Treatment regimens for neuromyelitis optica spectrum disorder attacks: a retrospective cohort study

Stanislas Demuth (1) , Maxime Guillaume , Bertrand Bourre , Jonathan Ciron (2, 3) , Hélène Zephir , Yoann Sirejacob , Anne Kerbrat , Christine Lebrun-Frenay , Caroline Papeix , Laure Michel , David Laplaud , Sandra Vukusic , Elisabeth Maillart , Mikael Cohen , Bertrand Audoin (4, 5) , Romain Marignier , Nicolas Collongues

1 Department of Neurology, University Hospital Strasbourg
2 CRC-SEP Toulouse - Centre Ressources et Compétences sclérose en plaques (CRC-SEP) [CHU Toulouse]
3 Infinity - Institut Toulousain des Maladies Infectieuses et Inflammatoires
4 Neurologie, maladies neuro-musculaires [Hôpital de la Timone - APHM]
5 CRMBM - Centre de résonance magnétique biologique et médicale

Background: Neuromyelitis optica spectrum disorder (NMOSD) attacks require an urgent probabilistic anti-inflammatory therapeutic strategy. As inadequately treated attacks result in disability, there is a need to identify the optimal attack-treatment regimen. Our study aimed to identify predictors of outcome after a first attack in patients with an NMOSD presentation and propose the best treatment strategy. Methods: 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. We studied the first attack for any independent locations of clinical core characteristic of NMOSD, in treatment-naïve patients. 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 used ordinal logistic regression to infer statistical associations with the outcome. Results: We included 211 attacks among 183 patients (104 with anti-AQP4 antibodies, 60 with anti-MOG antibodies, and 19 double seronegative). Attack treatment regimens comprised corticosteroids (n = 196), plasma exchanges (PE; n = 72) and intravenous immunoglobulins (n = 6). Complete recovery was reached in 40 attacks (19%) at 6 months. The treatment response was "good" in 134 attacks (63.5%). There was no improvement in EDSS score in 50 attacks (23.7%). MOG-antibody seropositivity and short delays to PE were significantly and independently associated with better recovery and treatment response. Conclusions: We identified two prognostic factors: serostatus (with better outcomes among MOG-Ab-positive patients) and the delay to PE. 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.

Mots clés: Neuromyelitis optica Therapeutics MOGAD Corticosteroids Plasma exchanges Relapses; Neuromyelitis optica; Therapeutics; MOGAD; Corticosteroids; Plasma exchanges; Relapses

Domaines: Médecine humaine et pathologie

Stanislas Demuth, Maxime Guillaume, Bertrand Bourre, Jonathan Ciron, Hélène Zephir, et al.. Treatment regimens for neuromyelitis optica spectrum disorder attacks: a retrospective cohort study. Journal of Neuroinflammation, 2022, 19, ⟨10.1186/s12974-022-02420-2⟩. ⟨hal-03963595⟩