儿童抗接触相关蛋白2抗体自身免疫性脑炎1例并文献复习

Anti-Caspr2 antibody-associated encephalitis in a child: a case report and literature review

目的探讨抗接触相关蛋白2(Caspr2)抗体自身免疫性脑炎的临床特点。方法回顾1例Caspr2抗体脑炎患儿的临床资料,并复习相关文献。结果女性患儿,5岁,表现为近期记忆力下降、易动怒、认知功能受损、幻觉、睡眠紊乱等;血清Caspr2抗体阳性,除外其他疾病后诊断Caspr2抗体脑炎。文献检索到资料完整的Caspr2抗体脑炎50例,男43例、女7例,起病年龄最小为本例患儿5岁,另有1例8岁,其余均为成人起病;边缘性脑炎32例(62. 75%),莫旺综合征19例(37. 25%);最常见的临床症状是记忆受损,癫痫发作,精神症状如焦虑、淡漠、易激惹、幻觉或妄想、周围神经高兴奋性、小脑共济失调及自主神经紊乱等;痊愈或好转44例(86.27%),合并肿瘤8例(15.69%),复发8例(15.69%)。结论 Caspr 2抗体脑炎比较少见尤其是儿童罕见,如患者有边缘性脑炎症状,且合并周围神经高兴奋性、共济失调、自主神经症状等需考虑Caspr2抗体脑炎可能。

Objective To explore the clinical features of anti-Caspr2 antibody-associated encephalitis.Method The clinical data of anti-Caspr2 antibody-associated encephalitis in a child were retrospectively reviewed.Results A 5-year-old girl manifested as recent memory loss,irritability,cognitive impairment,hallucination,sleep disorders,and so on.The child had positive serum Caspr2 antibody,and was diagnosed with Caspr2 antibody encephalitis after exclusion of other diseases.The literature search retrieved 50 cases of Caspr2 antibody encephalitis with complete data,including 43 male cases and 7 female cases.The minimum onset age of the disease was 5 years in this case.In addition,1 patient developed the disease at the age of 8,while the rest developed in adulthood.There were 32 cases (62.75%) of marginal encephalitis and 19 cases (37.25%) of morvan’s syndrome.The most common clinical symptoms were impaired memory,epileptic seizures,and psychiatric symptoms such as anxiety,apathy,irritability,hallucinations or delusions,peripheral nerve hyperexcitability (PNH),cerebellar ataxia and autonomic nervous disorders.Forty-four cases (86.27%) recovered or improved,8 cases (15.69%) were complicated with tumors and 8 cases (15.69%) relapsed.Conclusion Caspr2 antibody encephalitis is relatively rare,especially in children.If the patient has marginal encephalitis symptoms,combining with PNH,ataxia and autonomic nerve symptoms,the possibility of Caspr2 antibody encephalitis should be considered.