获得性肝脑变性二例

Two cases of acquired hepatocerebral degeneration

目的探讨获得性肝脑变性(AHCD)患者的临床及MRI特点。方法报道2例AHCD患者的临床及MRI资料,结合文献进行分析。结果 2例患者均有肝硬化病史,临床上例1表现为反复发生的主观性眩晕、精神行为异常,例2表现为头晕/眩晕,2例患者均未见运动障碍、小脑性共济失调等症状。本组2例患者MRI上均显示双侧基底核区、大脑脚对称性的T_1WI高信号。其中例1双侧内囊、大脑脚T_2Wl/FLAIR、DWI/ADC也呈稍高信号;例2双侧大脑脚DWI呈稍高信号,相应区域ADC相呈等信号,同时可见额叶、颞叶、顶叶、海马萎缩。结论 AHCD是一类与慢性肝病相关的神经系统损害综合征,临床表现复杂多样,其MRI特征表现为双侧基底核区、大脑脚对称性T_1WI高信号,可见部分患者DWI上亦呈高信号。

Objective To explore the clinical and neuroimaging characteristics of acquired hepatocerebral degeneration（AHCD）. MethodThe clinical and MRI datas of 2 patients with AHCD were reported and analyzed in combination with the literature. Results 2 cases had a history of definite cirrhosis. Case 1 represented as recurrent subjective vertigo and mental disturbance, while as case 2 had dizziness and vertigo. Nevertheless 2 patients had no symptoms such as dyskinesia, cerebellar ataxia and so on. Magnetic resonance imaging（MRI） of the brain revealed symmetrical high T1 signals in the bilateral basal ganglia and cerebral peduncle.Case 1 also had slightly higher signal of bilateral internal capsule and cerebral peduncle on T2WI/FLAIR and DWI/ADC. While case 2 showed a slightly higher signal on the bilateral cerebral peduncle on DWI and equal signals on ADC in the corresponding region. Frontal lobe, temporal lobe, parietal lobe, and hippocampus atrophy were also seen at the same time. ConclusionAHCD is a neurological impairment syndrome associated with chronic liver diseases. The clinical manifestations are diverse and complex. The MRI features of AHCD are bilateral symmetrical high T1 signals in the bilateral basal ganglia and cerebral peduncle, which also showed high signal on DWI in some patients.