Publications by authors named "Miguel Viana-Baptista"

62 Publications

Shotgun mass spectrometry-based lipid profiling identifies and distinguishes between chronic inflammatory diseases.

EBioMedicine 2021 Aug 24;70:103504. Epub 2021 Jul 24.

iNOVA4Health, CEDOC, NOVA Medical School, NMS, Universidade Nova de Lisboa, 1169-056 Lisboa, Portugal. Electronic address:

Background: Localized stress and cell death in chronic inflammatory diseases may release tissue-specific lipids into the circulation causing the blood plasma lipidome to reflect the type of inflammation. However, deep lipid profiles of major chronic inflammatory diseases have not been compared.

Methods: Plasma lipidomes of patients suffering from two etiologically distinct chronic inflammatory diseases, atherosclerosis-related vascular disease, including cardiovascular (CVD) and ischemic stroke (IS), and systemic lupus erythematosus (SLE), were screened by a top-down shotgun mass spectrometry-based analysis without liquid chromatographic separation and compared to each other and to age-matched controls. Lipid profiling of 596 lipids was performed on a cohort of 427 individuals. Machine learning classifiers based on the plasma lipidomes were used to distinguish the two chronic inflammatory diseases from each other and from the controls.

Findings: Analysis of the lipidomes enabled separation of the studied chronic inflammatory diseases from controls based on independent validation test set classification performance (CVD vs control - Sensitivity: 0.94, Specificity: 0.88; IS vs control - Sensitivity: 1.0, Specificity: 1.0; SLE vs control - Sensitivity: 1, Specificity: 0.93) and from each other (SLE vs CVD ‒ Sensitivity: 0.91, Specificity: 1; IS vs SLE - Sensitivity: 1, Specificity: 0.82). Preliminary linear discriminant analysis plots using all data clearly separated the clinical groups from each other and from the controls, and partially separated CVD severities, as classified into five clinical groups. Dysregulated lipids are partially but not fully counterbalanced by statin treatment.

Interpretation: Dysregulation of the plasma lipidome is characteristic of chronic inflammatory diseases. Lipid profiling accurately identifies the diseases and in the case of CVD also identifies sub-classes.

Funding: Full list of funding sources at the end of the manuscript.
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http://dx.doi.org/10.1016/j.ebiom.2021.103504DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8330692PMC
August 2021

Secondary Trigeminal Neuralgia: Do Not Forget the Pontine Ischemic Infarction.

Eur Neurol 2021 1;84(5):391-392. Epub 2021 Jul 1.

Department of Neurology, Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental, Lisbon, Portugal.

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http://dx.doi.org/10.1159/000517361DOI Listing
July 2021

Direct oral anticoagulants in treatment of cerebral venous thrombosis: a systematic review.

BMJ Open 2021 02 16;11(2):e040212. Epub 2021 Feb 16.

Department of Medicine, University of Ottawa and The Ottawa Hospital Research Institute, Ottawa, Ontario, Canada.

Objectives: Current guidelines do not recommend direct oral anticoagulants (DOACs) to treat cerebral venous thrombosis (CVT) despite their benefits over standard therapy. We performed a systematic review to summarise the published experience of DOAC therapy in CVT.

Data Sources: MEDLINE, Embase and COCHRANE databases up to 18 November 2020.

Eligibility Criteria: All published articles of patients with CVT treated with DOAC were included. Studies without follow-up information were excluded.

Data Extraction And Synthesis: Two independent reviewers screened articles and extracted data. A risk of bias analysis was performed.

Primary And Secondary Outcome Measures: Safety data included mortality, intracranial haemorrhage (ICH) or other adverse events. Efficacy data included recurrent CVT, recanalisation rates and disability by modified Rankin Scales (mRS).

