• Matthias Baumann, Eva-Maria Hennes, Kathrin Schanda, Michael Karenfort, Barbara Kornek, Rainer Seidl, Katharina Diepold, Heinz Lauffer, Iris Marquardt, Jurgis Strautmanis, Steffen Syrbe, Silvia Vieker, Romana Höftberger, Markus Reindl, and Kevin Rostásy.
• Department of Paediatrics I, Paediatric Neurology, Medical University of Innsbruck, Innsbruck, Austria.
• Mult. Scler. 2016 Dec 1; 22 (14): 1821-1829.

BackgroundMyelin 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).ObjectiveTo delineate the clinical, cerebrospinal fluid (CSF) and radiological features of paediatric MDEM with MOG antibodies.MethodsClinical course, serum antibodies, CSF, magnetic resonance imaging (MRI) studies and outcome of paediatric MDEM patients were reviewed.ResultsA 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.ConclusionChildren with MDEM and persisting MOG antibodies constitute a distinct entity of relapsing demyelinating events and extend the spectrum of MOG antibody-associated diseases.© The Author(s), 2016.

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