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Viana-Baptista M.,New University of Lisbon | Viana-Baptista M.,Centro Hospitalar Lisbon Ocidental
Journal of Neurology | Year: 2012

Fabry disease (FD) is a rare inherited disorder of the metabolism, associated with renal, cardiac, and cerebrovascular complications. Ischemic and hemorrhagic stroke in FD present with a similar proportion to that observed in the general population, but usually at an early age. Ischemic stroke may result from cardiac embolism, large and small vessel disease, while hemorrhagic stroke is usually attributed to hypertension. Deposition of glycosphingolipids in endothelial cells results in a specific FD vasculopathy that contributes to the different vascular phenotypes. Neuroimaging features of cerebrovascular involvement in FD include white matter lesions, dolichoectasia, and the "pulvinar sign", a T1 MRI hyperintensity of the posterior thalamus. The role of enzymatic replacement therapy in the prevention of stroke remains to be established, but its utilization should be considered in FD stroke patients, for prevention of renal and cardiac complications, together with general prevention measures. Enzymatic replacement therapy increased our awareness of FD, underlining the importance of incomplete phenotypes in specific settings such as stroke. An overview of studies on the prevalence of FD in stroke patients is presented. Available data suggest that prevalence of FD is similar to some of the rare causes of stroke usually considered, and that classic features of the disease may be absent or more subtle. Moreover, FD should be considered in both cryptogenic and all-cause stroke. The role of FD in stroke on a multifactorial basis and the identification of a putative "stroke variant" are questions that need to be further elucidated in future studies. © 2011 Springer-Verlag. Source

Leal A.J.R.,Hospital Julio de Matos | Leal A.J.R.,University of Lisbon | Lopes R.,University of Coimbra | Ferreira J.C.,Centro Hospitalar Lisbon Ocidental
Clinical Neurophysiology | Year: 2013

Objective: The aim of the study was to demonstrate the dynamics and structure of the epileptic network and provide a tentative correlation with the clinical manifestations, in a symptomatic case of Panayiotopoulos syndrome (PS). Methods: JP, 5-year-old girl. Gestational period and developmental milestones were normal. At age 4. years, two episodes of recurrent vomiting, tonic eye deviation and consciousness impairment lasting for about 30. min occurred. Multifocal spikes were apparent over frontal areas in the EEG and MRI demonstrated an inferior parietal lobe (IPL) lesion. Results: A long-term 35-channel scalp EEG was obtained, which was processed with a Blind Source Separation algorithm. The most significant components with a dipolar field were submitted to source analysis and the recovered generators used to build the nodes of a brain network associated with each spike type. Analysis of information flow supported epileptic propagation from the left parietal lobe to both frontal and temporal lobes around spike peak. The good spatial overlap with physiological networks controlling eye movements, autonomic functions and consciousness, provides a tentative explanation to the diverse clinical manifestations of PS. Conclusions: Spreading patterns of epileptic activity form an extended network in PS. Significance: An epileptic focus in an IPL can reproduce both neurophysiological and clinical features of PS. © 2012 International Federation of Clinical Neurophysiology. Source

Nina J.,Centro Hospitalar Lisbon Ocidental | Nina J.,New University of Lisbon
Acta Medica Portuguesa | Year: 2014

Ebolavirosis, like Marburgvirosis, are African zoonosis, and for both the primary animal reservoir are bats. It is a typical acute haemorrhagic fever, characterized by a high lethality rate. In an outbreak, the human index case became infected after contact with an infected animal or its blood, in most cases during hunting. Secondary human cases became infected after close contact with another human case, with infected human fluids or with a recent dead corps of a human case. These viruses are easily transmitted by direct contact or by contact with patient body fluids, mainly blood. As such, health professionals working under suboptimal conditions usually constitute a large share of Ebola victims. At the moment, the treatment is only supportive, but several drugs are almost ready to be tried in human trials. There is no vaccine approved, but again there are several very promising in the pipeline. © Ordem dos Médicos 2014. Source

Caldeira D.,University of Sfax | Amaral T.,Centro Hospitalar Lisbon Ocidental | David C.,University of Sfax | David C.,Centro Hospitalar Lisbon Norte | And 2 more authors.
Journal of Renal Nutrition | Year: 2011

Objective: To systematically review educational strategies for phosphorus reduction in patients with hyperphosphatemia and chronic kidney disease (CKD). Design: Systematic review with meta-analysis. Data Sources: CENTRAL, MEDLINE, EMBASE, and mRCT databases were assessed in June 2010. Study Selection: Randomized controlled trials evaluating educational strategies related to diet in hyperphosphatemic patients with CKD. Data Extraction and Synthesis Method: Study characteristics, phosphorus levels, and calcium-phosphorus product levels were retrieved. Jadad scale was used for quality assessment. Mean difference (MD) and 95% confidence intervals (CIs) were calculated by random effects method. Results: Seven randomized controlled trials were retrieved with a total of 524 patients with hyperphosphatemia and CKD. Educational strategies reduced phosphorus levels with an MD of -0.72 mg/dL (95% CI: -1.11 to -0.33, P < .01). Sensitivity analysis of trials with follow-up of <4 months did not show any benefit of the intervention, but educational intervention for ≥4 months showed an MD of -1.07 (95% CI: -1.49 to -0.64, P < 01). Calcium-phosphorus product level was improved in 227 evaluated patients from 5 trials with an MD of -5.22 mg2/dL2 (95% CI: -9.48 to -0.98, P = 02, and I2 = 58%). Sensitivity analysis removed the source of heterogeneity and resulted in an MD of -3.02 (95% CI: -6.51 to 0.47, P = 09). Conclusions: Education helped reduce phosphorus levels in hyperphosphatemic patients with CKD, particularly those on dialysis. © 2011 National Kidney Foundation, Inc. Source

Parreira R.,New University of Lisbon | Centeno-Lima S.,New University of Lisbon | Lopes A.,New University of Lisbon | Portugal-Calisto D.,Unidade de Clinica Tropical IHMT UNL | And 4 more authors.
Eurosurveillance | Year: 2014

A concurrent dengue virus serotype 4 and chikungunya virus infection was detected in a woman in her early 50s returning to Portugal from Luanda, Angola, in January 2014. The clinical, laboratory and molecular findings, involving phylogenetic analyses of partial viral genomic sequences amplified by RT-PCR, are described. Although the circulation of both dengue and chikungunya viruses in Angola has been previously reported, to our knowledge this is the first time coinfection with both viruses has been detected there. Source

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