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富含亮氨酸胶质瘤失活蛋白 1 抗体相关脑炎患儿 22 月龄 1 例报告

Leucine-rich glioma-inactivated protein 1 antibody-associated encephalitis in a 22-month-old girl: a case report.

机构信息

Department of Pediatrics, Affiliated Hospital of ChiFeng University, ChiFeng, Inner Mongolia Autonomous Region, China.

出版信息

BMC Pediatr. 2023 Aug 8;23(1):389. doi: 10.1186/s12887-023-04191-y.

Abstract

BACKGROUND

LGI-1 antibody-associated encephalitis is a type of autoimmune encephalitis with a lower prevalence than NMDAR antibody-associated encephalitis. LGI-1 antibody-associated encephalitis is the second most prevalent of all autoimmune encephalitides. LGI-1 antibodies interfere with the interactions of inter-synaptic proteins to produce clinical manifestations (N Engl J Med 378:840-851, 2018).

CASE PRESENTATION

Leucine-rich glioma-inactivated protein 1 (LGI-1) antibody-associated encephalitis is a subtype of autoimmune encephalitis with a low incidence. We report a case of a girl aged 22 months with convulsive seizures, psycho-behavioral abnormalities, sleep disorders, and limb tremors. This patient was diagnosed with LGI-1 antibody-associated encephalitis based on electroencephalography (EEG) examinations and autoimmune encephalitis antibody analyses. A combined therapy of anti-epileptic and immunosuppressant drugs was effective in controlling the patient's neurological symptoms.

CONCLUSIONS

The incidence of LGI-1 antibody-associated encephalitis is low and it occurs mostly in middle-aged and elderly patients, although it occasionally occurs in pediatric patients. To the best of our knowledge, this report describes the youngest patient with LGI-1 antibody-associated encephalitis. Following timely diagnosis, administration of anti-epileptic and immunosuppressant therapy was remarkably effective.

摘要

背景

LGI-1 抗体相关脑炎是一种自身免疫性脑炎,其发病率低于 NMDA 受体抗体相关脑炎。LGI-1 抗体相关脑炎是所有自身免疫性脑炎中第二常见的类型。LGI-1 抗体干扰突触间蛋白的相互作用,产生临床表现(N Engl J Med 378:840-851, 2018)。

病例介绍

富亮氨酸胶质瘤失活 1 蛋白(LGI-1)抗体相关脑炎是一种发病率较低的自身免疫性脑炎亚型。我们报告了一例 22 月龄女孩,表现为癫痫发作、精神行为异常、睡眠障碍和肢体震颤。该患者根据脑电图(EEG)检查和自身免疫性脑炎抗体分析诊断为 LGI-1 抗体相关脑炎。抗癫痫和免疫抑制剂药物的联合治疗对控制患者的神经症状有效。

结论

LGI-1 抗体相关脑炎的发病率较低,多发生于中老年人,但也偶见于儿科患者。据我们所知,本报告描述了最年轻的 LGI-1 抗体相关脑炎患者。及时诊断后,给予抗癫痫和免疫抑制剂治疗效果显著。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d78b/10408089/f5b4c321a197/12887_2023_4191_Fig1_HTML.jpg

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