肝豆状核变性是一种常染色体隐性遗传所致的铜代谢障碍性疾病。是目前可用药物治疗的几种先人性代谢缺陷性疾病之一，本病治疗效果与确诊及治疗早晚关系十分密切。但本病早期表现复杂，较易误诊。现将我院近10年误诊12例报告分析如下。1临床资料本组男7例，女5例，年龄6－55岁 Hepatolenticular degeneration is an autosomal recessive genetic disorder of copper metabolism. Is currently available drug treatment of several human metabolic defects of the disease, the treatment of this disease and the diagnosis and treatment sooner or later the relationship is very close. However, the performance of the early complex disease, more easily misdiagnosed. My hospital now misdiagnosed 12 cases in 10 years report as follows. 1 Clinical data The group of 7 males and 5 females, aged 6-55 years