罕见病 疑难病

以失语为主要表现的抗N- 甲基-D- 天冬氨酸抗体受体脑炎2 例报告及文献复习

  • WANG Susu ,
  • YI Mingji ,
  • ZHANG Ying
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  • 青岛大学附属医院神经内分泌儿科(山东青岛 266000)

网络出版日期: 2021-07-01

Anti-N-methyl-D-aspartate antibody receptor encephalitis with aphasia: report of two cases and literature review

  • 王素素,衣明纪,张颖
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  • Department of Neuroendocrinology, Affiliated Hospital of Qingdao University, Qingdao 266000 , Shandong, China

Online published: 2021-07-01

摘要

目的 探讨以失语为主要表现的抗 N- 甲基 -D- 天冬氨酸抗体受体(抗 NMDAR)脑炎的临床特点。方法 回顾分析2例以失语为主要表现的抗NMDAR脑炎患儿的临床资料,并进行文献复习。结果 男女患儿各1例,分别为 3、5岁,首发症状均为失语,伴精神行为异常、癫痫等。患儿血及脑脊液抗NMDA抗体均阳性。颅脑磁共振成像检查均未 见明显异常。男性患儿脑电图为双侧额极、额、前颞、中线区棘慢波发放;女性患儿未见明显异常。2例患儿均给予免疫 球蛋白及甲基泼尼松龙冲击治疗,效果好。结论 抗NMDAR脑炎表现形式多样,儿童出现不明原因失语者,需警惕抗 NMDAR脑炎的可能。

本文引用格式

WANG Susu , YI Mingji , ZHANG Ying . 以失语为主要表现的抗N- 甲基-D- 天冬氨酸抗体受体脑炎2 例报告及文献复习[J]. 临床儿科杂志, 2021 , 39(7) : 531 . DOI: 10.3969/j.issn.1000-3606.2021.07.012

Abstract

To explore the clinical characteristics of anti-N-methyl-D-aspartate antibody receptor (antiNMDAR) encephalitis with aphasia as the main symptom. Methods The clinical data of anti-NMDAR encephalitis with aphasia as the main manifestation in 2 children were retrospectively analyzed, and the literature was reviewed. Results In both children, one boy aged 3 years and one girl aged 5 years, the first symptom was aphasia, accompanied by abnormal mental behavior and epilepsy. Blood and cerebrospinal fluid anti-NMDA antibodies were positive in both children. No obvious abnormality was found in both by their brain magnetic resonance imaging. The electroencephalogram of the boy showed spinous and slow waves in bilateral frontal pole, frontal, anterior temporal and midline areas while no obvious abnormality was found in the electroencephalogram of the girl. Both children were treated with immunoglobulin and methylprednisolone, and a nice therapeutic effect was achieved. Conclusion The manifestations of anti-NMDAR encephalitis are various. In children with unexplained aphasia clinicians should be alert to the possibility of anti-NMDAR encephalitis.
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