The Clinical and Imaging Characteristics Associated With Neurological Sequelae of Pediatric Patients With Acute Necrotizing Encephalopathy

Frontiers in Pediatrics

article

Hong-Min Zhu¹  Chun-Quan Cai¹  Zhi-Sheng Liu²  Si-Min Zhang⁶  Cong Yao⁷  Meng-Qing Luo²  Hui-Jing Ma⁸  Tao Lei⁹  Chun-Hui Yuan^1,10  Ge-Fei Wu²  Jia-Sheng Hu² 

[1] Pediatric Clinical College of Tianjin Medical University;Department of Neurology, Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology;Tianjin Children's Hospital (Children's Hospital of Tianjin University);Tianjin Pediatric Research Institute, Tianjin Children's Hospital (Children's Hospital of Tianjin University);Tianjin Key Laboratory of Birth Defects for Prevention and Treatment;Department of Emergency, Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology;Health Care Department, Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology;Department of Radiology, Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology;Department of Rehabilitation, Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology;Department of Laboratory Medicine, Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology

关键词: acute necrotizing encephalopathy;    pediatric patients;    neurological sequelae;    magnetic resonance imaging;    brain lesion;   

DOI  :  10.3389/fped.2021.655074

学科分类：社会科学、人文和艺术（综合）

来源: Frontiers

【 摘 要 】

Background: Acute necrotizing encephalopathy of childhood (ANE) is a rare but rapidly progressing encephalopathy. Importantly, the exact pathogenesis and evidence-based treatment is scarce. Thus, we aimed to identify the clinical, imaging, and therapeutic characteristics that associated with prognosis of pediatric ANE patients. Methods: A retrospective study was conducted on pediatric patients with ANE who were admitted to Wuhan Children's Hospital between January 2014 and September 2019. All cases met the diagnostic criteria for ANE proposed by Mizuguchi in 1997. The clinical information and follow-up data were collected. The prognostic factors were analyzed by trend chi-square test and Goodman–Kruskal gamma test. Results: A total of 41 ANE patients ranging in age from 8.9 to 142 months were included in this study. Seven cases (17%) died, and the other 34 survivors had different degrees of neurological sequelae. Factors tested to be significantly correlated with the severity of neurological sequelae were the intervals from prodromal infection to acute encephalopathy (G = −0.553), conscious disturbance ( r = 0.58), endotracheal intubation ( r = 0.423), elevation of alanine aminotransferase ( r = 0.345), aspartate aminotransferase ( r = 0.393), and cerebrospinal fluid protein ( r = 0.490). In addition, dynamic magnetic resonance imaging (MRI) evaluation on follow-up revealed that the total numbers of brain lesion location (χ 2 = 6.29, P 3 months), and the presence of cavitation predict severe neurological sequelae in pediatric patients with ANE. Early treatments, including steroid therapy and IVIG, had no correlation with better outcomes. Further studies are needed to establish a consensus guideline for the management of ANE.

【 授权许可】

CC BY