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肝移植受者获得性肝脑变性

Acquired hepatocerebral degeneration in a liver transplant recipient.

作者信息

Soffer D, Sherman Y, Tur-Kaspa R, Eid A

机构信息

Department of Pathology Neuropathology, Hadassah Medical Center, Jerusalem, Israel.

出版信息

Acta Neuropathol. 1995;90(1):107-11. doi: 10.1007/BF00294467.

Abstract

A 47-year-old-man lapsed into coma 12 h after liver transplantation, and remained comatose until death 38 days later. Prior to transplantation he had repeated episodes of hepatic encephalopathy, but no fixed neurological signs. Autopsy revealed typical features of acquired hepatocerebral degeneration with diffuse but patchy pseudolaminar cortical necrosis, variable amount of neuronal loss in the cerebral cortex, basal ganglia and other areas, and proliferation of Alzheimer type II glia. In addition, there was central pontine and extensive extrapontine myelinolysis involving the lateral and medical geniculate bodies, the thalamus, internal capsule, fornix, mamillothalamic tract, white matter bundles in the caudate and pallidum, the oculomotor nuclei and the foliar white matter of the cerebellum. The distinction between myelinolytic lesions and lesions due to hepatocerebral degeneration was not always clear. Although neurological complications and brain lesions are rather common after liver transplantation, there have been no reports of acquired hepatocerebral degeneration in liver transplant recipients. Our data lend support to the idea that a single prolonged comatose episode, due to hepatic dysfunction, may induce permanent parenchymal brain damage.

摘要

一名47岁男性在肝移植术后12小时陷入昏迷,直至38天后死亡一直昏迷不醒。移植前他曾反复出现肝性脑病,但无固定神经体征。尸检显示为获得性肝脑变性的典型特征,有弥漫性但呈斑片状的假层状皮质坏死、大脑皮质、基底神经节及其他区域不同程度的神经元丢失,以及Ⅱ型阿尔茨海默胶质细胞增生。此外,存在中央桥脑及广泛的脑桥外髓鞘溶解,累及外侧及内侧膝状体、丘脑、内囊、穹窿、乳头丘脑束、尾状核和苍白球的白质束、动眼神经核及小脑叶白质。髓鞘溶解病变与肝脑变性所致病变之间的区别并不总是很清楚。虽然肝移植术后神经并发症和脑病变相当常见,但此前尚无肝移植受者发生获得性肝脑变性的报道。我们的数据支持这样一种观点,即由于肝功能障碍导致的单次长时间昏迷发作可能会引起永久性脑实质损伤。

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