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[单中心2020年儿童自身免疫性脑炎诊断方法的临床验证]

[Clinical validation of the 2020 diagnostic approach for pediatric autoimmune encephalitis in a single center].

作者信息

Wang J D, Xie L, Fang X, Zhuo Z H, Jin P N, Fan X L, Li H Y, Kong H M, Wang Y, Wang H L

机构信息

Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.

出版信息

Zhonghua Er Ke Za Zhi. 2022 Aug 2;60(8):786-791. doi: 10.3760/cma.j.cn112140-20220111-00039.

Abstract

To evaluate the value of the 2020 diagnostic criteria (Cellucci criteria) for pediatric autoimmune encephalitis (AE) in children with suspected AE in a single center. The clinical data of 121 children hospitalized at the First Affiliated Hospital of Zhengzhou University from October 2019 to October 2021, with a diagnosis of suspected AE, were retrospectively collected and analyzed. The children were divided into definite antibody-positive AE (dAPAE), probable antibody-negative AE (prANAE), possible AE (pAE) and non-AE groups according to the Chinese expert consensus and the Graus criteria. A new diagnosis was made according to the Cellucci criteria which was compared with the clinical diagnosis to evaluate the diagnostic value of the Cellucci criteria. The Mann-Whitney test, Kruskal-Wallis test, and χ test were used to compare the differences among groups. The sensitivity and specificity were used to evaluate efficacy of the Cellucci criteria. Among the 121 children, 72 were males and 49 were females, with an age of 10.3 (6.5, 14.0) years at disease onset. There were 99 cases diagnosed as AE according the clinical diagnosis (58 males and 41 females), of which 43 cases were diagnosed as dAPAE, 14 cases as prANAE and 42 cases as pAE, and the other 22 cases were not AE (14 males and 8 females). The top 2 initial symptoms in the 99 children with AE were seizures (53 cases, 53.5%) and abnormal mental behaviors (35 cases, 35.4%). And the most common symptoms during the course of the disease were abnormal mental behaviors (77 cases, 77.8%) and seizures (64 cases, 64.6%). There were statistically differences in the incidence of consciousness disorders, autonomic dysfunctions during the course of the disease and the length of hospitalization among the 4 groups (χ=21.63, 13.74, =22.60, all <0.05). Ninety-six of the 121 children were tested for AE-related antibodies, of which 45 cases (46.9%) were antibody-positive. According to the Cellucci criteria, 42 cases were diagnosed as dAPAE, 34 cases as prANAE and 14 cases as pAE. Compared with the clinical diagnosis, the sensitivity of the Cellucci criteria for the diagnosis of the 3 types of AE were 93.02%, 92.86% and 87.88%, and the specificity were 96.23%, 74.39% and 86.36%, respectively. The Cellucci criteria has a high sensitivity and specificity for the diagnosis of pAE and dAPAE in the clinical management of children with suspected AE, while a high sensitivity but low specificity for the diagnosis of prANAE. Therefore, it is recommended to apply the Cellucci criteria selectively in clinical practice according to the actual situation, especially in the diagnosis of prANAE.

摘要

评估2020年儿童自身免疫性脑炎(AE)诊断标准(Cellucci标准)在单中心疑似AE患儿中的应用价值。回顾性收集并分析2019年10月至2021年10月在郑州大学第一附属医院住院的121例诊断为疑似AE的患儿的临床资料。根据中国专家共识和Graus标准,将患儿分为确诊抗体阳性AE(dAPAE)、可能抗体阴性AE(prANAE)、可能AE(pAE)和非AE组。根据Cellucci标准进行重新诊断,并与临床诊断进行比较,以评估Cellucci标准的诊断价值。采用Mann-Whitney检验、Kruskal-Wallis检验和χ检验比较组间差异。采用敏感性和特异性评估Cellucci标准的诊断效能。121例患儿中,男72例,女49例,发病年龄为10.3(6.5,14.0)岁。根据临床诊断,99例诊断为AE(男58例,女41例),其中43例诊断为dAPAE,14例诊断为prANAE,42例诊断为pAE,另外22例不是AE(男14例,女8例)。99例AE患儿最初的前两位症状为癫痫发作(53例,53.5%)和精神行为异常(35例,35.4%)。疾病过程中最常见的症状为精神行为异常(77例,77.8%)和癫痫发作(64例,64.6%)。4组患儿在疾病过程中意识障碍、自主神经功能障碍的发生率及住院时间差异有统计学意义(χ=21.63,13.74,=22.60,均<0.05)。121例患儿中96例检测了AE相关抗体,其中45例(46.9%)抗体阳性。根据Cellucci标准,42例诊断为dAPAE,34例诊断为prANAE,14例诊断为pAE。与临床诊断相比,Cellucci标准对3种类型AE诊断的敏感性分别为93.02%、92.86%和87.88%,特异性分别为96.23%、74.39%和86.36%。Cellucci标准在疑似AE患儿的临床管理中对pAE和dAPAE的诊断具有较高的敏感性和特异性,而对prANAE的诊断敏感性高但特异性低。因此,建议在临床实践中根据实际情况选择性应用Cellucci标准,尤其是在prANAE的诊断中。

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