Abstract：
BACKGROUND: Acute hyperammonemic encephalopathy is associated with distinct brain MRI findings, namely, hyperintensity in T2-weighted sequences as well as restricted diffusion in diffusion-weighted imaging with accentuation in the insular cortex and cingulate gyrus. The pathophysiology and the histopathological correlates of these characteristic MRI findings are largely unknown.
METHODS: We present a 57-year-old male with a history of chronic alcohol abuse, liver cirrhosis and portal hypertension, and a clinical syndrome (variceal bleeding, depression of consciousness, seizures), elevated plasma ammonia levels, and characteristic brain MRI abnormalities suggestive of acute hyperammonemic encephalopathy. A postmortem histopathological examination revealed extensive hypoxic ischemic encephalopathy without evidence for metabolic encephalopathy. No episodes of prolonged cerebral hypoxemia were documented throughout the course of the disease. We conducted a review of the literature, which exhibited no reports of hyperammonemic encephalopathy in association with characteristic brain MRI findings and a consecutive histopathological examination.
CONCLUSIONS: This is the first report of a patient with acute hyperammonemic encephalopathy together with characteristic brain MRI findings and a histopathological correlation. Although characteristic MRI findings of acute hyperammonemic encephalopathy were present, a histopathological examination revealed only hypoxic pathology without signs of metabolic encephalopathy.
METHODS: We present a 57-year-old male with a history of chronic alcohol abuse, liver cirrhosis and portal hypertension, and a clinical syndrome (variceal bleeding, depression of consciousness, seizures), elevated plasma ammonia levels, and characteristic brain MRI abnormalities suggestive of acute hyperammonemic encephalopathy. A postmortem histopathological examination revealed extensive hypoxic ischemic encephalopathy without evidence for metabolic encephalopathy. No episodes of prolonged cerebral hypoxemia were documented throughout the course of the disease. We conducted a review of the literature, which exhibited no reports of hyperammonemic encephalopathy in association with characteristic brain MRI findings and a consecutive histopathological examination.
CONCLUSIONS: This is the first report of a patient with acute hyperammonemic encephalopathy together with characteristic brain MRI findings and a histopathological correlation. Although characteristic MRI findings of acute hyperammonemic encephalopathy were present, a histopathological examination revealed only hypoxic pathology without signs of metabolic encephalopathy.
摘要：
背景：急性高血氨脑病与不同的脑MRI表现有关，即,T2加权序列中的高强度以及弥散加权成像中的受限弥散，并在岛叶皮层和扣带回中加重。这些特征性MRI表现的病理生理学和组织病理学相关性在很大程度上是未知的。
方法：我们介绍了一名57岁的男性，有慢性酒精滥用史，肝硬化和门脉高压，和临床综合征（静脉曲张出血，意识抑郁,缉获物)，血浆氨水平升高，和特征性脑MRI异常提示急性高血氨脑病。死后组织病理学检查显示广泛的缺氧缺血性脑病，没有代谢性脑病的证据。在整个疾病过程中，没有记录到长期的脑低氧血症发作。我们对文献进行了回顾，没有与特征性脑MRI发现和连续组织病理学检查相关的高氨血症脑病的报告。
结论：这是首例急性高血氨脑病患者的报告，并伴有特征性脑MRI表现和组织病理学相关性。尽管存在急性高血氨脑病的特征性MRI表现，组织病理学检查仅显示缺氧病理，无代谢性脑病的迹象。
方法：我们介绍了一名57岁的男性，有慢性酒精滥用史，肝硬化和门脉高压，和临床综合征（静脉曲张出血，意识抑郁,缉获物)，血浆氨水平升高，和特征性脑MRI异常提示急性高血氨脑病。死后组织病理学检查显示广泛的缺氧缺血性脑病，没有代谢性脑病的证据。在整个疾病过程中，没有记录到长期的脑低氧血症发作。我们对文献进行了回顾，没有与特征性脑MRI发现和连续组织病理学检查相关的高氨血症脑病的报告。
结论：这是首例急性高血氨脑病患者的报告，并伴有特征性脑MRI表现和组织病理学相关性。尽管存在急性高血氨脑病的特征性MRI表现，组织病理学检查仅显示缺氧病理，无代谢性脑病的迹象。
