摘要
目的研究量化评价肝豆状核变性(WD)核磁共振(MRI)严重程度的方法,分析MRI异常信号和临床特点的关系。方法选取WD初诊患者60例(脑型40例,肝型20例)、正常对照20例。对所有患者进行神经系统评分,肝功能检查,铜代谢检查。所有入选者进行头部MRI检查。分别对T2呈像上各部位的高信号和低信号范围和程度进行评分。结果 100%的脑型和80%的肝型WD患者脑部MRI出现异常信号。脑型WD尾状核、丘脑、小脑、中脑、脑桥高信号范围评分高于肝型患者(P=0.024、0.033、0.210、0.036、0.019),程度评分高于肝型(P=0.038、0.044、0.037、0.043、0.020);脑型壳核高信号程度高于肝型(P=0.021);脑型WD丘脑低信号范围及程度高于肝型(P=0.001、0.035);脑型壳核低信号程度高于肝型(P=0.017)。言语清晰度得分与壳核高信号范围、壳核高信号程度得分呈正相关(r=0.379、0.387,P=0.047、0.042)。共济失调的程度得分和小脑高信号程度得分呈正相关(r=0.313,P=0.042)。震颤程度得分和丘脑高信号范围、高信号程度得分呈正比(r=0.372、0.337,P=0.034、0.039)。影像学评分与血清铜、尿铜无相关性。肝型WD患者头部影像学评分与肝功能等级无相关性。结论 WD头部影像学异常的比例较高。通过对T2高、低异常信号的范围和程度评分的方法,可以分别量化评价WD患者脑部铜沉积、神经损伤的程度。WD患者的神经症状与特定核团的神经损伤有关。
Objective To quantitatively evaluate the severity of magnetic resonance image(MRI) in Wilson disease(WD)patients and analyze the relationship between MRI abnormal signals and clinical features.Methods 60 cases of newly diagnosed WD patients(40 cases of cerebral type,20 cases of hepatic type)and 20 cases of normal control were selected.Neurological scores,liver function tests and copper metabolism tests were performed on all subjects.All the subjects underwent head MRI.The range and degree of high signal and low signal of each part on the T2 image were scored respectively.The results were analyzed statistically.Results The range scores of high signal in caudate nucleus,thalami,cerebellum,midbrain,pons of cerebral type WD patients were higher than that of hepatic type(P = 0.024,0.033,0.210,0.036,0.019),the level scores of high signal in these areas were higher than that of hepatic type(P = 0.038,0.044,0.037,0.043,0.020).The level scores of high signal in putamen of cerebral type were higher than that of hepatic type(P = 0.021).The scores of range and degree of low signal in thalami of cerebral type were higher than that of hepatic type(P = 0.001,0.035).The scores of low signal degree of cerebral type in the shell nucleus were higher than that of hepatic type(P = 0.017).Speech articulation scores were positively correlated with high signal range and high signal degree scores(r = 0.379,0.387,P = 0.047,0.042).The degree scores of ataxia were positively correlated with the degree scores of high signal in cerebellar(r = 0.313,P = 0.042).The tremor degree scores were positively correlated to the thalamic high signal range and high signal degree score(r = 0.372,0.337,P = 0.034,0.039).There was no correlation between the imaging score and serum and urine copper.There was no correlation between imaging score and liver function grade in hepatic type WD patients.Conclusions The proportion of WD brain imaging abnormality was higher.By scoring the range and degree of T2 high and low abnormal signals,the degree of brain copper d
作者
李爱萍
周香雪
蒲小勇
何荣兴
李洵桦
梁秀龄
LI Aiping;ZHOU Xiangxue;PU Xiaoyong;HE Rongxing;LI Xunhua;LIANG Xiuling(Department of Neurology,Dongguan People′s Hospital,Dongguan 523000,China)
出处
《实用医学杂志》
CAS
北大核心
2019年第13期2142-2147,共6页
The Journal of Practical Medicine
基金
国家自然科学基金青年项目(编号:81500968)
关键词
肝豆状核变性
磁共振
量化
神经症状
铜代谢
核团
Wilson disease
magnetic resonance imaging
quantization
neural symptoms
cop per metabolism
nucleus
