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疑似自身免疫性脑炎患儿的自身免疫性脑炎抗体谱及临床特征。

Autoimmune encephalitis antibody profiles and clinical characteristics of children with suspected autoimmune encephalitis.

机构信息

Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Paediatrics, Chongqing, China.

出版信息

Dev Med Child Neurol. 2024 Apr;66(4):483-492. doi: 10.1111/dmcn.15762. Epub 2023 Oct 2.

Abstract

AIM

To identify the spectrum of autoimmune encephalitis antibody biomarkers (AE-Abs) in children with suspected autoimmune encephalitis and explore the clinical features indicating AE-Abs presence.

METHOD

We included children with suspected autoimmune encephalitis who underwent AE-Abs tests at the Children's Hospital of Chongqing Medical University between June 2020 and June 2022. Clinical features suggestive of AE-Abs were analysed based on AE-Abs test results.

RESULTS

A total of 392 children were tested for AE-Abs with suspected autoimmune encephalitis. Of these, 49.5% were male, with a median age of 7 years 11 months (6 months-17 years 11 months); 93.6% (367/392) of all patients had both serum and cerebrospinal fluid (CSF) tests performed. The antibody-positive rate in the cohort was 23.7% (93/392), the serum antibody-positive rate was 21.9% (84/384), and the CSF antibody-positive rate was 20.8% (78/375). Eleven different AE-Abs were detected. Serum analysis revealed that N-methyl-D-aspartate receptor immunoglobulin-G (NMDAR-IgG) (15.1%) was greater than myelin oligodendrocyte glycoprotein (MOG)-IgG (14.6%) and glial fibrillary acidic protein (GFAP)-IgG (3.3%). CSF analysis revealed that NMDAR-IgG (16.3%) was greater than MOG-IgG (13.8%) and GFAP-IgG (3.3%). Compared with antibody-negative patients, antibody-positive patients were more often female (odds ratio [OR] 1.86, p = 0.03), with memory impairment (OR 2.91, p = 0.01) and sleep disorders (OR 2.08, p = 0.02).

INTERPRETATION

In children, the most frequent AE-Abs detected were NMDAR-IgG and MOG-IgG. Female sex, memory impairment, and sleep disorders predict a higher likelihood of AE-Abs.

摘要

目的

鉴定疑似自身免疫性脑炎患儿的自身免疫性脑炎抗体生物标志物(AE-Abs)谱,并探讨提示 AE-Abs 存在的临床特征。

方法

我们纳入了 2020 年 6 月至 2022 年 6 月在重庆医科大学儿童医院接受 AE-Abs 检测的疑似自身免疫性脑炎患儿。根据 AE-Abs 检测结果分析提示 AE-Abs 存在的临床特征。

结果

共有 392 例疑似自身免疫性脑炎患儿接受 AE-Abs 检测,其中男性占 49.5%(194/392),中位年龄为 7 岁 11 个月(6 个月-17 岁 11 个月);93.6%(367/392)的患儿均同时进行了血清和脑脊液(CSF)检测。该队列的抗体阳性率为 23.7%(93/392),血清抗体阳性率为 21.9%(84/384),CSF 抗体阳性率为 20.8%(78/375)。共检测到 11 种不同的 AE-Abs。血清分析显示,N-甲基-D-天冬氨酸受体免疫球蛋白-G(NMDAR-IgG)(15.1%)高于髓鞘少突胶质细胞糖蛋白(MOG)-IgG(14.6%)和胶质纤维酸性蛋白(GFAP)-IgG(3.3%)。CSF 分析显示,NMDAR-IgG(16.3%)高于 MOG-IgG(13.8%)和 GFAP-IgG(3.3%)。与抗体阴性患者相比,抗体阳性患者更常为女性(比值比 [OR] 1.86,p=0.03),且更易出现记忆障碍(OR 2.91,p=0.01)和睡眠障碍(OR 2.08,p=0.02)。

结论

在儿童中,最常见的 AE-Abs 是 NMDAR-IgG 和 MOG-IgG。女性、记忆障碍和睡眠障碍提示更有可能存在 AE-Abs。

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