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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
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results,CD 8 T lymphocytes,CD 4 /CD 8 ,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
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lymphocytes and CD 4 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
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severe and the total T cells and CD 4 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
抗 N- 甲基 -D- 天冬氨酸受体(NMDAR)脑炎是 有效的治疗干预,提高抗 NMDAR 脑炎患儿的生活质量。
由于自身抗体与神经系统表面的受体相互作用,引起中 1 对象与方法
枢神经系统神经元的兴奋性改变,从而导致一系列相应 1.1 研究对象 选取 2016 年 1 月至 2021 年 12 月于
临床症状的疾病 [1] 。该病平均发病年龄为 21 岁,女性 河北医科大学附属河北省儿童医院神经内科诊断为抗
和儿童较多见,18 岁以下的患者约占 38% [2] 。因此该 NMDAR 脑炎的住院患儿 54 例为研究对象。纳入标准:
病在儿童中越来越受到重视,但由于该病具有临床表现 (1)临床资料完整;(2)年龄≤ 16 周岁;(3)抗
多样、儿童精神症状很难被早期发现等特点,给临床诊 NMDAR 抗体检测(血和 / 或脑脊液)结果阳性;(4)
断带来巨大困难 [3] 。随着人们对该病的认识,目前抗 符合 2016 年 GRAUS 等 [5] 提出的抗 NMDAR 脑炎诊断
NMDAR 脑炎已成为最常见的非感染性脑炎 [4] 。2016 标准。排除标准:(1)未在河北省儿童医院完成治疗者;
年 GRAUS 等 [5] 在柳叶刀神经病学杂志上发表了新的抗 (2)抗 NMDAR 抗体阴性者;(3)非河北省儿童医院
NMDAR 脑炎诊断标准。目前公认的抗 NMDAR 脑炎的 初诊者。本研究已获得河北省儿童医院医学伦理委员会
一线治疗方案主要为皮质类固醇激素、静脉注射丙种球 的审核批准(伦理审查编号:202202),且患儿及其监
蛋白及血浆置换;二线治疗方案为环磷酰胺、利妥昔单 护人对本研究的研究内容、目的等均已知晓,并签署了
抗等 [6] ;但用药剂量、用药周期及一线方案之间是否 知情同意书。
联合治疗,目前仍有争议 [7] 。抗 NMDAR 脑炎临床恢 1.2 抗 NMDAR 脑炎诊断标准 2016 年 GRAUS 等 [5]
复较慢,常需数周至数月,且 12.0%~31.4% 的患者可 将抗 NMDAR 脑炎诊断标准分为拟诊和确诊。拟诊标准:
能会复发。综上所述,抗 NMDAR 脑炎是中枢神经系统 病程 <3 个月,并且具备精神行为异常、语言功能障碍、
炎症性疾病,具有诊断困难、治疗方案多样、复发率较 运动障碍、癫痫发作、意识水平下降、自主神经功能障
高等特点。迄今为止,关于儿童抗 NMDAR 脑炎复发有 碍,共 6 项临床症状中的 4 项;辅助检查中脑电图和脑
关的临床特征及辅助检查结果鲜有文献报道,且初次发 脊液检查有一项异常,并且在尽可能排除其他疾病后可
病年龄、早期临床症状与复发的确切关系尚不清楚。因 以诊断。确诊标准:上述 6 项临床症状中表现出 1 项症
此本研究通过对抗 NMDAR 脑炎患儿临床资料的回顾性 状;脑脊液中自身免疫性抗体阳性或血清中抗体阳性并
分析,以期观察早期临床症状及辅助检查,分析复发相 经过验证检测后确定抗体阳性,尽可能排除其他疾病后
关因素,并对抗 NMDAR 脑炎患儿复发风险进行评估, 可诊断。
从而早期确定可能存在复发风险的患儿,以便给予及时 1.3 研究方法 收集患儿的一般资料(性别、年龄、