富亮氨酸胶质瘤失活1蛋白抗体阳性边缘系统脑炎一例临床特点
Clinical characteristics of one patient with leucine-rich glioma inactivated-1 antibody positive limbic encephalitis
目的 探讨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、诊断及免疫治疗反应,以期引起临床重视.方法 报道国内首例LGi1抗体阳性边缘系统脑炎患者的诊治经过,结合临床表现、结构及功能影像学、脑电图、免疫治疗反应以及相关文献,分析该疾病的特点.结果 患者为老年男性,表现为亚急性起病的近期记忆功能减退,抗癫痫药物抵抗的频繁、短暂的面-臂肌张力障碍发作,顽固性低钠血症,影像学提示颞叶内侧区受累.血和脑脊液LGi1抗体阳性.经静脉丙种球蛋白治疗,临床痊愈.结论 LGi1抗体阳性边缘系统脑炎有独特的临床表现和较好的免疫治疗效果,及早诊治可明显改善预后.
更多Objective To study the clinical features,diagnosis and immunotherapy response of leucine-rich glioma inactivated-1 (LGi1)-antibody positive limbic encephalitis (LE).Methods A case of anti-LGi1 LE in a 68-year-old male was reported.The related literature was reviewed and clinical features were summarized.Results Anti-LGi1 LE is characterized by subacute development of amnesia,frequent,brief,and drug-resistant faciobrachial dystonic seizures,refractory hyponatremia,mesial temporal lobe abnormalities on magnetic resonance imaging and positron emission tomography,and a good response to intravenous immunoglobulin therapy.Conclusion Early recognition and appropriate immunotherapy for patients with LE associated with anti-LGi1 antibody may prevent irreversible injury to limbic function.
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