Journal
NEUROHOSPITALIST
Volume 12, Issue 1, Pages 90-95Publisher
SAGE PUBLICATIONS LTD
DOI: 10.1177/19418744211008351
Keywords
neuroinflammation; infection; cytokine storm; neuropathology
Categories
Funding
- [NIH 2R25NS070680]
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Acute necrotizing encephalopathy (ANE) is a rare and life-threatening disease associated with viral triggers. We presented a case of a pregnant woman who developed ANE and progressed to brain death within 36 hours of symptom onset, highlighting the importance of early diagnosis.
Acute necrotizing encephalopathy (ANE) is a rare and life-threatening disease. It is caused by a cytokine-mediated injury to the brain with characteristic hemorrhagic and edematous lesions involving the bilateral thalami, brainstem, and other subcortical structures. The disease is commonly associated with antecedent viral triggers such as influenza, parainfluenza, and more recently, SARS-CoV-2, with subsequent neurologic deterioration occurring within days to weeks. Here, we present a case of a pregnant adult woman who developed a hyperacute form of ANE, progressing to brain death within 36 hours of symptom onset. Her diagnosis was confirmed via brain imaging, CSF studies, and neurohistopathological analysis. This case highlights the importance of establishing an early diagnosis for this under-recognized disease, and also suggests an association between ANE and early pregnancy.
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