ANE Awareness
What is ANE (Acute Necrotizing Encephalopathy)
Acute Necrotizing Encephalopathy is a disease characterized by brain injury (encephalopathy) that usually follows an acute, mostly viral, infection. Influenza is the leading associated virus. 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.
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)”.
Although the clinical course and the prognosis of ANE is 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. The best treatment of ANE is still under investigation but may include corticosteroids and anticytokine therapies, including TNFa antagonists.
Disclaimer: The information in this website is intended as a general information/ discussion guideline only.It should never be construed as medical advice.For further information contact your local health care provider.If you have any questions regarding health concerns, seek medical assistance from a qualified health professional
“It’s a bit more complicated than “ANE is caused by a mutation in a gene” or “it is caused by a virus” or “it is caused by environmental factors”. In reality it is a combination of all of these. Your genes encode proteins that make up your cells. These proteins have various functions including how your body reacts to viruses and the environment. Disentangling all of this is very complicated. In the lab we can see that cells cannot survive without a RanBP2 protein – so it is likely that ANE-associated mutations do not completely disable the gene but alter it in a very particular way so that the body’s reaction to certain viral infections is altered”.
ALEXANDER F. PALAZZO,
ASSOCIATE PROFESSOR ,
TORONTO CANADA