肝豆状核变性的初始及随访脑磁共振成像结果及其与临床病程的相关性

Initial and follow-up brain MRI findings and correlation with the clinical course in Wilson's disease.

作者信息

Roh J K, Lee T G, Wie B A, Lee S B, Park S H, Chang K H

机构信息

Department of Neurology, Seoul National University, College of Medicine, Republic of Korea.

出版信息

Neurology. 1994 Jun;44(6):1064-8. doi: 10.1212/wnl.44.6.1064.

DOI:10.1212/wnl.44.6.1064

PMID:8208401

Abstract

We performed pretreatment brain MRIs in 25 patients with neurologically symptomatic Wilson's disease (WD) and clinical and MRI follow-up in 16 of them. All 25 pretreatment MRIs revealed abnormalities, with abnormal high-signal intensity (HSI) in bilateral thalami being the most common (92%). HSI lesions in the brainstem (84%) and the basal ganglia (72%) were also common. Brain atrophy was present in 88% of the 25 patients. In the follow-up period of 5 to 24 months, during which the patients were treated with D-penicillamine, both HSI lesions and neurologic symptoms improved in 88% of the 16 patients, but the brain atrophy did not change.

摘要

我们对25例有神经症状的威尔逊病（WD）患者进行了治疗前脑部磁共振成像（MRI）检查，并对其中16例进行了临床及MRI随访。所有25例治疗前MRI均显示异常，双侧丘脑异常高信号强度（HSI）最为常见（92%）。脑干（84%）和基底节（72%）的HSI病变也很常见。25例患者中有88%存在脑萎缩。在5至24个月的随访期内，患者接受青霉胺治疗，16例患者中有88%的HSI病变和神经症状均有改善，但脑萎缩没有变化。