富亮氨酸胶质瘤失活1蛋白抗体相关自身免疫性脑炎的临床特点

Leucine-rich glioma inactivated-1 protein antibody associated autoimmune encephalitis

目的:探讨富亮氨酸胶质瘤失活1蛋白(LGI1)抗体相关自身免疫性脑炎的临床特点、治疗及转归。方法:报道1例LGI1抗体相关自身免疫性脑炎的临床特点及治疗反应,结合文献分析该病的临床特点。结果:患者为中年女性,亚急性起病的近期记忆功能减退、癫痫、情绪和睡眠障碍;检验发现低钠血症;血和脑脊液LGI1抗体阳性;MRI示双侧海马、岛叶及基底节异常信号;MRS示双侧海马区神经元丢失;PET-CT示双侧尾状核、壳核、杏仁核及海马区代谢增高。经静脉类固醇激素治疗达到临床痊愈。结论:自身抗体相关免疫性脑炎有特征性临床表现、特异性抗体,在单一的激素冲击治疗后明显好转,后期随访中仍有进一步好转至基本痊愈。

Objective： To explore the clinical features and therapeutic outcomes of leucine-rich glioma inactivated-1（ LGIl） protein antibody associated autoimmune encephalitis（ Anti-LGI1 AE）. Methods： Report the clinical characteristics and therapeutic response of one case with anti-LGI1 AE and review of literatures. Results： A middle-aged woman was admitted to the hospital due to subacute onset of declination of recent-memory function,epilepsy as well as mood and sleep disorder. Hyponatremia was noted. Blood and cerebrospinal fluid to the LGIl antibody was positive. MRI studies revealed abnormal signals at bilateral hippocampus,insular and its basal ganglia; MRS indicated the loss of neurons in the bilateral hippocampus; PET-CT showed the increasing metabolism of bilateral caudate nucleus,putamen nucleus,amygdala and hippocampus. Intravenous steroid therapy achieved clinical cure. Conclusion： Anti-LGI1 AE has its characteristic clinical manifestations and specific antibody. Steroid hormone therapy alone can obviously relieve clinical symptoms. In the later follow-up,the patient has further improvement and recovery.