Results: 33 studies met inclusion criteria. One randomised controlled trial, 5 observational cohorts and 27 case series or studies reported 279 patients treated with DOAC for CVT: 41% dabigatran, 47% rivaroxaban, 10% apixaban and 2% edoxaban, in addition to 315 patients treated with standard therapy. The observational cohorts showed a similar risk of death in DOAC and standard therapy arms (RR 2.12, 95% CI 0.29 to 15.59). New ICH was reported in 2 (0.7%) DOAC-treated patients and recurrent CVT occurred in 4 (1.5%). A favourable mRS between 0 and 2 was reported in 94% of DOAC-treated patients, more likely than standard therapy in observational cohorts (RR 1.13, 95% CI 1.02 to 1.25).

Conclusion: The evidence for DOAC use in CVT is limited although suggests sufficient safety and efficacy despite variability in timing and dose of treatment. This systematic review highlights that further rigorous trials are needed to validate these findings and to determine optimal treatment regimens.
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http://dx.doi.org/10.1136/bmjopen-2020-040212DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7888326PMC
February 2021

Prognostic value of kappa free light chains determination in first-ever multiple sclerosis relapse.

J Neuroimmunol 2020 10 5;347:577355. Epub 2020 Aug 5.

Neurology Department, Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental, Portugal; NOVA Medical School, Universidade Nova de Lisboa, Portugal.

Given its highly variable clinical course, an unmet need for objective prognostic assessment in Multiple Sclerosis (MS) persists. In this work, we suggest that CSF kappa free light chains (KFLC) determination at first relapse may provide insight into future disease activity and disability worsening. We quantified KFLC by nephelometry in paired CSF/serum samples of 28 patients, collected within one month of first-ever MS relapse, and explored correlations with clinical data on disease activity, retrospectively registered across a median follow-up time of 79 months. We documented KFLC ratio (CSF-FKLC/Serum-KFLC) as an independent predictor of second relapse occurrence and disability worsening at follow-up, in this cohort.
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http://dx.doi.org/10.1016/j.jneuroim.2020.577355DOI Listing
October 2020

Letter by Ramos et al Regarding Article, "Neuroimaging in Ischemic Stroke Is Different Between Men and Women in the DEFUSE 3 Cohort".

Stroke 2020 05 26;51(5):e83. Epub 2020 Mar 26.

Department of Neurology, Hospital de Egas Moniz, Centro Hospitalar Lisboa Ocidental, Portugal.

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http://dx.doi.org/10.1161/STROKEAHA.120.029009DOI Listing
May 2020

Posterior reversible encephalopathy syndrome associated with the use of chemotherapeutic agents: a rare complication after treatment with vinorelbine.

BMJ Case Rep 2020 Feb 16;13(2). Epub 2020 Feb 16.

Neurology, Centro Hospitalar de Lisboa Ocidental EPE, Lisboa, Portugal.

The posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological syndrome characterised by a combination of headache, encephalopathy, seizures and visual disturbances, associated with high-intensity abnormalities on T2-weighted images affecting subcortical white and grey matter of the occipital and parietal lobes. Among other causes, PRES has been associated with the use of several medications including chemotherapeutic agents. Here we report a case of a 65-year-old patient with squamous cell carcinoma of the lung treated with cisplatin/vinorelbine. Following the second administration of vinorelbine, she was admitted to the hospital for a generalised seizure. Blood pressure was just slightly elevated and, except for drowsiness, she had a near-normal neurological examination. MRI corroborated the diagnosis. Vinorelbine-induced PRES has been reported only once in the literature, also in association with cisplatin. Our case underlines the role of vinorelbine and suggests that its association with cisplatin in this setting may enhance the risk of PRES.
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http://dx.doi.org/10.1136/bcr-2019-229319DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7046420PMC
February 2020

Undetermined stroke genesis and hidden cardiomyopathies determined by cardiac magnetic resonance.

Neurology 2020 01 2;94(1):e107-e113. Epub 2019 Dec 2.

From the Department of Neurology (A.C.F., D.P., P.N.A., T.P.e.M., J.M.F.), Hospital de Santa Maria, Institute of Molecular Medicine, University of Lisboa; Department of Neurology (J.P.M., M.V.-B.), Hospital Egas Moniz; Department of Cardiology (T.G., F.J.P., A.G.A.), Hospital de Santa Maria, University of Lisboa; and Department of Neurology (N.I.), Hospital Beatriz Ângelo, Lisbon, Portugal.

