磁共振成像在儿童肝豆状核变性临床各期中的变化

Magnetic resonance imaging features in different clinical stages of childhood Wilson′s disease

目的 了解儿童肝豆状核变性 (hepatolenticulardegeneration ,HLD)临床各期中的磁共振成像 (MRI)表现 ,以评价MRI在肝豆状核变性疗效及预后判断中的意义。方法 对临床确诊为HLD的 3 0例未治和初治患者及 2 7例随访患者应用荷兰Philips 0 5T超导MRI系统进行脑及肝常规序列扫描 ,并结合临床资料综合分析。结果 5 7例患者中 3 7例 ( 64 9%)MRI显示脑和 (或 )肝异常信号 ,18例 ( 3 1 6%)脑和肝同时受累。脑部异常者 ,基底节是最常见的受累部位 ,其次是丘脑 ,多表现为双侧对称性T2 W高信号或 (和 )T1W低信号。肝脏异常者 ,表现为多发性弥漫性圆型T1W高信号 ,T2 W低信号。未治及初治患者首次MRI异常率 (脑、肝 )明显高于随访患者 ( χ2 =12 5 ,P <0 0 1) ,疗程与MRI异常率成反比。 2 8例正规治疗患者 ( 19例的随访患者及 9例初治患者 )的随访MRI异常率与治疗前病程 (均在 5年以内 )无关 ,( χ2 =2 44,P >0 0 5 )。未治、短程或不规则治疗与MRI异常明显相关。多数病灶经合理治疗后可随临床好转而逐渐消失或减少。治疗不当且总病程超过 10年者 ,MRI呈脑软化、肝内巨大结节等严重异常信号 ,并伴有明显的临床症状和体征 ,复治后临床及MRI改善缓慢。结论 肝豆状核变性经合理治疗 ,肝及脑部MRI异常信号可?

Objectives To explore the findings of magnetic resonance imaging (MRI) in different stages of Wilson′s disease and to evaluate the value of MRI in the evaluation of therapeutic effect and prognosis. Methods Fifty-seven patients (30 untreated or newly-treated patients and 27 follow-up patients) with Wilson′s disease underwent routine SE sequence MRI of brain and liver. Of them 10 untreated or newly-treated patients and 3 follow-up patients had 2-3 times of MRI examinations with an interval of 1～1.5 years. Clinical data with MRI were analysed. Results Thirty-seven patients had abnormal MRI findings of the brain or/and liver (64.9%). Eighteen patients had abnormal MRI findings of both brain and liver (31.6%). In 29 patients with abnormal MRI of the brain (50.9%), 21 patients (36.8%) had lesion in putamen, 12 (21.1%) in caudatum, 7(12.3%) in thalamus, 2(3.5%) in internal capsule, 1(1.8%) in cerebral gray matter, 1(1.8%) in brainstem and 1(1.8%) in cerebellum. Three patients showed brain atrophy (5.3%). Most of patients showed a pattern of bilateral and symmetric T 2W high signal intensity or/ and T 1W low intensity. Only 2 patients showed T 1W high signal intensity. Twenty-six patients had abnormal MRI of the liver (45.6%), showing multiple rounded T 1 high signal intensity and T 2 low signal intensity. The abnormal rate of the first MRI in untreated or newly-treated patients was markedly higher than that of follow-up patients (χ 2=12.50, P<0.01). Less MRI abnormalities were found with the prolongation of treatment. In 28 patients receiving adequate treatments there was no correlation between the abnormal rate of follow-up MRI after the treatment and the courses of illness before treatment (χ 2=2.44,P>0.05). The major lesion might disappear or decrease after rational treatments. Abnormal MRI was found to be closely associated with short-term treatment or inadequate treatment. The MRI showed severe lesions such as encephalomalacia or large nodus of liver in patients receiving inadequate dosage of penicillamine at the beginning of treatment or discontinuing their treatment and having duration of the disease over ten years. In such cases the clinical and MRI improvement was slow during their treatment. Conclusions Hepatic and brain lesion may disappear after rational treatment with D-penicillamine; MRI was helpful in understanding therapeutic effect and prognosis of the Wilson′s disease.