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威爾遜氏病(Wilson's disease)之高磁場MRI所見－二病例報告
High Field MRI Findings of Wilson's Disease-2 Cases of Report
摘要
本文報告二例Wilson's disease使用1.0-T高磁場MRI之發現。此二例為18歲及22歲女孩，具典型臨床症狀，血清銅及ceruloplasmin降低，尿液銅排泄增加，眼角膜有Kayser-Fleisher rings,其中1例接受CT檢查，顯示典型的CT發現，即基底核密度變低，側腦室前角變寬。二例皆接受MRI檢查，MRI可以看到更多，更明顯的雙側對稱性高訊號強度病灶，位於基底神經核，腦幹，視丘，有1例甚至顳葉海馬回也有，這些高訊號病灶代表病理學上所見的腦組織壞死，神經元及髓鞘脫失。而兩例皆可見豆狀核的蒼白球訊號變得極低（很暗），代表銅的過份沈積，這是CT及低磁場MRI所看不到的。
並列摘要
Wilson's disease (hepatolenticular degeneration) is an uncommon inherited disorder of copper metabolism characterized by abnormal depositon of copper in various tissues, most pronounced in the liver and brain, and causes brain tissue damages in basal ganglia, thalami, brain- stem, dentate nuclei or even in the cortex, subcortex of cerebrum. CT scan of the brain in Wilson's disease demonstrates hypodensity in the above areas and atrophic change in the basal ganglia of brainstem, but not the deposition of copper. After the advent of MRI, the low field MRI may show multiple hyperintensive lesions in the above areas in long TR sequence and has been reported to be more sensitive than CT in detecting lesions. However, the low field MRI can not depict the deposition of copper either. In recent years, the high field MRI shows not only the lesions in basal ganglia, brainstem or brain parenchyma more clearly but also the deposition of copper, because the paramagnatic effect of copper shortens the T2 relaxation time, causing very low signal intensity on T 2 weighted image. Here we report two cases of Wilson's disease diagnosed by 1.0-T MRI. They were female patients, 18 and 22 years old. Both had typical symptoms and signs of Wilson's disease, and were confirmed by biochemical examination with markedly decreased serum copper and ceruloplasmin, and elevation of urine secretion of copper. The long TR images showed hyperintensive lesions in the basal ganglia (mainly in the corpus striatum), thalami and midbrain. One case even had lesions in bilateral hippocampus of temporal lobes. The most unique finding was very low signal intensity in the bilateral globus pallidus which reflected the depostion of copper.
延伸閱讀
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