自身免疫性脑炎6例临床分析

The clinical characteristics of autoimmune encephalitis 6 cases

目的探讨自身免疫性脑炎的临床特点。方法回顾性分析6例自身免疫性脑炎患者的临床表现、辅助检查及治疗。结果 6例患者中5例有首发症状为精神异常、记忆力减退、言语障碍,1例癫痫大发作为首发症状,主要临床表现有精神行为异常、认知障碍、癫痫发作、不自主运动、意识障碍、低通气障碍、心律失常;4例颅脑MRI有异常信号,6例脑电图均以背景慢波改变为主;6例脑脊液均以淋巴细胞反应为主;4例脑脊液NMDA受体抗体阳性,1例脑脊液LGI1蛋白抗体阳性,1例脑脊液GABAB受体抗体阳性。1例并发肺癌。5例给予人免疫球蛋白0.4g/(kg·d),5 d及甲强龙1 000 mg冲击治疗,1例因病情较轻仅给予地塞米松10 mg/d静滴。4例出院时或出院后电话随访病情好转;2例自动出院中断治疗,电话随访出院1~2个月后死亡。结论以精神异常、认知功能障碍、癫痫、意识障碍、低通气障碍为主要表现,脑脊液以淋巴细胞反应为主,脑MRI可有异常信号,也可正常,须考虑自身免疫性脑炎可能,进一步行自身免疫性脑炎相关抗体检查,尽早给予免疫治疗,以改善预后。

Objective The purpose of this study is to explore the clinical characteristics of autoimmune encephalitis.Methods A retrospective analysis of clinical manifestation,auxiliary examination and treatment was conducted for 6 patients with autoimmune encephalitis. Results 5 out of 6 cases initially suffered from mental disorder,memory loss and speech disorder,and 1 case had the primary symptom of grand mal. The major manifestations were mental disorder,cognitive disorder,epileptic attack,involuntary movement,conscious disturbance,hypoventilation and arrhythmias. There were 4 cases with abnormal signals in MRI,and the change of background slow waves were dominant in 6 cases. Cerebrospinal fluids of all 6 cases mainly had lymphocyte reactions. Additionally,the cerebrospinal fluid of 4 cases were positive in NMDA receptor antibodies,and 1 case with positive LGl1 protein antibody,as well as GABAB receptor antibody was positive in 1 case. Besides,1 case suffered from lung cancer. Moreover,5 cases were treated with human immunoglobulin（ 0. 4 g/（ kg·d） for 5 days and 1000 mg methylprednisolone,while 1 milder case was only dosed with dexamethasone（ 10 mg/d）. The conditions of 4 patients were improved after discharging from the hospital. Two patients voluntarily discontinued therapy were confirmed dead after discharging from hospital 1-2 months by follow-up call. Conclusion Autoimmune encephalitis should be considered when mental disorder,cognitive disorders,epilepsy,conscious disturbance and hypoventilation are as the principal symptoms with lymphocyte reactions in cerebrospinal fluids,as well as normal or abnormal brain MRI. Further examine on autoimmune antibodies should be performed and immunotherapy ought to be applied as early as possible in order to improve the prognosis.