Retrospective Study
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jun 6, 2022; 10(16): 5196-5207
Published online Jun 6, 2022. doi: 10.12998/wjcc.v10.i16.5196
Ovarian teratoma related anti-N-methyl-D-aspartate receptor encephalitis: A case series and review of the literature
Shan-Ji Li, Min-Hua Yu, Jie Cheng, Wen-Xin Bai, Wen Di
Shan-Ji Li, Min-Hua Yu, Department of Obstetrics and Gynecology, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China
Jie Cheng, Center for Reproductive Medicine, Shanghai Jiao Tong University, Shanghai 200135, China
Wen-Xin Bai, Department of Medicine, Shanghai Jiao Tong University, Shanghai 200025, China
Wen Di, Department of Obstetrics and Gynecology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China
Wen Di, Shanghai Key Laboratory of Gynecologic Oncology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China
Wen Di, State Key Laboratory of Oncogenes and Related Genes, Shanghai Cancer Institute, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, 200127, China
Author contributions: Li SJ and Yu MH contributed equally to this work; all authors contributed to the design and conduct of study, and approved the submission of this work for publication.
Supported by the Shanghai Municipal Commission of Health and Family Planning, No. 2017ZZ02016; the Funding from National Key Research and Development Program of China, No. 2021YFC2700400; the National Natural Science Foundation of China, No. 81974454; and the Shanghai Municipal Key Clinical Specialty, the Clinical Research Plan of SHDC, No. SHDC2020CR6009-002.
Institutional review board statement: The study was approved by the Ethic Committee of Shanghai Jiao Tong University.
Informed consent statement: Informed consent was obtained from all patients for participation in this study and the publication of results.
Conflict-of-interest statement: None of the authors have any conflict of interest to report.
Data sharing statement: Not available.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Wen Di, MD, PhD, Professor, Department of Obstetrics and Gynecology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, No. 160 Pujian Road, Pudong New Area, Shanghai 200127, China. diwen163@163.com
Received: July 12, 2021
Peer-review started: July 12, 2021
First decision: July 26, 2021
Revised: August 15, 2021
Accepted: April 9, 2022
Article in press: April 9, 2022
Published online: June 6, 2022
Processing time: 324 Days and 23.6 Hours
Abstract
BACKGROUND

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a rare but important complication of ovarian teratoma. Between July 2012 and December 2019, six patients with ovarian teratoma-associated anti-NMDAR encephalitis were enrolled in our hospital and their clinical characteristics, treatment, and follow-up were reviewed. We also conducted a systematic literature review of ovarian teratoma related anti-NMDAR encephalitis reports between January 2014 and December 2019.

AIM

To better understand anti-NMDAR encephalitis through literature review and patients enrolled in our hospital.

METHODS

The six patients enrolled in the study were those diagnosed with anti-NMDAR encephalitis. Their history, clinical manifestations, and medications were recorded and optimum treatment provided in addition to maintaining a record of the follow-ups. In addition, we also extensively surveyed the literature and provide summarized data from 155 published cases of anti-NMDAR encephalitis from 130 case reports. PubMed and Scopus were the sources of these publications and the time period covered was 6 years ranging from January 2014 through December 2019.

RESULTS

The six patients enrolled for this study presented with typical symptoms resulting in a diagnosis of ovarian teratoma induced anti-NMDAR encephalitis. Appropriate interventions led to a positive outcome in all the patients, with five of six patients reporting full recovery and the sixth patient recovering with a few deficits. No death was recorded. The literature survey comprising of 155 patients cases across 130 case reports of anti-NMDAR encephalitis clearly indicated an upward trend in the reports/diagnosis in China, particularly in the surveyed time from 2014 through 2019. The majority of patients (150/155) underwent surgical intervention resulting in positive outcome. No treatment intervention was mentioned for one case while the four patients who were not surgically operated succumbed to the disease.

CONCLUSION

Suspected anti-NMDAR encephalitis should be quickly evaluated for anti-NMDAR antibodies since early diagnosis is important. In case of a tumor, its earliest and complete removal is recommended. Finally, early use of corticosteroids and IgG-depleting strategies (intravenous immunoglobulin or plasma exchange) may improve outcome.

Keywords: Ovarian teratoma; Anti-N-methyl-D-aspartate receptor encephalitis; Immunotherapy; Surgery

Core Tip: We describe our findings from six cases from our own hospital and, in addition, we review 155 cases reported in 130 case reports. We describe the symptoms, diagnosis, and treatment, as well as prognosis. Our results indicate the importance of an early and quick intervention through surgery and immunotherapy, failing which the condition can be fatal.