Previous retrospective studies suggest the superiority of PE in AQP4-Ab-positive patients (NMOSD–AQP4+), with better outcomes when initiated early. There is no randomized clinical trial for the treatment of NMOSD relapses but there have been efforts to reach a consensus. As inadequately treated attacks result in disability, there is a need to identify the optimal attack treatment regimen. Consequently, despite MOGAD having a broader and less clearly defined spectrum of manifestations than classical NMOSD as defined by the IPND criteria, we focused on NMOSD phenotypical first presentations, and thus used the term NMOSD–MOG+ in this report.Īttack treatment strategy in NMOSD relies on various combinations of high-dose intravenous corticosteroids and apheresis methods including plasma exchanges (PE), immunoadsorption (IA) or intravenous immunoglobulins (IVIG). During this period, if an NMOSD phenotype is observed, there is an urgent need to instigate a powerful anti-inflammatory therapeutic strategy. The first attack of these patients must, therefore, be considered diagnostically uncertain until the results of the serological status are known. Despite this, AQP4-Ab-positive and MOG-Ab-positive patients can have a similar initial presentation, corresponding to an NMOSD phenotype Such phenotypes have been well described : longitudinally extensive transverse myelitis, severe optic neuritis (ON), area postrema/diencephalic/brainstem or encephalic syndrome. Indeed, patients positive for MOG-Ab included in the NMOSD set of criteria have recently been individualized as having MOG-associated diseases (MOGAD), and there is some debate about the classification of MOGAD in NMOSD. However, AQP4-Ab-positive patients and MOG-Ab-positive patients have different immunopathologic and clinical courses and the nosography is currently evolving rapidly. According to the most recent diagnostic criteria, NMOSD may be associated with anti-aquaporin 4 (AQP4) antibodies targeting astrocytes, with anti-myelin oligodendrocyte glycoprotein (MOG) antibodies targeting oligodendrocytes or with double seronegative status (NMOSD–DN, i.e., seronegative for AQP4 and MOG antibodies). Neuromyelitis optica spectrum disorder (NMOSD) attacks are more damaging than multiple sclerosis (MS) attacks and require more aggressive treatments. We, therefore, argue for a more aggressive anti-inflammatory management of the first attacks suggesting an NMOSD presentation, with the early combination of PE with corticosteroids. We identified two prognostic factors: serostatus (with better outcomes among MOG-Ab-positive patients) and the delay to PE. MOG-antibody seropositivity and short delays to PE were significantly and independently associated with better recovery and treatment response. There was no improvement in EDSS score in 50 attacks (23.7%). The treatment response was “good” in 134 attacks (63.5%). Complete recovery was reached in 40 attacks (19%) at 6 months. Attack treatment regimens comprised corticosteroids ( n = 196), plasma exchanges (PE n = 72) and intravenous immunoglobulins ( n = 6). We included 211 attacks among 183 patients (104 with anti-AQP4 antibodies, 60 with anti-MOG antibodies, and 19 double seronegative). We used ordinal logistic regression to infer statistical associations with the outcome. The primary outcome was the evolution of the Expanded Disability Status Scale (EDSS) score at 6 months, stratified in two ways to account for recovery (return to baseline EDSS score) and treatment response (classified as “good” if the EDSS score decreased by ≥ 1 point after a nadir EDSS score ≤ 3, or by ≥ 2 points after a nadir EDSS score > 3). We studied the first attack for any independent locations of clinical core characteristic of NMOSD, in treatment-naïve patients. We performed a retrospective cohort study on the French national NMOSD registry (NOMADMUS), a nested cohort of the French multiple sclerosis observatory (OFSEP) recruiting patients with NMOSD presentations in France. Our study aimed to identify predictors of outcome after a first attack in patients with an NMOSD presentation and propose the best treatment strategy. As inadequately treated attacks result in disability, there is a need to identify the optimal attack-treatment regimen. Neuromyelitis optica spectrum disorder (NMOSD) attacks require an urgent probabilistic anti-inflammatory therapeutic strategy.
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