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小小孩,大问题:抗 N-甲基-D-天冬氨酸受体脑炎!

Little Children, Bigger Problems: Anti-N-Methyl D-Aspartate Receptor Encephalitis!

机构信息

Pediatric Neurology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.

Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore, India.

出版信息

Indian J Pediatr. 2019 Nov;86(11):1048-1050. doi: 10.1007/s12098-019-03000-8. Epub 2019 Jun 13.

Abstract

Anti N-methyl D-aspartate receptor (NMDAR) antibody positive autoimmune encephalitis is a well-described entity both in adults as well as in children. The authors report an 18-mo-old boy who presented with seizures, movement disorder, cognitive impairment, and behavioral disturbances. Anti-NMDAR encephalitis was confirmed by detection of NMDAR antibodies in the cerebrospinal fluid. The patient was administered pulse corticosteroids and intravenous immunoglobulin followed by further immunomodulatory therapy. No tumor was detected on screening. The child remained unresponsive to treatment. The authors wish to highlight the occurrence of anti-NMDAR encephalitis in young toddlers and the poor response to therapy often seen in very young children.

摘要

抗 N-甲基-D-天冬氨酸受体 (NMDAR) 抗体阳性自身免疫性脑炎在成人和儿童中均有明确的描述。作者报告了一例 18 个月大的男孩,他出现了癫痫发作、运动障碍、认知障碍和行为障碍。通过检测脑脊液中的 NMDAR 抗体,确定为抗 NMDAR 脑炎。该患者接受了脉冲皮质类固醇和静脉注射免疫球蛋白治疗,随后进行了进一步的免疫调节治疗。筛查未发现肿瘤。患儿对治疗无反应。作者希望强调在幼儿中发生抗 NMDAR 脑炎的情况,以及在非常年幼的儿童中经常观察到的治疗反应不佳的情况。

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