This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
Infection-induced acute encephalopathy 3 (IIAE3) is the susceptibility to recurrent acute necrotizing encephalopathy (ANE). ANE refers to the brain lesions that develop within days following the onset of an acute viral illness caused by influenza A, influenza B, parainfluenza II, human herpes virus 6, coxsackie virus, or an enterovirus. Although most cases occurs before six years of age, first episodes have been observed in teenagers and adults. ANE begins within 12 hours to three or four days of the first viral symptoms. Symptoms include fever, cough, congestion, vomiting, and diarrhea in the first few days, followed by neurological problems, such as seizures, hallucinations, difficulty coordinating movements (ataxia), or abnormal muscle tone. Most affected individuals go into a coma, which usually lasts for a number of weeks. The condition is described as “acute” because the episodes of illness are time-limited. It is caused by mutations in the RANBP2 gene. Inheritance is autosomal dominant. In many cases, treatment involves corticosteroids, as well as immunoglobulin therapy, plasmapheresis, and TNFα antagonists. In some cases, treatment is not required.
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