Despite improved recognition and diagnosis of neuromyelitis spectrum disorder (NMOSD), management remains a challenge. Patients of African/Asian ethnicity and those with a clinical phenotype involving the brainstem/medulla probably are most at risk for disease progression and/or death. Factors that determine mortality risk include age at diagnosis, relapse rates and poor recovery from attacks (
Kitley et al., 2012). No data exists regarding the prognosis or treatment of poorly responsive seronegative NMOSD. Plasma exchange (PLEX) therapy and IV steroids are used to treat relapses regardless of antibody status, but chronic management of seronegative NMOSD remains undefined. Rituxan is likely more efficacious in NMOSD patients compared to mycophenolate mofetil (MMF) in both seropositive or negative NMOSD (
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Prognostic factors and disease course in aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder from the United Kingdom and Japan.
Brain. 2012; 135: 1834-1849
Poupart et al., 2020) but the approval of Eculizumab (Solaris, Alexion) for AQP4 seropositive patients adds complexity to treatment options. The recently approved CD19-directed humanized monoclonal antibody, inebilizumab (Uplinza) is only indicated for patients with APQP4 Ab seropositive status although it was also tried in seronegative patients with inconclusive results.
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Zephir H on behalf of the NOMADMUS study group. Evaluation of efficacy and tolerability of first-line therapies in NMOSD.
Neurology. 2020; 94: e1645-e1656
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- Prognostic factors and disease course in aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder from the United Kingdom and Japan.Brain. 2012; 135: 1834-1849
- Zephir H on behalf of the NOMADMUS study group. Evaluation of efficacy and tolerability of first-line therapies in NMOSD.Neurology. 2020; 94: e1645-e1656
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Published online: July 03, 2020
Accepted: July 1, 2020
Received in revised form: June 30, 2020
Received: June 27, 2020
© 2020 Elsevier B.V. All rights reserved.