Key result
SARS-CoV-2 and RANBP2 mutations are linked to rapid acute necrotizing encephalopathy progression via CNS hyper-inflammation.
Why the study?
Acute necrotizing encephalopathy (ANE) is a rare childhood neurological disorder linked to RANBP2 mutations and SARS-CoV-2 infection causing hyper-inflammatory CNS invasion.
Highlights a rare case of acute necrotizing encephalopathy associated with COVID-19 and RANBP2 mutation in a pediatric patient.
Hyper-inflammatory response may link SARS-CoV-2 and RANBP2 in pediatric ANE; leaves open whether immunomodulation alters outcomes.
Acute necrotizing encephalopathy (ANE) is a rare childhood neurological disorder. The familiar type of ANE (ANE1) is associated with a mutation in the RANBP2 gene. Both SARS-CoV-2 and RANBP2 mutations are responsible for the manifestation of a hyper-inflammatory response that invades the central nervous system and plays a key role in the rapid progression of encephalopathy.
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Christou et al. (2023) studied this question. SARS-CoV-2 and RANBP2 mutations trigger a central nervous system hyper-inflammatory response driving rapid progression of acute necrotizing encephalopathy.
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