Clinical and prognostic aspects of patients with the Neuromyelitis Optica Spectrum Disorder (NMOSD) from a cohort in Northeast Brazil.

ABSTRACT

INTRODUCTION: Neuromyelitis optica spectrum disorders (NMOSD) is a rare inflammatory and demyelinating disease of the central nervous system (CNS) more frequent in women and Afro-descendants. No previous epidemiological or prognostic study has been conducted in the region of the state of Bahia, Brazilian Northeast. OBJECTIVE: To evaluate clinical and prognostic aspects in patients with NMOSD from a cohort in northeastern Brazil. MATERIAL AND METHODS: A single-center retrospective study was conducted with consecutive patients diagnosed with NMOSD. Clinical and epidemiological characteristics were described. The degree of disability was expressed by the Expanded Disability Status Scale (EDSS). Worsening disability were analyzed through negative binomial regression adjusted for disease duration. RESULTS: Ninety-one patients were included, 72 (79.1%) female and 67 (73.6%) afro descendants. Mean age at onset was 36 (± 14) years and 73.3% were anti-aquaporin-4 antibody positive. Isolated transverse myelitis (32.9%) and isolated optic neuritis (22.4%) were the most frequent initial clinical syndromes. After multivariate analysis, optic neuritis (RR = 0.45; 95% CI = 0.23 - 0.88; p = 0.020) and dyslipidemia (RR = 0.40; 95% CI = 0.20 - 0.83; p = 0.014) were associated with slower disease progression. Area postrema involvement (RR = 6.70; 95% CI = 3.31 - 13.54; p < 0.001) and age at onset (RR = 1.03; 95% CI = 1.01 - 1.05; p = 0.003) were associated with faster disease progression. CONCLUSIONS: In the first clinical and prognostic study in northeastern Brazil, we identified area postrema involvement, age at onset, optic neuritis at fist syndrome and dyslipidemia as the main prognostic factors associated with disease progression.