富亮氨酸胶质瘤失活1蛋白抗体阳性边缘叶脑炎的临床特点并文献复习  

Clinical analysis of positive leueine-rich glioma inactived-1 antibody associated limbic encephalitis and literature review

作　　者：李志方[1] 董丽彬[1] 孙彬彬[1] 樊双义[1] 

机构地区：[1]军事医学科学院附属医院神经内科,北京100071

出　　处：《卒中与神经疾病》2017年第5期444-446,449,共4页Stroke and Nervous Diseases

摘　　要：目的探讨富亮氨酸胶质瘤失活1蛋白(LGI1)抗体阳性边缘叶脑炎的临床特点。方法对本院收治的1例LGI1抗体阳性的边缘叶脑炎患者的诊治过程进行回顾性分析并复习相关文献。结果患者为青年女性,亚急性起病,临床主要表现为记忆力下降、癫痫、闭经、情绪障碍。血和脑脊液LGI1抗体阳性;MRI检查T2及Flair序列可见双侧颞叶内侧、海马高信号病灶;PET-CT示双侧颞叶内侧、海马代谢活性对称性降低。经激素和免疫球蛋白联合治疗,疗效显著。结论 LGI1抗体阳性边缘叶脑炎以记忆力下降、癫痫发作为主要临床表现;病灶主要累及双侧颞叶内侧、海马;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 To explore the clinical features and therapeutic outcomes of positive leueine-rich glioma inactived-1 antibody（ Anti-LGI1） associated 1 imbic encephalitis. Methods One case with anti-LGI1 1 imbic encephalitis was analyzed retrospectively,and the literature was reviewed. Results A young woman was admitted to the hospital due to subacute onset of declination of recent-memory function,epilepsy,mood disorder and amenorrhea. LGI1 antibody both in blood and cerebrospinal fluid was positive. T2 or flair MRI scans revealed abnormal signals in bilateral medial temporal lobes and hippocampus. While PET-CT showed the decreased metabolism of bilateral medial temporal lobes and hippocampus. And glucocorticoid combined intravenous immunoglobulins（ IVIg） showed remarkable outcome. Conclusion Anti-LGI1 1 imbic encephalitis was characterized by memory function impairment,epilepsy,mood disorder with specific LGI1 antibody. Bilateral medial temporal lobes and hippocampus were major affected while immune therapeutic effect was significant. Prompt diagnosis and treatment might be helpful for patients in recovery.

关 键 词：抗富亮氨酸胶质瘤失活1蛋白 边缘叶脑炎 免疫治疗 

分 类 号：R512.3[医药卫生—内科学]