Hypoxic-Ischemic Encephalopathy Mimicking Acute Necrotizing Encephalopathy

Abstract Background 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. Patient We describe a previously healthy 9-month-old girl who presented with upper respiratory symptoms,...

Full description

Saved in:
Bibliographic Details
Published in:Pediatric neurology 2015, Vol.52 (1), p.110-114
Main Authors: Wang, Kevin Y., BS, Singer, Harvey S., MD, Crain, Barbara, MD, PhD, Gujar, Sachin, MBBS, MD, Lin, Doris D.M., MD, PhD
Format: Article
Language:English
Subjects:
acute necrotizing encephalopathy
Brain - pathology
Diagnosis, Differential
Fatal Outcome
Female
Humans
Hypoxia-Ischemia, Brain - diagnosis
Hypoxia-Ischemia, Brain - pathology
Hypoxia-Ischemia, Brain - therapy
hypoxic-ischemic encephalopathy
Infant
influenza
Influenza A virus
Leukoencephalitis, Acute Hemorrhagic - diagnosis
Magnetic Resonance Imaging
Neurology
pediatric
Pediatrics
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Abstract Background 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. Patient 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. Conclusion Combined clinical and neuroimaging features may be suggestive but not sufficient for the diagnosis of acute necrotizing encephalopathy.
ISSN:0887-8994
1873-5150
DOI:10.1016/j.pediatrneurol.2014.09.009