Acute necrotizing encephalopathy is a rare childhood syndrome associated with distinct and unifying neuroimaging features that are often used for the diagnosis of this entity.

We describe a previously healthy 9-month-old girl who presented with upper respiratory symptoms, suspected seizures, and positive nasopharyngeal rapid antigen test for influenza A virus. Magnetic resonance imaging revealed signal abnormality in both thalami, bilateral caudate nuclei, brainstem tegmentum, subcortical white matter, and cerebellar hemispheres, suggestive of acute necrotizing encephalopathy. She subsequently had a cardiac arrest, was placed on extracorporeal membrane oxygenation, and treated with methylprednisone, intravenous immunoglobulin, and plasmapheresis without apparent clinical response. On autopsy, neuropathology showed evidence of hypoxic-ischemic injury but lacked evidence of hemorrhagic necrosis, which is typically associated with acute necrotizing encephalopathy.

Combined clinical and neuroimaging features may be suggestive but not sufficient for the diagnosis of acute necrotizing encephalopathy.