A Favorable Treatment Outcome in RANBP2 and Influenza Associated Acute Necrotizing Encephalitis. (December 2022)

• Record Type: Journal Article
• Authors: Galan, Fernando; Nordli, Douglas R.; Yazdani, Milad; Klein, Jessica
• Is Part Of: Child neurology open. Volume 9(2022)
• Publication Date: 2022-12
• Subjects: encephalitis -- cognition -- children -- autoimmune; Pediatric neurology -- Periodicals; 618.928005
• DOI: 10.1177/2329048X221143689
• Languages: English
• ISSNs: 2329-048X

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.