Pediatric anti-NMDAR encephalitis with demyelination on brain MRI: A single center study

作者全名："Li, Yuhang; Luo, Hanyu; Zheng, Yaxin; Zhou, Lvli; Jiang, Yan; Li, Xiujuan; Ma, Jiannan; Jiang, Li"

作者地址："[Li, Yuhang; Luo, Hanyu; Zheng, Yaxin; Zhou, Lvli; Jiang, Yan; Li, Xiujuan; Ma, Jiannan; Jiang, Li] Chongqing Med Univ, Natl Clin Res Ctr Child Hlth & Disorders, Dept Neurol, Minist Educ,Key Lab Child Dev Disorders,Chongqing, 2 Zhongshan Rd, Chongqing 400013, Peoples R China"

通信作者："Ma, JN; Jiang, L (通讯作者)，Chongqing Med Univ, Natl Clin Res Ctr Child Hlth & Disorders, Dept Neurol, Minist Educ,Key Lab Child Dev Disorders,Chongqing, 2 Zhongshan Rd, Chongqing 400013, Peoples R China."

来源：MULTIPLE SCLEROSIS AND RELATED DISORDERS

ESI学科分类：NEUROSCIENCE & BEHAVIOR

WOS号：WOS:001105592500001

JCR分区：Q2

影响因子：2.9

年份：2023

卷号：80

期号：　

开始页：　

结束页：　

文献类型：Article

关键词：Anti-N-methyl-D-aspartate receptor; encephalitis; Concomitant; Demyelination; Myelin oligodendrocyte glycoprotein; Pediatrics

摘要："Objective: To explore the clinical characteristics, immunotherapy response, and prognosis of pediatric anti-Nmethyl-D-aspartate receptor (NMDAR) encephalitis associated with demyelination on brain magnetic resonance (MRI). Methods: We retrospectively reviewed the medical records of children diagnosed with anti-NMDAR encephalitis in our hospital between January 2016 and December 2021. All children with evidence of demyelination on brain MRI were included. Results: A total of 183 anti-NMDAR encephalitis children were included; 8.7 % (16/183) of them had demyelination on brain MRI. Nine were positive for myelin oligodendrocyte glycoprotein (MOG)-IgG, while two were positive for both MOG-IgG and glial fibrillary acidic protein (GFAP)-IgG. Four patients had a history of acquired demyelinating syndromes and encephalitis, respectively, while nine (56.3 %) had atypical symptoms of antiNMDAR encephalitis. All children had supratentorial demyelination on brain MRI; four of them had additional infratentorial lesions. All children received first-line immunotherapy; four were administered repeated first-line immunotherapy and/or rituximab because of poor initial response. During the follow-up, 37.5 % (6/16) of the children relapsed, but all responded well to immunotherapy. There were no significant differences in mRS score before immunotherapy, response to first-line immunotherapy, and long-term prognosis between antiNMDAR encephalitis children with and without demyelination. However, patients with demyelination were more likely to have a history of acquired demyelinating syndromes or unexplained cortical encephalitis and to relapse. Conclusion: Pediatric anti-NMDAR encephalitis can co-occur with demyelination and has a high rate of MOG-IgG positivity. A history of acquired demyelinating syndromes or unexplained cortical encephalitis and atypical symptoms may indicate demyelination in children with anti-NMDAR encephalitis. Pediatric anti-NMDAR encephalitis with demyelination is more likely to relapse and needs a closer follow-up. However, it remains unknown whether more intensive immunotherapy is required in these patients."

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