Serial outcomes in acute necrotising encephalopathy of childhood: A medium and long term study

Acute necrotising encephalopathy (ANEC) is a severe, debilitating childhood disorder. We used the ANEC scoring system (ANE-ss) and standardised neurodevelopmental scores to objectively characterise medium and long term outcomes.

Retrospective review of children with ANEC at KK Women's and Children's Hospital, Singapore, from 2005 to 2012. ANE-ss was determined from clinical features and neuroimaging, and neurodevelopmental scores (Pediatric Glasgow Outcome Scale Extended, Pediatric Cerebral Performance Category scale and Pediatric Overall Performance Category scale) were applied at 1, 6, 12 and 24months post diagnosis.

Seven patients with ANEC were studied. All had a viral prodrome with fever, and encephalopathy at presentation, and received immunotherapy (steroids or immunoglobulin). ANE-ss scores were medium risk in 4 patients and high risk in 3 patients. One died (high risk ANE-ss) and outcome was determined in the 6 survivors. At 1month post diagnosis, 3 patients (50%) were mildly affected and 3 (50%) were severely affected. Morbidity rates improved by 12months, with 67% and 33.3% scoring in the mildly affected and severely affected ranges, respectively. Medium risk patients did well with majority having little or no neurological deficits and good outcome scores.

Mortality and severe morbidity correlated well with high risk ANE-ss. However, our patients with medium risk ANE-ss had good neurodevelopmental sequelae. Serial disability scoring is useful in evaluating the progress of ANEC patients on follow up. Assessment at 1month post diagnosis can aid prognostication of long term outcome.