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富含亮氨酸的胶质瘤失活蛋白1(LGI-1)介导的边缘叶脑炎伴抗利尿激素分泌异常综合征:一例报告

Leucine-rich glioma-inactivated protein 1 (LGI-1) mediated limbic encephalitis associated with syndrome of inappropriate antidiuretic hormone secretion: a case report.

作者信息

Whiles Emily, Joshi Hareesh, Prachi Prachi, Kavuri Venkaiah, Sagi Satyanarayana V

机构信息

Department of Diabetes and Endocrinology, North West Anglia Trust, Peterborough City Hospital, UK.

Department of Acute Medicine, North West Anglia Trust, Peterborough City Hospital, UK.

出版信息

Oxf Med Case Reports. 2020 Jan 31;2020(1):omz136. doi: 10.1093/omcr/omz136. eCollection 2020 Jan.

Abstract

Autoantibodies to leucine-rich glioma-inactivated protein 1 (LGI-1) are associated with inflammation of the limbic system. Faciobrachial dystonic seizures are pathognomonic for LGI1-antibiodies and their treatment with immunotherapy is effective in seizure control with a potential to prevent cognitive decline. We report a 57-year-old man who presented to the emergency department with recurrent seizures, visual hallucinations and severe memory impairment over a seven-week period; he reported a background of alcohol excess. Initial investigations revealed hyponatremia, indicating syndrome of inappropriate anti-diuretic hormone secretion. Magnetic resonance imaging of the brain revealed bilateral asymmetrical high-T2 and low-T1 signal in the medial temporal lobes. Serum immunofluorescence assay tested positive for LGI-1 antibody. Patient responded to treatment with levetiracetam, intravenous methylprednisolone and five plasma exchange sessions. Patient remains on a maintenance dose of prednisolone and azathioprine. It is imperative that clinicians recognize signs of autoimmune encephalitis in order to curb long-term sequelae and improve clinical outcomes.

摘要

富含亮氨酸的胶质瘤失活蛋白1(LGI-1)自身抗体与边缘系统炎症相关。面臂肌张力障碍性癫痫是LGI1抗体的特征性表现,免疫疗法治疗此类癫痫对控制发作有效,且有可能预防认知功能下降。我们报告一名57岁男性,在7周内反复出现癫痫发作、视幻觉和严重记忆障碍,前来急诊科就诊;他有过量饮酒史。初步检查发现低钠血症,提示抗利尿激素分泌异常综合征。脑部磁共振成像显示双侧内侧颞叶不对称高T2和低T1信号。血清免疫荧光检测LGI-1抗体呈阳性。患者对左乙拉西坦、静脉注射甲泼尼龙及五次血浆置换治疗有反应。患者继续服用泼尼松龙和硫唑嘌呤维持剂量。临床医生必须认识到自身免疫性脑炎的体征,以控制长期后遗症并改善临床结局。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/05ea/6996037/f461b3133593/omz136f1.jpg

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