8例自身免疫性脑炎的病例分析并文献复习

Autoimmune encephalitis associated with anti-neuronal antibodies: case analysis and literature review

目的探讨自身免疫性脑炎的临床表现、磁共振(MRI)图像、脑脊液特点及治疗转归。方法回顾性分析我院8例(自2012年1月-2016年1月)自身免疫性脑炎患者的临床资料。结果 8例患者平均发病年龄为44岁,其中有4例抗NMDA受体脑炎,3例LGI1蛋白抗体阳性边缘系统脑炎,1例抗GABABR脑炎,所有患者(100%)均有认知功能损害和精神症状;5例(62%)出现癫痫发作;3例(37%)出现不自主运动和自主神经功能障碍。4例(50%)患者脑电图存在异常;4例(50%)患者有磁共振检查异常信号;1例发现肺癌。结论以认知功能损害、精神行为异常、癫痫发作为主要表现的患者,要警惕自身免疫性脑炎的可能,以免误漏诊。

Objective To explore the characteristics of clinical presentations ,magnetic resonance imaging （MRI）, cerebrospinal fluid （CSF） and treatment outcome of autoimmune encephalitis （LE）.Methods The clinical data of 8 pa-tients with AE were retrospectively analyzed .Results Among these patients ,4 of them were proved to have anti-N-methyl-D-aspartate receptor （ NMDAR） encephalitis ,while 3 of them got diagnosis of anti-leucine-rich glioma-inactivated protein 1 antibody（LGI1） encephalitis,and the other 1 had anti-γ-aminobutyric acid B receptor （GABABR） antibody encephalitis. All the patients presented cognitive and memory dysfunction and psychological symptoms .About 62%of the patients had a seizure,about 37%of the patients had involuntary movement and autonomic nerve dysfunction .About 50%of the patients showed abnormal EEG .About 50%patients had abnormal MRI signal .Lung mass was detected in 1 patient.Conclusion Testing for autoimmune disease related antibodies in blood and cerebrospinal fluid is necessary for patients with cognitive impairment ,mental and behavior disorder and new onset epilepsy .AE should be considered in order to avoid misdiagnosis .