Abstract General Information
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.
Area
NMO, ADEM and CIS
Authors
ANA CAROLINA AMARAL DE ANDRADE, MARIANA MOREIRA SOARES DE SÁ, GABRIEL DE DEUS VIERA, ALFREDO DAMASCENO