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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。
In this paper, 121 cases of hepatolenticular degeneration accompanied by mental disorders were analyzed and analyzed. The onset age of 6 to 43 years, an average of 18.4 years old. Mental symptoms as the first symptom of a total of 30 cases, accounting for 24.8%. Psychiatric symptoms are mainly emotional instability, mandatory dumbfounding and mood swings, showing some of the features of organic psychosis that predominates in the basal ganglia and frontal lobes. Corneal K-F ring and blood copper oxidase or plasma ceruloplasmin reduction is the main diagnostic basis, the positive rate of head CT scan is high. However, the specificity of the former two. Treatment emphasizes drive copper therapy and antipsychotics and use, but close observation of liver function.