Childhood Anti-NMDA Receptor Encephalitis
- 1k Downloads
To study the clinical profile, and outcome of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.
This is a retrospective case series of children <12 y of age, diagnosed with anti-NMDAR encephalitis at a tertiary care institute during the period, May 2013 through June 2015.
Twenty patients were tested for suspected anti-NMDAR encephalitis over this 2 y period. Of these, six children were positive for anti-NMDAR antibodies. Four of these six children had completed treatment and two are currently receiving immunotherapy. Behavioral changes, psychosis, seizures and oro-lingual-facial dyskinesia were the presenting features. Extreme irritability, insomnia and mutism were noted in all the children. The symptoms were persistent, and the course was progressive over 4–8 wk duration. Neuroimaging and electroencephalography were non-specific. Intravenous pulse methylprednisolone and immunoglobulins were used as first-line therapeutic agents. Only one patient responded to first line immunotherapy; five out of six children required second-line immunotherapy. One patient recovered following rituximab, and two patients showed a good response to cyclophosphamide pulse therapy; two patients are currently under treatment with second line immunotherapeutic agents. Tumor screen was negative in all children.
Anti-NMDAR encephalitis is rare but a potentially treatable condition. Timely recognition is essential because treatment is entirely different from other viral encephalitis. Aggressive immunotherapy is the key to a favourable outcome.
KeywordsAutoimmune encephalitis Encephalopathy Neuropsychiatric Neuroimmunology
The authors wish to thank Dr. Josep Dalmau, (ICREA Senior Investigator, Institutd’InvestigacionsBiomèdiques August Pi iSunyer (IDIBAPS), Hospital Clinic, University of Barcelona, Spain) for his support in testing the samples.
RS, AGS: Patient management, draft of manuscript and review of literature; JKS: Patient management, critical review of manuscript for important intellectual content and final approval of the version to be published; NS: Patient management, critical review of manuscript for important intellectual content and final approval of the version to be published; PS: Clinician-in-charge, concept and design of the study, critical review of manuscript for important intellectual content and final approval of the version to be published. She will act as guarantor for the paper.
Compliance with Ethical Standards
Conflict of Interest
Source of Funding
- 4.Gable MS, Sheriff H, Dalmau J, Tilley DH, Glaser CA. The frequency of autoimmune N-methyl-D-aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the California encephalitis project. Clin Infect Dis. 2012;54:899–904.CrossRefPubMedPubMedCentralGoogle Scholar
- 12.Armangue T, Titulaer MJ, Málaga I, et al. Pediatric anti-N-methyl-D-aspartate receptor encephalitis-clinical analysis and novel findings in a series of 20 patients. J Pediatr 2013;162:850–6. e2.Google Scholar
- 29.Kashyape P, Taylor E, Ng J, Krishnakumar D, Kirkham F, Whitney A. Successful treatment of two pediatric cases of anti-NMDA receptor encephalitis with cyclophosphamide: the need for early aggressive immunotherapy in tumour negative pediatric patients. Eur J Paediatr Neurol. 2012;16:74–8.CrossRefPubMedGoogle Scholar