富亮氨酸胶质瘤失活1蛋白抗体阳性边缘系统脑炎7例临床分析

Clinical analysis of 7 patients with leucine-rich glioma inactivated-1 protein antibody positive limbic encephalitis

目的总结富亮氨酸胶质瘤失活1蛋白(LGI1)抗体阳性边缘系统脑炎临床特点及免疫治疗反应,以提高对该病的认识。方法分析2012-03-2014-07在天坛医院神经内科就诊的7例LGI1抗体阳性边缘系统脑炎患者的临床、实验室检查资料,总结不同治疗方案患者的预后。结果 7例患者以精神行为异常、认知功能下降、颞叶癫痫为主要临床表现。均行脑脊液检查,其中4例常规、生化正常,3例表现为蛋白轻度升高。3例患者出现顽固性低钠血症。所有患者肿瘤标志物、影像学筛查均未发现肿瘤,副肿瘤抗体(抗-Hu、-Yo、-Ri、-Ma2)阴性。4例脑电图检查异常,表现为局灶性癫痫样放电或节律减慢。4例MRI检查Flair及T2序列可见颞叶内侧高信号病灶。7例患者经免疫调节治疗预后良好。结论 LGI1抗体阳性边缘系统脑炎以认知功能下降、癫痫发作为主要临床表现,常伴有低钠血症,极少伴有肿瘤;病灶累及颞叶内侧为主,T2或Flair序列为高信号;具有较好的免疫治疗效果,及时的诊断及治疗有助于患者的恢复。

Objective We summarize the clinical features and immunotherapeutic response of leucine- rich glioma inactivated -1 protein （LGI1） antibody positive limbic encephalitis to deepen the understanding of this disease. Methods We analyzed the clinical features of 7 patients who were admitted to Tiantan hospital from March 2012 to July 2014, and observed the prognosis of different treatments. Results Seven patients mainly presented with psychiatric symptoms, memory loss, and epilepsy. Of the 7 patients who underwent cerebrospinal fluid （CSF） examination, 4 were found to have normal CSF tests while 3 had mildly increased protein concentrations. Three of the 7 patients were found to have hyponatremia. Screening for malignant tumors of the chest and abdomen revealed normal findings. Paraneoplastic antibodies （anti-Ha,-Yo, -Ri, -Ma2） were negative in all the 7 patients. Four electroencephalographs showed slow wave activity or epileptic form discharges. Magnetic resonance imaging （MRI） was normal or showed T2WI and flair phase high signal in the medial temporal lobes. All patients treated with immunomodulatory therapy had favorable outcomes. Conclusions Most patients develop the classic syndrome of limbic encephalltis and is distinguishable from other limbic encephalitis because of hyponatremia, a weak association with cancer. T2 or flair MRI scans mainly show high signals in the medial temporal lobes. Anti-LGI1 encephalitis is a kind of treatable condition. And timely diagnosis and treatment may yield a favorable prognosis.