tetano
Editor, Senior Moderator
Child Neurol Open
. 2022 Dec 13;9:2329048X221143689.
doi: 10.1177/2329048X221143689. eCollection 2022 Jan-Dec.
A Favorable Treatment Outcome in RANBP2 and Influenza Associated Acute Necrotizing Encephalitis
Fernando Galan[SUP] 1 2 [/SUP], Douglas R Nordli[SUP] 2 [/SUP], Milad Yazdani[SUP] 1 [/SUP], Jessica Klein[SUP] 3 [/SUP]
Affiliations
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.
Keywords: autoimmune; children; cognition; encephalitis.
. 2022 Dec 13;9:2329048X221143689.
doi: 10.1177/2329048X221143689. eCollection 2022 Jan-Dec.
A Favorable Treatment Outcome in RANBP2 and Influenza Associated Acute Necrotizing Encephalitis
Fernando Galan[SUP] 1 2 [/SUP], Douglas R Nordli[SUP] 2 [/SUP], Milad Yazdani[SUP] 1 [/SUP], Jessica Klein[SUP] 3 [/SUP]
Affiliations
- PMID: 36530174
- PMCID: PMC9755544
- DOI: 10.1177/2329048X221143689
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.
Keywords: autoimmune; children; cognition; encephalitis.