Morbi mortality in Brazilian patients with recurrent neuromyelitis optica

Abstract

The chronic form of Devic´s disease with relapsing remitting course was only recognized in the 90´s . Neuromyelitis optica (NMO) affect mainly Asian and African women. The aim of this study was to evaluate the long term prognosis and quality of life in NMO patients from Rio de Janeiro, where the majority of the population is Afro descendent. In a cohort of 1444 patients followed in Hospital da Lagoa since 1990 (Figure 1) we selected NMO by 2006 criteria and analyzed long term disability, health quality of life by SF-36 and mortality. The classification of the NMOSD syndromes (2015) were applied. 122 Recurrent NMO patients, 89.3% women, 70.55 % black, mean disease onset of 31.30 years (3 – 70 years), 18.9% pediatric forms (<18 years) were analyzed. 827 acute events were registered [TM (53%), ON (31%), [ON+TM] (9%), brainstem syndrome (4%), encephalopathy (3%), diencephalic syndrome (0.81%)] . Prevention treatment included azatioprin, micophenolato and rituximabe. At last assessment after the average of 12.7 years ± 8.98 (1-40), 39 % of the patients had paraplegia and bilateral amaurosis (Devic like-syndrome), 83.6% severe bilateral dysfunction and 59.8% severe motor dysfunction. The SF-36 scores were under 50 in all domains. 46 patients died (38%) died, 9 unknown data. They were classified as SDNMO (2015) AQP4-Ab positive (40,2%), negative (23,8%) or unknown (36,1%). NMO represented 11% of all IIDD patients. Recurrent NMO patients developed severe disability, had a high reduction in quality of life and and high mortalily.