自身免疫性脑炎患儿的临床分析

Clinical analysis of children with autoimmune encephalitis

目的探讨儿童自身免疫性脑炎(AE)的临床特点、治疗及预后。方法回顾性分析2014年12月至2022年9月贵阳市妇幼保健院收治的39例AE患儿的临床资料。结果39例AE患儿中,13例为抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎、1例为抗γ-氨基丁酸B型受体(GABA BR)脑炎、1例为抗二肽基肽酶样蛋白(DPPX)脑炎、1例为抗接触蛋白相关蛋白2(CASPR2)脑炎、1例为抗CV2脑炎,余22例中17例AE患儿的抗体为阴性,5例未进行抗体检测。AE患儿的主要症状为癫痫发作、认知及行为障碍、意识障碍、运动障碍、睡眠障碍及自主神经功能障碍等,所有患儿均未合并肿瘤。39例中有36例患儿行一线免疫治疗,其中24例好转,12例无效;一线免疫治疗无效的4例患儿行二线免疫治疗,其中2例有效,2例无效。结论AE患儿的临床特点总体与成人患者相似,但儿童不同类型抗体AE有其自身特点,肿瘤发生率低,预后相对成人较好。抗体阴性的AE和可能的AE标准具有重要的临床实践价值。

Objective To explore the clinical characteristics,therapy and prognosis of childhood autoimmune encephalitis(AE).Methods Clinical data was retrospectively analyzed for 39 children with AE admitted to Guiyang Maternal and Child Health Hospital from December 2014 to September 2022.Results Among 39 children with AE,there were 13 children with anti N-methyl-D-aspartate receptor(NMDAR)encephalitis,1 child withγ-Aminobutyric acid B-type receptor(GABABR)encephalitis,1 child with anti-dipeptidyl-peptidase like protein 6(DPPX)encephalitis,1 child with anti-contact protein-related protein 2(CASPR2)encephalitis and 1 child with anti-CV2 encephalitis.Among the remaining 22 children,17 children were negative for antibodies,and 5 children did not undergo antibody examination.The main symptoms of AE patients included epileptic seizures,cognitive and behavioral disorders,consciousness disorders,motor disorders,sleep disorders and autonomic dysfunction.All children did not have concurrent tumors.The main symptoms of children with AE included epileptic seizures,cognitive and behavioral disorders,consciousness disorders,motor disorders,sleep disorders,and autonomic dysfunction,etc..All children did not have tumors.Among 39 children,36 children underwent the first-line immunotherapy,which were effective in 24 children,ineffective in 12 children.Four children underwent the second-line immunotherapy,which were effective in 2 children,ineffective in 2 children.Conclusion The clinical characteristics of children with AE are generally similar to those of adult patients with AE,but children with AE carrying different types of antibody have their own characteristics,with lower tumor incidence and relatively better prognosis than adult patients with AE.