肝豆状核变性(Wilson病)是一种好发于青少年的铜代谢障碍的常染色体隐性遣传病。本病的主要特征是脑部基底节尤其是豆状核的变性、肝硬变、肾损害以及眼部的病变。如治疗不及时,可致死亡。本病的眼部表现主要有:④角膜Kayser-FIeischer环(简称K-F环);③晶状体葵花状混浊;③斜视、集合力不足、调节幅度减少等眼肌改变;④视网膜内黄色或灰色小点,静脉充盈;⑤暗适应机能下降。国内关于本病的眼科报道不多,且多侧重于对角膜K-F环的描述,未有专门关于眼肌病变的报告。本文对1982年以来确诊为肝豆状 Hepatolenticular degeneration (Wilson’s disease) is an autosomal recessive catastrophic disorder that attacks copper metabolism in adolescents. The main features of this disease are the degeneration of the basal ganglia, especially lentiform nuclei, cirrhosis, renal damage and ocular lesions. If treatment is not timely, can cause death. The ocular manifestations of the disease are: ④ corneal Kayser-FIeischer ring (referred to as KF ring); ③ lens sunflower-like opacity; ③ strabismus, lack of aggregate strength, regulatory changes such as the reduction of ocular muscle; ④ retinal yellow or gray dots , Vein filling; ⑤ dark adaptation function decline. Ophthalmology of the disease is not much domestic coverage, and more focused on the corneal K-F ring description, there is no special report on ocular muscle lesions. This article has been diagnosed since 1982, liver bean