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Title

DESCRIPTION OF THE THERAPEUTIC RESPONSE IN NEUROMYELITIS OPTICA ANTI-AQUAPORIN ANTIBODY POSITIVE IV

Abstract

Introduction: Neuromyelitis Optica (NMO) is a clinical condition that has been studied deeply in recent years and therapeutic advances have been achieved, but unfortunately, in our country, NMO lacks highly effective therapeutic options. Objectives: Evaluating the demographic aspects, clinical evolution and responding to maintenance therapies, in patients with positive Anti APQIV NMO. Methodology: Reviewing the medical records for 46 patients with a diagnosis of NMO and positive AQPIV antibody. Results: Female accounted for 84.8% of the sample and white individuals accounted for 78.7%. The median age at the time of the first outbreak was 36 years. The most common of first symptomatic event was for those of single topography (Optic neuritis – 39.1%, followed by longitudinally extensive transverse myelitis – 19.5%, area postrema syndrome – 15.2% and trunk syndrome – 2.17%), while multi-site events involving the regions above, occur in 17.4%. Most patients, 41.3%, took between 1 and 5 years to start disease-modifying therapy after the first symptomatic event and most of these patients received Azathioprine as initial treatment – 78.2%, followed by 6.5 % treated with cyclophosphamide and 4.2% received mitoxantrone or rituximab. Before starting maintenance therapy, 47.8% of patients had between 1 and 5 relapses per year. The annualized relapse rate was reduced after the start of maintenance therapy, with 61.1% of patients who started therapy with azathioprine being free of relapses, while no patients who received initial therapy with rituximab, cyclophosphamide, or mitoxantrone had a post-treatment relapse. The therapy of choice after initial treatment failure or intolerance was Rituximab in 45% of cases and most of these patients did not need a third therapy – 77.7%. Conclusion: As it is a little-known pathology, there is a delay between the first manifestations until the correct diagnosis then starting of maintenance treatment, with consequent impact on the clinical outcome.