摘要
本文综合了伴有精神障碍的肝豆状核变性121例并进行分析。起病年龄6～43岁,平均18.4岁。以精神症状为首发症状者共30例,占24.8％。精神症状以情感不稳、强制性哭笑和情绪波动为主,显示以脑基底节和额叶病变为主的器质性精神病的部分特点。角膜K-F环和血铜氧化酶吸光度或血浆铜蓝蛋白减低为主要诊断依据,头颅CT扫描的阳性率虽高。然特异性不如前二者。治疗强调驱铜治疗和抗精神病药物并用,但需严密观察肝功能。
From domestic literatures, one hundred and twenty one patients With Wilson's disease associated with mental disturbances, including the present group of 48 cases, were analysed. Among them, 82 were males and 39 females, with a ratio of 2.1:1. The age of onset of Wilson's disease ranged from 6 to 43 years, with an average of 18.4 years. Mental distubrance as an initial sympotom in the 121 patients was seen in 30 cases (24.8%). Emotional lability, forced laughing and crying and mood fluctuation were the dominant symptoms. These symptoms were characteristic of partial organic brain syndrome of the basal ganglia and frontal lobe. The presence of Kayser-Fleischer ring on the cornea and reduction of bloodcooper oxidase activity and plasma ceruloplamin were the essential diagnostic procedures. CT scan of the brain revealed highly positive rate, but the specificity was not as high as former two procedures. Promoting the excretion of cooper and antipsychotic drugs should be given together in order to control the neuropsychiatric symptoms. Liver function has to be examined and observed carefully.
出处
《北京医学》
CAS
北大核心
1991年第3期139-142,共4页
Beijing Medical Journal
