Mierzewska-Schmidt Magdalena, Piwowarczyk Anna, Szymanska Krystyna, Ciaston Michal, Podsiadly Edyta, Przybylski Maciej, Pagowska-Klimek Izabela
Department of Pediatric Anesthesiology and Intensive Therapy, Medical University of Warsaw, 02-091 Warsaw, Poland.
Department of Pediatrics with Clinical Assessment Unit, Medical University of Warsaw, 02-091 Warsaw, Poland.
Biomedicines. 2024 Dec 18;12(12):2877. doi: 10.3390/biomedicines12122877.
Epstein-Barr virus (EBV) usually causes mild, self-limiting, or asymptomatic infection in children, typically infectious mononucleosis. The severe course is more common in immunocompromised patients. Neurological complications of primary infection, reactivation of the latent infection, or immune-mediated are well-documented. However, few published cases of fatal EBV encephalitis exist. Case presentation We report a case of a 5.5-year-old immunocompetent girl with fulminant EBV encephalitis fulfilling the criteria for the recently proposed subtype Acute Fulminant Cerebral Edema: (AFCE). The child presented with fever, vomiting, altered mental status, and ataxia. Her initial brain CT (computed tomography) scan was normal. On day 2 she developed refractory status epilepticus requiring intubation, ventilation, and sedation for airway protection and seizure control. Magnetic resonance imaging (MRI) scan showed cytotoxic brain edema. Despite intensive treatment, including acyclovir, ceftriaxone, hyperosmotic therapy (3% NaCl), intravenous immunoglobulins (IVIG), corticosteroids, as well as supportive management, on day 5 she developed signs of impending herniation. Intensification of therapy (hyperventilation, deepening sedation, mannitol) was ineffective, and a CT scan demonstrated generalized brain edema with tonsillar herniation. EBV primary infection was confirmed by serology and qPCR in blood samples and post-mortem brain tissue. An autopsy was consistent with the early phase of viral encephalitis. Conclusions This case confirms that normal or non-specific CT and MRI scans do not exclude encephalitis diagnosis if clinical presentation fulfills the diagnostic criteria. The implementation of prophylactic anticonvulsants could improve outcomes. Intracranial pressure (ICP) monitoring should be considered in AFCE for better ICP management. Decompressive craniectomy might be a life-saving option in refractory cases. An encephalitis management algorithm is proposed.
爱泼斯坦-巴尔病毒（EBV）通常在儿童中引起轻度、自限性或无症状感染，典型的是传染性单核细胞增多症。严重病程在免疫功能低下的患者中更为常见。原发性感染、潜伏感染的重新激活或免疫介导的神经系统并发症已有充分记录。然而，已发表的致命性EBV脑炎病例很少。病例报告 我们报告一例5.5岁免疫功能正常的女孩，患有暴发性EBV脑炎，符合最近提出的急性暴发性脑水肿（AFCE）亚型的标准。患儿出现发热、呕吐、精神状态改变和共济失调。她最初的脑部计算机断层扫描（CT）正常。第2天，她出现难治性癫痫持续状态，需要插管、通气和镇静以保护气道并控制癫痫发作。磁共振成像（MRI）扫描显示细胞毒性脑水肿。尽管进行了强化治疗，包括阿昔洛韦、头孢曲松、高渗疗法（3%氯化钠）、静脉注射免疫球蛋白（IVIG）、皮质类固醇以及支持性治疗，但在第5天她出现了即将脑疝的迹象。强化治疗（过度通气、加深镇静、甘露醇）无效，CT扫描显示广泛性脑水肿伴小脑扁桃体疝。血液样本和尸检脑组织的血清学和定量聚合酶链反应（qPCR）证实为EBV原发性感染。尸检结果与病毒性脑炎的早期阶段一致。结论 本病例证实，如果临床表现符合诊断标准，正常或非特异性的CT和MRI扫描不能排除脑炎诊断。预防性使用抗惊厥药物可能改善预后。对于AFCE，应考虑进行颅内压（ICP）监测以更好地管理ICP。在难治性病例中，减压性颅骨切除术可能是挽救生命的选择。提出了一种脑炎管理算法。
