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MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 2: Epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome

Please always quote using this URN: urn:nbn:de:bvb:20-opus-165570
  • Background A subset of patients with neuromyelitis optica spectrum disorders (NMOSD) has been shown to be seropositive for myelin oligodendrocyte glycoprotein antibodies (MOG-IgG). Objective To describe the epidemiological, clinical, radiological, cerebrospinal fluid (CSF), and electrophysiological features of a large cohort of MOG-IgG-positive patients with optic neuritis (ON) and/or myelitis (n = 50) as well as attack and long-term treatment outcomes. Methods Retrospective multicenter study. Results The sex ratio was 1:2.8 (m:f).Background A subset of patients with neuromyelitis optica spectrum disorders (NMOSD) has been shown to be seropositive for myelin oligodendrocyte glycoprotein antibodies (MOG-IgG). Objective To describe the epidemiological, clinical, radiological, cerebrospinal fluid (CSF), and electrophysiological features of a large cohort of MOG-IgG-positive patients with optic neuritis (ON) and/or myelitis (n = 50) as well as attack and long-term treatment outcomes. Methods Retrospective multicenter study. Results The sex ratio was 1:2.8 (m:f). Median age at onset was 31 years (range 6-70). The disease followed a multiphasic course in 80% (median time-to-first-relapse 5 months; annualized relapse rate 0.92) and resulted in significant disability in 40% (mean follow-up 75 ± 46.5 months), with severe visual impairment or functional blindness (36%) and markedly impaired ambulation due to paresis or ataxia (25%) as the most common long-term sequelae. Functional blindness in one or both eyes was noted during at least one ON attack in around 70%. Perioptic enhancement was present in several patients. Besides acute tetra-/paraparesis, dysesthesia and pain were common in acute myelitis (70%). Longitudinally extensive spinal cord lesions were frequent, but short lesions occurred at least once in 44%. Fourty-one percent had a history of simultaneous ON and myelitis. Clinical or radiological involvement of the brain, brainstem, or cerebellum was present in 50%; extra-opticospinal symptoms included intractable nausea and vomiting and respiratory insufficiency (fatal in one). CSF pleocytosis (partly neutrophilic) was present in 70%, oligoclonal bands in only 13%, and blood-CSF-barrier dysfunction in 32%. Intravenous methylprednisolone (IVMP) and long-term immunosuppression were often effective; however, treatment failure leading to rapid accumulation of disability was noted in many patients as well as flare-ups after steroid withdrawal. Full recovery was achieved by plasma exchange in some cases, including after IVMP failure. Breakthrough attacks under azathioprine were linked to the drug-specific latency period and a lack of cotreatment with oral steroids. Methotrexate was effective in 5/6 patients. Interferon-beta was associated with ongoing or increasing disease activity. Rituximab and ofatumumab were effective in some patients. However, treatment with rituximab was followed by early relapses in several cases; end-of-dose relapses occurred 9-12 months after the first infusion. Coexisting autoimmunity was rare (9%). Wingerchuk’s 2006 and 2015 criteria for NMO(SD) and Barkhof and McDonald criteria for multiple sclerosis (MS) were met by 28%, 32%, 15%, 33%, respectively; MS had been suspected in 36%. Disease onset or relapses were preceded by infection, vaccination, or pregnancy/delivery in several cases. Conclusion Our findings from a predominantly Caucasian cohort strongly argue against the concept of MOG-IgG denoting a mild and usually monophasic variant of NMOSD. The predominantly relapsing and often severe disease course and the short median time to second attack support the use of prophylactic long-term treatments in patients with MOG-IgG-positive ON and/or myelitis.show moreshow less

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Metadaten
Author: Sven Jarius, Klemens Ruprecht, Ingo Kleiter, Nadja Borisow, Nasrin Asgari, Kalliopi Pitarokoili, Florence Pache, Oliver Stich, Lena-Alexandra Beume, Martin W. Hümmert, Marius Ringelstein, Corinna Trebst, Alexander Winkelmann, Alexander Schwarz, Mathias Buttmann, Hanna Zimmermann, Joseph Kuchling, Diego Franciotta, Marco Capobianco, Eberhard Siebert, Carsten Lukas, Mirjam Korporal-Kuhnke, Jürgen Haas, Kai Fechner, Alexander U. Brandt, Kathrin Schanda, Orhan Aktas, Friedemann Paul, Markus Reindl, Brigitte Wildemann
URN:urn:nbn:de:bvb:20-opus-165570
Document Type:Journal article
Faculties:Medizinische Fakultät / Neurologische Klinik und Poliklinik
Language:English
Parent Title (English):Journal of Neuroinflammation
Year of Completion:2016
Volume:13
Issue:280
Source:Journal of Neuroinflammation (2016) 13:280
DOI:https://doi.org/10.1186/s12974-016-0718-0
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
Tag:Aquaporin-4 antibodies (AQP4-IgG, NMO-IgG); Autoantibodies; Azathioprine; Barkhof criteria; Cerebrospinal fluid; Electrophysiology; Evoked potentials; Glatiramer acetate; IPND criteria; Infections; Interferon beta; International consensus diagnostic criteria for neuromyelitis optica spectrum disorders; Longitudinally extensive transverse myelitis; Magnetic resonance imaging; McDonald criteria; Methotrexate; Multiple sclerosis; Myelin oligodendrocyte glycoprotein antibodies (MOG-IgG); Natalizumab; Neuromyelitis optica spectrum disorders (NMOSD); Ofatumumab; Oligoclonal bands; Optic neuritis; Outcome; Pregnancy; Rituximab; Therapy; Transverse myelitis; Treatment; Vaccination; Wingerchuk criteria 2006 and 2015
Release Date:2020/05/22
Contributing Corporation:in cooperation with the Neuromyelitis Optica Study Group (NEMOS)
Licence (German):License LogoCC BY: Creative-Commons-Lizenz: Namensnennung 4.0 International