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病例报告:自身免疫性脑炎患者中抗LGI1抗体与抗mGluR2抗体共存

Case Report: Coexistence of anti-LGI1 and anti-mGluR2 antibodies in an autoimmune encephalitis patient.

作者信息

Zhang Xudong, Ma Fei, Geng Qingqing, Luo Changjiang, Qu Chuanqiang

机构信息

Department of Neurology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, China.

Department of Neurology, Jinan Shizhong District People's Hospital, Jinan, China.

出版信息

Front Immunol. 2025 May 30;16:1609389. doi: 10.3389/fimmu.2025.1609389. eCollection 2025.

Abstract

Autoimmune encephalitis (AE) encompasses a broad group of inflammatory encephalopathies mediated by immune responses against central nervous system (CNS) antigens. With the expanding spectrum of identified anti-neuronal antibodies and their increasing clinical recognition, the number of confirmed AE cases has risen. Notably, cases involving concurrent positivity for multiple anti-neuronal antibodies have emerged, complicating both diagnosis and treatment. To date, no published reports have described the co-occurrence of anti-leucine-rich glioma-inactivated 1 (LGI1) antibody and anti-metabotropic glutamate receptor 2 (mGluR2) antibody in AE patients. We report a case of a 61-year-old woman presenting with impaired responsiveness, gait disturbance, and language disorders. Serological and cerebrospinal fluid (CSF) analyses revealed positivity for both LGI1 and mGluR2 antibodies. The anti-LGI1 antibody titers were 1:32+ (serum) and 1:1+ (CSF), while anti-mGluR2 antibody titers were 1:100+ (serum) and 1:10+ (CSF). Based on clinical manifestations and diagnostic findings, the patient was diagnosed with AE with concurrent anti-LGI1 and anti-mGluR2 antibody positivity. The patient received intravenous immunoglobulin (IVIG) and methylprednisolone pulse therapy (500 mg/day), resulting in symptomatic improvement. Following discharge, maintenance therapy with oral prednisone acetate and mycophenolate mofetil was initiated. At the one-week follow-up, her condition remained stable; however, she succumbed to death at the two-week follow-up due to complications from poor oral intake.

摘要

自身免疫性脑炎(AE)涵盖了一大类由针对中枢神经系统(CNS)抗原的免疫反应介导的炎症性脑病。随着已鉴定的抗神经元抗体谱的扩大及其临床认知度的提高,确诊的AE病例数量有所增加。值得注意的是,出现了多种抗神经元抗体同时呈阳性的病例,这使得诊断和治疗都变得复杂。迄今为止,尚无已发表的报告描述AE患者中抗富含亮氨酸胶质瘤失活1(LGI1)抗体和抗代谢型谷氨酸受体2(mGluR2)抗体同时出现的情况。我们报告一例61岁女性患者,表现为反应迟钝、步态障碍和语言障碍。血清学和脑脊液(CSF)分析显示LGI1和mGluR2抗体均呈阳性。抗LGI1抗体滴度血清为1:32+,脑脊液为1:1+;而抗mGluR2抗体滴度血清为1:100+,脑脊液为1:10+。根据临床表现和诊断结果,该患者被诊断为同时存在抗LGI1和抗mGluR2抗体阳性的AE。患者接受了静脉注射免疫球蛋白(IVIG)和甲泼尼龙冲击治疗(500毫克/天),症状有所改善。出院后,开始使用口服醋酸泼尼松和霉酚酸酯进行维持治疗。在一周的随访中,她的病情保持稳定;然而,在两周的随访中,她因口服摄入不良引发的并发症死亡。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ff6/12162904/ba4028b87bd9/fimmu-16-1609389-g001.jpg

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