Wang Yan, Younce John R, Perlmutter Joel S, Mar Soe S
Department of Neurology, Washington University School of Medicine, Saint Louis, MO, USA.
Department of Radiology, Washington University School of Medicine, Saint Louis, MO, USA.
Neurohospitalist. 2021 Oct;11(4):351-355. doi: 10.1177/1941874421991370. Epub 2021 Feb 5.
Acute necrotizing encephalopathy (ANE) is a rare para-infectious encephalopathy that classically occurs in children. However, ANE should be considered in the differential diagnosis of adults with symmetric brain lesions after a prodromal illness given recent reports of coronavirus disease of 2019 (COVID-19) to presumably cause ANE in adults. We report a case of a 29-year-old male presenting with fever, malaise, and rapid deterioration into coma. Brain magnetic resonance imaging revealed multifocal symmetric areas of diffusion restriction and surrounding vasogenic edema involving bilateral thalami, pons and cerebellar hemispheres with a core of susceptibility artifact, and minimal thalamic contrast enhancement, most consistent with ANE. Extensive infectious workup revealed isolated and in his urine. Despite the severe encephalopathy on initial presentation, the patient improved with intravenous antibiotics and supportive management with minimal residual deficits at 9 months follow-up. We aim to provide an overview of the radiological features, differential diagnosis, treatment and prognosis of ANE. Becoming familiarized with this rare but devastating disease will improve detection, treatment, and ultimately prognosis, especially in the era of a new pandemic.
急性坏死性脑病(ANE)是一种罕见的感染后脑病,典型地发生于儿童。然而,鉴于最近有关于2019冠状病毒病(COVID-19)可能导致成人ANE的报道,在患有前驱疾病后出现对称性脑病变的成人鉴别诊断中应考虑ANE。我们报告一例29岁男性病例,该患者出现发热、不适,并迅速恶化为昏迷。脑部磁共振成像显示多灶性对称的扩散受限区域以及周围血管源性水肿,累及双侧丘脑、脑桥和小脑半球,伴有磁敏感伪影核心,丘脑对比增强轻微,最符合ANE表现。广泛的感染性检查仅在其尿液中发现[此处原文似乎不完整]。尽管初始表现为严重脑病,但患者经静脉使用抗生素及支持治疗后病情改善,9个月随访时遗留轻微功能缺损。我们旨在概述ANE的放射学特征、鉴别诊断、治疗及预后。熟悉这种罕见但具有毁灭性的疾病将改善其检测、治疗,并最终改善预后,尤其是在新的大流行时代。