Clinical, laboratory, radiologic profile, and outcome in acute necrotizing encephalopathy of childhood (ANEC) – A case series

Abstract

Acute necrotizing encephalopathy of childhood (ANEC) is a potentially devastating illness characterized by fever, acute encephalopathy, and bilateral thalamic lesions. This case series is a retrospective review of clinical, laboratory, neuroimaging data of 19 children diagnosed with ANEC over 9 years at a single-center from south India. The median age was 84 months (interquartile range 25.5–120 months). All children had acute febrile encephalopathy, 12 had seizures and hypotension each, 8 had vomiting, and 5 had abnormal posturing. The liver transaminases were raised almost all children. All had bilateral thalamic lesions followed by lesions in cerebral white-matter, brain stem, and cerebellar white matter. Microbial etiology was established in 9 patients (7 dengue, 1 H1N1, 1 influenza A). Neurodeficits at discharge were seen in 63% and at follow-up in 33.3% cases. Neurodeficits at follow-up were seen in all cases with age 48 months age (P = 0.018).

Keywords

• acute necrotizing encephalopathy of childhood
• neuroimaging
• neurologic outcome
• steroids