Zorgdrager Marcel, Cuperus Frans J C, de Haas Robbert J
Department of Radiology, University Medical Centre Groningen, University of Groningen, 9713 GZ Groningen, The Netherlands.
Department of Gastro-Enterology and Hepatology, University Medical Centre Groningen, University of Groningen, 9713 GZ Groningen, The Netherlands.
Diagnostics (Basel). 2023 Feb 3;13(3):572. doi: 10.3390/diagnostics13030572.
Hyperammonemia can lead to encephalopathy and may be accompanied by a diagnostic dilemma. Imaging as well as biochemical analyses are the cornerstone for identifying possible underlying causes such as severe liver disease or urea cycle defect. We report a case of a patient that presented with neurological deficits based on hyperammonemia in the presence of a large pancreatic neuroendocrine tumor (PNET) and portosystemic shunts in the liver. Prior cases are rather scarce, and the exact mechanism is not fully understood. The case illustrates the added value of a multimodality imaging approach in patients presenting with hyperammonemia-induced encephalopathy.
高氨血症可导致脑病，且可能伴有诊断难题。影像学检查以及生化分析是识别可能的潜在病因（如严重肝病或尿素循环缺陷）的基石。我们报告了一例患者，该患者因高氨血症出现神经功能缺损，同时存在巨大胰腺神经内分泌肿瘤（PNET）和肝脏门体分流。既往病例相当少见，确切机制尚未完全明确。该病例说明了多模态成像方法在高氨血症所致脑病患者中的附加价值。
