富亮氨酸胶质瘤失活1蛋白抗体阳性边缘性脑炎临床分析及文献复习

Clinical Analysis of Leucine-Rich Glioma-Inactivated Protein 1 Antibody-Positive Limbic Encephalitis and Review of Literature

目的:总结富亮氨酸胶质瘤失活1蛋白(LGI1)抗体阳性边缘性脑炎的临床和影像特点及诊疗预后。方法:报道我院1例LGI1抗体阳性相关边缘性脑炎并文献复习。结果:患者女性,60岁,表现为渐进性加重的记忆力减退、癫痫发作(全身强直阵挛发作,面-臂肌张力障碍发作)、低钠血症和轻度精神行为异常。颅脑MRI-T2/Flair序列提示双侧颞叶(左侧为甚)内侧、海马异常高信号。脑脊液抗LGI1抗体阳性(++)。经激素治疗症状有所改善。检索既往报道LGI1抗体阳性边缘性脑炎患者237例,多数呈急性、亚急性起病,最常见是记忆障碍、癫痫(含面-臂肌张力障碍发作)和低钠血症,头颅MRI(特别是MRI-T2/Flair序列)显示单侧或者双侧海马区、颞叶异常多见,早期免疫治疗预后良好。结论:LGI1抗体阳性相关边缘性脑炎有其特有的临床特点,免疫治疗可明显改善患者预后。

Objective:To summarize the clinical and imaging features,treatment,and prognosis of leucine-rich glioma-inactivated protein 1(LGI1)antibody-positive limbic encephalitis.Methods:In this paper,1 case of LGI1 antibody-positive limbic encephalitis treated in our hospital was reported,and the relevant literature was reviewed.Results:The patient was a 60-year-old female who presented with progressive memory loss,seizures(including generalized tonic-clonic seizure and faciobrachial dystonic seizure),hyponatremia,and mild mental and behavioral abnormalities.The brain MRI-T2/Flair sequence showed abnormally high signal intensity in the medial temporal lobe and hippocampus on both sides of the temporal lobe(especially the left).The cerebrospinal fluid was positive for anti-LGI1 antibody(++).After corticosteroids treatment,the patient’s symptoms improved.We collected data of 237 cases of LGI1 antibody-positive limbic encephalitis from PubMed.Most patients showed acute and sub-acute onset,and the most common symptoms were memory impairment,epilepsy(including facial-brachial dystonia),and hyponatremia.Cerebral MRI(especially the MRI-T2/FLAIR sequence)in most patients showed abnormalities in the unilateral or bilateral hippocampal and temporal lobes.Prognosis was improved when immunotherapy was initiated at an early stage.Conclusion:LGI1 antibody-positive limbic encephalitis has unique clinical characteristics,and immunotherapy can significantly improve prognosis.