title: Anti-N-methyl-D-aspartate receptor encephalitis: a newly recognized inflammatory brain disease in children.
abstract: OBJECTIVE
  Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a newly recognized antineuronal antibody-mediated inflammatory brain disease that causes severe psychiatric and neurologic deficits in previously healthy children. The present study was undertaken to describe characteristic clinical features and outcomes in children diagnosed as having anti-NMDAR encephalitis.
  
  
  METHODS
  Consecutive children presenting over a 12-month period with newly acquired psychiatric and/or neurologic deficits consistent with anti-NMDAR encephalitis and evidence of central nervous system (CNS) inflammation were screened. Children were included in the study if they had confirmatory evidence of anti-NMDAR antibodies in the serum and/or cerebrospinal fluid. Features at clinical presentation and results of investigations were recorded. Type and duration of treatment and outcomes at last followup were documented.
  
  
  RESULTS
  Seven children were screened, and 3 children with anti-NMDAR encephalitis were identified. All patients presented with neurologic and/or psychiatric abnormalities, seizures, speech disorder, sleep disturbance, and fluctuating level of consciousness. The 2 older patients had more prominent psychiatric features, while the younger child had significant autonomic instability and prominent involuntary movement disorder. None had an underlying tumor. Immunosuppressive therapy resulted in near or complete recovery; however, 2 of the patients had early relapse necessitating re-treatment.
  
  
  CONCLUSIONS
  Anti-NMDAR encephalitis is an important cause of neuropsychiatric deficits in children, which must be included in the differential diagnosis of CNS vasculitis and other inflammatory brain diseases. Early diagnosis and treatment are essential for neurologic recovery.
authors.affiliation: University of Toronto and Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Nadia
authors.initials: N
authors.lastname: Luca
authors.forename: Tassalapa
authors.initials: T
authors.lastname: Daengsuwan
authors.forename: Josep
authors.initials: J
authors.lastname: Dalmau
authors.forename: Kevin
authors.initials: K
authors.lastname: Jones
authors.forename: Gabrielle
authors.initials: G
authors.lastname: deVeber
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Kobayashi
authors.forename: Ronald M
authors.initials: RM
authors.lastname: Laxer
authors.forename: Susanne M
authors.initials: SM
authors.lastname: Benseler
mesh_terms: D000293:Adolescent; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D001921:Brain / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D004660:Encephalitis / Q000097:blood / Q000175:diagnosis* / Q000276:immunology*; D005260:Female; D006801:Humans; D008297:Male; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D016194:Receptors, N-Methyl-D-Aspartate