合并多种抗体阳性自身免疫性脑炎临床分析

Clinical analysis of autoimmune encephalitis with multiple anti-neuronal antibodies

目的总结自身免疫性脑炎合并多种抗神经元抗体阳性患者的临床特点及意义。方法回顾性分析郑州大学第一附属医院2015-01-2019-05确诊的7例合并多种抗神经元抗体阳性自身免疫性脑炎患者的临床表现、实验影像学检查及治疗效果,并进行相关文献复习。结果255例自身免疫性脑炎患者仅出现7例合并多抗体阳性患者,其中4例抗NMDAR抗体阳性患者分别合并抗LGI1抗体、抗-Ma2、抗-Yo抗体阳性,2例抗GABABR脑炎分别合并抗Hu、抗amphiphsin阳性,1例抗LGI1脑炎合并抗Hu阳性。6例免疫治疗有效好转,1例对症治疗后好转,其中2例患者病情严重并于治疗后半年死亡,余5例均明显好转。结论多种抗神经元抗体阳性的自身免疫性脑炎并不多见,临床表现更复杂多样,极易引起误诊或漏诊。潜在肿瘤风险更大,合并的肿瘤类型可能更广泛,加重病情、增加复发率及病死率,需引起高度重视,全面的实验室检查及定期复查是必要的。

Objective To summarize the clinical characteristics and significance of autoimmune encephalitis with multiple antibody positive patients.Methods The clinical manifestations,experimental imaging examination and therapeutic effects of 7 patients with multiple antibody positive AE diagnosed in the first affiliated Hospital of Zhengzhou University from January 2015 to May 2019 were analyzed retrospectively,and the related literatures were reviewed.Results Only 7 patients with multiple antibodies were found in 255 patients with AE.Among them,4 patients with positive anti-NMDAR antibody were accompanied with positive anti-LGI1 antibody,anti-Ma2 and anti-Yo antibody,2 patients of anti-GABABR encephalitis with positive anti-Hu and anti-amphiphsin,and 1 patient of anti-LGI1 encephalitis with positive anti-Hu.6 cases were effectively improved by immunotherapy and 1 case was improved after symptomatic treatment.Among them,2 cases were in serious condition and died half a year after treatment,and the other 5 cases were significantly improved.Conclusion Multiple antibody positive AE is rare,and the clinical manifestations are more complex and diverse,which is easy to cause misdiagnosis or missed diagnosis.The potential risk of tumor is higher and the type of tumor is more extensive,which may aggravate the condition,increase the recurrence rate and mortality,and need to be paid more attention to.Comprehensive laboratory examination and regular review are of great significance.