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| Item Type: | Article |
|---|---|
| Title: | 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 |
| Creators Name: | Jarius, S., Ruprecht, K., Kleiter, I., Borisow, N., Asgari, N., Pitarokoili, K., Pache, F., Stich, O., Beume, L.A., Hümmert, M.W., Ringelstein, M., Trebst, C., Winkelmann, A., Schwarz, A., Buttmann, M., Zimmermann, H., Kuchling, J., Franciotta, D., Capobianco, M., Siebert, E., Lukas, C., Korporal-Kuhnke, M., Haas, J., Fechner, K., Brandt, A.U., Schanda, K., Aktas, O., Paul, F., Reindl, M. and Wildemann, B. |
| Abstract: | 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 blindess 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. |
| Keywords: | Myelin Oligodendrocyte Glycoprotein Antibodies (MOG-IgG), Autoantibodies, Neuromyelitis Optica Spectrum Disorders (NMOSD), Aquaporin-4 Antibodies (AQP4-IgG, NMO-IgG), Optic Neuritis, Transverse Myelitis, Longitudinally Extensive Transverse Myelitis, Magnetic Resonance Imaging, Cerebrospinal Fluid, Oligoclonal Bands, Electrophysiology, Evoked Potentials, Treatment, Therapy, Methotrexate, Azathioprine, Rituximab, Ofatumumab, Interferon {beta}, Glatiramer Acetate, Natalizumab, Outcome, Pregnancy, Infections, Vaccination, Multiple Sclerosis, Barkhof Criteria, McDonald Criteria, Wingerchuk Criteria 2006 and 2015, IPND Criteria, International Consensus Diagnostic Criteria for Neuromyelitis Optica Spectrum Disorders |
| Source: | Journal of Neuroinflammation |
| ISSN: | 1742-2094 |
| Publisher: | BioMed Central |
| Volume: | 13 |
| Number: | 1 |
| Page Range: | 280 |
| Date: | 28 October 2016 |
| Official Publication: | https://doi.org/10.1186/s12974-016-0718-0 |
| PubMed: | View item in PubMed |
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