title: Clinical and neuroradiological differences of paediatric acute disseminating encephalomyelitis with and without antibodies to the myelin oligodendrocyte glycoprotein.
abstract: BACKGROUND
  Myelin oligodendrocyte glycoprotein (MOG) antibodies have been recently described in children with acute disseminating encephalomyelitis (ADEM), but the clinical and neuroradiological characterisation of this subgroup is lacking.
  
  
  OBJECTIVE
  To compare the clinical and neuroradiological features of paediatric ADEM with and without MOG antibodies.
  
  
  METHODS
  Clinical course, cerebrospinal fluid (CSF)-, MRI studies, outcome and MOG status of 33 paediatric ADEM prospectively studied were reviewed.
  
  
  RESULTS
  MOG antibodies (median 1:2560; range 1:160-1:20 480) were detected in 19 children with ADEM. The majority of children showed a decline of serum MOG-IgG titres over time. Children with MOG antibodies did not differ in their age at presentation, sex ratio, the presence of oligoclonal bands, clinical symptoms or initial severity, apart from a higher CSF cell count (p=0.038), compared with children without MOG antibodies. In addition, further relapsing demyelinating episodes associated with MOG antibodies were observed only in children with MOG antibodies. All 19 children with MOG antibodies had a uniform MRI pattern, characterised by large, hazy and bilateral lesions and the absence of atypical MRI features (eg, mainly small lesions, well-defined lesions), which was significantly different compared to that of children without MOG antibodies (p=0.003; and p=0.032, respectively). In addition, children with MOG antibodies had involvement of more anatomical areas (p=0.035) including the myelon characterised by a longitudinally extensive transverse myelitis (p=0.003), more often a complete resolution of lesions (p=0.036) and a better outcome (p=0.038).
  
  
  CONCLUSIONS
  Patients with ADEM with MOG antibodies in our cohort had a uniform MRI characterised by large, bilateral and widespread lesions with an increased frequency of longitudinal extensive transverse myelitis and a favourable clinical outcome in contrast to children lacking MOG antibodies.
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics I, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: M
authors.initials: M
authors.lastname: Baumann
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics I, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: K
authors.initials: K
authors.lastname: Sahin
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics I, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: C
authors.initials: C
authors.lastname: Lechner
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics I, Innsbruck Medical University, Innsbruck, Austria Clinical Department of Neurology, Klinikum Bogenhausen, Munich, Germany.
authors.forename: E M
authors.initials: EM
authors.lastname: Hennes
authors.affiliation: Clinical Department of Neurology, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: K
authors.initials: K
authors.lastname: Schanda
authors.affiliation: Clinical Department of Neurology, Innsbruck Medical University, Innsbruck, Austria The Feinstein Institute for Medical Research, Center for Autoimmune and Musculoskeletal Diseases, Manhasset, New York, USA.
authors.forename: S
authors.initials: S
authors.lastname: Mader
authors.affiliation: Department of General Pediatrics, Pediatric Neurology, Heinrich Heine University Düsseldorf, Düsseldorf, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Karenfort
authors.affiliation: Behandlungszentrum Vogtareuth, Vogtareuth, Germany.
authors.forename: C
authors.initials: C
authors.lastname: Selch
authors.affiliation: Department of Pediatrics, Division of Neuropediatrics and Social Pediatrics, University Hospital, Aachen, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Häusler
authors.affiliation: Department of Pediatrics, Landes- Frauen- und Kinderklinik Linz, Linz, Austria.
authors.forename: A
authors.initials: A
authors.lastname: Eisenkölbl
authors.affiliation: Department of Pediatrics, Bozen Hospital, Bozen, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Salandin
authors.affiliation: Department of Pediatrics, Graz Medical University, Graz, Austria.
authors.forename: U
authors.initials: U
authors.lastname: Gruber-Sedlmayr
authors.affiliation: Department of Pediatric Neurology and Developmental Medicine, Dr von Hauner Children's Hospital, University of Munich, Munich, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Blaschek
authors.affiliation: Department of Pediatrics, Klinikum rechts der Isar, Technische Universität, Munich, Germany.
authors.forename: V
authors.initials: V
authors.lastname: Kraus
authors.affiliation: Pediatric Neurology, Department of Pediatrics, Klinikum Dritter Orden, Munich, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Leiz
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, Klinikum Mutterhaus der Borromäerinnen, Trier, Germany.
authors.forename: J
authors.initials: J
authors.lastname: Finsterwalder
authors.affiliation: Radiological Institute, Kettenbrücke, Innsbruck, Austria.
authors.forename: T
authors.initials: T
authors.lastname: Gotwald
authors.affiliation: Department of Neuroradiology, Medical University of Innsbruck, Innsbruck, Austria Department of Neurology, Paracelsus Medical University of Salzburg, Salzburg, Austria.
authors.forename: G
authors.initials: G
authors.lastname: Kuchukhidze
authors.affiliation: Clinical Department of Neurology, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: T
authors.initials: T
authors.lastname: Berger
authors.affiliation: Clinical Department of Neurology, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: M
authors.initials: M
authors.lastname: Reindl
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics I, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: K
authors.initials: K
authors.lastname: Rostásy
mesh_terms: D000293:Adolescent; D001323:Autoantibodies / Q000097:blood*; D001921:Brain / Q000276:immunology / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004673:Encephalomyelitis, Acute Disseminated / Q000175:diagnosis* / Q000276:immunology*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008279:Magnetic Resonance Imaging; D008297:Male; D009103:Multiple Sclerosis / Q000175:diagnosis / Q000276:immunology; D063308:Myelin-Oligodendrocyte Glycoprotein / Q000276:immunology*; D009188:Myelitis, Transverse / Q000175:diagnosis / Q000276:immunology; D009471:Neuromyelitis Optica / Q000175:diagnosis / Q000276:immunology; D011379:Prognosis; D011446:Prospective Studies; D013116:Spinal Cord / Q000276:immunology / Q000473:pathology
chemical_list: D001323:Autoantibodies; D007074:Immunoglobulin G; C571326:MOG protein, human; D063308:Myelin-Oligodendrocyte Glycoprotein
keywords: MRI; NEUROIMMUNOLOGY; PAEDIATRIC NEUROLOGY