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Research paper
Clinical and neuroradiological differences of paediatric acute disseminating encephalomyelitis with and without antibodies to the myelin oligodendrocyte glycoprotein
  1. M Baumann1,
  2. K Sahin1,
  3. C Lechner1,
  4. E M Hennes1,2,
  5. K Schanda3,
  6. S Mader3,4,
  7. M Karenfort5,
  8. C Selch6,
  9. M Häusler7,
  10. A Eisenkölbl8,
  11. M Salandin9,
  12. U Gruber-Sedlmayr10,
  13. A Blaschek11,
  14. V Kraus12,
  15. S Leiz13,
  16. J Finsterwalder14,
  17. T Gotwald15,
  18. G Kuchukhidze16,17,
  19. T Berger3,
  20. M Reindl3,
  21. K Rostásy1
  1. 1Division of Pediatric Neurology, Department of Pediatrics I, Innsbruck Medical University, Innsbruck, Austria
  2. 2Clinical Department of Neurology, Klinikum Bogenhausen, Munich, Germany
  3. 3Clinical Department of Neurology, Innsbruck Medical University, Innsbruck, Austria
  4. 4The Feinstein Institute for Medical Research, Center for Autoimmune and Musculoskeletal Diseases, Manhasset, New York, USA
  5. 5Department of General Pediatrics, Pediatric Neurology, Heinrich Heine University Düsseldorf, Düsseldorf, Germany
  6. 6Behandlungszentrum Vogtareuth, Vogtareuth, Germany
  7. 7Department of Pediatrics, Division of Neuropediatrics and Social Pediatrics, University Hospital, Aachen, Germany
  8. 8Department of Pediatrics, Landes- Frauen- und Kinderklinik Linz, Linz, Austria
  9. 9Department of Pediatrics, Bozen Hospital, Bozen, Italy
  10. 10Department of Pediatrics, Graz Medical University, Graz, Austria
  11. 11Department of Pediatric Neurology and Developmental Medicine, Dr von Hauner Children's Hospital, University of Munich, Munich, Germany
  12. 12Department of Pediatrics, Klinikum rechts der Isar, Technische Universität, Munich, Germany
  13. 13Pediatric Neurology, Department of Pediatrics, Klinikum Dritter Orden, Munich, Germany
  14. 14Division of Pediatric Neurology, Department of Pediatrics, Klinikum Mutterhaus der Borromäerinnen, Trier, Germany
  15. 15Radiological Institute, Kettenbrücke, Innsbruck, Austria
  16. 16Department of Neuroradiology, Medical University of Innsbruck, Innsbruck, Austria
  17. 17Department of Neurology, Paracelsus Medical University of Salzburg, Salzburg, Austria
  1. Correspondence to Professor Kevin Rostásy, Division of Pediatric Neurology, Department of Pediatrics I, Medical University Innsbruck, Anichstrasse 35, Innsbruck A-6020, Austria; Kevin.Rostasy{at}uki.at

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.

  • MRI
  • PAEDIATRIC NEUROLOGY
  • NEUROIMMUNOLOGY

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