Abstract
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.
Highlights
Acute necrotizing encephalopathy (ANE), first proposed by Mizuguchi et al in 1995, is a rare but distinctive type of acute encephalopathy with global distribution [1]
An increasing number of cases were later reported in Western countries [3,4,5,6,7,8,9,10,11,12,13] including adult ones [14, 15], suggesting a global distribution of ANE without racial predilection
Gadolinium-contrast magnetic resonance imaging (MRI) has been reported useful in identifying lesions at the very early stage of ANE when conventional computed tomography (CT), MRI, and even diffusion-weighted imaging (DWI) and apparent diffusion coefficient (ADC) show no abnormalities [37]
Summary
Acute necrotizing encephalopathy (ANE), first proposed by Mizuguchi et al in 1995, is a rare but distinctive type of acute encephalopathy with global distribution [1]. Most initially reported ANE cases were Japanese and Taiwanese children, leading to the suspicion that the disease was related to racial factors [2]. An increasing number of cases were later reported in Western countries [3,4,5,6,7,8,9,10,11,12,13] including adult ones [14, 15], suggesting a global distribution of ANE without racial predilection. We summarize the up-to-date knowledge of the etiology, pathogenesis, clinical manifestations, and radiological features, as well as current treatment and prognosis of ANE
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