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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 and auxiliary findings of leucine-rich glioma inactivated 1 (LGI1) antibody-associated limbic encephalytis (LE). Methods We retrospectively analyzed the basic characteristics, clinical manifestations and laboratory and imaging findings of 6 patients with LGI1 antibody-associated LE. Results Six cases of LE were all subacute onset, and LGI1 antibody was positive in both serum and cerebrospinal fluid (CSF). Six patients had near memory loss and abnormal mental behavior, of which 4 patients had near memory loss as the first symptom. Five patients had epileptic seizures, 2 cases of involuntary movement, 1 case of intractable hiccups; 5 cases of serum sodium <135mmol / L, 1 case of cerebrospinal fluid biochemistry and routine examination of mild abnormalities; 4 cases of EEG, light and severe abnormalities 2 Cases; head MRI examination, 1 case showed bilateral bilateral hippocampal and temporal lobe abnormal signals, 1 case showed abnormal signals around the left ventricle. Conclusion The clinical manifestations of LG associated with LGI1 antibody are lack of specificity. Decreased memory and uninduced hyponatremia are relatively unique manifestations.