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儿童自身免疫性脑炎

Autoimmune encephalitis in children.

作者信息

Erol I

机构信息

Department of Pediatrics, Baskent University, Adana, Turkey.

出版信息

Minerva Pediatr. 2013 Jun;65(3):295-305.

PMID:23685381
Abstract

Autoimmune encephalitis is a heterogeneous group of disorders characterized by cognitive and behavioral decline due to an immune reaction against neuronal antigens. There is increasing evidence that autoimmune encephalitis represents a significant subgroup of encephalitis in children, which are defined by the presence of antibodies against important proteins involved in neurotransmission. The distinction between the different causes of autoimmune encephalitis is important for the patient, as there is a marked difference in therapeutic response; specifically, autoimmune encephalitis associated with the classical onconeuronal antibody is unresponsive to treatment, while autoimmune encephalitis in association with antibodies against surface proteins may respond to immunomodulation. Autoimmune encephalitis may be classified into forms with prevalent involvement of the grey matter (polioencephalitis), white matter (leucoencephalitis), or endothelial cells (vasculitis). The subject of this review includes polioencephalitis, which encompasses syndromes in which there is a loss and/or alteration of neuronal function and in which autoantibodies can be detected in the serum or CSF.

摘要

自身免疫性脑炎是一组异质性疾病,其特征是针对神经元抗原的免疫反应导致认知和行为衰退。越来越多的证据表明,自身免疫性脑炎是儿童脑炎的一个重要亚组,其定义为存在针对参与神经传递的重要蛋白质的抗体。区分自身免疫性脑炎的不同病因对患者很重要,因为治疗反应存在显著差异;具体而言,与经典肿瘤神经元抗体相关的自身免疫性脑炎对治疗无反应,而与针对表面蛋白的抗体相关的自身免疫性脑炎可能对免疫调节有反应。自身免疫性脑炎可分为以灰质(脑灰质炎)、白质(白质脑炎)或内皮细胞(血管炎)普遍受累为特征的类型。本综述的主题包括脑灰质炎,它涵盖了存在神经元功能丧失和/或改变且血清或脑脊液中可检测到自身抗体的综合征。

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