1W images and long T2W images,and 8 patients showed short T2W images. There were significant differences in mean age between group A and group B. There were nonsignificant differences between the course and abnormal part in the brain （ P 〉 0.05 ）. Conclusion MRI inspection plays an important role in diagnosis for patients with Wilson disease. The abnormal findings focus on lentiform nucleus, brainstem, caudate nucleus and thalamus nuclei. Short T2W images was characteristic finding of Wilson disease. Dysarthria was related with lentiform nucleus ,dystonia was related with midbrain, and tremor was related with thalamus nuclei.

关 键 词：肝豆状核变性 颅脑 磁共振成像 临床表现 

分 类 号：R742.4[医药卫生—神经病学与精神病学]