肝豆状核变性合并肝性脊髓病2例报道及文献复习

Hepatic Myelopathy Complicated with HLD ： Literature Review and Report of 2 Cases

目的：通过对肝豆状核变性合并肝性脊髓病l临床表现的分析，探讨其发病机制及诊治要点。方法：回顾性分析我院收治的2例肝豆状核变性合并肝性脊髓病患者的临床资料，并对相关文献进行复习。结果：2例患者均表现为缓慢进行性痉挛性截瘫，无肌肉萎缩、感觉障碍及括约肌功能障碍。化验检查均有铜生化的异常和角膜K—F环。经驱铜保肝营养神经治疗后症状改善。结论：在临床上遇到原因不明的肝脏损害、进行性痉挛性截瘫患者，除考虑到肝性脊髓病的可能外，应进一步检查铜生化和角膜有无K—F环，确定是否由肝豆状核变性引起，及早确诊以利及时治疗。

Objective：To analyze the clinical manifestation of HLD complicated with hepatic myelopathy and to explore its pathogenesis and diagnostic cure points. Methods： To analyze the clinical datas of two patients of Hbi complicated with HLD in our hospital and review the related literatures. Results： These two patients were manifested as a slow and progressive spastic paraplegia, no muscle myatrophy, sense handicap and musculus sphincter functional disorder. They also had abnormal metabolism of copper and corneal K - F ring. Their clinic manifestations got better progress after copper chelating treatment. Conclusions：Clinically, for patients with unexplained liver damage and progressive spastic paraplegia, in addition to considering the possibility of hepatic myelopathy, copper biochemical indices should be measured and cornea should be inspected for K - F ring, to determine whether the symptoms are caused by HLD, thus ensure the early diagnosis and treatment.