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Original Research ARTICLE Provisionally accepted The full-text will be published soon. Notify me

Front. Neurol. | doi: 10.3389/fneur.2019.00906

Pediatric autoimmune encephalitis: case series from two Chinese tertiary pediatric neurology centers

 Jianzhao Zhang1,  Taoyun Ji2,  Qian Chen3, Yanan Jiang2, Huan Cheng2, Ping Zheng2, Wenqiang Ma2, Ting Lei2, Yao Zhang2, Yiwen Jin2,  Cuijie Wei2, Ye Wu2, Xingzhi Chang2, Xinhua Bao2,  Yuehua Zhang2, Hui Xiong2, Xinna Ji3, Shuo Feng3,  Haitao Ren4, Jian Yang3 and  Yuwu Jiang2*
  • 1neurology department, capital Institute of Pediatrics, China, Beijing, China
  • 2Peking University First Hospital, China
  • 3Children's Hospital of Capital Institute of Pediatrics, China
  • 4Peking Union Medical College Hospital (CAMS), China

Background and purpose: We retrospectively analyzed the clinical characteristics of children with autoimmune encephalitis (AE) in two Chinese tertiary pediatric neurology centers. We also compared the anti-NMDAR encephalitis with and without co-positive MOG antibody, as well as the specific autoantibody positive AE and autoantibody-negative but probable AE.
Methods: A retrospective study of children (0–18 years old) with AE in Peking University First Hospital and Children's Hospital Affiliated to Capital Institute of Pediatrics was carried out from May 2012 to January 2017. Demographics, clinical features, laboratory and imaging findings, outcome, and co-positive with MOG antibody were analyzed.
Results: A total of 103 children had AE, 89 (86.4%) had anti-NMDAR encephalitis, 2 (1.9%) had anti-LGI1 encephalitis, 1 (0.9%) had anti-CASPR2 encephalitis, and 11 (10.7%) were diagnosed as autoantibody-negative but probable AE. Among the 89 children with anti-NMDAR encephalitis, 35 were males, and 54 were females. The follow-up time was 1–3 years. A total of 15 cases (15/89, 16.9%) with anti-NMDAR encephalitis had co-positive MOG antibody (serum or cerebrospinal fluid or both). These patients were more likely to experience relapse later in life (P=0.014). We had two cases with anti-LGI1 encephalitis, that is, one with sleep disorder onset, and the other one with seizure onset, both of which recovered after treatment. One case with anti-CASPR2 encephalitis was treated with antiepileptic drug and fully recovered. There were 11 cases diagnosed as autoantibody negative but probable AE who had relatively poorer outcome than those with autoantibody-positive AE (15.2%, 14/89). However, the difference was not significant (P=0.08). Only one 12-year-old girl with NMDAR-antibody AE had ovarian teratoma.
Conclusion: Most subjects with AE in our Chinese cohort had anti-NMDAR AE, which had relatively good prognosis. Children with anti-LGI1 or anti-CASPR2 encephalitis were rare and showed good response on immunotherapy. Co-positive MOG antibody were relatively common in anti-NMDAR encephalitis, which was related to high relapse rate. In our study, the prognosis of autoantibody negative but probable AE seemed worse than that of specific autoantibody positive AE.
Keywords: NMDAR, autoimmune encephalitis, child, prognosis

Keywords: NMDAR, autoimmune encephalitis, prognosis, Child, MOG

Received: 10 Oct 2018; Accepted: 05 Aug 2019.

Copyright: © 2019 Zhang, Ji, Chen, Jiang, Cheng, Zheng, Ma, Lei, Zhang, Jin, Wei, Wu, Chang, Bao, Zhang, Xiong, Ji, Feng, Ren, Yang and Jiang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: MD, PhD. Yuwu Jiang, Peking University First Hospital, Beijing, Beijing Municipality, China, jiangyuwu@bjmu.edu.cn