Treatment Timing, EEG, Neuroimaging, and Outcomes After Acute Necrotizing Encephalopathy in Children

Abstract

Background: Acute necrotizing encephalopathy (ANE) is a rare condition associated with rapid progression to coma and high incidence of morbidity and mortality. Methods: Clinical, electroencephalographic (EEG), and brain magnetic resonance imaging (MRI) characteristics and immunomodulatory therapy timing were retrospectively analyzed in children with ANE. ANE severity scores (ANE-SS) and MRI scores were also assessed. The associations of patient characteristics with 6-month modified Rankin scale (mRS) and length of hospitalization were determined using either univariate linear regression or one-way analysis of variance. Results: 7 children were retrospectively evaluated. Normal EEG sleep spindles ( P = .024) and early treatment ( R 2 = .57, P = .030) were associated with improved outcomes (ie, decreased mRS). Higher ANE-SS ( R 2 = .79, P = .011), higher age ( R 2 = .62, P = .038), and presence of brainstem lesions ( P = .015) were associated with longer length of hospitalization. Other patient characteristics were not significantly associated with mRS or length of hospitalization. Conclusion: Early immunomodulatory therapy and normal sleep spindles are associated with better functional outcome in children with ANE.