Objective: To determine whether cardiac magnetic resonance imaging (CMR) could be useful in identifying previously undiagnosed cardiomyopathies in a cohort of patients with ischemic stroke who underwent standard etiologic investigation and to describe the type and frequency of these cardiomyopathies.

Methods: We performed a subanalysis of a previously collected prospective cohort of patients with ischemic stroke. Patients with structural changes on echocardiography that are considered causal for stroke in the Trial of Org 10172 in Acute Stroke Treatment (TOAST) classification were excluded. A 3T CMR was performed. We compared the frequency of the cardiomyopathies that we found with reference values for the general population.

Results: One hundred thirty-two patients with a mean age of 68.4 years were included. In 7 patients (5.3%, 95% confidence interval 2.59%-10.54%) CMR identified cardiomyopathy. Four patients had hypertrophic cardiomyopathy, 2 had restrictive cardiomyopathy, and 1 had noncompaction cardiomyopathy. Six of these patients had been classified after standard evaluation as having undetermined stroke and 1 patient as having cardioembolic stroke (atrial fibrillation). We found a higher frequency of hypertrophic cardiomyopathy in the entire cohort and in the undetermined cause group compared to the general population (3.03% and 5.81% vs 0.2%, respectively, = 0.001 and < 0.001). The frequency of noncompaction cardiomyopathy was also higher in our cohort (0.76% vs 0.05%, respectively, < 0.001).

Conclusions: Although rare, cardiomyopathies should be considered as a possible cause of ischemic stroke classified as of undetermined etiology after standard evaluation.
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http://dx.doi.org/10.1212/WNL.0000000000008698DOI Listing
January 2020

Vascular White Matter Lesions in Young Adults: A Neurology Outpatient Clinic Registry.

Eur Neurol 2019 21;82(1-3):23-31. Epub 2019 Nov 21.

Department of Neuroradiology, Hospital Egas Moniz, Centro Hospitalar de Lisboa Ocidental, Lisboa, Portugal.

Introduction: Although frequently assumed to be age-related changes, vascular white matter lesions (WML) are sometimes found in young adults. Etiology is usually attributed to sporadic small vessel disease; nevertheless, genetic disorders may also be implicated. We aimed to characterize the population of young adults with vascular WML in Neurology outpatient clinics.

Methods: Neurologists from 12 Portuguese hospitals were invited to include patients aged 18-55 years evaluated in consultation, with vascular WML on MRI, scoring II or III in the Fazekas scale. Central imaging validation was performed by 2 independent, blinded, Neuroradiologists. Demographic and clinical data were collected as well as results of investigations performed.

Results: During 2 years, 77 patients were included (mean age 47.7 years). Vascular risk factors were present in 88.3% patients (hypertension in 53.2%) and previous history of stroke in 36.4%. Patients without history of stroke were younger (46.6 ± 7.2 vs. 49.6 ± 3.9 years, p = 0.045) and had fewer vascular risk factors (p < 0.001). They were more frequently females (87.8 vs. 46.4%, p < 0.001), and headache (30.6 vs. 3.6%, p = 0.007), contrary to focal symptoms (16.3 vs. 53.6%, p = 0.001), was the most frequent reason of referral. Etiological investigations performed differed between Neurologists. A genetic disorder was identified in 6 out of 58 patients (CADASIL n = 5; COL4A1 n = 1).

Conclusion: Young adults with vascular WML evaluated in Neurology outpatient clinics concentrate in the oldest age groups. Vascular risk factors should be screened carefully in this population. Among patients without history of stroke, females largely outweigh males. Diagnostic investigations performed do not follow a standardized protocol.
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http://dx.doi.org/10.1159/000504409DOI Listing
June 2020

Global Outcome Assessment Life-long after stroke in young adults initiative-the GOAL initiative: study protocol and rationale of a multicentre retrospective individual patient data meta-analysis.

