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Outcomes in influenza and RANBP2 mutation-associated acute necrotizing encephalopathy of childhood
Nurin Chatur1,2, Carmen Yea1, Birgit Ertl-Wagner3
1Department of Pediatrics, Division of Neurology, Hospital for Sick Children, Division of Neuroscience and Mental Health, SickKids Research Institute, Toronto, Ontario, Canada.
Insights
Influenza infection in acute necrotizing encephalopathy of childhood (ANEC) increases hemorrhage risk. RANBP2 mutations predict relapse but not overall outcomes in pediatric patients.
Area of Science:
- Pediatric Neurology
- Neuroinflammation
- Infectious Diseases
Background:
- Acute necrotizing encephalopathy of childhood (ANEC) is a rare neurological disorder characterized by acute brain lesions.
- Identifying factors influencing ANEC prognosis is crucial for patient management.
Purpose of the Study:
- To investigate clinical and imaging predictors of RANBP2 mutations, influenza association, and long-term outcomes in ANEC.
- To understand the role of influenza and RANBP2 gene status in ANEC severity and recurrence.
Main Methods:
- Retrospective chart review of ANEC patients (2012-2020) at a tertiary pediatric center.
- Inclusion criteria: acute inflammatory lesions in basal ganglia and pons.
- Data collected: presenting features, imaging, RANBP2 gene testing, influenza status, therapies, and outcomes.
Main Results:
- Twenty ANEC patients (mean age 3y 6mo) were analyzed; 14 female, 6 male.
- Ten patients tested positive for influenza; seven had RANBP2 mutations.
- Influenza-positive patients showed higher hemorrhage likelihood (p=0.048), thalamic hemorrhage (p=0.035), and diffusion restriction (p=0.035).
Conclusions:
- Influenza-associated ANEC correlates with increased hemorrhage and thalamic swelling.
- RANBP2 mutation status predicts disease relapse but not overall patient outcomes.
Aim:
To evaluate clinical and imaging features in patients with acute necrotizing encephalopathy of childhood (ANEC) to identify predictors of RANBP2 mutations, influenza association, and long-term outcomes.
Method:
A retrospective chart review in patients with ANEC (2012-2020) seen at a tertiary pediatric center was performed. Children were included if they had acute inflammatory lesions in the basal ganglia and pons. Variables included presenting features, imaging characteristics, RANBP2 gene testing, nasopharyngeal swab findings, therapies, and long-term outcomes.
Results:
Twenty patients were included (average age at presentation 3y 6mo, interquartile range 3y 7mo, SD 2y 8mo; 14 females, six males). Three of the 20 experienced recurrences; one of the 20 died. Ten patients were influenza positive. Seven patients were RANBP2 mutation positive. A higher likelihood of hemorrhage was observed in patients who were influenza positive compared to influenza negative (p=0.048). Patients with influenza had a higher degree of thalamic hemorrhage (2, p=0.035) and greater extent of diffusion restriction (3, p=0.035) in semiquantitive analysis.
Interpretation:
Children with ANEC who are positive for influenza are more likely to have hemorrhage and greater thalamic swelling. RANBP2 status was predictive of relapse but not predictive of overall outcome.
