１引言肝豆状核变性是一种以铜代谢障碍为主要特征的常染色体隐性遗传的神经系统疾病犤１犦。由于铜在体内逐渐蓄积，临床上主要表现为肝损害和锥体外系病变的慢性铜中毒症状。然而，肝豆状核变性又是少数几种可治疗的神经系统遗传性疾病之一，早期诊断并早期给予驱铜治疗，则 1 Introduction Hepatolenticular degeneration is an autosomal recessive neurological disease characterized by copper metabolism disorders. Due to the gradual accumulation of copper in the body, the main clinical manifestations of liver damage and extrapyramidal lesions of chronic copper poisoning symptoms. However, hepatolenticular degeneration is one of the few treatable neurological hereditary diseases, early diagnosis and early treatment with copper flooding, then