Department of Neurology, Massachusetts General Hospital, 55 Fruit Street, WACC 8-835, Boston, MA, USA.
Department of Neurology, University of Iowa Hospitals and Clinics, Iowa City, IA, USA.
J Neurovirol. 2022 Feb;28(1):172-176. doi: 10.1007/s13365-021-01042-3. Epub 2021 Dec 30.
Acute necrotizing encephalopathy (ANE) is a rare complication of coronavirus disease 2019 (COVID-19) secondary to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. The condition is typically diagnosed based on characteristic neuroimaging findings in the context of active viral respiratory symptoms. We present a rare case of COVID-19-associated ANE presenting with expressive aphasia and encephalopathy in the absence of active respiratory symptoms. Initial evaluation revealed bilateral thalamic lesions and a mild neutrophilic-predominant pleocytosis on cerebrospinal fluid analysis, the latter of which has not been described in previously published cases. Presence of these atypical features prompted extensive diagnostic evaluation. Metagenomic next-generation sequencing on cerebrospinal fluid did not detect the presence of pathogenic nucleic acids. Thalamic biopsy revealed perivascular neutrophilic inflammation suggestive of small vessel vasculitis with surrounding hemorrhage and necrosis. Ultimately, the diagnosis was made following detection of SARS-CoV-2 serologies and after exclusion of alternative etiologies. The patient was successfully treated with a short course of high-dose methylprednisolone with favorable outcome.
急性坏死性脑病（ANE）是一种罕见的 2019 年冠状病毒病（COVID-19）并发症，继发于严重急性呼吸综合征冠状病毒 2（SARS-CoV-2）感染。该疾病通常基于活跃的病毒性呼吸道症状背景下的特征性神经影像学表现进行诊断。我们报告了一例罕见的 COVID-19 相关 ANE 病例，表现为表达性失语和脑病，而无活跃的呼吸道症状。初步评估显示双侧丘脑病变和脑脊液分析中轻度嗜中性粒细胞为主的白细胞增多症，后者在以前发表的病例中未描述。这些非典型特征的存在促使进行了广泛的诊断评估。脑脊液的宏基因组下一代测序未检测到致病核酸的存在。丘脑活检显示血管周围嗜中性粒细胞炎症，提示伴有周围出血和坏死的小血管血管炎。最终，在检测到 SARS-CoV-2 血清学并排除其他病因后作出诊断。该患者成功接受了短疗程大剂量甲基强的松龙治疗，结果良好。
