Abstract | 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.
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Authors | Lianhong Wu, Hailun Peng, Yan Jiang, Ling He, Li Jiang, Yue Hu |
Journal | International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience
(Int J Dev Neurosci)
Vol. 82
Issue 5
Pg. 447-457
(Aug 2022)
ISSN: 1873-474X [Electronic] United States |
PMID | 35688614
(Publication Type: Journal Article)
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Copyright | © 2022 International Society for Developmental Neuroscience. |
Topics |
- Brain Diseases
(diagnostic imaging)
- Child
- Epstein-Barr Virus Infections
- Herpesvirus 4, Human
- Humans
- Influenza A Virus, H1N1 Subtype
- Leukoencephalitis, Acute Hemorrhagic
(diagnostic imaging, genetics)
- Magnetic Resonance Imaging
- Male
- Retrospective Studies
- Seizures
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