肝豆状核变性腹部B超、腹部CT、脑CT和MRI表现

目的分析肝豆状核变性(WD)病例的腹部B超、肝脾脑CT和脑MRI特点,初步探讨WD影像学检查意义和方法选择.方法回顾69例WD,依据最主要症状分为脑型、肝型和肾型3组,分析各组中腹部B超、肝脾脑CT和脑MRI检查的主要特点.结果 60例患者进行了腹部B超检查,其中59例(98.3%)发现异常声像,以肝实质异常声像、脾大和门脉高压声像为特点,声像结果与临床表现有一定相关性.5例肝脾CT检查以小结节性肝硬化改变和脾大为主要特点.11例患者行脑CT检查,无神经症状者脑CT无异常,有神经症状者50%发现异常病灶,主要特点是基底节区和丘脑对称性低密度灶和脑萎缩.7例患者进行MRI检查,6例发现异常信号,均为对称分布斑片状长T1长T2信号,分布部位主要为基底节、丘脑和脑干,5例发现脑萎缩.结论影像学检查对WD诊断有重要意义,可用于病情判断和鉴别诊断.WD脑CT或MRI检查以基底节区和丘脑改变为特点.

Abdominal ultrasonography, CT, and brain CT, MRI features in patients with Wilson's disease

Objective To analyze the characteristics of abdominal ultrasonography, abdominal and brain computed tomography (CT), brain magnetic resonance imaging (MRI) of Wilson's disease (WD), and to explore the significance of imaging examination to WD patients and selection of favorable methods. Methods Sixty nine WD patients were grouped into brain type, liver type and kidney type according to their major clinical symptoms. The imaging examination results by abdominal ultrasonography, CT, and brain MRI were summarized and the major characteristics were explored. Results Sixty patients received abdominal ultrasonography examination, and 59 (98.3%) of them displayed abnormal image, characterized by various extent of liver parenchyma abnormality, splenomegaly, and portal hypertension. These image features were, to some extent, correlated with clinical signs. Abdominal CT examination of five cases showed small nodular hepatic cirrhosis and splenomegaly as the main distinguishing features. Among 11 patients subjected to brain CT examination, the cases without neurological symptoms had normal CT image, while half of those with neurological signs were found abnormal. The main CT features were symmetric low density areas in basal ganglia and thalamus and brain atrophy. Six of 7 patients exposed to brain MRI examination were found to have abnormal signals, all of which were low signal on T1 weighted images and high signals on T2 weighted images and symmetrically distributed in basal ganglia, thalamus and brain stem. Five of 7 were found to inflict with brain atrophy. Conclusion Imaging examination is of great significance to WD diagnosis. Liver ultrasonography and CT are useful for judgment of the severity of the disease and for differential diagnosis. CT and MRI examination showed that alterations in basal ganglia and thalamus constituted the major features of brain.