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Favorable outcomes in acute necrotizing encephalopathy in a child treated with hypothermia
Wendy S Vargas1, Sabiha Merchant, Gail Solomon
1Department of Pediatric Neurology, New York Presbyterian Hospital and Weill Cornell Medical College, New York, New York 10065, USA.
Insights
Acute necrotizing encephalopathy, a rare childhood brain disorder, is linked to influenza. Early diagnosis and treatment, including hypothermia, significantly improve outcomes for affected children.
Area of Science:
- Pediatric Neurology
- Infectious Diseases
Background:
- Acute necrotizing encephalopathy (ANE) is a rare, severe neurological disorder predominantly affecting young children in East Asia.
- It typically follows an upper respiratory infection and is associated with influenza viruses.
Observation:
- The case describes a 4-year-old Caucasian girl presenting with fever, altered consciousness, and seizures.
- Influenza A was confirmed via nasal swab, and magnetic resonance imaging revealed characteristic brain lesions.
Findings:
- The hallmark of ANE includes multifocal, symmetric brain lesions in the thalami, brainstem, white matter, cerebellum, and medulla.
- Historically, ANE had a dismal prognosis with high mortality and severe neurological sequelae.
Implications:
- Prompt recognition and management of ANE are crucial for improving patient outcomes.
- Treatment with hypothermia and anti-inflammatory agents may lead to favorable recovery, even in non-Asian populations.
Abstract:
Acute necrotizing encephalopathy predominately affects young children in Japan, Taiwan, and Korea. It manifests with fever, altered mental status, and seizures 2-5 days after the onset of upper respiratory infection. It is commonly associated with influenzas A, B, and H1N1. The hallmark of the encephalopathy involves multifocal, symmetric brain lesions affecting the bilateral thalami, brainstem tegmentum, cerebral periventricular white matter, cerebellum, and medulla, as visualized by computed tomography and magnetic resonance imaging. Prognoses were uniformly dismal before 1980, with high mortality rates and severe neurologic sequelae in survivors. We describe a previously healthy 4-year-old Caucasian girl who presented with fever, alterations of consciousness, and convulsions. Nasal swab revealed her to be influenza A-positive, and her magnetic resonance imaging was diagnostic of the disease. Prompt recognition of the disease and treatment with hypothermia and anti-inflammatory agents led to a favorable outcome.
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