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首页> 美国卫生研究院文献>Oxford Medical Case Reports >Leucine-rich glioma-inactivated protein 1 (LGI-1) mediated limbic encephalitis associated with syndrome of inappropriate antidiuretic hormone secretion: a case report
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Leucine-rich glioma-inactivated protein 1 (LGI-1) mediated limbic encephalitis associated with syndrome of inappropriate antidiuretic hormone secretion: a case report

机译：富含亮氨酸的神经胶质瘤灭活蛋白1（LGI-1）介导的边缘性脑炎与抗利尿激素分泌异常综合征相关：一例报告

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摘要

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.
机译：富含亮氨酸神经胶质瘤灭活蛋白1（LGI-1）的自身抗体与边缘系统的炎症有关。面臂肌张力障碍性癫痫是LGI1抗体的致病原，其免疫疗法治疗可有效控制癫痫发作，并有可能预防认知功能下降。我们报告了一名57岁的男子，他在七周内反复出现癫痫发作，视觉幻觉和严重的记忆障碍，来到急诊科。他报告有过量饮酒的背景。初步调查显示低钠血症，表明抗利尿激素分泌不当综合征。大脑的磁共振成像显示内侧颞叶的双侧不对称高T2和低T1信号。血清免疫荧光法检测LGI-1抗体呈阳性。患者对左乙拉西坦，静脉注射甲基强的松龙和五次血浆置换疗程反应良好。患者保持维持剂量的泼尼松龙和硫唑嘌呤。临床医生必须认识到自身免疫性脑炎的体征，以遏制长期后遗症和改善临床结局。

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