Effect of rituximab on disease activity in latin American patients with anti-aquaporin-4 (+) neuromyelitis optica spectrum disorder.

OBJECTIVES: The aim of the present study is to explore the efficacy of rituximab in patients with Neuromyelitis Optica spectrum disorders (NMOsd) with positive AQP4-IgG serostatus. PATIENTS AND METHODS: In this single center retrospective study, we recruited seropositive anti-AQP4 NMOsd patients who received treatment with Rituximab (RTX) for at least 2 years. Demographics were described and annualized relapse rate (AAR) and survival analysis were performed for time to relapse with Rituximab. All p values ≤0.05 we considered statistically significant. RESULTS: A total of 15 patients (100 % female) were identified. Mean age of disease onset was 34 ±â€¯11 years, mean time of disease was 8.11 ±â€¯4.04 years and the median number of relapses was 5 (2-16). Ten patients received an immunosuppressive agent before RTX. Mean age of RTX initiation was 37 ±â€¯12 with a mean treatment duration of 52 ±â€¯28 months. The median ARR before and after treatment with RTX was 2.08 vs 0.00, respectively, with a difference of -2.08 (p < 0.001) CONCLUSIONS: This study shows a statistically significant reduction in the ARR and an increase in the relapse-free rate in AQP4-IgG NMOsd patients treated with RTX. These findings support the use of rituximab in our population, and indirectly suggests that its prompt use could modify the course of the disease.

Doença priônica: relato do primeiro caso notificado da forma esporádica da doença de Creutzfeldt-Jakob em Brasília, Distrito Federal / Creutzfeldt-Jakob disease sporadic form: report of the first notified case in Brasília, Distrito Federal

A doença de Creutzfeldt-Jakob é uma encefalopatia espongiforme causada por príon, que leva à demência rapidamente progressiva e pode se manifestar com ampla heterogeneidade de sintomas neurológicos e psiquiátricos. Relata-se o primeiro caso notificado de paciente do Distrito Federal com o diagnóstico da doença, confirmado por método histopatológico.

Creutzfeldt-Jakob disease is a spongiform encephalopathy caused by prion, leading rapidly to progressive dementia with a wide range of neurologic and psychiatric symptoms. We report the first notified case of a patient resident in Brasília, Distrito Federal, Brazil, with Creutzfeldt-Jakob disease confirmed by pathological study.