BMJ Open 2019 11 14;9(11):e031144. Epub 2019 Nov 14.

Neurology Department, University of Maryland School of Medicine, Baltimore, Maryland, USA.

Introduction: Worldwide, 2 million patients aged 18-50 years suffer a stroke each year, and this number is increasing. Knowledge about global distribution of risk factors and aetiologies, and information about prognosis and optimal secondary prevention in young stroke patients are limited. This limits evidence-based treatment and hampers the provision of appropriate information regarding the causes of stroke, risk factors and prognosis of young stroke patients.

Methods And Analysis: The Global Outcome Assessment Life-long after stroke in young adults (GOAL) initiative aims to perform a global individual patient data meta-analysis with existing data from young stroke cohorts worldwide. All patients aged 18-50 years with ischaemic stroke or intracerebral haemorrhage will be included. Outcomes will be the distribution of stroke aetiology and (vascular) risk factors, functional outcome after stroke, risk of recurrent vascular events and death and finally the use of secondary prevention. Subgroup analyses will be made based on age, gender, aetiology, ethnicity and climate of residence.

Ethics And Dissemination: Ethical approval for the GOAL study has already been obtained from the Medical Review Ethics Committee region Arnhem-Nijmegen. Additionally and when necessary, approval will also be obtained from national or local institutional review boards in the participating centres. When needed, a standardised data transfer agreement will be provided for participating centres. We plan dissemination of our results in peer-reviewed international scientific journals and through conference presentations. We expect that the results of this unique study will lead to better understanding of worldwide differences in risk factors, causes and outcome of young stroke patients.
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http://dx.doi.org/10.1136/bmjopen-2019-031144DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6887075PMC
November 2019

Autonomic nervous system response to remote ischemic conditioning: heart rate variability assessment.

BMC Cardiovasc Disord 2019 09 9;19(1):211. Epub 2019 Sep 9.

CEDOC - NOVA Medical School, Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Campo Mártires da Pátria, 130, 1169-056, Lisboa, Portugal.

Background: Remote ischemic conditioning (RIC) is a procedure applied in a limb for triggering endogenous protective pathways in distant organs, namely brain or heart. The underlying mechanisms of RIC are still not fully understood, and it is hypothesized they are mediated either by humoral factors, immune cells and/or the autonomic nervous system. Herein, heart rate variability (HRV) was used to evaluate the electrophysiological processes occurring in the heart during RIC and, in turn to assess the role of autonomic nervous system.

Methods: Healthy subjects were submitted to RIC protocol and electrocardiography (ECG) was used to evaluate HRV, by assessing the variability of time intervals between two consecutive heart beats. This is a pilot study based on the analysis of 18 ECG from healthy subjects submitted to RIC. HRV was characterized in three domains (time, frequency and non-linear features) that can be correlated with the autonomic nervous system function.

Results: RIC procedure increased significantly the non-linear parameter SD2, which is associated with long term HRV. This effect was observed in all subjects and in the senior (> 60 years-old) subset analysis. SD2 increase suggests an activation of both parasympathetic and sympathetic nervous system, namely via fast vagal response (parasympathetic) and the slow sympathetic response to the baroreceptors stimulation.

Conclusions: RIC procedure modulates both parasympathetic and sympathetic autonomic nervous system. Furthermore, this modulation is more pronounced in the senior subset of subjects. Therefore, the autonomic nervous system regulation could be one of the mechanisms for RIC therapeutic effectiveness.
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http://dx.doi.org/10.1186/s12872-019-1181-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6734354PMC
September 2019

Bismuth encephalopathy- a rare complication of long-standing use of bismuth subsalicylate.

BMC Neurol 2019 Aug 29;19(1):212. Epub 2019 Aug 29.

Neurology Department. Hospital Egas Moniz, Centro Hospitalar de Lisboa Ocidental, Lisbon, Portugal.

