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Myelin Oligodendrocyte Glycoprotein MOG35-55 Induced Experimental Autoimmune Encephalomyelitis EAE in C57BL/6 Mice
Published on: April 15, 2014
Clinical and imaging features of children with autoimmune encephalitis and MOG antibodies
Andreas Wegener-Panzer1, Robert Cleaveland1, Eva-Maria Wendel1
1From the Department of Radiology (A.W.-P., R.C.), Children's Hospital Datteln, University Witten/Herdecke; Department of Pediatrics (E.-M.W.), Olgahospital, Klinikum Stuttgart, Germany; Division of Pediatric Neurology (M. Baumann, C.L.), Department of Pediatrics I, Medical University of Innsbruck, Austria; Department of Pediatric Neurology (A.B., K.R.), Children's Hospital Datteln, University Witten/Herdecke; Department of Pediatrics (M.H.), Division of Neuropediatrics and Social Pediatrics, Medical University RWTH Aachen, Germany; Charité-Universitätsmedizin Berlin (E.K.), Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Germany; Department of Neuropediatrics (E.K.), Berlin, Germany; Department of Pediatrics and Adolescent Medicine (E.R.-F., M. Breu), Medical University of Vienna; St. Anna Children´s Hospital (E.R.-F.), Vienna, Austria; Klinik für Kinder und Jugendmedizin (Ö.S.), Evangelisches Krankenhaus Hamm, Germany; Department of Neuropediatrics (A.D.M.), Developmental Neurology and Social Pediatrics, Children's Hospital, University of Duisburg-Essen, Germany; Department of Pediatrics (R.P.), Christliches Kinderhospital Osnabrück, Germany; Department of Pediatric Neurology (M.P.), Children's Hospital Auf der Bult, Hannover Germany; Department of Neonatology (C.R.), Pediatric Intensive Care, Sleep Medicine, Children's Hospital Datteln, University Witten/Herdecke, Germany; Institute of Neurology (R.H.), Medical University of Vienna, Austria; Neuroimmunology (F.L.), Institute of Clinical Chemistry and Department of Neurology, Christian-Albrechts-University Kiel and Medical University Hospital Schleswig-Holstein, Germany; and Clinical Department of Neurology (M.R.), Medical University of Innsbruck, Austria.
Objective:
To describe the presentations, radiologic features, and outcomes of children with autoimmune encephalitis associated with myelin oligodendrocyte glycoprotein antibodies (MOG abs).
Methods:
Identification of children fulfilling the diagnostic criteria for possible autoimmune encephalitis (AE) and testing positive for serum MOG abs. Chart review and comprehensive analysis of serum MOG abs using live cell assays and rat brain immunohistochemistry.
Results:
Ten children (4 girls, 6 boys) with AE and serum MOG abs were identified. The median age at onset was 8.0 years (range: 4-16 years). Children presented with a combination of encephalopathy (10/10), headache (7/10), focal neurologic signs (7/10), or seizures (6/10). CSF pleocytosis was common (9/10, median 80 white cell count/μL, range: 21-256). Imaging showed cortical and deep gray matter involvement in all in addition to juxtacortical signal alterations in 6/10 children. No involvement of other white matter structures or contrast enhancement was noted. MOG abs were detected in all children (median titer 1:640; range: 1:320-1:10,540). Nine children had a favorable outcome at discharge (modified Rankin scale of < 2). Five of 10 children had up to 3 additional demyelinating relapses associated with persisting MOG abs. One child had NMDA receptor (NMDAR) abs at initial presentation. A second child had a third demyelinating episode with MOG abs with overlapping NMDAR encephalitis.
Discussion:
AE associated with serum MOG abs represents a distinct form of autoantibody-mediated encephalitis in children. We therefore recommend including MOG abs testing in the workup of children with suspected AE.
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