Acute Necrotizing Encephalopathy: An Underrecognized Clinicoradiologic Disorder

作者：Xiujuan Wu, Wei Wu, Wei Pan, Limin Wu, Kangding Liu, Hongliang Zhang · 发表于：Mediators of Inflammation · 年份：2015 · DOI：10.1155/2015/792578 · 被引用次数：226 · 研究领域：Infectious Encephalopathies and Encephalitis、Viral Infections and Immunology Research、Long-Term Effects of COVID-19

Acute necrotizing encephalopathy (ANE) is a rare but distinctive type of acute encephalopathy with global distribution. Occurrence of ANE is usually preceded by a virus-associated febrile illness and ensued by rapid deterioration. However, the causal relationship between viral infections and ANE and the exact pathogenesis of ANE remain unclear; both environmental and host factors might be involved. Most cases of ANE are sporadic and nonrecurrent, namely, isolated or sporadic ANE; however, few cases are recurrent and with familial episodes. The recurrent and familial forms of ANE were found to be incompletely autosomal-dominant. Further the missense mutations in the gene encoding the nuclear pore protein Ran Binding Protein 2 (RANBP2) were identified. Although the clinical course and the prognosis of ANE are diverse, the hallmark of neuroradiologic manifestation of ANE is multifocal symmetric brain lesions which are demonstrated by computed tomography (CT) or magnetic resonance imaging (MRI). The treatment of ANE is still under investigation. We summarize the up-to-date knowledge on ANE, with emphasis on prompt diagnosis and better treatment of this rare but fatal disease.