Clinical features and imaging manifestations of acute necrotizing encephalopathy in children

Purpose We summarized the clinical features of acute necrotizing encephalopathy (ANE) in children. Methods Clinical data of 14 children with ANE at one center from January 2017 to January 2020 were retrospectively analyzed. ANE severity score (ANE-ss) was used to assess ANE severity, and the Glasgow Outcome Scale-Extended was used to assess functional outcomes. Results Peak incidence was between 1 and 3 years of age (71%), and a large percentage of males were affected (79%). The main manifestations included fever (100%), seizure (86%), and impaired consciousness (100%). Seven patients (58%) developed status epilepticus. The etiology was identified in 10 patients (71%) and mainly included H1N1 (36%) and Epstein-Barr virus (29%).Complications included multiple organ failure (MOF), predominantly liver (36%), heart (21%) and kidney (7%) failure, gastrointestinal hemorrhage (21%), hypernatremia (7%), hematuria (7%), disseminated intravascular coagulation (7%), and shock (7%). Pleocytosis was observed in two patients, and increased cerebrospinal fluid protein was found in 11 patients. A missense mutation in RANBP2 (c.1754C>T: p.Thr585Met) was observed in one patient. Magnetic resonance imaging revealed increased T2 and T1 signal density in multifocal and symmetric brain lesions (bilateral thalami, 100%) in all patients during the acute phase. There were no deaths. Nine children retained neurological sequelae affecting movement, cognition, speech, vision, and/or seizure. Four children recovered almost completely. There was a significant correlation between risk classification and outcome by ANE-ss. Conclusion ANE is a group of clinical and imaging syndromes. Most patients have severe neurological sequelae, and ANE may have lower mortality. And ANE-ss can assess prognosis.