title: MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 1: Frequency, syndrome specificity, influence of disease activity, long-term course, association with AQP4-IgG, and origin creator: Jarius, Sven creator: Ruprecht, Klemens creator: Kleiter, Ingo creator: Borisow, Nadja creator: Asgari, Nasrin creator: Pitarokoili, Kalliopi creator: Pache, Florence creator: Stich, Oliver creator: Beume, Lena-Alexandra creator: Hümmert, Martin W. creator: Trebst, Corinna creator: Ringelstein, Marius creator: Aktas, Orhan creator: Winkelmann, Alexander creator: Buttmann, Mathias creator: Schwarz, Alexander creator: Zimmermann, Hanna creator: Brandt, Alexander U. creator: Franciotta, Diego creator: Capobianco, Marco creator: Kuchling, Joseph creator: Haas, Jürgen creator: Korporal-Kuhnke, Mirjam creator: Lillevang, Soeren Thue creator: Fechner, Kai creator: Schanda, Kathrin creator: Paul, Friedemann creator: Wildemann, Brigitte creator: Reindl, Markus subject: 610 subject: 610 Medical sciences Medicine description: Background: Antibodies to myelin oligodendrocyte glycoprotein (MOG-IgG) have been suggested to play a role in a subset of patients with neuromyelitis optica and related disorders. Objective: To assess (i) the frequency of MOG-IgG in a large and predominantly Caucasian cohort of patients with optic neuritis (ON) and/or myelitis; (ii) the frequency of MOG-IgG among AQP4-IgG-positive patients and vice versa; (iii) the origin and frequency of MOG-IgG in the cerebrospinal fluid (CSF); (iv) the presence of MOG-IgG at disease onset; and (v) the influence of disease activity and treatment status on MOG-IgG titers. Methods: 614 serum samples from patients with ON and/or myelitis and from controls, including 92 follow-up samples from 55 subjects, and 18 CSF samples were tested for MOG-IgG using a live cell-based assay (CBA) employing full-length human MOG-transfected HEK293A cells. Results: MOG-IgG was detected in 95 sera from 50 patients with ON and/or myelitis, including 22/54 (40.7%) patients with a history of both ON and myelitis, 22/103 (21.4%) with a history of ON but no myelitis and 6/45 (13.3%) with a history of longitudinally extensive transverse myelitis but no ON, and in 1 control patient with encephalitis and a connective tissue disorder, all of whom were negative for AQP4-IgG. MOG-IgG was absent in 221 further controls, including 83 patients with AQP4-IgG-seropositive neuromyelitis optica spectrum disorders and 85 with multiple sclerosis (MS). MOG-IgG was found in 12/18 (67%) CSF samples from MOG-IgG-seropositive patients; the MOG-IgG-specific antibody index was negative in all cases, indicating a predominantly peripheral origin of CSF MOG-IgG. Serum and CSF MOG-IgG belonged to the complement-activating IgG1 subclass. MOG-IgG was present already at disease onset. The antibodies remained detectable in 40/45 (89%) follow-up samples obtained over a median period of 16.5 months (range 0–123). Serum titers were higher during attacks than during remission (p < 0.0001), highest during attacks of simultaneous myelitis and ON, lowest during acute isolated ON, and declined following treatment. Conclusions: To date, this is the largest cohort studied for IgG to human full-length MOG by means of an up-to-date CBA. MOG-IgG is present in a substantial subset of patients with ON and/or myelitis, but not in classical MS. Co-existence of MOG-IgG and AQP4-IgG is highly uncommon. CSF MOG-IgG is of extrathecal origin. Serum MOG-IgG is present already at disease onset and remains detectable in the long-term course. Serum titers depend on disease activity and treatment status. publisher: BioMed Central date: 2016 type: Article type: info:eu-repo/semantics/article type: NonPeerReviewed format: application/pdf identifier: https://archiv.ub.uni-heidelberg.de/volltextserverhttps://archiv.ub.uni-heidelberg.de/volltextserver/22059/1/12974_2016_Article_717.pdf identifier: DOI: identifier: urn:nbn:de:bsz:16-heidok-220593 identifier: Jarius, Sven ; Ruprecht, Klemens ; Kleiter, Ingo ; Borisow, Nadja ; Asgari, Nasrin ; Pitarokoili, Kalliopi ; Pache, Florence ; Stich, Oliver ; Beume, Lena-Alexandra ; Hümmert, Martin W. ; Trebst, Corinna ; Ringelstein, Marius ; Aktas, Orhan ; Winkelmann, Alexander ; Buttmann, Mathias ; Schwarz, Alexander ; Zimmermann, Hanna ; Brandt, Alexander U. ; Franciotta, Diego ; Capobianco, Marco ; Kuchling, Joseph ; Haas, Jürgen ; Korporal-Kuhnke, Mirjam ; Lillevang, Soeren Thue ; Fechner, Kai ; Schanda, Kathrin ; Paul, Friedemann ; Wildemann, Brigitte ; Reindl, Markus (2016) MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 1: Frequency, syndrome specificity, influence of disease activity, long-term course, association with AQP4-IgG, and origin. Journal of Neuroinflammation, 13 (279). pp. 1-16. ISSN 1742-2094 relation: https://archiv.ub.uni-heidelberg.de/volltextserver/22059/ rights: info:eu-repo/semantics/openAccess rights: Please see front page of the work (Sorry, Dublin Core plugin does not recognise license id) language: eng