Olubiyi Olutayo Ibukunolu, Zamora Carlos, Jewells Valerie, Hunter Senyene E
Division of Neuroradiology, Department of Radiology, University of North Carolina, Chapel Hill, North Carolina, USA.
Division of Pediatric Neurology, Department of Neurology, University of North Carolina, Chapel Hill, North Carolina, USA.
BJR Case Rep. 2022 Nov 1;8(6):20220019. doi: 10.1259/bjrcr.20220019.
A young male child presented with recurrent episodes of seizures and altered mental status following febrile episodes on three separate occasions between his first and third birthdays. Laboratory evaluations identified SARS-CoV-2 infection during the first episode and no infective agents or antibodies in the cerebrospinal fluid during all the episodes. Brain imaging with CT and MRI revealed bilaterally symmetric patchy hemorrhagic necrotic foci in the deep brain nuclei and medial temporal lobes, prompting suspicion for an underlying predisposition to recurrent acute hemorrhagic necrotizing encephalopathy. Gene analysis confirmed a mutation in the RAN-binding protein-2 (RANBP2) gene. The patient made good recovery following treatment with IVIG, steroids and plasmapheresis, and follow-up brain imaging showed no progression of brain lesions. Early suspicion from characteristic imaging features in appropriate clinical settings will inform timely appropriate treatment and better outcome. We therefore provided short review of imaging features of acute hemorrhagic necrotizing encephalopathy.
一名幼龄男童在1岁至3岁期间，有三次在发热后出现反复发作的癫痫和精神状态改变。实验室评估发现在首次发作时感染了新冠病毒，且在所有发作期间脑脊液中均未发现感染因子或抗体。脑部CT和MRI成像显示双侧对称的深部脑核和内侧颞叶有斑片状出血性坏死灶，这引发了对复发性急性出血性坏死性脑病潜在易感性的怀疑。基因分析证实RAN结合蛋白2（RANBP2）基因存在突变。患者在接受静脉注射免疫球蛋白、类固醇和血浆置换治疗后恢复良好，后续脑部成像显示脑损伤无进展。在适当的临床环境中，根据特征性影像学表现尽早怀疑，将有助于及时进行适当治疗并取得更好的预后。因此，我们对急性出血性坏死性脑病的影像学特征进行了简要综述。
