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抗N-甲基-D-天冬氨酸受体抗体阳性和阴性儿童自身免疫性脑炎的临床及脑电图特征分析

Clinical and EEG characteristics analysis of autoimmune encephalitis in children with positive and negative anti-N-methyl- D-aspartate receptor antibodies.

作者信息

Tong Li-Li, Yang Xiao-Fan, Zhang Shu-Qian, Zhang Dong-Qing, Yang Xiao-Rong, Li Bao-Min, Sheng Gui-Mei

机构信息

Department of Pediatrics, Qilu Hospital of Shandong University, Ji'nan, China.

出版信息

Ann Palliat Med. 2020 Sep;9(5):2575-2585. doi: 10.21037/apm-19-484. Epub 2020 Aug 27.

Abstract

BACKGROUND

Autoimmune encephalitis is complex and varied, but it is a curable disease. However, the diagnosis and treatment of children with Autoimmune encephalitis remains challenging. Therefore, we conducted this study to analyze the clinical features, electroencephalogram (EEG) characteristics, treatment and prognosis of autoimmune encephalitis in children with negative and positive anti-N-methyl-D-aspartate receptor (NMDAR) antibody.

METHODS

From January 2015 to January 2017, 28 child patients with autoimmune encephalitis were hospitalized in the Neural Ward of the Children's Medical Center, Qilu Hospital of Shandong University. Inclusion criteria were based on the diagnostic criteria for autoimmune encephalitis published in Lancet Neurology in 2016. The clinical, EEG and imaging data were summarized. The clinical features, treatment regimen, follow-up and prognosis were also analyzed.

RESULTS

Among these 28 child patients, 10 patients had positive anti-NMDAR antibody, while 18 patients had negative anti-NMDAR antibody. The clinical manifestations, EEG findings and seizures were similar (P>0.05) between these two groups. All 28 child patients were treated with methylprednisolone shock and human immunoglobulin. The response to immunotherapy was similar between these two groups (P>0.05).

CONCLUSIONS

The clinical manifestation of autoimmune encephalitis is complex and varied, but it is a curable disease. Immunotherapy should be considered as soon as possible, with or without autoantibodies. Most of the child patients had a good prognosis, while some of them had the sequelae of epilepsy, mild mental symptoms, and dyskinesia. It is necessary to improve the understanding of autoimmune encephalitis with/without positive antibodies, and make diagnosis and treatment as soon as possible, in order to improve the prognosis.

摘要

背景

自身免疫性脑炎复杂多样,但为可治愈性疾病。然而,儿童自身免疫性脑炎的诊断和治疗仍具有挑战性。因此,我们开展本研究以分析抗N-甲基-D-天冬氨酸受体(NMDAR)抗体阴性和阳性的儿童自身免疫性脑炎的临床特征、脑电图(EEG)特点、治疗及预后。

方法

2015年1月至2017年1月,山东大学齐鲁医院儿童医学中心神经科病房收治28例自身免疫性脑炎患儿。纳入标准基于2016年《柳叶刀·神经病学》发表的自身免疫性脑炎诊断标准。总结临床、EEG及影像学资料。分析临床特征、治疗方案、随访及预后情况。

结果

这28例患儿中,10例抗NMDAR抗体阳性,18例抗NMDAR抗体阴性。两组间临床表现、EEG表现及癫痫发作情况相似(P>0.05)。28例患儿均接受甲泼尼龙冲击及人免疫球蛋白治疗。两组免疫治疗反应相似(P>0.05)。

结论

自身免疫性脑炎临床表现复杂多样,但为可治愈性疾病。无论有无自身抗体,均应尽早考虑免疫治疗。多数患儿预后良好,部分患儿有癫痫、轻度精神症状及运动障碍等后遗症。有必要提高对抗体阳性或阴性自身免疫性脑炎的认识,尽早诊断和治疗,以改善预后。

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