[PS18-3B-2] Acute Necrotizing Encephalopathy in Children: A Rare but Important Neurological Condition
Acute necrotizing encephalopathy (ANE) is a rare but unique, diffuse encephalopathy that may affect infants and children. It may be manifested by rapid deterioration in the level of consciousness and seizures after a febrile viral illness such as influenza A and B. However, there seem to be no obvious causality between viral infections and ANE and the pathogenic mechanism of ANE remain unclear. The putative mechanism is that some viruses trigger cytokine formation which breaks down blood brain barrier in particular regions of brain resulting in localized edema or hemorrhage.
It was initially reported in Japan, Korea, Taiwan, suggesting ethnic factors, but an increasing number of cases were later reported in other western countries. Most cases are sporadic, but a missense mutation in nuclear pore gene RANBP2 has been identified in familial and recurrent cases. Furthermore, it is also believed that altered immune responses may contribute to ANE, based on individual HLA genotypes.
ANE shows a wide variety of different clinical manifestations, but the diagnostic hall mark is symmetric, multifocal brain lesions involving bilateral thalami, upper brainstem tegmentum and other regions on magnetic resonance imaging (MRI). Even though the treatment of ANE is still limited, anti-inflammatory, immunomodulatory therapy or hypothermia might be considered especially in early childhood, in which fatal cases have been frequently reported. This lecture will summarize the up-to-date knowledge on ANE in many aspects.
It was initially reported in Japan, Korea, Taiwan, suggesting ethnic factors, but an increasing number of cases were later reported in other western countries. Most cases are sporadic, but a missense mutation in nuclear pore gene RANBP2 has been identified in familial and recurrent cases. Furthermore, it is also believed that altered immune responses may contribute to ANE, based on individual HLA genotypes.
ANE shows a wide variety of different clinical manifestations, but the diagnostic hall mark is symmetric, multifocal brain lesions involving bilateral thalami, upper brainstem tegmentum and other regions on magnetic resonance imaging (MRI). Even though the treatment of ANE is still limited, anti-inflammatory, immunomodulatory therapy or hypothermia might be considered especially in early childhood, in which fatal cases have been frequently reported. This lecture will summarize the up-to-date knowledge on ANE in many aspects.