Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Child Neurology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Pathology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Pediatr Neurol. 2022 Feb;127:1-5. doi: 10.1016/j.pediatrneurol.2021.11.003. Epub 2021 Nov 12.
We report the clinical, radiological, laboratory, and neuropathological findings in support of the first diagnosis of lethal, small-vessel cerebral vasculitis triggered by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in a pediatric patient.
A previously healthy, eight-year-old Hispanic girl presented with subacute left-sided weakness two weeks after a mild febrile illness. SARS-CoV-2 nasopharyngeal swab was positive. Magnetic resonance imaging revealed an enhancing right frontal lobe lesion with significant vasogenic edema. Two brain biopsies of the lesion showed perivascular and intraluminal lymphohistiocytic inflammatory infiltrate consistent with vasculitis. Despite extensive treatment with immunomodulatory therapies targeting primary angiitis of the central nervous system, she experienced neurological decline and died 93 days after presentation. SARS-CoV-2 testing revealed positive serum IgG and positive cerebrospinal fluid IgM. Comprehensive infectious, rheumatologic, hematologic/oncologic, and genetic evaluation did not identify an alternative etiology. Postmortem brain autopsy remained consistent with vasculitis.
This is the first pediatric presentation to suggest that SARS-CoV-2 can lead to a fatal, postinfectious, inflammatory small-vessel cerebral vasculitis. Our patient uniquely included supportive cerebrospinal fluid and postmortem tissue analysis. While most children recover from the neurological complications of SARS-CoV-2, we emphasize the potential mortality in a child with no risk factors for severe disease.
我们报告了临床、影像学、实验室和神经病理学检查结果，以支持首例由严重急性呼吸综合征冠状病毒 2（SARS-CoV-2）引发的致命性小血管脑血管炎的诊断，该患者为儿科患者。
一名既往健康的 8 岁西班牙裔女孩，在轻度发热疾病后两周出现亚急性左侧无力。SARS-CoV-2 鼻咽拭子检测呈阳性。磁共振成像显示右侧额叶有增强的病变，伴有明显的血管源性水肿。病变的两次脑活检显示血管周围和管腔内淋巴组织细胞炎症浸润，符合血管炎的表现。尽管针对中枢神经系统原发性血管炎的免疫调节治疗广泛，但她的神经功能仍出现了恶化，并在发病后 93 天死亡。SARS-CoV-2 检测显示血清 IgG 阳性和脑脊液 IgM 阳性。全面的传染病、风湿病、血液肿瘤学/肿瘤学和遗传学评估未发现替代病因。尸检大脑仍符合血管炎的表现。
这是首例提示 SARS-CoV-2 可导致致命性、感染后、炎症性小血管脑血管炎的儿科病例。我们的患者独特之处在于支持性的脑脊液和尸检组织分析。尽管大多数儿童从 SARS-CoV-2 的神经系统并发症中恢复，但我们强调在无严重疾病风险因素的儿童中，这种疾病可能会导致死亡。
