Matthias Baumann1, Eva-Maria Hennes2, Kathrin Schanda3, Michael Karenfort4, Barbara Kornek5, Rainer Seidl6, Katharina Diepold7, Heinz Lauffer8, Iris Marquardt9, Jurgis Strautmanis10, Steffen Syrbe11, Silvia Vieker12, Romana Höftberger13, Markus Reindl3, Kevin Rostásy14. 1. Department of Paediatrics I, Paediatric Neurology, Medical University of Innsbruck, Innsbruck, Austria. 2. Olga Hospital, Children's Hospital Stuttgart, Stuttgart, Germany. 3. Clinical Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria. 4. Department of General Paediatrics, Neonatology and Paediatric Cardiology, University Children's Hospital, Heinrich Heine University Düsseldorf, Düsseldorf, Germany. 5. Department of Neurology, Medical University of Vienna, Vienna, Austria. 6. Department of Paediatrics, Medical University of Vienna, Vienna, Austria. 7. Department of Paediatric Neurology, Children's Hospital, Kassel, Germany. 8. Department of Neuropaediatrics and Metabolic Diseases, Greifswald University Hospital, Greifswald, Germany. 9. Paediatric Neurology, University Children's Hospital, Oldenburg, Germany. 10. Department of Neurology, Children's Clinical University Hospital, Riga, Latvia. 11. University Hospital for Children and Adolescents, Leipzig, Germany. 12. Children's Hospital Bayreuth, Bayreuth, Germany. 13. Institute of Neurology, Medical University of Vienna, Vienna, Austria. 14. Department of Paediatric Neurology, Children's Hospital Datteln, Witten/Herdecke University, Datteln, Germany k.rostasy@kinderklinik-datteln.de.
Abstract
BACKGROUND: 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). OBJECTIVE: To delineate the clinical, cerebrospinal fluid (CSF) and radiological features of paediatric MDEM with MOG antibodies. METHODS: Clinical course, serum antibodies, CSF, magnetic resonance imaging (MRI) studies and outcome of paediatric MDEM patients were reviewed. RESULTS: 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. CONCLUSION: Children with MDEM and persisting MOG antibodies constitute a distinct entity of relapsing demyelinating events and extend the spectrum of MOG antibody-associated diseases.
BACKGROUND: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). OBJECTIVE: To delineate the clinical, cerebrospinal fluid (CSF) and radiological features of paediatric MDEM with MOG antibodies. METHODS: Clinical course, serum antibodies, CSF, magnetic resonance imaging (MRI) studies and outcome of paediatric MDEM patients were reviewed. RESULTS: 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. CONCLUSION:Children with MDEM and persisting MOG antibodies constitute a distinct entity of relapsing demyelinating events and extend the spectrum of MOG antibody-associated diseases.
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