肝豆状核变性是一种常染色体隐性遗传、铜代谢障碍性疾病。该病临床表现复杂,易造成误诊。本院自1973年以来,经住院确诊为肝豆状核变性而在院外误诊者14例,其中误诊为精神分裂症、肝硬化及慢性肝炎各两例,脑干病变3例,神经官能症4例,闭塞性脑血管病、扭转痉挛及震颤麻痹各1例。兹结合部分病例,对误诊原因加以 Hepatolenticular degeneration is an autosomal recessive, copper metabolic disorder. The clinical manifestations of the disease is complex, easily lead to misdiagnosis. Since 1973, our hospital has been diagnosed with hepatolenticular degeneration by inpatients and misdiagnosed outside the hospital in 14 cases, misdiagnosed as schizophrenia, cirrhosis and chronic hepatitis in two cases, three cases of brain stem lesions, neurosis 4 Cases, occlusive cerebrovascular disease, torsion spasm and paralysis of paralysis in 1 case. In combination with some cases, the reasons for misdiagnosis