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

Objective: 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. Methods: 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 U test, Kruskal-Wallis test, and χ2 test were used to compare the differences among groups. The sensitivity and specificity were used to evaluate efficacy of the Cellucci criteria. Results: 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 (χ2=21.63, 13.74, H=22.60, all P<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. Conclusions: 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.目的： 评价儿童自身免疫性脑炎（AE）2020年诊断标准（Cellucci标准）在单中心疑似AE患儿中的应用价值。 方法： 回顾性收集和分析2019年10月至2021年10月在郑州大学第一附属医院住院治疗且疑似AE的121例患儿的临床资料，参照“中国自身免疫性脑炎诊治专家共识”和Graus标准进行临床诊断，分为确诊的抗体阳性AE组、拟诊的抗体阴性AE、可能的AE组和非AE组。再根据Cellucci标准作出新诊断，与临床诊断比较，评价其诊断价值。组间比较采用Mann-Whitney U检验、Kruskal-Wallis检验和χ2检验，Cellucci标准的效能采用灵敏度和特异度评价。 结果： 121例患儿中男72例、女49例，起病年龄10.3（6.5，14.0）岁。临床诊断99例AE（男58例、女41例），包括确诊的抗体阳性AE组43例、拟诊的抗体阴性AE组14例和可能的AE组42例，非AE组22例（男14例、女8例）。99例AE患儿首发症状前2位为癫痫发作（53例，53.5%）、精神行为异常（35例，35.4%），病程中以精神行为异常（77例，77.8%）和癫痫发作（64例，64.6%）常见。不同诊断4组间病程中意识障碍、自主神经功能障碍发生率以及住院时间差异均有统计学意义（χ2=21.63、13.74，H=22.60，均P<0.05）。96例患儿行AE相关抗体检测，45例（46.9%）抗体阳性。应用Cellucci标准诊断确诊的抗体阳性AE 42例、拟诊的抗体阴性AE 34例和可能的AE 14例；参照临床诊断，Cellucci标准诊断上述3类AE的灵敏度依次为93.02%、92.86%、87.88%，特异度依次为96.23%、74.39%、86.36%。 结论： Cellucci标准在疑似AE患儿临床诊疗中诊断可能的AE和确诊的抗体阳性AE时有较高的灵敏度和特异度，诊断拟诊的抗体阴性AE时灵敏度高而特异度较低，建议临床实践中根据实际情况选择性应用，尤其是在诊断拟诊的抗体阴性AE时。.