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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