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DOI: 10.1055/s-0034-1390553
Clinical and Radiological Features of Children with MOG-positive and MOG-negative ADEM
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.