A Favorable Treatment Outcome in RANBP2 and Influenza Associated Acute Necrotizing Encephalitis.

Fernando Galan, Douglas R Nordli, Milad Yazdani, Jessica Klein
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引用次数: 1

Abstract

In current literature, there is uncertainty in the pathophysiology and management of influenza-associated Acute Necrotizing Encephalitis. Because of this and the rarity of the disease, no clear treatment guidelines exist. It is thought that treatment after 24 h of symptom onset or known brainstem involvement are poor predictors of outcome. Here, we present a case that provides support for aggressive management of the inflammatory cascade with combination high-dose steroid, immunoglobulin, and anti-viral therapy with oseltamivir despite initiation after 24 h from symptom onset, brainstem involvement, and a pathogenic RANBP2 gene mutation which mechanistically increases oxidative stress, cytokine effects, and possibly viral invasion into brain tissue and vasculature.

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RANBP2和流感相关急性坏死性脑炎的良好治疗结果
在目前的文献中,在流感相关的急性坏死性脑炎的病理生理学和管理方面存在不确定性。由于这种疾病的罕见性,目前还没有明确的治疗指南。人们认为,在症状出现24小时后进行治疗或已知脑干受累是预后的不良预测指标。在这里,我们提出了一个病例,为炎症级联的积极管理提供了支持,联合使用大剂量类固醇、免疫球蛋白和奥司他韦抗病毒治疗,尽管在症状出现、脑干累及和致病性RANBP2基因突变24小时后开始治疗,该基因突变在机制上增加了氧化应激、细胞因子作用,并可能增加病毒侵入脑组织和血管。
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