Acute Necrotizing Encephalopathy: Case Report

Background: Acute Necrotizing Encephalopathy (ANE) is a rare disease that follows viral infections, most commonly influenza. Patients present with altered mental status and rapidly progress into a comatose state. Characteristic bilateral thalamic and brainstem lesions are seen on magnetic resonance imaging scans. Most early cases were isolated to East Asian countries such as Japan and Taiwan, with very few cases reported in the United States and Europe. Results/Conclusions: Recent studies of sporadic and familial cases of ANE have found a missense mutation in the Ran Binding Protein 2 (RANBP2) gene. We describe the clinical and radiological findings and treatment of a 14 year old female with recurrent RANBP2 positive ANE.