富亮氨酸胶质瘤失活1蛋白A自身抗体相关边缘性脑炎6例临床分析

Leucine-rich glioma inactivated 1 protein antibody associated limbic encephalitis in 6 cases

目的总结富亮氨酸胶质瘤失活1蛋白A(leucine-rich glioma inactivated 1,LGI1)抗体相关边缘性脑炎(limbic encephalytis,LE)的临床特征及辅助检查结果。方法回顾性分析6例LGI1抗体相关LE患者的基本特征、临床表现及实验室和影像学检查结果。结果 6例LE均为亚急性起病,血清及脑脊液LGI1抗体均为阳性;6例患者均有近记忆力减退及精神行为异常,其中4例以近记忆力减退为首发症状;5例有癫痫性发作,2例有不自主运动,1例发生顽固性呃逆;5例患者血清钠<135mmol/L,1例脑脊液生化和常规检查轻度异常;4例行脑电图检查者,轻、重度异常各2例;头颅MRI检查,1例示双侧海马及颞叶内侧异常信号,1例示左侧脑室旁有异常信号。结论 LGI1抗体相关LE临床表现缺乏特异性,近记忆下降和无诱因低钠血症是相对独特的表现。

Objective To summarize the clinical features of leucine-rich glioma inactivated-1 protein （LGI1） antibody associated limbic encephalitis （LE） and the auxiliary examination results. Methods The basic characteristics, clinical features, results of laboratory and imaging examination results were retrospectively analyzed in 6 patients with LGI1 antibody associated LE. Results All these 6 patients with LE presented subacute onset, with positive LGI protein antibody in serum and cerebrospinal fluid. All patients were found recent memory decline and psychological behavior disorders, in which 4 patients initially presented significant recent memory decline. Five patients experienced epileptic seizure, 2 appeared involuntary movements, and 1 presented intractable hiccup. The serum sodium concentration was lower than 135 mmol/L in 5 patients, the cerebrospinal fluid examination was slightly abnormal in 1, and the electroencephalogram was slightly abnormal in 2 and severely abnormal in 2. The cranial MRI found abnormal appearance in bilateral hippocampus and inside temporal lobe in 1, and beside the left cerebral ventricle in 1. Conclusion The clinical manifestations of LGI1 antibody associated LE lacks specificity, recent memory decline and byponatremia with no inducement are its relatively unique manifestation.