Background: Drugs containing bismuth, although usually safe, may rarely cause neurotoxicity.

Case Presentation: We describe the case of a 44-year-old woman treated with bismuth subsalicylate for about 20 years, who developed abnormal behaviour and postural instability in two weeks. On examination, she had greyish discoloration of teeth, was confused and presented generalized myoclonic jerks. In the next days, her clinical condition deteriorated, with a reduction in alertness and more exuberant myoclonus. Brain MRI was unremarkable. CSF revealed mild elevation of protein content (47 mg/dL; reference range: 15-45 mg/dL) and elevation of white blood cell count (10/μL). Bismuth levels in urine (375 μg/L), serum (260 μg/L) and CSF (21.4 μg/L) samples were highly above the threshold for toxicity. Following supportive treatment and bismuth discontinuation, she made a full recovery within weeks.

Conclusions: Although rare, bismuth encephalopathy should be considered in patients presenting with subacute encephalopathy and myoclonus. This encephalopathy can be subacute even after a chronic exposure. Cessation of bismuth can lead to a complete resolution in weeks.
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http://dx.doi.org/10.1186/s12883-019-1437-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6714398PMC
August 2019

Intravenous Thrombolysis for Acute Ischemic Stroke After Recent Myocardial Infarction: Case Series and Systematic Review.

Stroke 2019 10 22;50(10):2813-2818. Epub 2019 Aug 22.

Stroke Unit, Department of Neurology, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Portugal (L.A.K., T.P.-e.-M., C.F.).

Background and Purpose- The safety of IV r-tPA (intravenous tissue-type plasminogen activator) for acute ischemic stroke (AIS) treatment after recent myocardial infarction (MI) is still a matter of debate. We studied the safety of delivering IV r-tPA to AIS patients with a MI within the preceding 3 months. Methods- Retrospective review of consecutive AIS admitted to 2 tertiary university hospitals' and systematic literature review for AIS patients with history of MI in the previous 3 months. Patients were divided into 2 groups: treated or not treated with standard IV r-tPA dose for AIS. Cardiac complications (cardiac rupture/tamponade, intracardiac thrombus embolization, or life-threatening arrhythmias) were compared between groups and assessed by type of MI (non-ST-segment-elevation myocardial infarction [STEMI], or STEMI) and time elapsed between vascular events. Results- One hundred and two patients were included; 46 (45.1%) were derived from literature review. Median age (interquartile range) was 64 (53-75) years old, and 69 (67.6%) were men. Forty-seven (46.1%) received IV r-tPA. In the treated group, 25 (53.2%) and 23 (48.9%) patients had, respectively, concurrent AIS and MI and STEMI, in comparison with 12 (21.8%; =0.002) and 36 (65.5%; =0.110) patients in the nontreated. Four (8.5%) IV r-tPA-treated patients died from confirmed or presumed cardiac rupture/ tamponade, all with a STEMI in the week preceding stroke. This complication occurred in 1 (1.8%) patients in the nontreated group (=0.178). There were no differences in thrombus embolization (1 [2.1%) versus 2 [3.6]; =1.000) and life-threatening arrhythmias (3 [6.4%) versus 7 [12.7]; =0.335). No non-STEMI patients receiving IV r-tPA had cardiac complications. Conclusions- In patients with AIS and recent or concurrent MI, MI type and the time elapsed between the 2 events should be taken into consideration when deciding to deliver IV r-tPA. Although recent non-STEMI or concurrent events seem safe, STEMI in the week preceding stroke should prompt caution. The low number of events and publication bias may have influenced our conclusions.
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http://dx.doi.org/10.1161/STROKEAHA.119.025630DOI Listing
October 2019

Underuse of Prophylactic Treatment Among Portuguese Patients with Primary Headache: A Retrospective Observational Study.

J Oral Facial Pain Headache 2019 24;33(3):331–336. Epub 2019 Apr 24.

Aims: To evaluate prescription of prophylactic treatment before and after consultation in a neurology headache clinic and to determine predictors for prophylactic treatment and clinical improvement.

