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酒精性和非酒精性终末期肝病患者的间脑和小脑病理学

Diencephalic and cerebellar pathology in alcoholic and nonalcoholic patients with end-stage liver disease.

作者信息

Kril J J, Butterworth R F

机构信息

Department of Pathology, The University of Sydney, Royal Prince Alfred Hospital, Australia.

出版信息

Hepatology. 1997 Oct;26(4):837-41. doi: 10.1002/hep.510260405.

Abstract

Formalin-fixed sections from the brains of 36 patients (30 alcoholic and 6 nonalcoholic) with autopsy-proven cirrhosis who died while in a hepatic coma were stained with hematoxylin and eosin, and examined for the presence of diencephalic, cerebellar, pontine, and basal ganglia lesions. Significant neuropathology was identified in 23 of 36 cases consisting of mammillary body and thalamic lesions characteristic of Wernicke encephalopathy (WE) (9 cases, all alcoholic patients) and cerebellar degeneration (20 cases, 17 alcoholic and 3 nonalcoholic patients). Clinical diagnosis of WE had been entertained during life in only 2 of these patients. All cases, alcoholic and nonalcoholic, manifested mild to severe Alzheimer's type II astrocytosis. No cases of central pontine myelinolysis nor acquired (non-Wilsonian) hepatocerebral degeneration were found. These findings show that the brains of a high proportion of cirrhotic patients with end-stage liver disease manifest concomitant unsuspected diencephalic and cerebellar pathology. The high incidence of WE underscores the need for early sustained treatment of alcoholic cirrhotic patients with vitamin B1. Evaluation of the neurological sequelae of liver transplantation, particularly of alcoholic patients with end-stage liver disease, may require a careful neurological and radiological assessment both before and after surgery.

摘要

对36例经尸检证实患有肝硬化且死于肝昏迷的患者(30例酒精性肝硬化患者和6例非酒精性肝硬化患者)的大脑进行福尔马林固定切片,用苏木精和伊红染色,并检查间脑、小脑、脑桥和基底神经节病变情况。36例中有23例发现明显的神经病理学改变,包括韦尼克脑病(WE)特征性的乳头体和丘脑病变(9例,均为酒精性患者)以及小脑变性(20例,17例酒精性患者和3例非酒精性患者)。这些患者中生前仅有2例曾考虑过WE的临床诊断。所有病例,无论酒精性还是非酒精性,均表现出轻度至重度的II型阿尔茨海默样星形细胞增多症。未发现中央桥脑髓鞘溶解症及获得性(非威尔逊病性)肝脑变性病例。这些发现表明,高比例的晚期肝病肝硬化患者大脑存在未被怀疑的间脑和小脑病变。WE的高发病率凸显了对酒精性肝硬化患者早期持续给予维生素B1治疗的必要性。评估肝移植的神经后遗症,尤其是晚期肝病酒精性患者的神经后遗症,可能需要在手术前后进行仔细的神经学和放射学评估。

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