[Acute necrotizing encephalopathy]

Abstract

Acute necrotizing encephalopathy is a syndrome of acute encephalopathy characterized by bilateral thalamic lesions and prevalent among children in East Asia. The antecedent infection is usually a viral disease with high fever, such as influenza and exanthem subitum. There are neurologic symptoms, such as coma, convulsion and brainstem dysfunction, and findings of systemic organ involvement. Despite the efficacy of corticosteroids in some cases, the prognosis is often poor with a high mortality and severe neurologic sequelae. Recent studies have elucidated the genetic background, providing a clue to the pathogenesis.