Aspectos clínicos e prognósticos de pacientes com diagnóstico de doença do espectro da neuromielite óptica

Abstract

Introduction: Neuromyelitis optica spectrum disease (DENMO) is a rare inflammatory and demyelinating disease of the central nervous system (CNS), more frequent in women and people of African descent. No previous epidemiological or prognostic studies have been carried out in the region of the state of Bahia, northeastern Brazil. Objective: To identify demographic characteristics and factors associated with greater disease progression in patients with DENMO in the State of Bahia. Material and Methods: A single-center retrospective study was conducted with consecutive patients diagnosed with DENMO. Clinical and epidemiological characteristics were described. The degree of disability was expressed by the Expanded Disability Status Scale (EDSS). The main outcome of the study was the progression index, defined by the ratio between the EDSS and the duration of the disease in months. Predictors of disease progression were identified using negative binomial regression adjusted for disease duration. Results: Ninety-one patients were included, 72 (79.1%) of whom were female and 67 (73.6%) were of African descent. The mean age at onset was 36 (± 14) years and 73.3% were positive for the anti-aquaporin-4 antibody. Isolated transverse myelitis (32.9%) and isolated optic neuritis (22.4%) were the most frequent initial clinical syndromes. After multivariate analysis, optic neuritis (RR = 0.49; 95% CI=0.29 – 0.84; p = 0.009) and dyslipidemia (RR = 0.50; 95% CI=0.26 – 0.96; p= 0.038) were associated with slower disease progression. Involvement of the postrema area (RR = 6.59; 95% CI = 3.56 – 12.21; p < 0.001) and age at onset (RR = 1.01; 95% CI = 1.00 – 1.03; p = 0.047) were associated with a more rapid progression of the disease. Conclusions: In the first clinical and prognostic study in northeastern Brazil, we identified the involvement of the area postrema and the older age of onset as factors associated with the progression of the disease; and optic neuritis as the initial syndrome and dyslipidemia as the main protective factors.