儿童抗接触蛋白关联蛋白2抗体脑炎4例报道并文献分析

Anti-Contact Protein Associated Protein 2 Antibody Encephalitis in Children:a Report of 4 Cases and Related Literature Analysis

目的 对儿童抗接触蛋白关联蛋白2(Caspr2)抗体脑炎的临床资料进行探讨,提高对儿童自身免疫性脑炎认识。方法分析潍坊市人民医院儿科2020年6月至2022年7月间4例抗Caspr2抗体脑炎的临床资料,并结合相关文献进行讨论。结果 4例患儿中3例男孩,1例女孩,年龄2~11岁,病程14~21d,主要临床表现为发热3例,呕吐4例,精神差2例,抽搐3例,头痛1例,认知障碍1例,病理征阳性3例,颅脑CT异常1例,颅脑MRI异常3例,脑电图异常4例。其中2例接受免疫球蛋白及激素治疗,2例为单独激素治疗。患儿出院后随访2~14月,电话及门诊随访,未再复发。结论 儿童抗Caspr2抗体自身免疫性脑炎较少见,主要表现抽搐、精神异常、认知障碍、神经性疼痛等,反复出现中枢神经系统受累患儿需尽早完善自身免疫性脑炎抗体检查。

Objective To explore the clinical data of children with anti-contact protein associated Protein 2(Caspr2)antibody encephalitis and to improve the understanding of children with autoimmune encephalitis.Methods To analyze the clinical data of 4 cases of anti-Caspr2 antibody encephalitis in Pediatrics Department of Weifang People’s Hospital from June,2020 to July,2022,and to summarize the related literature.Results In 4 cases,there were 3 boys and 1 girl aged 2-11 years.The course of disease was 14-21 days.The main clinical manifestations were fever in 3 cases,vomiting in 4 cases,poor mental state in 2 cases,convulsion in 3 cases,headache in 1 case,cognitive impairment in 1 case,and positive pathological signs in 3 cases.There were 1 abnormal brain CT,3 abnormal brain MRI and 4 abnormal electroencephalogram.Among them,2 cases received immunoglobulin and hormone therapy,and 2 cases received hormone therapy alone.During 2-14 months of follow-up after discharge with telephone and outpatient follow-up,no recurrence was identified.Conclusion Children with autoimmune encephalitis associated with anti-Caspr2 antibody are rare,and the main symptoms are convulsions,mental disorders,cognitive impairment,and neuropathic pain.For children with repeated central nervous system,clinicians need to improve the detection of antibodies to autoimmune encephalitis timely.