Neuromyelitis optica in Portugal (NEMIPORT) – A multicentre study

Abstract

Results: Sixty-seven patients fulfilled the inclusion criteria. They were mainly Caucasian, 55 female. Median age at onset was 32.0 years and mean follow-up 7.4 ± 6.0 years. Twenty-one patients were definite NMO and optic neuritis (ON) the most frequent initial presentation. Forty-six were classified as NMO spectrum disorders. The main subtypes were recurrent ON and single longitudinally extensive transverse myelitis. Twenty-four patients had positive AQP4-IgG. Twenty-three had other circulating autoantibodies. Fifteen out of 67 patients had concomitant autoimmune disease. There was a significant correlation between the presence of autoimmune disease and the positivity for AQP4-IgG. Five patients died, all definite NMO. Conclusion: This is the first study about this rare disease in Portugal. Demographic features were similar to other studies. The existence of concomitant autoimmune disease was significantly associated with seropositivity for AQP4-IgG.

Clinical and Prognostic Aspects of Patients With The Neuromyelitis Optic Spectrum Disorder (NMOSD) From a Cohort in Northeast Brazil

“…With regards to comorbid autoimmune diseases, the frequency in our sample is similar (10.2%) to what was shown in some previous studies [6,8]. When compared to others studies, however, where up to a third of patients presented this type of comorbidity, our percentage was lower [10,11,22]. In our study, we observed that Hashimoto's thyroiditis was the most frequent autoimmune disease, followed by systemic lupus erythematosus, comorbidities that were previously associated with NMOSD [29].…”

“…Although in some studies the seropositivity of AQP4-IgG antibody has been associated with more severe impairment [15,26,27]. Others, similar to our results, showed no association [11,22].…”

“…This could be due to the population pro le of the study location, the city of Salvador, state of Bahia, which concentrates the highest proportion of black people outside Africa [13]. The higher prevalence of NMOSD among Afro-descendant populations explains the sample size of this study, which is comparable to prior multicenter studies that evaluated epidemiological aspects of entire countries [10,11].…”

“…. Other authors found a lower frequency of AQP4-IgG seropositivity [8,11]. This divergence can be attributed to the used and the timing of the antibody test.…”

“…NMOSD is a rare disease with an estimated prevalence of 0.3-4.4/100,000, more frequent in women and Afro-descendants [5]. Few epidemiological studies have been carried out in Europe, Asia, the United States and Latin America [6][7][8][9][10][11][12]. No studies were found in northeastern Brazil or in the State of Bahia, State with the highest percentage of blacks outside Africa [13].…”