Clinical Features And Imaging Manifestations Of Acute Necrotizing Encephalopathy In Children

Objective: 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-ss can assess prognosis.