儿童肝豆状核变性患者脑部磁共振与临床表现的相关性

目的探讨儿童肝豆状核变性患者治疗前后的脑部MRI表现及其与临床表现的相关性。方法分析50例初诊儿童肝豆状核变性患者,其中20例随访复查脑部MRI,15例经青霉胺治疗后回顾性分析,然后把MRI资料与临床表现作相关分析。结果根据首次脑部MRI表现将患者分成3组:第1组(n=23):脑部MRI正常;第2组(n=15):脑部MRI表现双基底节区T1高信号;第3组(n=12):脑部MRI表现基底节区T2高信号。3组患者在中位年龄、神经系统表现和Child-Pugh肝功能分级方面差异有统计学意义(P<0.01)。驱铜治疗后,随访复查脑部MRI发现,临床治疗效果与脑部MRI的影像变化之间密切相关(P<0.01)。结论3组肝豆状核变性患者的脑部MRI表现与临床表现呈明显相关性,治疗后也呈密切相关,故脑部MRI检查对肝豆状核变性的诊断与疗效评价具有重要价值。

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

Objective To analyze the correlation between the findings by MRI in brain of children with Wilson disease and clinical manifestations before and after treatment.Methods The data of 50 patients with Wilson disease at first diagnosis were retrospectively analyzed.Among the 50 patients,20 patients were followed-up and examined by MRI,and 15 patients were retrospectively analyzed after treatment with penicillamine,then the correlation between the findings by MRI and clinical manifestations were analyzed.Results The patients were divided into 3 groups according to initial findings by MRI.Group I(n=23)showed normal findings by MRI;group II(n=15)was characterized by T1-weighted images with increased signal intensity in the globus pallidus followed by putamen,midbrain and caudate nucleus;Group III(n=12)showed T2-weighted images with increased signal intensity in the putamen followed by caudate nucleus,globus pallidus,thalamus,midbrain and pons.There were significant differences in mean age,manifestations of nervous system and Child-Pugh liver function classification among the 3 groups(P<0.01).after clearing copper therapy,the changes showed by MRI at follow-up were closely correlated with clinical efficacy(P<0.01).Conclusion The manifestations in brain showed by MRI in children with Wilson disease is closely related with therapeutic effect,so did that after treatment,as a result,MRI inspection plays an important role in evaluating the therapeutic effect for patients with Wilson disease.