Background. Acute necrotizing encephalopathy (ANE) is a rare encephalopathy occurring after minor infection in children. ANE was initially described in 1995 in Asian children. The brain lesions are remarkably similar among patients with ANE, including white matter, bilateral thalamic, brain stem, and cerebellar lesions. Most children experience a monophasic illness but there is a predisposition to recurrent episodes in patients who have (dominant/de novo) mutarion in RANBP2 gene.
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