基于影像学方法的肝豆状核变性疾病阶段划分研究  

Stage division of Wilson's disease based on imaging indexes

作　　者：周香雪[1] 廖健 刘颖洁 肖霞 覃浩玲[2] Zhou Xiangxue;Liao Jian;Liu Yinjie;Xiao Xia;Qin Haolin(National Key Clinical Department and Key Discipline of Neurology,Guangdong Provincial Key Laboratory for Diagnosis and Treatment of Major Neurological Diseases,Department of Neurology,First Affiliated Hospital of Sun Yat-sen University,Guangzhou 510080,China;Imaging Department,First Affiliated Hospital of Sun Yat-Sen University,Guangzhou 510080,China)

机构地区：[1]中山大学附属第一医院神经科,广东省重大神经疾病诊治研究重点实验室,国家临床重点专科和国家重点学科,广州510080 [2]中山大学附属第一医院影像科,广州510080

出　　处：《中华神经医学杂志》2023年第4期368-373,共6页Chinese Journal of Neuromedicine

基　　金：广东省自然科学基金面上项目(2021A515012407)。

摘　　要：目的探讨基于影像学方法对肝豆状核变性(WD)患者进行疾病阶段划分的可行性,并评价不同疾病阶段患者的临床特点差异。方法选择自2015年7月至2022年6月于中山大学附属第一医院神经科住院治疗的60例WD患者(脑型40例,肝型20例)及20例与患者年龄、性别相匹配的健康志愿者(对照组)为研究对象。对所有受试者进行头颅磁敏感成像(SWI)、弥散张量成像(DTI)、磁共振波谱成像(MRS)检查,选取苍白球、尾状核、壳核、丘脑、小脑、中脑、桥脑7个感兴趣区,分别进行各向异性值(FA)、相位值(CP)、N-乙酰基门冬氨酸/肌酸(NAA/Cr)值测定,并根据DTI、SWI、MRS结果将患者分为金属沉积期(CP值下降,FA值无明显异常,NAA/Cr值无明显下降)、纤维损伤期(FA值明显异常,NAA/Cr值无明显下降)、神经元坏死期(NAA/Cr值明显下降),进一步对不同疾病阶段患者及对照组的临床资料(改良Young量表评分、肝功能Child-Pugh分级、血清铜含量、尿铜含量)及影像学指标(FA值、CP值、NAA/Cr值)进行比较。结果60例WD患者中,处于金属沉积期患者共19例(包括18例肝型和1例脑型),纤维损伤期患者共28例(包括2例肝型和26例脑型),神经元坏死期患者共13例(均为脑型)。(1)与金属沉积期组和纤维损伤期组比较,神经元坏死期组患者尿铜含量均明显降低,差异均有统计学意义(P<0.05)。金属沉积期组、纤维损伤期组和神经元坏死期组患者的改良Young量表评分依次升高;金属沉积期组患者的Child-Pugh分级高于纤维损伤期组、神经元坏死期组。(2)与金属沉积期组和神经元坏死期组比较,纤维损伤期组患者苍白球、黑质的CP值明显降低;与金属沉积期组和纤维损伤期组患者比较,神经元坏死期组患者壳核的FA值明显降低,苍白球、丘脑和尾状核的NAA/Cr值明显降低,差异均有统计学意义(P<0.05)。结论基于影像学方法对WD患者进行疾病阶段划分�Objective To explore the feasibility of staging Wilson's disease(WD)based on imaging indexes,and evaluate the clinical characteristic differences of WD patients at different stages.Methods Sixty WD patients(40 with cerebral type and 20 with hepatic type)hospitalized in Department of Neurology,First Affiliated Hospital of Sun Yat-sen University from July 2015 to June 2022 and 20 age-and gender-matched normal controls were selected.All subjects accepted susceptibility-weighted imaging(SWI),diffusion tensor imaging(DTI),magnetic resonance spectroscopy(MRS);7 regions of interest were selected:globus pallidus,caudate nucleus,putamen,thalamus,cerebellum,midbrain and pons;their fractional anisotropy(FA),corrected phase(CP),N-acetylaspartate/creatine(NAA/Cr)values were measured.According to DTI,SWI and MRS results,WD patients were divided into group of metal deposition stage(decreased CP,normal FA and NAA/Cr),group of fiber damage stage(abnormal FA,normal NAA/Cr),and group of neuron necrosis stage(decreased NAA/Cr);the clinical data(modified Young scale scores,Child-Pugh grading of liver function,serum copper content,and urinary copper content)and imaging indexes(FA,CP,and NAA/Cr)among the 3 groups and control group were compared.Results Among the 60 patients,19 patients were at metal deposition stage(including 18 with liver type and 1 with brain type),28 patients at fiber injury stage(including 2 with liver type and 26 with brain type),and 13 patients at neuron necrosis stage(all brain type).(1)Compared with group of metal deposition stage and fiber damage stage,group of neuron necrosis stage had significantly decreased urinary copper content(P<0.05).The modified Young scale scores in groups of metal deposition stage,fiber injury stage and neuronal necrosis stage increased successively;Child-Pugh grading in group of metal deposition stage was higher than that in groups of fiber injury stage and neuronal necrosis stage.(2)Compared with groups of metal deposition stage and neuron necrosis stage,CP values in the globus pal

关 键 词：肝豆状核变性 疾病阶段 磁敏感成像 弥散张量成像 磁共振波谱成像 

分 类 号：R742.4[医药卫生—神经病学与精神病学]