Browsing by Author "Santana, I"
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- Diagnostic value of CSF protein profile in a Portuguese population of sCJD patientsPublication . Baldeiras, IE; Ribeiro, MH; Pacheco, P; Machado, A; Santana, I; Cunha, L; Oliveira, CRThe clinical diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) is difficult, and reliable markers are highly desired. In this work we assess the value of several cerebrospinal fluid (CSF) markers for sCJD diagnosis. Within the framework of the Portuguese Epidemiological Surveillance Program for Human Prion Diseases, CSF samples from 71 patients with clinically suspected sCJD, 30 definite sCJD and 41 non-CJD patients, were analysed for the presence of 14-3-3 protein. CSF levels of tau (t-tau), and phosphorylated tau (p-tau181), S-100b and beta amyloid (Abeta42) proteins were determined. The influence of clinical and genetic characteristics on CSF markers sensitivity was also evaluated. Protein 14-3-3 was detected in 29/30 sCJD patients and 9/41 non-CJD patients. Extremely elevated t-tau and S-100b protein levels were found in sCJD patients, while p-tau181 levels were only slightly elevated and Abeta42 showed no differences compared to controls. 14-3-3 was the most sensitive parameter (97%), but its specificity was low (78%); sensitivity/specificity for other proteins were: S-100b-93/93%, t-tau-93/95%, with maximum accuracy being obtained by a combination of tests (14-3-3 combined with either t-tau or S-100b, or combining S-100b with t-tau/Abeta42 or p-tau/t-tau ratios). The sensitivity of 14-3-3, as well as of p-tau181/t-tau ratio, was decreased in younger patients with long disease duration, with the PrP-2 isotype and MV genotype. Both 14-3-3, t-tau and S-100b are sensitive markers for sCJD, but 14-3-3 specificity seems to be lower in this special clinical setting of rapidly progressing dementias. We propose that in cases with a 14-3-3 weak positive result, or in young patients with long disease duration, a second CSF marker would be valuable for the diagnosis of sCJD.
- A large outbreak of Legionnaires’ Disease in an industrial town in PortugalPublication . George, F; Shivaji, T; Pinto, CS; Serra, LA; Valente, J; Albuquerque, MJ; Vicêncio, PC; San-Bento, A; Diegues, P; Nogueira, PJ; Marques, T; Rebelo, H; Costa, F; Rodrigues, R; Nunes, A; Borges, V; Gomes, JP; Sampaio, D; Barreiro, P; Duarte, S; Carpinteiro, D; Mendonça, J; Silva, C; Vieira, L; Simões, MJ; Gonçalves, P; Nunes, B; Dias, C; Machado, J; Almeida, F; Goncalves, EA; Carvalho, L; Viterbo, P; Jardim, D; Lacasta, N; Boavida, F; Perez, A; Santana, I; Matias, P; Banza, N; Rabacal, CBackground We describe the investigation and control of an outbreak of Legionnaires’ disease in Portugal in October, November and December 2014. Methods Confirmed cases were individuals with pneumonia, laboratory evidence of Legionella pneumophila serogroup 1 and exposure, by residence, occupational or leisure to the affected municipalities. 49 possible sources were reduced to four potential sources, all industries with wet cooling system, following risk assessment. We geo-referenced cases’ residences and the location of cooling towers defining four study areas 10 km buffer centered on each cooling tower system. We compared the number of cases with expected numbers, calculated from the outbreak's attack rates applied to 2011 census population. Using Stones’ Test, we tested observed to expected ratios for decline in risk, with distance up to 10 km four directions. Isolates of Legionella pneumophila were compared using molecular methods. Results We identified 403 cases, 377 of which were confirmed, 14 patients died. Patients became ill between 14 October and 2 December. A NE wind and thermal inversion were recorded during the estimated period of exposure. Disease risk was highest in people living south west from all of the industries identified and decreased with distance (p < 0.001). 71 clinical isolates demonstrated an identical SBT profile to an isolate from a cooling tower. Whole genome sequencing identified an unusual L. pneumophila subsp. fraseri serogroup 1 as the outbreak causative strain, and confirmed isolates’ relatedness. Conclusions Industrial wet cooling systems, bacteria with enhanced survival characteristics and a combination of climatic conditions contributed to the second largest outbreak of Legionnaires’ disease recorded internationally.
- Primary Bilateral Thalamic Astrocytoma Presenting With Head Tremor, Ataxia, and DementiaPublication . Machado, A; Ribeiro, M; Rodrigues, M; Ferreira, C; Almeida, R; Santana, I; Castro, L; Carpenter, S
- The Portuguese who could no longer speak French: primary progressive aphasia in a bilingual manPublication . Machado, A; Rodrigues, M; Simões, S; Santana, I; Soares-Fernandes, J