Influenza-Associated Acute Necrotizing Encephalopathy in Siblings.

doi:10.1093/jpids/piy033

Paper

Influenza-Associated Acute Necrotizing Encephalopathy in Siblings.

Published Aug 17, 2018 · Ashley Howard, T. Uyeki, J. Fergie

Journal of the Pediatric Infectious Diseases Society

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33

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2

Influential Citations

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Abstract

Encephalopathy is an important complication associated with influenza, most frequently observed in young children, with a wide range of severity. The most severe category of influenza-associated encephalopathy (IAE) is acute necrotizing encephalopathy (ANE), characterized by high frequency of neurologic sequelae and fatal outcomes. We report two young siblings who developed fever and seizures with altered mental status. Influenza A(H1N1)pdm09 virus infection was identified in upper respiratory tract specimens from both patients, and neuroimaging revealed bilateral inflammatory lesions, consistent with acute necrotizing encephalopathy. Neither child had received influenza vaccination. Both children progressed to critical illness and required invasive mechanical ventilation. In addition to critical care management, both patients received high-dose corticosteroids, mannitol, anticonvulsants, and antiviral treatment of influenza. The older child recovered fully and was discharged 2 weeks after illness onset, but the younger sibling developed severe brainstem edema and cerebellar tonsillar herniation, and died on illness day 11. Both children tested positive for Ran Binding Protein 2 (RANBP2) gene mutations. RANBP2 is a genetic polymorphism associated with recurrent episodes of necrotizing encephalitis with respiratory viral infections. Annual influenza vaccination is especially important for ANE survivors, with or without RANBP2 mutations, their household contacts, and caregivers. During influenza season, close monitoring of any child with a history of neurological complications associated with respiratory illness is indicated, with prompt initiation of antiviral treatment with onset of acute respiratory illness, and influenza testing performed by molecular assay.

Case Report

Study Snapshot

Influenza-associated acute necrotizing encephalopathy can be fatal in young children, with RANBP2 gene mutations increasing the risk of recurrence.

PopulationOlder adults (50-71 years)

Sample size24

MethodsObservational

OutcomesBody Mass Index projections

ResultsSocial networks mitigate obesity in older groups.

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Paper

Influenza-Associated Acute Necrotizing Encephalopathy in Siblings.

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References

Familial acute necrotizing encephalopathy without RANBP2 mutation: Poor outcome

Unknown gene mutations may link acute necrotizing encephalopathy to a more severe outcome and male sex predisposition in East Asia.

RANBP2 mutation and acute necrotizing encephalopathy: 2 cases and a literature review of the expanding clinico-radiological phenotype.

ANE1 with RANBP2 mutation is a rare and potentially fatal condition in young children, with a higher incidence in younger age groups and equal occurrence between male and female patients.

Familial acute necrotizing encephalopathy due to mutation in the RANBP2 gene

Acute necrotizing encephalopathy due to RANBP2 mutations can cause severe episodes in children and adults, with a large clinical heterogeneity and potential for prophylaxis and symptomatic management.

Influenza encephalopathy and related neuropsychiatric syndromes

Influenza can cause severe CNS disorders, such as acute encephalopathy, and milder neuropsychiatric disorders, with outcomes varying from death to recovery.

National Surveillance of Influenza-Associated Encephalopathy in Japan over Six Years, before and during the 2009–2010 Influenza Pandemic

The 2009-2010 pandemic pandemic had a higher incidence of influenza-associated encephalopathy (IAE) compared to seasonal IAE, with a U-shaped pattern in the 0-4-year age group.

Citations

Recommendations for Prevention and Control of Influenza in Children, 2024-2025: Technical Report.

The American Academy of Pediatrics recommends annual influenza vaccination for children aged 6 months and older, with antiviral medications for treatment and prevention, for the 2024-2025 season.

Mortality risk factors in children with influenza-associated encephalopathy admitted to the paediatric intensive care unit between 2009 and 2021.

Children with influenza-associated encephalopathy have a high mortality rate, with vomiting, loss of consciousness, lymphopenia, alanine aminotransferase, and serum sodium concentration being related to mortality.

Recommendations for Prevention and Control of Influenza in Children, 2023-2024.

The American Academy of Pediatrics recommends annual influenza vaccination for children aged 6 months and older, with antiviral medications for treatment and prevention, for the 2023-2024 season.

Acute necrotizing encephalopathy in elderly: An unusual cause

Acute necrotizing encephalopathy in elderly patients is an unusual cause, but can be managed effectively in intensive care units.

Risk factors for mortality in fulminant acute necrotizing encephalopathy following influenza A in an adolescent boy

Adolescent boy with fulminant acute necrotizing encephalopathy after influenza A had high risk factors for mortality, emphasizing the importance of recognizing and preventing risk factors in diseases.