Chinese General Practice ›› 2022, Vol. 25 ›› Issue (21): 2629-2634.DOI: 10.12114/j.issn.1007-9572.2022.0047

Special Issue: 儿科最新文章合集

• Original Research • Previous Articles     Next Articles

Clinical Features and Recurrence-related Factors of Anti-N-methyl-D-aspartate Receptor Encephalitis in Children

  

  1. 1. Department of Neurology, Hebei Children's Hospital, Hebei Medical University, Shijiazhuang 050000, China
    2. The Second Hospital of Hebei Medical University, Shijiazhuang 050000, China
  • Received:2022-01-11 Revised:2022-04-11 Published:2022-07-20 Online:2022-05-24
  • Contact: Suzhen SUN
  • About author:
    ZHANG K, WANG C Z, LIU K, et al. Clinical features and recurrence-related factors of anti-N-methyl-D-aspartate receptor encephalitis in children[J]. Chinese General Practice, 2022, 25 (21) : 2629-2634.

儿童抗N-甲基-D-天冬氨酸受体脑炎的临床特征及其复发相关因素分析

  

  1. 1.050000 河北省石家庄市,河北医科大学附属河北省儿童医院神经内科
    2.050000 河北省石家庄市,河北医科大学第二临床医学院
  • 通讯作者: 孙素真
  • 作者简介:
    张凯,王彩贞,刘康,等.儿童抗N-甲基-D-天冬氨酸受体脑炎的临床特征及其复发相关因素分析[J].中国全科医学,2022,25(21):2629-2634.[www.chinagp.net] 作者贡献:张凯进行文章的构思与设计,撰写论文;王彩贞进行资料收集、整理;刘康进行研究的实施与可行性分析;孙素真负责文章的质量控制及审校、对文章整体负责,监督管理。

Abstract:

Background

At present, the recurrence rate of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is relatively high. But there are relatively few studies and most medical staff are not aware of it.

Objective

A comparative study of children with recurrent and non-recurrent anti-NMDAR encephalitis was conducted to improve clinicians' understanding of anti-NMDAR encephalitis and its recurrence-related factors, in order to provide evidence for individualized treatment of children with anti-NMDAR encephalitis and reduce the recurrence rate.

Methods

The clinical data of 54 hospitalized children diagnosed with anti-NDMAR encephalitis in the Department of Neurology, Hebei Children's Hospita, Hebei Medical University from January 2016 to December 2021 were retrospectively analyzed. According to the recurrence situation, the children were divided into non-recurrence group and recurrence group. The clinical characteristics of children with anti-NDMAR encephalitis were analyzed, including gender and age distribution, onset time, clinical symptoms and signs, routine examination of cerebrospinal fluid, imaging examination, electroencephalography, immunological examination, treatment and prognosis. The above-mentioned related indicators of the two groups were compared.

Results

The male-to-female ratio of the 54 children was 1.16∶1, and the school-age group (≥7 years old) accounted for 51.8%; The onset time was higher in summer (June-August) (33.3%) . The clinical symptoms were diverse, and the most common clinical symptom was epilepsy, accounting for 61.1%; 38 cases (70.4%) had abnormal cerebrospinal fluid routine examination results, mainly manifested as increased leukocyte and mildly increased protein in cerebrospinal fluid; MRI examination of brain of 25 children (46.3%) showed abnormal signals, the most common abnormal signal was in the frontal lobe, followed by the basal ganglia and thalamus; 6 cases (11.1%) were given immunoglobulin for ≥2 rounds, and 5 cases (9.3%) were given hormone shock ≥ after 2 rounds, 7 cases (13.0%) received second-line immunotherapy, 31 cases (57.4%) received antiepileptic drugs; 26 cases (48.1%) achieved complete remission. There was no significant difference in gender, age distribution, onset time, cerebrospinal fluid routine examination results, abnormal proportion of brain MRI examination results, video EEG examination results, CD8+T lymphocytes, CD4/CD8, total B lymphocytes, IgA, IgM, IgG, disease course before treatment, proportion of immunoglobulin ≥ 2 rounds, proportion of hormone shock ≥ 2 rounds, proportion of second-line immunotherapy, proportion of antiepileptic drugs, mRS at discharge, and proportion of complete remission between two groups (P>0.05) . The total T lymphocytes and CD4+ T lymphocytes in the recurrence group were lower than those in the non-recurrence group (P<0.05) .

Conclusion

The clinical symptoms of children with anti-NMDAR encephalitis are diverse, but the early clinical symptoms are severe and the total T cells and CD4+ T cells are significantly reduced after admission, so the children who are not easy to achieve complete remission after standard immunotherapy should be alert to the risk of recurrence.

Key words: Anti-N-methyl-D-aspartate receptor encephalitis, Child, Recurrence, Disease attributes

摘要:

背景

目前抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎患儿复发比例较高,而相关研究较少,大部分医务人员对其认识不足。

目的

对复发和非复发的抗NMDAR脑炎患儿进行对比研究,以提高临床医生对抗NMDAR脑炎及其复发相关因素的认识,进而为抗NMDAR脑炎患儿的个性化治疗及降低复发率提供依据。

方法

回顾性分析2016年1月至2021年12月于河北医科大学附属河北省儿童医院神经内科诊断为抗NDMAR脑炎的54例住院患儿的临床资料,根据患儿复发情况分为非复发组和复发组。分析抗NDMAR脑炎患儿临床特征,包括性别和年龄分布、发病时间、临床症状及体征、脑脊液常规检查情况、影像学检查情况、脑电图检查情况、免疫学检查情况、治疗及预后情况,并对两组的上述相关指标进行比较。

结果

54例患儿男女比为1.16∶1,学龄期组(≥7岁)占比较高为51.8%;发病时间以夏季(6—8月)占比较高(33.3%);临床症状表现多样,最常见的临床症状为癫痫发作,占61.1%;38例(70.4%)患儿出现脑脊液常规检查结果异常,主要表现为脑脊液白细胞升高和蛋白轻度升高;25例(46.3%)患儿颅脑磁共振成像(MRI)检查显示异常,异常信号最常见于额叶,其次为基底核及丘脑;6例(11.1%)给予免疫球蛋白治疗≥2轮,5例(9.3%)给予激素冲击治疗≥2轮,7例(13.0%)给予二线免疫治疗,31例(57.4%)给予抗癫痫药治疗;26例(48.1%)达到完全缓解。两组性别和年龄分布情况、发病时间、脑脊液常规检查结果、颅脑MRI检查结果异常比例、视频脑电图检查结果、CD8+T淋巴细胞、CD4/CD8、总B淋巴细胞、IgA、IgM、IgG比较、治疗前病程、免疫球蛋白治疗≥2轮比例、激素冲击治疗≥2轮比例、二线免疫治疗比例、应用抗癫痫药比例、出院时改良的Rankin量表评分(mRS)、完全缓解比例比较,差异无统计学意义(P>0.05)。复发组总T淋巴细胞及CD4+T淋巴细胞较未复发组降低(P<0.05)。

结论

抗NMDAR脑炎患儿的临床症状表现多样,但早期临床症状较重、入院后总T淋巴细胞及CD4+T淋巴细胞明显降低、给予规范免疫治疗后不易达到完全缓解的患儿应警惕复发的风险。

关键词: 抗N-甲基-D-门冬氨酸受体脑炎, 儿童, 复发, 疾病特征