Acute Necrotizing Encephalopathy in a Four-Year-Old Boy

Diagnostics (Basel). 2021 Mar 22;11(3):568. doi: 10.3390/diagnostics11030568.

Abstract

Acute necrotizing encephalopathy is a devastating clinico-radiological syndrome characterized by fulminant neurological deterioration after an antecedent febrile illness, as well as the imaging hallmark of bilateral thalamic involvement. Herein, we describe a 4-year-old boy with typical clinical and neuroimaging features of acute necrotizing encephalopathy. The bithalamic swelling led to a block of cerebrospinal fluid circulation at the foramen of Monro, thereby causing the mild dilatation of lateral ventricles. The periventricular areas could, therefore, have been potentially affected by the acute necrotizing encephalopathy per se and/or transependymal edema secondary to obstructive hydrocephalus. The information from diffusion imaging allows for differentiation between these two pathophysiological processes.

Keywords: acute necrotizing encephalopathy; hydrocephalus; thalamus.