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.