The Diagnostic Challenge and Prognosis of Autoimmune Encephalitis in Children: A Single-Center Retrospective Study

医学 自身免疫性脑炎 脑电图 脑炎 磁共振成像 强的松 脑脊液 回顾性队列研究 儿科 疾病 甲基强的松龙 人口统计学的 内科学 免疫学 放射科 病毒 人口学 精神科 社会学
作者
Jingjing Dong,Xiaoli Yi
出处
期刊:Pediatric Neurology [Elsevier]
卷期号:146: 103-109 被引量:2
标识
DOI:10.1016/j.pediatrneurol.2023.06.014
摘要

Autoimmune encephalitis (AE) is an immune-mediated encephalitis; nevertheless, its diagnosis in children remains challenging. This study aimed to reveal the clinical characteristics, diagnostic processes, and therapeutic outcomes of AE in children.A total of 18 children with AE were enrolled. Antibody assay was performed in the cerebrospinal fluid (CSF) and serum samples by indirect immunofluorescence. Electroencephalography (EEG) and magnetic resonance imaging (MRI) were monitored to reflect abnormal neural signals. In addition, demographics data, neurological symptoms, therapeutic strategies, and outcomes were recorded and analyzed.Convulsion (50.00%) and emotional disturbance (44.44%) were common clinical symptoms of AE. The biochemical parameters in the CSF had a relatively low diagnostic value. Antibodies in the CSF were dominant in the diagnosis of AE but those in the serum were limited in the diagnosis of anti-MOG or anti-LGI1 AE. In addition, all children showed abnormalities in EEG (72.22%) or MRI (66.67%). Methylprednisolone combined with sequential oral prednisone (83.33%) and gamma globulin (88.89%) were the dominant drugs, achieving an overall recovery rate of 72.22%. However, there were still two patients who had poor outcomes, including Patient 3 with a young age (two years old) and progressive symptom and Patient 12 with a long disease course before treatment (>120 days).The clinical manifestations of AE are varied in children. Antibody in the CSF was dominant, and EEG and MRI were instructive in the diagnosis of AE. Young age, progressive symptom, and prolonged disease course before treatment may contribute to poor outcomes.

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