title: N-methyl-D-aspartate receptor antibody-mediated neurological disease: results of a UK-based surveillance study in children.
abstract: OBJECTIVE
  N-methyl-D-aspartate receptor antibody (NMDAR-Ab) encephalitis is a well-recognised clinico-immunological syndrome that presents with neuropsychiatric symptoms cognitive decline, movement disorder and seizures. This study reports the clinical features, management and neurological outcomes of paediatric NMDAR-Ab-mediated neurological disease in the UK.
  
  
  METHODS
  A prospective surveillance study. Children with NMDAR-Ab-mediated neurological diseases were voluntarily reported to the British Neurological Surveillance Unit (BPNSU) from November 2010 to December 2011. Initial and follow-up questionnaires were sent out to physicians.
  
  
  RESULTS
  Thirty-one children fulfilled the criteria for the study. Eight presented during the study period giving an incidence of 0.85 per million children per year (95% CI 0.64 to 1.06); 23 cases were historical. Behavioural change and neuropsychiatric features were present in 90% of patients, and seizures and movement disorders both in 67%. Typical NMDAR-Ab encephalitis was reported in 24 children and partial phenotype without encephalopathy in seven, including predominantly psychiatric (four) and movement disorder (three). All patients received steroids, 22 (71%) received intravenous immunoglobulin, 9 (29%) received plasma exchange,and 10 (32%) received second-line immunotherapy. Of the 23 patients who were diagnosed early, 18 (78%) made a full recovery compared with only 1 of 8 (13%) of the late diagnosed patients (p=0.002, Fisher's exact test). Seven patients relapsed, with four needing additional second-line immunotherapy.
  
  
  CONCLUSIONS
  Paediatric NMDAR-Ab-mediated neurological disease appears to be similar to adult NMDAR-Ab encephalitis, but some presented with a partial phenotype. Early treatment was associated with a quick and often full recovery.
authors: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK Department of Paediatric Neurology, Birmingham Children's Hospital, Birmingham, UK.
authors: Sukhvir
authors: S
authors: Wright
authors: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.
authors: Yael
authors: Y
authors: Hacohen
authors: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.
authors: Leslie
authors: L
authors: Jacobson
authors: Department of Paediatric Neurology, Birmingham Children's Hospital, Birmingham, UK.
authors: Shakti
authors: S
authors: Agrawal
authors: Department of Paediatric Neurology, Birmingham Children's Hospital, Birmingham, UK.
authors: Rajat
authors: R
authors: Gupta
authors: Department of Paediatric Neurology, Birmingham Children's Hospital, Birmingham, UK.
authors: Sunny
authors: S
authors: Philip
authors: Department of Paediatric Neurology, Birmingham Children's Hospital, Birmingham, UK.
authors: Martin
authors: M
authors: Smith
authors: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK Children's Neurosciences, Evelina Children's Hospital @ Guy's and St Thomas' NHS Foundation Trust, King's Health Partners Academic Health Science Centre, London, UK.
authors: Ming
authors: M
authors: Lim
authors: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.
authors: Evangeline
authors: E
authors: Wassmer
authors: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.
authors: Angela
authors: A
authors: Vincent
mesh_terms: D000293:Adolescent; D001323:Autoantibodies / Q000097:blood*; D020274:Autoimmune Diseases of the Nervous System / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D004660:Encephalitis / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D005260:Female; D006801:Humans; D007167:Immunotherapy; D007223:Infant; D008297:Male; D011446:Prospective Studies; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology*; D011795:Surveys and Questionnaires; D006113:United Kingdom / Q000453:epidemiology
chemical_list: D001323:Autoantibodies; D016194:Receptors, N-Methyl-D-Aspartate
keywords: Autoantibody; Encephalitis; NMDA receptors; Neurology; immunotherapy
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