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小儿自身免疫性癫痫性脑病

Pediatric Autoimmune Epileptic Encephalopathies.

作者信息

Wright Sukhvir, Vincent Angela

机构信息

1 Department of Pediatric Neurology, Birmingham Children's Hospital, Birmingham, United Kingdom.

2 Nuffield Department of Clinical Neurosciences, John Radcliffe University Hospital, Oxford, United Kingdom.

出版信息

J Child Neurol. 2017 Mar;32(4):418-428. doi: 10.1177/0883073816685505. Epub 2017 Jan 6.

Abstract

Pediatric autoimmune epileptic encephalopathies are predominantly characterized by the presence of autoantibodies to the surface of neuronal proteins, for example, N-methyl-d-aspartate (NMDA) receptor antibodies, but also include diseases with non-cell surface antibodies (eg, anti-Hu, glutamic-acid decarboxylase antibodies). In some cases with distinct clinical and para-clinical features, an autoimmune epileptic encephalopathy can be diagnosed without the presence of an antibody and will also respond favorably to immunotherapy. In this review, we summarize the common presentations of pediatric autoimmune epileptic encephalopathies, treatments, and outcomes, and report recent findings in the field of epilepsy, encephalopathy, and the immune system.

摘要

儿童自身免疫性癫痫性脑病主要特征为存在针对神经元蛋白表面的自身抗体,例如N-甲基-D-天冬氨酸(NMDA)受体抗体,但也包括具有非细胞表面抗体的疾病(如抗Hu、谷氨酸脱羧酶抗体)。在一些具有独特临床和辅助临床特征的病例中,即使不存在抗体也可诊断自身免疫性癫痫性脑病,且其对免疫治疗也有良好反应。在本综述中,我们总结了儿童自身免疫性癫痫性脑病的常见表现、治疗方法及预后,并报告癫痫、脑病和免疫系统领域的最新研究结果。

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