Introduction:

Acute Necrotizing Encephalopathy of Childhood (ANEC) is characterized by a trivial febrile illness followed by impaired consciousness and/or seizures, bilateral thalamic lesions, along with increased liver enzymes without hyperammonemia, absence of CSF pleocytosis and increased CSF protein.

Methods:

Clinical profile and outcome of children diagnosed with ANEC in a North Indian tertiary care center between January 2014 and June 2018 were retrospectively reviewed from case records.

Results:

Out of 353 cases of Acute Encephalitis Syndrome, 10 children (2.8%, 7 boys, 70%; median age 2.5 years, range 1.5 -4.5 years) were found to have ANEC. Predominant clinical features observedwere encephalopathy (100%, 10/10), seizure (90%,9/10), extrapyramidal features: predominantly dystonia (90%, 9/10)hepatic transaminitis(90%, 9/10), features of raised intracranial pressure(20%, 2/10) and autonomic instability (20%, 2/10). Apart from bilateral thalami (100%),perventricular white matter, brain stem, putamen and cerebellum were variably affected in 4 children (40%).

Nine children (90%), responded favorably to initial immunotherapy withconcomitant intravenous immunoglobulin and dexamethasone followed by tapering oral steroids. Apart from one child who had previous history of Chikungunya, no specific viral etiologies were found in any other child. One child presented with recurrent ANEC had pathogenic autosomal dominant heterozygous mutation in RANBP2 genewas identified. At median follow up of 1 ½ year (range 1 month-4 years), 8 children (80%) had good clinical outcome(Pediatric Cerebral Performance Scale score 1-2)

Conclusion:

Timely identification and early institution of immunotherapy is imperative for favorable long term outcome in children with ANEC.