Clinical Features and Outcomes of Anti-N-Methyl-d-Aspartate Receptor Encephalitis in Infants and Toddlers

Changhong Ren; Weihua Zhang; Xiaotun Ren; Jiuwei Li; Changhong Ding; Xiaohui Wang; Haitao Ren; Fang Fang

doi:10.1016/j.pediatrneurol.2021.02.009

Clinical Features and Outcomes of Anti-N-Methyl-d-Aspartate Receptor Encephalitis in Infants and Toddlers

Pediatr Neurol. 2021 Jun:119:27-33. doi: 10.1016/j.pediatrneurol.2021.02.009. Epub 2021 Mar 12.

Authors

Changhong Ren¹, Weihua Zhang¹, Xiaotun Ren¹, Jiuwei Li¹, Changhong Ding¹, Xiaohui Wang¹, Haitao Ren², Fang Fang³

Affiliations

¹ Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Department of Neurology, Beijing, China.
² Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Department of Neurology, Beijing, China.
³ Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Department of Neurology, Beijing, China. Electronic address: fangfang@bch.com.cn.

PMID: 33838580
DOI: 10.1016/j.pediatrneurol.2021.02.009

Abstract

Objective: We describe the clinical features and outcomes of anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis in infants and toddlers.

Methods: This was a single-center retrospective study. Infants and toddlers who met the diagnostic criteria for anti-NMDAR encephalitis were recruited for the study. Data on clinical features, treatment, and long-term outcomes were collected retrospectively.

Results: A total of 41 patients (age range: six to 34 months; median age: 23 months; female: 19) were enrolled in this study. Nineteen (46%) patients exhibited classical anti-NMDAR encephalitis, whereas 22 (54%) patients exhibited anti-NMDAR encephalitis after viral encephalitis. There was a high presentation of movement disorders (100%), developmental regression (90%), abnormal behaviors (90%). All patients were administered first-line therapy, with only 17% of them being administered second-line immunotherapy. Two patients succumbed to the disease, whereas none of them relapsed. At the long-term follow-up (more than one year), 20 of 35 (57%) exhibited satisfactory outcomes (modified Rankin Scale ≤2). Compared with patients with classical anti-NMDAR encephalitis (n = 18), patients after viral encephalitis (n = 17) were more likely to have worse clinical outcomes. They exhibited a higher modified Rankin Scale/Pediatric Cerebral Performance Category score and more frequent seizures. A predictor of poor outcome was presentation after viral encephalitis (odds ratio 35.7, 95% confidence interval 4.64 to 275.03, P = 0.001).

Conclusion: Anti-NMDAR encephalitis in infants and toddlers clinically presents with movement disorders, developmental regression, and abnormal behaviors. Interestingly, this group had a higher proportion of patients after viral encephalitis, which is regarded as the only risk factor for poor outcomes.

Keywords: Anti-N-methyl-d-aspartate receptor; Encephalitis; Infants; Outcome; Pediatric; Toddlers; Viral encephalitis.

MeSH terms

Anti-N-Methyl-D-Aspartate Receptor Encephalitis / complications
Anti-N-Methyl-D-Aspartate Receptor Encephalitis / physiopathology*
Behavioral Symptoms / etiology
Child, Preschool
Disease Progression*
Encephalitis, Viral / complications
Encephalitis, Viral / physiopathology*
Female
Follow-Up Studies
Humans
Infant
Male
Movement Disorders / etiology
Neurodevelopmental Disorders / etiology
Outcome Assessment, Health Care*
Retrospective Studies
Severity of Illness Index