Abstract
Acute necrotizing encephalopathy (ANE) is a rare and devastating disorder characterized by fever and rapid progression to coma after common viral infections. Most cases of ANE are sporadic; however, the observation of multiple cases in the same family with recurrent episodes of ANE led to the identification of a genetic form of the disorder, called ANE1, and to the discovery of the causative mutation in RANBP2.
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References
Mizuguchi M, Abe J, Mikkaichi K, et al. Acute necrotising encephalopathy of childhood: a new syndrome presenting with multifocal, symmetric brain lesions. J Neurol Neurosurg Psychiatry. 1995;58:555–61.
Wu X, Wu W, Pan W, et al. Acute necrotizing encephalopathy: an underrecognized clinicoradiologic disorder. Mediat Inflamm. 2015;1–10.
Wong AM, Simon EM, Zimmerman RA, et al. Acute necrotizing encephalopathy of childhood: correlation of MR findings and clinical outcome. AJNR Am J Neuroradiol. 2006;27:1919–23.
Neilson DE, Adams MD, Orr CMD, et al. Infection-triggered familial or recurrent cases of acute necrotizing encephalopathy by mutations in a component of the nuclear pore, RANBP2. Am J Hum Genet. 2009;84:44–51.
Bergamino L, Capra V, Biancheri R, et al. Immunomodulatory therapy in recurrent acute necrotizing encephalopathy ANE1. Brain and Development. 2012;34:384–91.
Tenembaum S, Chitnis T, Ness J, for the International Pediatric MS Study Group. Acute disseminated encephalomyelitis. Neurology. 2007;68(Suppl 2):S23–36.
Neilson DE, Heidi S, Feiler HS, et al. Autosomal dominant acute necrotizing encephalopathy maps to 2q12.1-2q13. Ann Neurol. 2004;55:291–4.
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Alper, G. (2017). Acute Necrotizing Encephalopathy Mimicking ADEM. In: Waubant, E., Lotze, T. (eds) Pediatric Demyelinating Diseases of the Central Nervous System and Their Mimics. Springer, Cham. https://doi.org/10.1007/978-3-319-61407-6_2
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DOI: https://doi.org/10.1007/978-3-319-61407-6_2
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