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用脑磁共振(MRI)研究8例肝豆状核变性(WD)病人,其中7名也接受了脑CT扫描检查。本文的目的是证明MRI在WD的所见与临床表现、疾病进展和预后的相互关系。CT能显示WD脑损害,并已用于疗效随访。对照研究发现MRI优于CT。自从1988年以来,作者连续研究8名病人。全部病例行CT或MRI检查前进行体检。6名(11～27岁)有明显异常的神经症状,2名(5岁,9岁)无症状。诊断标准为:血清铜蓝蛋白、24小时排泄尿铜增加、接受肝活检的病人中肝铜含量增加。开始全部病例用D—青霉胺治疗,后2例由于血小板减少症用三乙烯羟化四胺氢氧化物(triethylene tetraminedihydrochloride)替代青霉胺。
Eight patients with hepatolenticular degeneration (WD) were studied with brain magnetic resonance imaging (MRI), and seven of them also underwent brain CT scans. The purpose of this article is to demonstrate the correlation between MRI findings and clinical manifestations, disease progression, and prognosis in WD. CT can show brain damage in WD and has been used for follow-up. The control study found that MRI is superior to CT. Since 1988, the author has studied eight patients in a row. All cases underwent CT or MRI examination before the medical examination. Six (11-27 years old) had significant abnormal neurological symptoms and two (5 and 9 years old) asymptomatic. Diagnostic criteria were: ceruloplasmin, increased urinary copper excretion in 24 hours, and increased liver copper levels in patients undergoing liver biopsy. All cases were initially treated with D-penicillamine, and in the latter two cases penicillamine was replaced with triethylene tetraminedihydrochloride due to thrombocytopenia.