The clinical features, treatment and outcomes of 33 children from Northwestern China with Anti-N-methyl-D-aspartate receptor encephalitis

ABSTRACT

Objectives

We analyzed the clinical features and outcomes of children with anti-N-methyl-D–aspartate receptor (anti-NMDAR) encephalitis in Northwestern China.

Methods

We retrospectively recruited 33 pediatric patients with anti-NMDAR encephalitis in Northwestern China from December 2013 to April 2020. The demographics, clinical features, treatments, and outcomes were reviewed.

Results

33 patients with anti-NMDAR encephalitis were enrolled in this study (a median age of 6.8 years, 20 females and 13 males). The initial symptoms included seizures (42.4%), psychiatric symptoms (39.4%), speech dysfunction (12.1%), and paralysis (6.1%). During the course of the disease, 31 patients (93.9%) presented with psychiatric symptoms, 29 patients (87.9%) presented with speech dysfunction, 25 patients (75.8%) presented with movement disorders and 24 patients (72.7%) presented with sleep disorders followed by seizures, consciousness disturbance, autonomic nervous dysfunction, paralysis, and hypoventilation. 12 patients (36.4%) had abnormal cerebrospinal fluid (CSF) findings, 10 patients (30.3%) exhibited abnormal brain magnetic resonance imaging (MRI) results, and 29 patients (87.9%) showed abnormal Electroencephalography (EEG) findings. None of the patients had tumors. All patients received first-line immunotherapy and 8 patients both received first and second-line immunotherapy. 30 of the 33 patients achieved good outcomes (score on the modified Rankin Scale [mRS] of 0–2), whilst the other 3 patients had poor outcomes (mRS score of 3–6).

Conclusions

Patients with higher CSF anti-NMDAR body titer were more likely to develop sleep disorders, consciousness disturbances and more severe disease states.

KEYWORDS:

• Anti-NMDAR encephalitis
• clinical feature
• treatment
• outcome
• children

Acknowledgments

We would like to thank all of the pediatricians, patients, and families who contributed to this study.

Disclosure statement

No potential conflict of interest was reported by the author(s).

Abbreviations

NMDAR: NMDA receptor

MRI: magnetic resonance imaging

EEG: electroencephalography

mRS: modified Rankin scale

CSF: cerebrospinal fluid

ICU: intensive care unit

Contributions

Xiangjun Dou wrote the first draft of the manuscript. Dongjing Li and Fang Wu performed the data analysis. Zhijing Wang, Mengmeng Niu, You Wu, Ting Deng were responsible for data collection. Dong Wang and Xia Li initially designed and supervised the study. All authors read and approved the final manuscript.

Compliance with ethical standards

The clinical data was extracted after the necessary approvals from the hospital ethics committee (final approval for publication obtained on 26/01/2019). Informed consent was obtained from the guardians of all the participants involved in the study.

Additional information

Funding

This work was supported by the Shaanxi Key Science and Technology Innovation Team Project(CN) [2020SF-006].