获得性肝脑变性25例临床分析

Acquired Hepatocerebral Degeneration: A Series of 25 Cases

目的提高对获得性肝脑变性认识。方法收集2016年1月至2019年2月中国医科大学附属盛京医院25例获得性肝脑变性患者的临床资料及影像学特点,结合文献进行总结分析。结果25例获得性肝脑变性患者均患有慢性肝病,肝脏影像学均显示肝硬化伴侧支循环形成,头颅MRI均显示苍白球T1WI高信号,部分伴有邻近部位受累,中脑9例,内囊3例,丘脑3例。25例患者均有不同程度的神经系统症状,以帕金森病样症状和小脑损害症状组合为主。同时合并有急性肝性脑病发作7例,经治疗急性肝性脑病后症状可缓解,但其帕金森病样症状及小脑损害症状未见明显缓解。结论获得性肝脑变性是与慢性肝病相关的神经系统慢性疾病,目前认为主要与微量元素锰离子沉积导致的基底节区病变相关。抗帕金森病药物治疗对症状无明显改善,早期肝移植可能是有效的治疗手段。

Aim To analyze the clinical data and imaging features of patients with acquired hepatocerebral degeneration ( AHD) and improve the understanding of the disease.Methods A total of 25 patients with acquired hepatocerebral degeneration were selected for study.Clinical and laboratory data were analyzed.Results 25 cases had cirrhosis and collateral circulation.MRI showed hyperintensity in the bilateral globus pallidus on T1-weighted magnetic resonance images ( T1WI),some of them were involved in adjacent sites,25 patients had varying degrees of neurological symptoms,with a combination of Parkinson-like symptoms and cerebellar damage symptoms.Seven patients had an episode of acute hepatic encephalopathy,the symptoms were relieved after the treatment,but the Parkinson-like symptoms and cerebellar damage symptoms were not significantly relieved.Conclusion Acquired hepatocerebral degeneration is a chronic neurological syndrome in patients with end stage liver disease.The accumulation of manganese may lead to neurodegeneration in basal ganglia.Antiparkinson drugs did not improve symptoms.Liver transplantation in early stage may improve outcomes.