Clinical features, prognostic factors of pediatric acute necrotizing encephalopathy: a 67-case retrospective analysis

Acute necrotizing encephalopathy is a rapidly and severe encephalopathic condition with no specific treatment, and a generally poor prognosis. This study aimed to investigate the clinical characteristics of pediatric Acute necrotizing encephalopathy, identify key prognostic factors influencing outcomes, and provide evidence to optimize clinical management.

We retrospectively reviewed the medical records of 67 pediatric ANE patients admitted to Wuhan Children's Hospital (2014-2022). Baseline demographics, clinical manifestations, laboratory and neuroimaging findings, treatments, and follow-up data were collected. Disease onset was defined as the starting point, and death or 12 months after discharge was set as the study endpoint. Prognostic factors associated with mortality and long-term outcomes were analyzed.

Most patients were aged under 4 years, with prodromal infections were mainly respiratory, with 32.8% associated with influenza virus infection. Eighteen patients died within 3 months, while all 49 survivors exhibited varying degrees of neurological sequelae during 12-month follow-up. Brain magnetic resonance imaging (MRI) commonly shows thalamic lesions, and as the disease progresses, hemorrhage, cystic degeneration, and atrophy may occur. Sixty-three patients received corticosteroids, of whom 21 were treated within 24 h of onset. Univariate logistic regression identified influenza virus infection, prodromal-to-encephalopathy interval ≤24 h, tracheal intubation, Glasgow Coma Scale (GCS) <5, prolonged APTT, and elevated PCT and IL-10 as risk factors for mortality. Multivariate logistic regression demonstrated that hemorrhage and atrophy on follow-up MRI were independent predictors of poor long-term outcome, whereas corticosteroid administration within 24 h of onset was an independent protective factor.

Clinicians should identify influenza-related prodromal infections early (≤24 h), dynamically monitor neuroimaging changes to detect structural brain alterations affecting long-term prognosis, and intervene promptly with glucocorticoid therapy within the 24-h.