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January 2020; 7 (1) ArticleOpen Access

Anti-NMDAR encephalitis

A single-center, longitudinal study in China

Xiaolu Xu, Qiang Lu, Yan Huang, Siyuan Fan, Lixin Zhou, View ORCID ProfileJing Yuan, Xunzhe Yang, Haitao Ren, Dawei Sun, Yi Dai, Huadong Zhu, Yinan Jiang, Yicheng Zhu, Bin Peng, Liying Cui, Hongzhi Guan
First published October 16, 2019, DOI: https://doi.org/10.1212/NXI.0000000000000633
Xiaolu Xu
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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Qiang Lu
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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Yan Huang
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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Siyuan Fan
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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Lixin Zhou
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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Jing Yuan
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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  • ORCID record for Jing Yuan
Xunzhe Yang
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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Haitao Ren
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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Dawei Sun
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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Yi Dai
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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Huadong Zhu
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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Yinan Jiang
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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Yicheng Zhu
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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Bin Peng
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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Liying Cui
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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Hongzhi Guan
From the Department of Neurology (X.X., Q.L., Y.H., S.F., L.Z., J.Y., X.Y., H.R., Y.Z., B.P., L.C., H.G.), Department of Gynecology and Obstetrics (D.S., Y.D.), Department of Emergency (H.Z.), and Department of Psychology (Y.J.), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; and Center of Neuroscience (L.C.), Chinese Academy of Medical Sciences.
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Full PDF
Citation
Anti-NMDAR encephalitis
A single-center, longitudinal study in China
Xiaolu Xu, Qiang Lu, Yan Huang, Siyuan Fan, Lixin Zhou, Jing Yuan, Xunzhe Yang, Haitao Ren, Dawei Sun, Yi Dai, Huadong Zhu, Yinan Jiang, Yicheng Zhu, Bin Peng, Liying Cui, Hongzhi Guan
Neurol Neuroimmunol Neuroinflamm Jan 2020, 7 (1) e633; DOI: 10.1212/NXI.0000000000000633

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Abstract

Objective To describe the detailed clinical characteristics, immunotherapy, and long-term outcomes of patients with anti-NMDA receptor (NMDAR) encephalitis in China.

Methods A single-center, prospective study. Patients who met the diagnostic criteria were enrolled from 2011 to 2017 and followed up. The clinical features, treatment, and long-term outcomes were collected prospectively. Factors affecting the long-term prognosis were analyzed.

Results The study included 220 patients. The most common clinical presentations were psychosis (82.7%) and seizures (80.9%). Of the patients, 19.5% had an underlying neoplasm; of which ovarian teratoma was 100% of tumors in females and only one male had lung cancer. Most patients (99.5%) received first-line therapy (glucocorticoids, IV immunoglobulin, or plasmapheresis alone or combined), and only 7.3% received second-line immunotherapy (rituximab, cyclophosphamide alone, or combined). Long-term immunotherapy (mycophenolate mofetil or azathioprine >1 year) was administered to 53.2% of patients. During the first 12 months, 207 (94.1%) patients experienced improvement, and 5 (2.3%) died, whereas 38 (17.3%) experienced relapses. At 12-month follow-up, 92.7% had favorable clinical outcomes (modified Rankin Scale score ≤2).

Conclusions Patients in China present with psychosis and seizure frequently but have a low percentage of underlying neoplasms. Re-enforced first-line immunotherapy is effective in managing anti-NMDAR encephalitis in the acute phase. Although relapse is relatively common, with combined first-line and long-term immunotherapy, most patients reached favorable outcomes.

Glossary

AE=
autoimmune encephalitis;
AQP4=
aquaporin-4;
AZA=
azathioprine;
CTX=
cyclophosphamide;
HSV=
herpes simplex virus;
ICU=
intensive care unit;
IQR=
interquartile range;
IVIG=
IV immunoglobulin;
MMF=
mycophenolate mofetil;
MOG=
myelin oligodendrocyte glycoprotein;
mRS=
modified Rankin Scale;
MTX=
methotrexate;
NMDAR=
NMDA receptor;
PE=
plasmapheresis;
PUMCH=
Peking Union Medical College Hospital;
RTX=
rituximab

Footnotes

  • Go to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.

  • ↵* These authors contributed equally to the manuscript.

  • The Article Processing Charge was funded by National Key Research and Development Program of China (Grant no. 2016YFC0901500); Center for Rare Diseases Research, Chinese Academy of Medical Sciences, Beijing, China (Grant no. 2016ZX310174-4); and Beijing Municipal Science and Technology Foundation (Grant no. Z161100000516094).

  • Received April 21, 2019.
  • Accepted in final form September 12, 2019.
  • Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

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