Acute Necrotizing Encephalopathy

OBJECTIVE: To describe the clinical profile of pediatric patients with acute necrotizing encephalopathy (ANE). STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: Aga Khan University Hospital, Karachi, Pakistan, from January 2014 to October 2017. METHODOLOGY: Retrospective review of medical records of all children aged 1 month to 16 years admitted with diagnosis of ANE was done. Diagnosis was based on the criteria of ANE described by Mizuguchi et al. the clinical profile, management and outcome were recorded. RESULTS: There were 17 patients. The mean age at presentation was 55.47 ± 59.13 months. The most common presentation was fever with altered consciousness and seizures. The mean length of stay was 11.7 ± 5.6 days. Viral etiology was established in three children. The managements of the patients were symptomatic and supportive; the combination of antibiotics, antivirals and anticonvulsants was the most frequently used regimen. Eleven out of seventeen (65%) patients required intensive care unit admission and mechanical ventilation; while others were managed in the special care unit. Three (17.6%) children died during the stay; while 10 (58.8%) children developed severe morbidity in the form of neurodevelopmental sequelae. CONCLUSION: The devastating outcome of ANE seemed to occur with increasing severity at the time of initial presentation; and the use of antivirals and immunomodulation did not alter the course of disease.