获得性肝性脑部变性的临床及影像学特点

Clinical and imaging features of acquired hepatocerebral degeneration

目的探讨获得性肝性脑部变性（AHCD）的临床及影像学特点。方法回顾性分析10例AHCD患者的临床资料。结果本组患者年龄45-75岁,平均（62.5±10.1）岁;病程2-36个月,平均（8.3±3.2）个月;有慢性肝病病史3-30年。临床表现为精神症状7例,认知功能障碍5例,震颤5例,不自主运动3例,构音障碍4例,共济失调3例。MRI均示有异常改变,表现为双侧苍白球（5例）、豆状核（3例）、壳核（1例）、尾状核（5例）、大脑脚（4例）、红核（2例）、脑桥（1例）部位对称性T1WI高信号,T2WI信号正常（7例）、稍高信号（2例）、高信号（1例）。结论 AHCD的临床特点是慢性肝病患者出现精神行为、智能障碍和帕金森综合征;其影像学特征在双侧基底节及脑干出现MRIT1 WI高信号,MRI检查有助于AHCD的诊断。

Objective To investigate the clinical and imaging features of acquired hepatocerebral degeneration（AHCD）.Methods The clinical data of 10 patients with AHCD were analyzed retrospectively.Results The age of the patients was 45 to 75 years old,the average age was（62.5±10.1）years old.Course of the disease was 2 to 36 months,and the average course was（8.3±3.2） months.Mean duration of the liver disease was 3 to 30 years.Clinical manifestation included psychiatric symptoms（7 cases）,cognitive function impairment（5 cases）,tremor（5 cases）,involuntary movement（3 cases）,dysarthria（4 cases） and ataxia（3 cases）.MRI abnormalities were found in all the patients,including T1WI high signal increased in globus pallidus（5 cases）,lentiform nucleus（3 cases）,putamen（1 case）,caudate nucleus（4 cases）,cerebral peduncles（4 cases）,red nucleus（2 cases） and pontine（1 case）on T2WI normal signal（7 cases）,slighty high signal（2 cases）,high signal（1 case）bilaterally.Conclusions The clinical features of AHCD are mental,cognitive and behavioral disorder and parkinsonism in patients with chronic liver disease.Imaging features are T1WI high signal intensity on MRI in certain region（such as bilateral basal ganglia and brain stem）.MRI examination may be helpful to diagnosis of AHCD.