Kalisky Itai, Maor Yaakov, Goldstein Lilach, Inbar Yael, Ben-Ari Ziv
Department of Gastroenterology and Liver Diseases, Hadassah Medical Organization and Faculty of Medicine, Hebrew University of Jerusalem, Israel.
Department of Gastroenterology and Liver Diseases, Hadassah Medical Organization and Faculty of Medicine, Hebrew University of Jerusalem, Israel, Department of Gastroenterology and Hepatology, Kaplan Medical Center, Rehovot, Israel.
Isr Med Assoc J. 2023 Jan;25(1):8-12.
Acquired hepatocerebral degeneration (AHD) is a neurologic complication of severe chronic liver disease (CLD) with portosystemic shunts. The proposed etiology is manganese accumulation in the brain tissue, especially in the basal ganglia. Combination of clinical manifestation, mostly extrapyramidal movement disorders, and hyperintensities on T1-weighted brain magnetic resonance imaging (MRI) is diagnostic. Although liver transplantation controversial, it is suggested for AHD.
To depict clinical and neuroimaging characteristics and response to treatments in patients diagnosed with AHD at Sheba Medical Center.
Review of patients with AHD diagnosis at the Liver Diseases Center at Sheba Medical Center between 2012 and 2017, data of clinical and neuroimaging, follow-up, and response to treatments, including liver transplantation were recorded.
Five patients with diagnosis of AHD were identified, median age at diagnosis 55 years (range 45-64 years). Four patients had cirrhosis at the time of AHD diagnosis. The main risk factor for AHD was the presence of portosystemic shunts. The most prevalent clinical manifestations were movement disorders, specifically a combination of extrapyramidal and cerebellar signs including instability, rigidity, tremor, bradykinesia, and cognitive impairment. Brain MRI revealed hyperintensities on T1-weighted images in the basal ganglia in all patients. Administration of antiparkinsonian drugs showed clinical improvement, whereas liver transplantation performed in two patients was not associated to neurological improvement.
AHD is related to portosystemic shunts. The combination of Parkinsonism and cerebellar signs and MRI pallidal lesions should alert physicians to the diagnosis. The role of liver transplantation in AHD is still controversial.
获得性肝性脑病（AHD）是严重慢性肝病（CLD）伴门体分流的一种神经并发症。其病因推测为锰在脑组织中蓄积，尤其是在基底节区。临床表现（主要是锥体外系运动障碍）与脑磁共振成像（MRI）T1加权像上的高信号相结合可作出诊断。尽管肝移植存在争议，但仍建议用于AHD的治疗。
描述在舍巴医疗中心被诊断为AHD的患者的临床和神经影像学特征以及对治疗的反应。
回顾2012年至2017年期间在舍巴医疗中心肝病中心诊断为AHD的患者，记录其临床和神经影像学数据、随访情况以及对治疗（包括肝移植）的反应。
确定了5例诊断为AHD的患者，诊断时的中位年龄为55岁（范围45 - 64岁）。4例患者在AHD诊断时已患有肝硬化。AHD的主要危险因素是门体分流的存在。最常见的临床表现是运动障碍，特别是锥体外系和小脑体征的组合，包括不稳定、僵硬、震颤、运动迟缓以及认知障碍。脑MRI显示所有患者基底节区T1加权像上有高信号。给予抗帕金森药物后临床症状有所改善，而2例接受肝移植的患者神经功能并未改善。
AHD与门体分流有关。帕金森综合征和小脑体征以及MRI苍白球病变的组合应提醒医生进行诊断。肝移植在AHD中的作用仍存在争议。
