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.