title: Childhood Anti-NMDA Receptor Encephalitis.
abstract: OBJECTIVE
  To study the clinical profile, and outcome of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Unit of Pediatric Neurology and Neurodevelopment, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India.
authors.forename: Renu
authors.initials: R
authors.lastname: Suthar
authors.affiliation: Unit of Pediatric Neurology and Neurodevelopment, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India.
authors.forename: Arushi Gahlot
authors.initials: AG
authors.lastname: Saini
authors.affiliation: Unit of Pediatric Neurology and Neurodevelopment, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India.
authors.forename: Naveen
authors.initials: N
authors.lastname: Sankhyan
authors.affiliation: Unit of Pediatric Neurology and Neurodevelopment, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India.
authors.forename: Jitendra Kumar
authors.initials: JK
authors.lastname: Sahu
authors.affiliation: Unit of Pediatric Neurology and Neurodevelopment, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India. doctorpratibhasinghi@gmail.com.
authors.forename: Pratibha
authors.initials: P
authors.lastname: Singhi
mesh_terms: D060426:Anti-N-Methyl-D-Aspartate Receptor Encephalitis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy; D002648:Child; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008297:Male; D011618:Psychotic Disorders / Q000209:etiology; D016194:Receptors, N-Methyl-D-Aspartate; D012189:Retrospective Studies; D012640:Seizures / Q000209:etiology
chemical_list: D016194:Receptors, N-Methyl-D-Aspartate
keywords: Autoimmune encephalitis; Encephalopathy; Neuroimmunology; Neuropsychiatric