Neuropediatrics 2014; 45 - fp048
DOI: 10.1055/s-0034-1390553

Clinical and Radiological Features of Children with MOG-positive and MOG-negative ADEM

M. Bauman 1, K. Sahin 1, C. Lechner 1, E. Hennes 1, K. Schanda 2, M. Karenfort 3, J. Koch 4, C. Selch 5, M. Häusler 6, V. Kraus 7, S. Mader 2, M. Salandin 8, U. Gruber-Sedlmeyer 9, M. Piepkorn 10, A. Blaschek 11, A. Eisenköbl 12, S. Leiz 13, J. Finsterwalder 14, T. Berger 2, M. Reindl 2, K. Rostasy 1
  • 1Medizinische Universität Innsbruck, Pädiatrie 1, Neuropädiatrie, Innsbruck, Austria
  • 2Medizinische Universität Innsbruck, Abteilung für Neurologie, Innsbruck, Austria
  • 3Heinrich Heine Universität, Klinik für Allgemeine Pädiatrie, Düsseldorf, Germany
  • 4Universitätskinderklinik Salzburg, Kinderklinik, Salzburg, Austria
  • 5Schön Klinik Vogtareuth, Klinik für Neuropädiatrie und Neurologische Rehabilitation, Tagesklinik für Neuropädiatrie, Vogtareuth, Germany
  • 6Universitätsklinikum Aachen, Klinik für Kinder- und Jugendmedizin, Neuropädiatrie, Aachen, Germany
  • 7Technische Universität München, Kinderklinik Schwabing, München, Germany
  • 8Kinderklinik, Bozen, Bozen, Italien
  • 9Universitäts-Klinik für Kinder- und Jugendheilkunde, Ambulanz für Neuropädiatrie und angeborene Stoffwechselerkrankungen, Graz, Austria
  • 10Kinderkrankenhaus Auf der Bult, Neuropädiatrie, Hannover, Germany
  • 11Haunersches Kinderspital, Neuropädiatrie, München, Germany
  • 12Landes-Frauen-und Kinderklinik Linz, Linz, Austria
  • 13Klinikum Dritter Orden, Klinik für Kinder- und Jugendmedizin, München, Germany
  • 14Klinikum Mutterhaus der Borromäerinnen, Trier, Abteilung für Kinderheilkunde, Trier, Germany

Background: Serum myelin oligodendrocyte glycoprotein (MOG) immunoglobulin (IgG) antibodies have recently been detected in pediatric acute disseminating encephalomyelitis (ADEM).

Aim: This article aims to further delineate the clinical and neuroradiological features of pediatric ADEM with serum antibodies against MOG.

Methods: A total of 39 pediatric patients with an episode of ADEM were recruited. The following outcome measures were obtained: clinical features, intrathecal IgG-synthesis, MRI findings and outcome. Cell-based immunofluorescence assay was used to measure serum IgG antibodies to MOG.

Results: All 39 children fulfilled the clinical criteria of ADEM. The cohort consisted of 17 girls and 22 boys with a median age of 5 years (range, 1-17 years). On the basis of the presence of different magnetic resonance imaging (MRI) features the cohort was divided into two groups: children with an MRI characterized by large, hazy, bilateral, and widespread lesions (n = 28) and children with at least two atypical MRI features in addition (e.g., unilateral lesions only and not widespread). Children from the first group presented at a younger age (median, 5 years; range, 1-17 years vs. median, 9 years range, 1-14 years; p = 0.037), had a higher cerebrospinal fluid cell count (p = 0.004) and a better clinical outcome (p = 0.003). In addition, lesions were detected in more anatomical areas when compared with children from the second group (p = 0.001) often combined with a resolution of signal changes (p < 0.001). Overall, six children with atypical MRI findings were assigned an alternative diagnosis on follow-up.

Serum MOG-IgG antibodies (median, 1:2,560; range, 1:160-1:20,480) were detected in 19 children all of whom had an MRI characterized by large, bilateral, and widespread MRI lesions. In 16 of 18 children with follow-up samples, titers declined over time (p < 0.001). On the basis of MOG status, this subgroup was further divided and analyzed. Apart from the observation that children with MOG antibodies had more often involvement of the myelon characterized by a longitudinally extensive transverse myelitis (LETM) (p = 0.039), children with a typical MRI had similar clinical features and clinical outcome.

Conclusion: The majority of children with ADEM and an MRI revealing hazy, bilateral, and widespread lesions had MOG antibodies declining overtime. They often have a LETM and resolution of white matter lesions combined with a favorable clinical outcome. Children with ADEM, atypical MRI features, and absent MOG antibodies have a high likelihood of an alternative diagnosis.