目的 分析儿童抗接触蛋白相关蛋白-2(contactin-associated protein 2,Caspr2)抗体相关自身免疫性脑炎的临床特点、诊疗过程及预后情况,以期提高儿科医生对此病的认识.方法 回顾性收集2019年6月至2022年6月山东大学附属儿童医院收治的抗Caspr2抗体相关脑炎患儿4例,分析其临床资料的特征并进行随访.结果 男女患儿各2例,年龄为1.3～7.0岁.4例均呈亚急性发病,首发症状表现为发热、惊厥发作4例,表现为自主神经功能障碍2例、头痛1例、精神行为异常1例;主要临床表现为意识障碍3例,言语障碍、运动障碍各2例,睡眠增多、反复头痛各1例.4例患儿头颅MRI检查和脑电图检查结果均存在异常.3例脑脊液Caspr2抗体阳性,4例血清Caspr2抗体阳性(其中1例复发时呈阳性).3例行糖皮质激素联合免疫球蛋白治疗,1例行支持治疗,出院时病情均好转.出院后18～28个月进行随访,1例出现复发,4例改良Rankin评分0～2分.结论 儿童抗Caspr2抗体相关脑炎临床表现不典型,常表现为意识障碍、发热、精神行为异常、睡眠障碍、癫痫、运动障碍、自主神经症状等.患儿对免疫治疗有较好的反应,早期诊断、早期治疗患儿预后良好.
Clinical analysis of 4 cases with anti-contactin-associated protein-like 2 antibody associated encephalitis in children
Objective To analyze the clinical features,diagnosis and treatment process,and prognosis of anti-contactin-associated protein-like 2(Caspr2)antibody associated encephalitis in children,in order to improve pediatricians'understanding of this disease.Methods Four patients diagnosed with Caspr2 antibody-associated encephalitis in the children's Hospital Affiliated to Shandong University from June 2019 to June 2022 were selected.We retrospectively analyzed the clinical data and followed up the patients.Results There were 2 male and 2 female children,aged from 1.3 to 7.0 years old.All 4 cases showed subacute onset,4 cases presenting with fever and convulsions,2 cases showing autonomic nerve dysfunction,1 case with headache,and 1 case with mental behavior abnormalities as the initial symptoms.Three cases had consciousness disorder as the main clinical manifestations,2 cases had speech disorder,2 cases had movement disorder,1 case had somnolence,and 1 case had recurrent headache.Head MRI and EEG examination results of all 4 children showed abnormalities.Caspr2 antibodies in cerebrospinal fluid were positive in 3 cases,and Caspr2 antibodies in serum were positive in 4 cases(of which 1 case was positive during recurrence).Three cases were treated with glucocorticoids combined with immunoglobulin,and 1 case received supportive treatment,all showing improvement at discharge.Follow-up visits were conducted 18 to 28 months after discharge,with 1 case showing recurrence,and the modified Rankin scores of 4 cases were 0 to 2 points.Conclusions The clinical manifestations of Caspr2 antibody associated encephalitis in children are atypical,often presenting as disturbance of consciousness,fever,mental and behavioral abnormalities,sleep disorders,epilepsy,movement disorders,autonomic nervous symptoms,etc.Children with this disease tend to respond well to immunotherapy,and early diagnosis and treatment could lead to good prognosis.
万方数据
维普
