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Acute necrotizing encephalopathy of childhood with radiographic progression over 10 hours

We report a 14-month-old girl who was examined for altered mentation. Although head CT on arrival was normal, CT and MRI 10 hours later demonstrated bilateral thalamic abnormalities. Results of an extensive laboratory evaluation were negative, and the patient was diagnosed with acute necrotizing encephalopathy of childhood (ANEC).

The patient, a 14-month-old girl of western European and African-American ancestry (no known Asian ancestry), with no medical or travel history, was brought to the emergency department after discovered unresponsive. She had been febrile for 2 days, with decreased appetite, but with apparently normal mentation until presentation. She was noted to have episodes of four-extremity extension, prompting loading with phenobarbital. Head CT at that time was normal (figure, A). CSF analysis revealed an erythrocyte count of 3 cells/mL, leukocyte count of 1 cell/mL, protein of 51 mg/dL, and glucose of 64 mg/dL. Urine and serum toxicology screens were negative. Blood gas, routine blood counts, chemistry, hepatic panel, and ammonia were all normal, except for a mild …