Browsing by Author "Sequeira, M"
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- A Case of Kawasaki Disease in PortugalPublication . Proença, R; Sousa, M; Vakil, K; Sequeira, M; Sykes, A; Marques, I; Malhado, JThis case report is believed to be the first case of Kawasaki disease in Portugal. An otherwise healthy 20 years old female was carefully examined and diagnosis of mucocutaneous lyrnphnode syndrome estab lished, based on: typical clinical picture, exclusion of other mimicking situations and middle term evolution of this patient. The A. A. wish to emphasize their diagnosis complied on C. D. C. criteria for Kawasaki disease. A short up dated briefing on this peculiar entity and geographycal pathology are included in this article.
- Late Onset Neuromyelitis Optica Spectrum Disorders (LONMOSD) from a Nationwide Portuguese Study: Anti-AQP4 Positive, Anti-MOG Positive and Seronegative SubgroupsPublication . Santos, E; Moura, J; Samões, R; Sousa, AP; Mendonça, T; Abreu, P; Guimarães, J; Correia, I; Durães, J; Sousa, L; Ferreira, J; de Sá, J; Sousa, F; Sequeira, M; Correia, AS; André, AL; Basílio, C; Arenga, M; Brás Marques, I; Perdigão, S; Alves, I; Santos, M; Salgado, V; Palos, A; Guerreiro, R; Isidoro, L; Boleixa, D; Carneiro, P; Neves, E; Martins Silva, A; Gonçalves, G; Sá, MJIntroduction: Several neuroimmunological disorders have distinct phenotypes according to the age of onset, as in multiple sclerosis or myasthenia gravis. It is also described that late onset NMOSD (LONMOSD) has a different phenotype. Objective: To describe the clinical/demographic characteristics of the LONMOSD and distinguish them from those with early onset (EONMOSD). Methods: From a nationwide Portuguese NMOSD study we analyzed the clinical/demographic characteristics of the LONMOSD. Results: From the 180 Portuguese patients 45 had disease onset after 50 years old, 80% were female. 23 had anti-AQP4 antibodies (51.1%), 13 anti-MOG antibodies (28.9%) and 9 were double seronegative (20.0%). The most common presenting phenotypes in LONMOSD were transverse myelitis (53.3%) and optic neuritis (26.7%), without difference from EONMOSD (p = 0.074). The mean EDSS for LONMOSD was 6.0 (SD=2.8), after a mean follow-up time of 4.58 (SD=4.47) years, which was significantly greater than the mean EDSS of EONMOSD (3.25, SD=1.80)(p = 0.022). Anti-AQP4 antibodies positive LONMOSD patients had increased disability compared to anti-MOG antibodies positive LONMOSD (p = 0.022). The survival analysis showed a reduced time to use a cane for LONMOSD, irrespective of serostatus (p<0.001). Conclusions: LONMOSD has increased disability and faster progression, despite no differences in the presenting clinical phenotype were seen in our cohort.
- Neuromyelitis Optica Spectrum Disorders: a Nationwide Portuguese Clinical Epidemiological StudyPublication . Santos, E; Rocha, AL; Oliveira, V; Ferro, D; Samões, R; Sousa, AP; Figueiroa, S; Mendonça, T; Abreu, P; Guimarães, J; Sousa, R; Melo, C; Correia, I; Durães, J; Sousa, L; Ferreira, J; Sá, J; Sousa, F; Sequeira, M; Correia, AS; André, AL; Basílio, C; Arenga, M; Mendes, I; Brás Marques, I; Perdigão, S; Felgueiras, H; Alves, I; Correia, F; Barroso, C; Morganho, A; Carmona, C; Palavra, F; Santos, M; Salgado, V; Palos, A; Nzwalo, H; Timóteo, A; Guerreiro, R; Isidoro, L; Boleixa, D; Carneiro, P; Neves, E; Martins Silva, A; Gonçalves, G; Leite, MI; Sá, MJIntroduction: Neuromyelitis optica spectrum disorder (NMOSD) is a rare disorder in which astrocyte damage and/or demyelination often cause severe neurological deficits. Objective: To identify Portuguese patients with NMOSD and assess their epidemiological/clinical characteristics. Methods: This was a nationwide multicenter study. Twenty-four Portuguese adult and 3 neuropediatric centers following NMOSD patients were included. Results: A total of 180 patients met the 2015 Wingerchuk NMOSD criteria, 77 were AQP4-antibody positive (Abs+), 67 MOG-Abs+, and 36 seronegative. Point prevalence on December 31, 2018 was 1.71/100,000 for NMOSD, 0.71/100,000 for AQP4-Abs+, 0.65/100,000 for MOG-Abs+, and 0.35/100,000 for seronegative NMOSD. A total of 44 new NMOSD cases were identified during the two-year study period (11 AQP4-Abs+, 27 MOG-Abs+, and 6 seronegative). The annual incidence rate in that period was 0.21/100,000 person-years for NMOSD, 0.05/100,000 for AQP4-Abs+, 0.13/100,000 for MOG-Abs+, and 0.03/100,000 for seronegative NMOSD. AQP4-Abs+ predominated in females and was associated with autoimmune disorders. Frequently presented with myelitis. Area postrema syndrome was exclusive of this subtype, and associated with higher morbidity/mortality than other forms of NMOSD. MOG-Ab+ more often presented with optic neuritis, required less immunosuppression, and had better outcome. Conclusion: Epidemiological/clinical NMOSD profiles in the Portuguese population are similar to other European countries.