30例儿童肝豆状核变性的MRI检查及随访

30 Cases MR Imaging and Follow up of Hepatolenticular Degeneration in Children

目的 :探讨MRI在儿童肝豆状核变性中的检查价值。材料和方法 :对临床和生化证实30例肝豆状核变性的儿童进行治疗前后的脑部及腹部的MRI检查。结果 :治疗前30例MRI中 ,19例 (63.35 % )病人有脑部异常信号 ,异常信号分布 :豆状核14例 (73.7% ) ,尾状核7例 (36.8% ) ,内囊6例 (31.6 % ) ,丘脑4例 (21.1 % ) ,脑干3例(15.8% )。4例 (21.1 % )有轻度或中度脑萎缩。11例(36.6%)病人肝内大量弥漫性结节状异常信号。23例(76.7%)病人有不同程度的脾脏肿大。治疗后复查 ,19例脑部异常信号中 ,17例病变变小或消失,1例没有明显变化 ,1例加重。11例肝脏病变中 ,10例病变消失或变小 ,1例加重。23例脾脏肿大没有明显变化。结论 :MRI对肝豆状核变性的诊断及预后具有重要意义。

Purpose:To evaluate MRI in diagnosing and follow up hepatolenticular degeneration(HLD)in chilˉdren.Materials and Methods:Pretreatment and follow up brain and abdomen MRI were performed in30children with clinically-and biochemically-proved HLD.Results:Abnormal signals on brain MRI were seen in19cases,which were distributed in lenticular(n=14,73.7%),the caudate head(n=7,36.8%),internal capsule(n=6,31.6%),the thalami(n=4,21.1%),the brain stem(n=3,15.8%).Mild or moderate encephalatrophy was found in4cases(21.1%).Nodular abnormal signs on liver were seen in11cases(36.6%).Splenomegaly was present in23cases(76.7%).On the follow-up MRI,among patients with brain lesions,17cases shrunk or disappeared,1case was unchanged,and1case was worse.Among patients with liver lesions,10cases shrunk or disappeared,1cases was worse.23cases of splenomegaly were unchanged.Conclusion:MRI is of great significance in making diagnosis and prognosis of HLD.