文章摘要
陈兴泳,傅瑜萍,陈艳佳,江秀龙.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[J].神经损伤功能重建,2020,15(12):722-724
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 En⁃cephalitis and Review of Literature
  
DOI:
中文关键词: 富亮氨酸胶质瘤失活1蛋白  边缘性脑炎  记忆力障碍  面臂肌张力障碍发作
英文关键词: leucine-rich glioma inactivation protein 1  limbic encephalitis  memory impairment  faciobrachial dystonic seizure
基金项目:国家自然科学基金 (No. 81771250); 福建省科技创新联 合资金项目(No. 2 017Y9065); 福建省自然科学基 金 卫 生 联 合 项 目 (No. 2020J011059, 2018J01255)
作者单位
陈兴泳,傅瑜萍,陈艳佳,江秀龙 福建医科大学省立 临床医学院福建 省立医院神经内科 
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中文摘要:
      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。
英文摘要:
      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.
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