Acute Necrotizing Encephalopathy: An Underrecognized Clinicoradiologic Disorder
Top Cited Papers
Open Access
- 1 January 2015
- journal article
- review article
- Published by Hindawi Limited in Mediators of Inflammation
- Vol. 2015, 1-10
- https://doi.org/10.1155/2015/792578
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.Keywords
Funding Information
- Young Scholars Program of Norman Bethune Health Science Center of Jilin University (2013205035, JDYY42013003, 3C113BK73428, 81241147)
This publication has 72 references indexed in Scilit:
- A novel autoantibody against ephrin type B receptor 2 in acute necrotizing encephalopathyJournal of Neuroinflammation, 2013
- Novel Influenza A (H1N1)-Associated Acute Necrotizing Encephalopathy: A Case ReportAnnals of Rehabilitation Medicine, 2013
- A case of acute necrotizing encephalopathy associated with parainfluenza virus infectionKorean Journal of Pediatrics, 2012
- Novel Human Reovirus Isolated from Children with Acute Necrotizing EncephalopathyEmerging Infectious Diseases, 2011
- Carnitine palmitoyl transferase II polymorphism is associated with multiple syndromes of acute encephalopathy with various infectious diseasesBrain & Development, 2011
- Neuropathologic Correlates for Diffusion Tensor Imaging in Postinfectious EncephalopathyPediatric Neurology, 2011
- Acute Necrotizing Encephalopathy in 3 BrothersPEDIATRICS, 2010
- Clinical Spectrum and Prognostic Factors of Acute Necrotizing Encephalopathy in ChildrenJournal of Korean Medical Science, 2010
- Outcome of acute necrotizing encephalopathy in relation to treatment with corticosteroids and gammaglobulinBrain & Development, 2009
- Infection-Triggered Familial or Recurrent Cases of Acute Necrotizing Encephalopathy Caused by Mutations in a Component of the Nuclear Pore, RANBP2American Journal of Human Genetics, 2009