Reis Elisabeth, Coolen Tim, Lolli Valentina
Erasme Hospital, BE.
J Belg Soc Radiol. 2020 Jan 30;104(1):9. doi: 10.5334/jbsr.2017.
Acute hyperammonemic encephalopathy is a rare but life-threatening condition that might complicate liver disease as well as non-hepatic conditions. It can lead to coma and death, secondary to brain edema and intracranial hypertension. We present three cases of acute hyperammonemic encephalopathy of different etiologies and the observed brain MRI findings. Symmetrical extensive cortical signal abnormalities, typically involving the insular and cingulate cortices, often showing restricted diffusion, are commonly described. These specific imaging features should be recognized by the radiologist since prompt treatment of the condition is paramount.
急性高氨血症性脑病是一种罕见但危及生命的疾病,可并发于肝脏疾病以及非肝脏疾病。它可导致昏迷和死亡,继发于脑水肿和颅内高压。我们报告了三例不同病因的急性高氨血症性脑病病例以及观察到的脑部MRI表现。通常描述为对称性广泛的皮质信号异常,典型地累及岛叶和扣带回皮质,常表现为扩散受限。放射科医生应认识到这些特定的影像学特征,因为及时治疗该疾病至关重要。