磁共振成像在儿童肝豆状核变性预后判断中的意义

目的探讨磁共振成像（MRI）在儿童肝豆状核变性（hepatolenticular degeneration，HLD）预后判断中的意义。方法对临床确诊为HLD的70例患儿应用MRI进行脑及肝常规序列扫描。其中25例予MRI动态观察，结合临床资料进行综合分析。结果70例患儿中48例显示脑或／和肝异常MRI。25例动态观察患者中12例MRI显示脑和肝异常信号，10例脑受累，3例肝受累。青霉胺剂量不足、初治年龄〉15岁或治疗前病程〉5年者，MRI病灶严重且改善缓慢或无变化。停药2个月以上MRI病灶即可重现，并伴有异常的神经系统症状和体征，但肝型患者可无临床症状和肝功能异常。动态MRI显示，治疗合理组临床及MRI病灶缓解率明显高于治疗不合理组；25例患儿经合理治疗1年以上临床及MRI逐渐好转直至消失，并都能正常学习与工作，13例缓解迁延及11例复发患者均存在治疗不合理因素，且MRI病灶严重，其中12／14例患者不能学习或工作。结论肝豆状核变性经合理治疗后，肝及脑部MRI异常信号可随临床改善而逐渐消失。治疗不合理者MRI病灶严重、改善缓慢、无变化或重现。MRI检查有助于肝豆状核变性预后的判断。

Values of magnetic resonance imaging in evaluating prognosis of childhood hepatolenticular degeneration

Objectives To observe the changes of magnetic resonance imaging (MRI) during the course of treat -ment for and to evaluate the values of MRI in evaluating prognosis of childhood hepatolenticular degeneration. Methods Seventy patients with Wilson' s disease received routine SE sequence MRI of brain and liver, clinical data were also analyzed. Results A total of 48 patients had abnormal MRI findings of the brain or and liver. Twenty-five of these 48 patients who initially had abnormal MRI findings were followed up by 2 to 4 serial MRI examinations at intervals of 1 ~ 1.5 years. Among 25 patients who had been followed up by MRI, 12 had lesions in both brain and liver, 10 in brain and 3 in liver. There were only slight or no improvement in MRI findings of patients who received inadequate dosage of D-penicillamine or patients who started treatment after fifteen years old or who had disease over five years before treatment. Abnormal MRI findings showed again two months later after treatment stopped, meanwhile patients presented obvious neural symptoms and signs. However patients of liver type may have normal liver function without clinical symptoms. Abnormalities of clinical presentations and MRI findings of 25 patients disappeared or decreased after appropriate treatments over one year and all went back to study and work, while slow improvement and recrudescence were seen in patients who had inappropriate treatment. Conclusions Hepatic and brain lesions shown in MRI gradually improved or disappeared after appropriate treatment. Patients received inappropriate treatments had neither clinical improvement nor better changes of their MRI. MRI was helpful in evaluating the prognosis of Wilson' s disease.