title: Paediatric brainstem encephalitis associated with glial and neuronal autoantibodies.
abstract: Central nervous system (CNS) autoantibodies have been reported in a range of neuroimmune diseases, but there has not been a systematic evaluation of autoantibodies in paediatric patients with brainstem encephalitis.
  
  
  
  Serum samples from 57 children (40 male, 17 female, median age 12y, range 0.6-18y) with a diagnosis of brainstem encephalitis were tested retrospectively for antibodies to GQ1b, aquaporin-4 (AQP4), myelin oligodendrocyte glycoprotein (MOG), N-methyl-D-aspartate receptor, LGI1, CASPR2, glycine receptor (GlyR), DPPX, and the voltage gated potassium channel (VGKC)-complex.
  
  
  
  Disease localized to the brainstem was seen in 19 patients: Bickerstaff's brainstem encephalitis (n=14) and clinically isolated syndrome (n=5). Polyfocal presentation was seen in 38 children, with predominantly white matter disease in 18 patients and grey matter in 20 patients. CNS surface antibodies were found in 22/57 patients (two patients with double positivity): GQIb (n=6), NMDAR (n=7), GlyR (n=5), MOG (n=5), and one AQP4. Three patients were positive for VGKC-complex antibodies. All patients were negative for antibodies to DPPX and the VGKC-complex antigens LGI1, CASPR2, and contactin-2. Although there were some partial differences in the presentations, the clinical features and outcomes did not relate clearly to the presence or absence of specific antibodies.
  
  
  
  As determined retrospectively, 39% of patients had cell surface antibodies. The results did not suggest any relationship with treatment or outcomes obtained but it is possible that specific antibody detection could be a helpful guide to more intensive immunotherapies in some cases.
authors.affiliation: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.
authors.forename: Yael
authors.initials: Y
authors.lastname: Hacohen
authors.affiliation: Department of Pediatrics, Wakayama National Hospital, Wakayama, Japan.
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Nishimoto
authors.affiliation: Department of Medicine, Yong Loo Ling School of Medicine, National University of Singapore, Singapore, Singapore.
authors.forename: Yuki
authors.initials: Y
authors.lastname: Fukami
authors.affiliation: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.
authors.forename: Bethan
authors.initials: B
authors.lastname: Lang
authors.affiliation: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.
authors.forename: Patrick
authors.initials: P
authors.lastname: Waters
authors.affiliation: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.|Children's Neurosciences, Evelina London Children's Hospital, at Guy's and St Thomas' NHS Foundation Trust, King's Health Partners Academic Health Science Centre, London, UK.
authors.forename: Ming J
authors.initials: MJ
authors.lastname: Lim
authors.affiliation: Department of Medicine, Yong Loo Ling School of Medicine, National University of Singapore, Singapore, Singapore.|Department of Physiology, Yong Loo Ling School of Medicine, National University of Singapore, Singapore, Singapore.
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Yuki
authors.affiliation: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.
authors.forename: Angela
authors.initials: A
authors.lastname: Vincent
mesh_terms: D000293:Adolescent; D051401:Aquaporin 4 / Q000276:immunology; D001323:Autoantibodies / Q000097:blood*; D001933:Brain Stem / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D004660:Encephalitis / Q000097:blood* / Q000473:pathology*; D005260:Female; D005732:Gangliosides / Q000276:immunology; D006801:Humans; D007223:Infant; D008297:Male; D063308:Myelin-Oligodendrocyte Glycoprotein / Q000276:immunology; D009457:Neuroglia / Q000378:metabolism*; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology; D018009:Receptors, Glycine / Q000276:immunology; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology; D012189:Retrospective Studies
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies; D005732:Gangliosides; D063308:Myelin-Oligodendrocyte Glycoprotein; D024642:Potassium Channels, Voltage-Gated; D018009:Receptors, Glycine; D016194:Receptors, N-Methyl-D-Aspartate; C039129:GQ1b ganglioside