title: Children with multiphasic disseminated encephalomyelitis and antibodies to the myelin oligodendrocyte glycoprotein (MOG): Extending the spectrum of MOG antibody positive diseases.
abstract: Myelin oligodendrocyte glycoprotein (MOG) antibodies have been described in children with acute disseminated encephalomyelitis (ADEM), recurrent optic neuritis, neuromyelitis optica spectrum disorders and more recently in children with multiphasic disseminated encephalomyelitis (MDEM).
  
  
  
  To delineate the clinical, cerebrospinal fluid (CSF) and radiological features of paediatric MDEM with MOG antibodies.
  
  
  
  Clinical course, serum antibodies, CSF, magnetic resonance imaging (MRI) studies and outcome of paediatric MDEM patients were reviewed.
  
  
  
  A total of 8 children with two or more episodes of ADEM were identified from a cohort of 295 children with acute demyelinating events. All children had persisting MOG antibodies (median titre: 1:1280). All ADEM episodes included encephalopathy, polyfocal neurological signs and a typical MRI. Apart from ADEM episodes, three children had further clinical attacks without encephalopathy. Median age at initial presentation was 3 years (range: 1-7 years) and median follow-up 4 years (range: 1-8 years). New ADEM episodes were associated with new neurological signs and new MRI lesions. Clinical outcome did range from normal (four of the eight) to mild or moderate impairment (four of the eight). A total of four children received monthly immunoglobulin treatment during the disease course.
  
  
  
  Children with MDEM and persisting MOG antibodies constitute a distinct entity of relapsing demyelinating events and extend the spectrum of MOG antibody-associated diseases.
authors: Department of Paediatrics I, Paediatric Neurology, Medical University of Innsbruck, Innsbruck, Austria.
authors: Matthias
authors: M
authors: Baumann
authors: Olga Hospital, Children's Hospital Stuttgart, Stuttgart, Germany.
authors: Eva-Maria
authors: EM
authors: Hennes
authors: Clinical Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria.
authors: Kathrin
authors: K
authors: Schanda
authors: Department of General Paediatrics, Neonatology and Paediatric Cardiology, University Children's Hospital, Heinrich Heine University Düsseldorf, Düsseldorf, Germany.
authors: Michael
authors: M
authors: Karenfort
authors: Department of Neurology, Medical University of Vienna, Vienna, Austria.
authors: Barbara
authors: B
authors: Kornek
authors: Department of Paediatrics, Medical University of Vienna, Vienna, Austria.
authors: Rainer
authors: R
authors: Seidl
authors: Department of Paediatric Neurology, Children's Hospital, Kassel, Germany.
authors: Katharina
authors: K
authors: Diepold
authors: Department of Neuropaediatrics and Metabolic Diseases, Greifswald University Hospital, Greifswald, Germany.
authors: Heinz
authors: H
authors: Lauffer
authors: Paediatric Neurology, University Children's Hospital, Oldenburg, Germany.
authors: Iris
authors: I
authors: Marquardt
authors: Department of Neurology, Children's Clinical University Hospital, Riga, Latvia.
authors: Jurgis
authors: J
authors: Strautmanis
authors: University Hospital for Children and Adolescents, Leipzig, Germany.
authors: Steffen
authors: S
authors: Syrbe
authors: Children's Hospital Bayreuth, Bayreuth, Germany.
authors: Silvia
authors: S
authors: Vieker
authors: Institute of Neurology, Medical University of Vienna, Vienna, Austria.
authors: Romana
authors: R
authors: Höftberger
authors: Clinical Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria.
authors: Markus
authors: M
authors: Reindl
authors: Department of Paediatric Neurology, Children's Hospital Datteln, Witten/Herdecke University, Datteln, Germany k.rostasy@kinderklinik-datteln.de.
authors: Kevin
authors: K
authors: Rostásy
mesh_terms: D001323:Autoantibodies / Q000134:cerebrospinal fluid*; D002648:Child; D002675:Child, Preschool; D020278:Demyelinating Autoimmune Diseases, CNS / Q000097:blood / Q000134:cerebrospinal fluid / Q000000981:diagnostic imaging / Q000503:physiopathology; D004679:Encephalomyelitis / Q000097:blood / Q000134:cerebrospinal fluid / Q000000981:diagnostic imaging / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D063308:Myelin-Oligodendrocyte Glycoprotein / Q000276:immunology*
chemical_list: D001323:Autoantibodies; C571326:MOG protein, human; D063308:Myelin-Oligodendrocyte Glycoprotein
keywords: Multiphasic disseminated encephalomyelitis; acquired demyelinating syndromes; acute disseminated encephalomyelitis; children; magnetic resonance imaging; myelin oligodendrocyte glycoprotein