摘要
目的:探讨肝豆状核变性的临床特点与头颅MRI特征,为早期诊断及治疗提供参考。方法:回顾性归纳分析12例肝豆状核变性患者的临床表现及神经影像学特点。结果:平均发病年龄23.5岁;以锥体外系症状为首发10例;性格改变1例;学习成绩下降1例,K-F环均为阳性;头颅MRI特征为对称性基底节区、丘脑、中脑及桥脑异常信号。结论:肝豆状核变性临床表现多样,血清铜蓝蛋白检测、角膜K-F环及头颅MRI检查对诊断本病有重要意义。
Objective:To investigate the clinical data and brain MRI feature of 12 patients with hepatolenticular degeneration(HLD)as to offering evidences of early diagnosis and treatment.Methods:The clinical data and brain MRI feature of 12 patients diagnosed by the criteria of HLD were retrospectively analyzed.Results:The mean age at HLD onset was 23.5 years old.The first symptoms marked by extrapyramidal signs,by character changes and by academic achievement decline respectively are 10 cases,1 case and 1 case.Cerul...
出处
《脑与神经疾病杂志》
2008年第6期697-699,共3页
Journal of Brain and Nervous Diseases