摘要目的 对3例不同类型的抗神经元表面抗原抗体阳性的边缘性脑炎患者的临床特点进行分析及比较. 方法 回顾性收集自2011年11月至2015年2月在天津市环湖医院神经内科收治的3例不同类型的抗神经元表面抗原抗体阳性的边缘性脑炎患者的临床资料,分别为抗N-甲基-D-天门冬氨酸(NMDA)受体脑炎(例1)、富亮氨酸胶质瘤失活因子(LGI1)抗体阳性的边缘性脑炎(例2)和γ-氨基丁酸B(GABAB)受体抗体阳性的边缘性脑炎(例3),分析并比较其在临床表现、辅助检查、治疗及预后等方面的特点. 结果 3例边缘性脑炎均有边缘系统受损相关的认知障碍、精神症状、癫痫发作、运动障碍及头MRI的T2WI或FLAIR出现高信号等表现;但由于不同的自身抗体介导,又有各自不同的特点.例1患者为年轻女性,表现出特异的口-面部异常运动、大量唾液分泌及中枢性低通气,予气管切开及机械通气,腹盆部CT提示伴发卵巢畸胎瘤.例2存在类似面-臂肌张力障碍发作的表现.例3在肾透明细胞癌术后发病,以癫痫大发作为主要表现.无论伴发肿瘤与否,3例均对免疫治疗反应较好,但均遗留不同程度的认知损害. 结论 由不同类型的抗神经元表面抗原抗体介导的边缘性脑炎有各自不同的临床特点,但均对免疫治疗敏感.

abstractsObjective To analyze and compare the clinical features of three patients with limbic encephalitis of different kinds of antibodies against neuronal cell surface.Methods The clinical data of three patients with limbic encephalitis,including one with anti-N-methyl-D-aspartate (NMDA) receptor encephalitis,one with anti-leucine-rich glioma-inactivated 1 (LGI1) antibody and one with antigamma-aminobutyric acid B (GABAB) receptor antibody;their clinical manifestations,outcomes,assistant examination results,therapies and prognoses were listed,analyzed and compared.Results All 3 patients presented cognitive impairment,psychiatric symptoms,seizures,abnormal movements and increased T2-weighted imaging or fluid-attenuated inversion-recovery signal on MR imaging;however,mediated by different antibodies,each patient possessed specific features.The young girl with anti-NMDA receptor encephalitis presented oro-facial dyskinesias,hypersalivation and hypoventilation,and had to get tracheotomy and mechanical ventilation,and ovarian teratoma was found through CT.The patient with anti-LGI1 antibodies manifested like faciobrachial dystonic seizure.The patient with anti-GABAB receptor antibody got the disease after nephrectomy due to renal clear cell carcinoma.With or without tumor,all the three patients responded well to immunotherapy.Conclusion Different kinds of limbic encephalitis with different antibody against neuronal cell surface have different clinical features,whereas,all of them are sensitive to immunotherapy.