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Jarius, S.; Paul, F.; Aktas, O.; Asgari, N.; Dale, R. C.; de Seze, J.; Franciotta, D.; Fujihara, K.; Jacob, A.; Kim, H. J.; Kleiter, I.; Kümpfel, T.; Levy, M.; Palace, J.; Ruprecht, K.; Saiz, A.; Trebst, C.; Weinshenker, B. G. and Wildemann, B. (2018): MOG-Enzephalomyelitis: Internationale Empfehlungen zu Diagnose und Antikörpertestung. In: Nervenarzt, Vol. 89, No. 12: pp. 1388-1399

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Abstract

Over the past few years, new-generation cell-based assays have demonstrated a robust association of autoantibodies to full-length human myelin oligodendrocyte glycoprotein (MOG-IgG) with (mostly recurrent) optic neuritis, myelitis and brainstem encephalitis, as well as with acute disseminated encephalomyelitis (ADEM)-like presentations. Most experts now consider MOG-IgG-associated encephalomyelitis (MOG-EM) a disease entity in its own right, immunopathogenetically distinct from both classic multiple sclerosis (MS) and aquaporin-4 (AQP4)-IgG-positive neuromyelitis optica spectrum disorders (NMOSD). Owing to a substantial overlap in clinicoradiological presentation, MOG-EM was often unwittingly misdiagnosed as MS in the past. Accordingly, increasing numbers of patients with suspected or establishedMS are currently being tested for MOG-IgG. However, screening of large unselected cohorts for rare biomarkers can significantly reduce the positive predictive value of a test. To lessen the hazard of overdiagnosing MOG-EM, which may lead to inappropriate treatment, more selective criteria for MOG-IgG testing are urgently needed. In this paper, we propose indications for MOG-IgG testing based on expert consensus. In addition, we give a list of conditions atypical for MOG-EM ("red flags") that should prompt physicians to challenge a positive MOG-IgG test result. Finally, we provide recommendations regarding assay methodology, specimen sampling and data interpretation, and propose for the first time diagnostic criteria for MOG-EM.

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