Clinical study of 41 children with acute necrotizing encephalopathy
10.3760/cma.j.cn101070-20200416-00655
- VernacularTitle:儿童急性坏死性脑病41例临床研究
- Author:
Hongmin ZHU
1
;
Cong YAO
;
Mengqing LUO
;
Zhiyao TIAN
;
Tao LEI
;
Gefei WU
;
Jiasheng HU
;
Zhisheng LIU
Author Information
1. 华中科技大学同济医学院附属武汉儿童医院神经内科，武汉 430016
- Keywords:
Acute necrotizing encephalopathy;
Child;
Clinical feature;
Magnetic resonance imaging;
Prognosis
- From:
Chinese Journal of Applied Clinical Pediatrics
2021;36(16):1257-1261
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical features and prognosis of acute necrotizing encephalopathy (ANE) in children.Methods:The clinical data and follow-up information of 41 pediatric patients with ANE treated in Wuhan Children′s Hospital, Tongji Medical College, Huazhong University of Science & Technology from January 2014 to September 2019 were retrospectively reviewed.Results:The 41 patients included 23 males and 18 females with the onset age of (4.4±3.2) years.The main prodromal symptoms were gastrointestinal (20/41 cases, 48.8%) and respiratory infections (19/41 cases, 46.3%). Acute encephalopathy progressed rapidly following the prodromal infection [29 cases (70.7%) ≤2 days], and patients had clinical manifestations of coma (32/41 cases, 78.0%), convulsion (32/41 cases, 78.0%), multiple organ dysfunction (27/41 cases, 65.9%), shock and disseminated intravascular coagulation were rarely occured, and 28 cases (68.3%) were admitted to intensive care unit for treatment.Brain magnetic resonance imaging (MRI) showed lesion involving thalamus (41/41 cases, 100.0%), periventricular white matter (34/41 cases, 82.9%), brainstem (31/41 cases, 75.6%), basal ganglia (26/41 cases, 63.4%), cerebral cortex and subcortex (20/41 cases, 48.8%) and cerebellum (18/41 cases, 43.9%). The common presentations on the apparent diffusion coefficient mapping of brain MRI were " tricolor pattern" or " bicolor pattern" of the thalamus.During follow-up (≥ 6 months), MRI showed that hemorrhage, cystic degeneration and atrophy changed dynamically with the progression of ANE.All cases were treated with glucocorticoids, 38 cases(92.7%) with intravenous immune globulin.Seven cases (17.1%) were died and the 34 survivors had different degrees of neurological dysfunction.Conclusions:ANE in children is a distinctive type of clinicoradiologic syndrome with rapid progression and various presentations.Brain MRI has typical imaging characteristics and dynamically indicates the progression of this disease.The treatment options are still limited, the prognosis is poor and the survivors are often with neurological dysfunction.
