Clinical Profile, Intensive Care Needs, and Short-Term Outcome of Acute Necrotizing Encephalopathy of Childhood: A Retrospective Study From a Tertiary Care Hospital in North India
- PMID: 40617070
- DOI: 10.1016/j.pediatrneurol.2025.06.016
Clinical Profile, Intensive Care Needs, and Short-Term Outcome of Acute Necrotizing Encephalopathy of Childhood: A Retrospective Study From a Tertiary Care Hospital in North India
Abstract
Background: Acute necrotizing encephalopathy of childhood (ANEC) is a rare parainfectious clinicoradiological syndrome characterized by rapid neurological deterioration and poor outcomes.
Methods: We conducted a retrospective study over 11 years (2014-2024) in the pediatric emergency and intensive care units of a quaternary hospital in North India. Children aged one month to 12 years diagnosed with ANEC were enrolled. Data on demographics, clinical features, laboratory and neuroimaging findings, etiology, management, and outcomes were collected. The ANEC Severity Score (ANE-SS) was calculated.
Results: Thirty-two children were included (median age, 4 [interquartile range, 1-7] years; 53.1% male). Common clinical features included altered state of consciousness (96.9%), fever (93.7%), seizures (78.1%), and signs of raised intracranial pressure (46.9%). Organ dysfunctions included encephalopathy (100%), transaminitis (56.2%), and thrombocytopenia (46.9%). Neuroimaging revealed bilateral thalamic involvement in all cases. Etiology was identified in 37.5%, most commonly dengue virus (21.9%), followed by H1N1 (6.2%). Intensive care interventions included mechanical ventilation (56.2%) and vasoactive drugs (31.2%). Immunomodulatory therapy included methylprednisolone (78.1%), intravenous immunoglobulin (25%), and tocilizumab (15.6%). Survival rate was 78.1%. At discharge, the median Pediatric Cerebral Performance Category score was 3 (3-4), indicating moderate to severe disability. High-risk ANE-SS was significantly associated with mortality (P = 0.007).
Conclusions: ANEC remains a severe pediatric encephalopathy with high neuromorbidity. Dengue virus was the most common trigger in this cohort. Early identification and intensive care support, along with immunomodulation, are key. ANE-SS may serve as a valuable prognostic tool.
Keywords: Acute necrotizing encephalopathy; Cytokine storm; Dengue virus; Immunotherapy; Magnetic resonance imaging; Methylprednisolone; Pediatric intensive care units; Pediatrics.
Copyright © 2025 Elsevier Inc. All rights reserved.
Conflict of interest statement
Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
Similar articles
-
Influenza-Associated Acute Necrotizing Encephalopathy in US Children.JAMA. 2025 Jul 30:e2511534. doi: 10.1001/jama.2025.11534. Online ahead of print. JAMA. 2025. PMID: 40736730
-
Clinical features and outcomes of nine children with acute necrotizing encephalopathy.Front Pediatr. 2025 Aug 6;13:1615960. doi: 10.3389/fped.2025.1615960. eCollection 2025. Front Pediatr. 2025. PMID: 40843075 Free PMC article.
-
Systemic Inflammatory Response Syndrome.2025 Jun 20. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan–. 2025 Jun 20. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan–. PMID: 31613449 Free Books & Documents.
-
Interventions for necrotizing soft tissue infections in adults.Cochrane Database Syst Rev. 2018 May 31;5(5):CD011680. doi: 10.1002/14651858.CD011680.pub2. Cochrane Database Syst Rev. 2018. PMID: 29851032 Free PMC article.
-
Heliox for croup in children.Cochrane Database Syst Rev. 2021 Aug 16;8(8):CD006822. doi: 10.1002/14651858.CD006822.pub6. Cochrane Database Syst Rev. 2021. PMID: 34397099 Free PMC article.
MeSH terms
LinkOut - more resources
Full Text Sources
Medical