自身免疫性脑炎的临床特点及治疗转归

目的 探讨不同类型自身免疫性脑炎(autoimmune encephalitis,AE)的临床特点及治疗转归.方法 分析北京天坛医院2012-2016年入院治疗的AE患者13例,对其临床表现、脑脊液、脑电图、神经影像学及治疗等相关资料进行回顾性分析.结果 按临床类型分类,2例为抗Hu抗体阳性AE,7例为抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎,4例为富亮氨酸胶质瘤失活基因1(LGI1)蛋白抗体阳性脑炎.所有患者均出现精神症状、近记忆力减退及癫痫发作.10例脑脊液白细胞数增高(其中7例为抗NMDAR脑炎),3例脑脊液蛋白升高,3例IgG寡克隆区带阳性.头颅MRI检查发现8例异常,位于海马、海马旁回及杏仁体,均表现为T2WI和FLAIR高信号,在FLAIR像上更清晰.所有患者均给予原发病治疗、抗癫痫治疗及免疫治疗,12例临床症状得到改善且影像学显示病灶缩小.结论 结合临床表现、实验室及神经影像学检查能有效地提高AE的早期诊断;对诊断明确的患者予以免疫治疗疗效更佳.

Clinical features and therapeutic outcomes of autoimmune encephalitis

Objective To investigate the clinical features and therapeutic outcomes of different types of autoimmune encephalitis (AE).Methods Clinical manifestations,serum and cerebrospinal fluid (CSF) immuno-tests,electro-encephalogram (EEG),MRI,treatments and prognosis were retrospectively reviewed in 13 AE patients,who were admitted to Beijing Tiantan Hospital from 2012 to 2016.Results Among the 13 AE cases,2 cases were with Hu antibodies,7 cases were with N-methyl-D-aspartate receptor (NMDAR) antibodies,and 4 cases were with leucine-rich glioma inactivated 1 (LGI1) antibodies.All cases presented with psychiatric symptoms,short memory loss,and epileptic seizures.Ten cases had elevated white cells (7 out of 10 were with anti-NMDAR AE),3 cases had elevated protein level and 3 cases had positive oligoclonal bands in CSF,respectively.Eight cases were abnormal in MRI,which was indicated by high signal on T2WI and FLAIR in the areas of hippocampus,parahippocampal gyrus and amygdaloid body.All patients received treatments towards primary disease,anti-epileptic and immunotherapy.The symptoms alleviated and lesions lessened on MRI in 12 patients.Conclusion Combination of clinical symptoms,laboratory tests,and neuroimaging examinations,can effectively improve the early diagnosis of AE.Patients with definite diagnosis of AE will benefit from early immune-based treatment.