National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Acute necrotizing encephalopathy


Other Names:
Acute necrotizing encephalitis; ANE
Subtypes:
Acute necrotizing encephalopathy (ANE) is a rare disease characterized by brain damage (encephalopathy) that usually follows an acute febrile disease, mostly viral infections.[1]. Most of the reported cases are from previously healthy Japanese and Taiwanese children, but it is now known that the disease may affect anybody in the world.  The symptoms of the viral infection (fever, respiratory infection, and gastroenteritis, among others) are followed by seizures, disturbance of consciousness that may rapidly progress to a coma, liver problems, and neurological deficits.[1][2] 

The disease is caused by both environmental factors and genetic factors. Usually, ANE develops secondary to viral infections, among which the influenza A, influenza B, and the human herpes virus 6, are the most common. ANE can be familial or sporadic, but both forms are very similar to each other. Most familial cases are caused by mutations in the RANBP2 gene, and are known as “infection-induced acute encephalopathy 3 (IIAE3)”.[2][3]  

Although the clinical course and the prognosis of ANE are diverse, the characteristic that is specific to the disease is the “multifocal symmetric brain lesions affecting the thalami, brain stem, cerebral white matter, and cerebellum” which can be seen on computed tomography (CT) or magnetic resonance imaging (MRI) exams. A picture of the brain may help you better visualize the areas of the brain affected. The best treatment of ANE is still under investigation but may include corticosteroids and anticytokine therapies, including TNFα antagonists.[2] 
Last updated: 2/23/2017

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In-Depth Information

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  1. Neilson D. Susceptibility to Infection-Induced Acute Encephalopathy 3. GeneReviews. December 4, 2014; https://www.ncbi.nlm.nih.gov/books/NBK258641/.
  2. Xiujuan Wu, Wei Wu, Wei Pan, Limin Wu, Kangding Liu & Hong-Liang Zhang. Acute Necrotizing Encephalopathy: An Underrecognized Clinicoradiologic Disorder. Mediators of Inflammation. 2015; 2015:https://www.hindawi.com/journals/mi/2015/792578/cta/.
  3. Suri M. Genetic basis for acute necrotizing encephalopathy of childhood. Dev Med Child Neurol. January, 2010; 52(1):4-5. http://onlinelibrary.wiley.com/doi/10.1111/j.1469-8749.2009.03495.x/full.