title: Paediatric anti-N-methyl-D-aspartate receptor encephalitis: The first Italian multicenter case series.
abstract: BACKGROUND
  Given the rarity of this condition, especially in children, there is a paucity of large reported paediatric case series of anti-N-methyl-d-aspartate receptor encephalitis.
  
  
  METHODS
  To contribute to define the features of this condition, we describe retrospectively a new nationwide case series of 20 children (50% females), referred by 13 Italian centres.
  
  
  RESULTS
  Mean age at onset was 8 years (range 3-17). Prodromal symptoms were reported in 31.6%; onset was with neurological symptoms in 70%, and with behavioural/psychiatric disturbances in 30%. Most patients developed a severe clinical picture (90%), and 41% experienced medical complications; children 12-18 years old seemed to be more severe and symptomatic than younger patients. All children received first-line immune therapy; second-line treatment was administered to 45%. Relapses occurred in 15%. At last follow-up (mean 23.9 months, range 5-82), 85% patients had mRS 0-1; this rate was higher among older patients, and in those receiving first immune therapy within 1 month.
  
  
  CONCLUSIONS
  Our case series confirms a symptomatologic core of paediatric anti-N-methyl-d-aspartate receptor encephalitis, even though displaying some distinctive features that may be explained by a specific genetic background or by the limited number of patients. The growing incidence of this condition, the relative age-dependent variability of its manifestations, the availability of immunotherapy and the possible better outcome with early treatment impose a high index of clinical suspicion be maintained. In the absence of data suggesting other specific etiologies, paediatricians should consider this diagnosis for children presenting with neurological and/or behavioural or psychiatric disturbances, regardless of age and gender.
authors: Paediatric Neurology Unit, Department of Woman's and Child's Health, University Hospital of Padua, Padua, Italy. Electronic address: stefano.sartori@unipd.it.
authors: Stefano
authors: S
authors: Sartori
authors: Paediatric Neurology Unit, Department of Woman's and Child's Health, University Hospital of Padua, Padua, Italy. Electronic address: margherita.nosadini@gmail.com.
authors: Margherita
authors: M
authors: Nosadini
authors: Child Neuropsychiatry Unit, Polytechnic University of the Marche, Ancona, Italy. Electronic address: elisabettacesaronix@gmail.com.
authors: Elisabetta
authors: E
authors: Cesaroni
authors: Paediatric Acute and Emergency Operative Unit and Department, Policlinico Vittorio Emanuele University Hospital, University of Catania, Italy. Electronic address: raffaelefalsaperla@hotmail.com.
authors: Raffaele
authors: R
authors: Falsaperla
authors: Department of Child Neuropsychiatry, Epilepsy Center, C. Poma Hospital, Mantua, Italy. Electronic address: giuseppe.capovilla@aopoma.it.
authors: Giuseppe
authors: G
authors: Capovilla
authors: Department of Child Neuropsychiatry, Epilepsy Center, C. Poma Hospital, Mantua, Italy. Electronic address: francesca.beccaria@aopoma.it.
authors: Francesca
authors: F
authors: Beccaria
authors: Unit of Child Neuropsychiatry, Head-Neck and Neuroscience Department, Giannina Gaslini Institute, Genoa, Italy. Electronic address: margheritamancardi@ospedale-gaslini.ge.it.
authors: Maria Margherita
authors: MM
authors: Mancardi
authors: Child and Adolescent Neuropsychiatry Unit, Department of Paediatrics, ARNAS Civico - Di Cristina, Palermo, Italy. Electronic address: giuseppe.santangelo@ospedalecivicopa.org.
authors: Giuseppe
authors: G
authors: Santangelo
authors: Paediatric Acute and Emergency Operative Unit and Department, Policlinico Vittorio Emanuele University Hospital, University of Catania, Italy. Electronic address: leandra.giunta@gmail.com.
authors: Leandra
authors: L
authors: Giunta
authors: Paediatric Neurophysiology Unit, Department of Paediatrics, University of Padua, Italy. Electronic address: boniver@pediatria.unipd.it.
authors: Clementina
authors: C
authors: Boniver
authors: Child Neuropsychiatry, Department of Life and Reproduction Sciences, University of Verona, Italy. Electronic address: gcantalupo@gmail.com.
authors: Gaetano
authors: G
authors: Cantalupo
authors: Child Neuropsychiatry, Department of Life and Reproduction Sciences, University of Verona, Italy. Electronic address: albertocapp@yahoo.it.
authors: Alberto
authors: A
authors: Cappellari
authors: Institute for Maternal and Child Health - IRCCS "Burlo Garofolo", Trieste, Italy. Electronic address: paola.costa@burlo.trieste.it.
authors: Paola
authors: P
authors: Costa
authors: Child Neuropsychiatry, Department of Life and Reproduction Sciences, University of Verona, Italy. Electronic address: dallabernardina@univr.it.
authors: Bernardo
authors: B
authors: Dalla Bernardina
authors: Unit of Clinical Neurophysiology, Department of Neurological Sciences, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy. Electronic address: robertino.dilena@policlinico.mi.it.
authors: Robertino
authors: R
authors: Dilena
authors: Department of Neurology, S. Raffaele Hospital, Milan, Italy. Electronic address: natalisora.mariagrazia@hsr.it.
authors: Maria Grazia
authors: MG
authors: Natali Sora
authors: Paediatric Neurology Unit, Department of Woman's and Child's Health, University Hospital of Padua, Padua, Italy. Electronic address: mariafederica.pelizza@gmail.com.
authors: Maria Federica
authors: MF
authors: Pelizza
authors: Child Neurology and Psychiatry Dept, Pediatric Hospital, Cagliari, Italy. Electronic address: d.pruna@tiscali.it.
authors: Dario
authors: D
authors: Pruna
authors: Neurology Unit, Bambino Gesù Children's Hospital, Rome, Italy. Electronic address: domenico.serino@opbg.net.
authors: Domenico
authors: D
authors: Serino
authors: Child and Adolescent Neuropsychiatry Unit, Department of Paediatrics, ARNAS Civico - Di Cristina, Palermo, Italy. Electronic address: francesca.vanadia@ospedalecivicopa.org.
authors: Francesca
authors: F
authors: Vanadia
authors: Neurology Unit, Bambino Gesù Children's Hospital, Rome, Italy. Electronic address: federico.vigevano@opbg.net.
authors: Federico
authors: F
authors: Vigevano
authors: Child Neuropsychiatry Unit, Polytechnic University of the Marche, Ancona, Italy. Electronic address: n.zamponi@tin.it.
authors: Nelia
authors: N
authors: Zamponi
authors: Institute for Maternal and Child Health - IRCCS "Burlo Garofolo", Trieste, Italy. Electronic address: caterina.zanus@burlo.trieste.it.
authors: Caterina
authors: C
authors: Zanus
authors: Paediatric Neurology Unit, Department of Woman's and Child's Health, University Hospital of Padua, Padua, Italy. Electronic address: irene.toldo@unipd.it.
authors: Irene
authors: I
authors: Toldo
authors: Paediatric Neurology Unit, Department of Woman's and Child's Health, University Hospital of Padua, Padua, Italy. Electronic address: suppiej@pediatria.unipd.it.
authors: Agnese
authors: A
authors: Suppiej
mesh_terms: D000293:Adolescent; D060426:Anti-N-Methyl-D-Aspartate Receptor Encephalitis / Q000175:diagnosis / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007167:Immunotherapy / Q000379:methods; D007558:Italy; D008297:Male; D012189:Retrospective Studies
keywords: Antibodies; Children; Encephalitis; Italy; NMDAR; Paediatric