获得性肝脑变性临床特点及MRI表现

The clinical characteristics and MRI findings of acquired hepatocerebral degeneration

获得性肝脑变性系一种临床少见的慢性脑病,与肝硬化、门静脉高压有关。筛选326例肝硬化病例中11例明确诊断为获得性肝脑变性,发生率约为3.37%。神经系统症状以静止性震颤(9例)、共济失调(7例)常见;MRI显示双侧苍白球均受累,T1WI为高信号,病变可同时累及双侧丘脑(1例)、中脑被盖(1例)、双侧内囊后肢(1例)和双侧壳核(1例)。多巴丝肼、左旋多巴等抗帕金森病药物治疗无明显效果。

Acquired hepatocerebral degeneration （AHD） is a rare chronic encephalopathy, and its etiology is usually correlated with cirrhosis and portal hypertension. In this study, clinical and radiological data of 326 patients with cirrhosis of the liver were retrospectively analyzed, among whom 11 patients were diagnosed as AHD, with the incidence of 3.37%. The most common neurological symptoms were static tremor （9 cases） and ataxia （7 cases）, Cerebral MRI showed T^WI hyperintensity in bilateral globus pallidus of all 11 patients. The lesions were also involved in bilateral thalamus （one case）, tegment of midbrain （one case）, bilateral posterior crus of internal capsule （one case） and bilateral putamen （one case）. Anti- Parkinson＇s disease drugs, such as levodopa and benserazide, had not presented satisfactory clinical effect.