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The Hypoxic Ischemic Encephalopathy Model of Perinatal Ischemia
Published on: November 19, 2008
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Hypoxic-ischemic encephalopathy mimicking acute necrotizing encephalopathy.
Kevin Y Wang1, Harvey S Singer2, Barbara Crain3
1Johns Hopkins University School of Medicine, Baltimore, Maryland.
Pediatric Neurology
|December 3, 2014
Summary
Acute necrotizing encephalopathy diagnosis can be challenging, even with characteristic imaging. This case highlights that neuroimaging alone may not be sufficient for definitive diagnosis in children.
Area of Science:
- Pediatric Neurology
- Neuroradiology
- Infectious Diseases
Background:
- Acute necrotizing encephalopathy (ANE) is a rare pediatric neurological disorder.
- Diagnosis often relies on characteristic neuroimaging findings.
- Influenza A virus is a known potential trigger for ANE.
Observation:
- A previously healthy 9-month-old girl presented with respiratory symptoms, seizures, and influenza A infection.
- MRI revealed signal abnormalities in the thalami, basal ganglia, brainstem, white matter, and cerebellum, consistent with ANE.
- The patient experienced cardiac arrest and was treated with ECMO, methylprednisone, IVIG, and plasmapheresis without improvement.
Findings:
- Autopsy revealed hypoxic-ischemic injury but lacked the typical hemorrhagic necrosis seen in ANE.
- The clinical presentation and imaging were suggestive of ANE, but neuropathology findings were atypical.
Implications:
- Clinical and neuroimaging findings alone may not be sufficient for a definitive diagnosis of ANE.
- This case underscores the importance of integrating all diagnostic data, including autopsy findings, for accurate diagnosis.
- Further research is needed to understand the spectrum of neuropathological findings in ANE.
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