Clinical evaluation of acute necrotizing encephalopathy in children

Xiaowei Fan; Li Huang; Suyun Li; Sida Yang; Yongling Song; Qinglian Chen; Yumei Xiong; Qiuyan Peng; Wencheng Ma; Dandan Hu; Peiqing Li

doi:10.3389/fped.2022.947693

Clinical evaluation of acute necrotizing encephalopathy in children

Front Pediatr. 2022 Aug 25:10:947693. doi: 10.3389/fped.2022.947693. eCollection 2022.

Authors

Affiliation

¹ Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangdong Provincial Clinical Research Center for Child Health, Guangzhou, China.

Abstract

Objective: Acute necrotizing encephalopathy (ANE) is a rare but severe encephalopathy and is associated with a high morbidity and mortality. We aimed to analyze and compare the clinical features and predictive indicators of pediatric ANE.

Materials and methods: This retrospective study included children with ANE diagnosed at Guangzhou Women and Children's Medical Center between November 2018 and January 2020. Pediatric patients' information, including clinical characteristics, laboratory tests, neuroelectrophysiology and brain magnetic resonance imaging (MRI) findings, MRI score, brainstem auditory evoked potential (BAEP) grades, ANE severity scores (ANE-SS), and modified Rankin scale (mRS), were collected.

Results: Twelve ANE patients were included. Among them, one patient (8.3%) died from brainstem dysfunction, one (8.3%) recovered and 10 (83.3%) experienced neurological sequelae. All patients had an initial viral infection and neurological symptoms such as acute disturbance of consciousness (ADOC) or seizure, and the interval from onset of the disease to neurological manifestations was 3 (1.25-3) days. MRI score-I ranged from 1 to 3 (1.8 ± 0.7), MRI score-II ranged from 1 to 4 (2.5 ± 1.1). ANE-SS varied from 1 to 6 (3.9 ± 1.3). The scores of mRS were from 0 to 6 (2.9 ± 1.7). Higher MRI score were associated with worse outcomes, while the BAEP grade and ANE-SS score were not significantly associated with mRS.

Conclusion: ANE is a severe encephalopathy syndrome with rapid progression, resulting in serious neurological sequelae. Compared with BAEP grade and ANE-SS, brain MRI shows more comprehensive advantages in predicting the prognosis of ANE patients. More in-depth research and better indicators are still needed to support the evaluation and treatment of ANE.

Keywords: acute necrotizing encephalopathy; acute necrotizing encephalopathy severity score; brainstem auditory evoked potential; children; magnetic resonance imaging scores; modified Rankin scale.