Methods: Clinical records of consecutive patients assessed in a neurologic headache clinic in Portugal and diagnosed with acute or chronic migraine and/or tension-type headache were assessed. Prescription of prophylaxis before and after the first visit to the clinic were compared. Logistic regression was used to evaluate predictors of the need for therapeutic intervention and clinical improvement.

Results: Among 409 patients (86.8% women; mean age 41.6 years), 315 (77%) had indication for prophylaxis, and 70 (22%) of these patients were already on prophylactic treatment. Among the 265 patients with information for follow-up, prophylactic treatment was added in 178 (67.2%), and there was a significant change in the number of treated patients between the first and second visits. Ongoing treatment was switched or the dose increased in 21 patients. Multivariate logistic regression revealed that women (odds ratio [OR] = 2.09, 95% confidence interval [CI] 1.1 to 3.97] and patients with medication overuse headache (MOH) (OR = 6.97, 95% CI 1.60 to 30.39) were more likely to need therapeutic intervention, whereas patients referred from the emergency room were less likely to need it (OR = 0.44, 95% CI 0.22 to 0.89). Of the 265 patients, 185 (69.8%) had improved at a follow-up. Having prophylactic treatment at the time of the second visit was associated with improvement (OR = 2.39, 95% CI 1.23 to 4.63; P = .01).

Conclusion: Women and medication overuse headache patients were more likely to need therapeutic intervention. However, only a minority of patients with treatment indication were treated before their first visit to the headache clinic. Prophylaxis prescription was associated with clinical improvement at follow-up.
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http://dx.doi.org/10.11607/ofph.2122DOI Listing
November 2019

The effect of enzyme replacement therapy on clinical outcomes in male patients with Fabry disease: A systematic literature review by a European panel of experts.

Mol Genet Metab Rep 2019 Jun 6;19:100454. Epub 2019 Feb 6.

Department of Paediatrics, University of Torino, Torino, Italy.

Background: Enzyme replacement therapy (ERT) with recombinant human α-galactosidase has been available for the treatment of Fabry disease since 2001 in Europe and 2003 in the USA. Treatment outcomes with ERT are dependent on baseline patient characteristics, and published data are derived from heterogeneous study populations.

Methods: We conducted a comprehensive systematic literature review of all original articles on ERT in the treatment of Fabry disease published up until January 2017. This article presents the findings in adult male patients.

Results: Clinical evidence for the efficacy of ERT in adult male patients was available from 166 publications including 36 clinical trial publications. ERT significantly decreases globotriaosylceramide levels in plasma, urine, and in different kidney, heart, and skin cell types, slows the decline in estimated glomerular filtration rate, and reduces/stabilizes left ventricular mass and cardiac wall thickness. ERT also improves nervous system, gastrointestinal, pain, and quality of life outcomes.

Conclusions: ERT is a disease-specific treatment for patients with Fabry disease that may provide clinical benefits on several outcomes and organ systems. Better outcomes may be observed when treatment is started at an early age prior to the development of organ damage such as chronic kidney disease or cardiac fibrosis. Consolidated evidence suggests a dose effect. Data described in male patients, together with female and paediatric data, informs clinical practice and therapeutic goals for individualized treatment.
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http://dx.doi.org/10.1016/j.ymgmr.2019.100454DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6365982PMC
June 2019

Analysis of riluzole's profile of use in a Central Hospital in Lisbon.

Patient Prefer Adherence 2018 6;12:2357-2361. Epub 2018 Nov 6.

Neurology Department, Centro Hospitalar de Lisboa Ocidental, Lisbon, Portugal.

Purpose: Riluzole is indicated to prolong life or delay the institution of mechanical ventilation in patients with amyotrophic lateral sclerosis (ALS). Clinical studies have shown that this drug prolongs survival, defined as living patients who are not intubated for mechanical ventilation and without tracheotomy. The purpose of this study is to characterize riluzole's use as well as the user population in order to contribute to a rational and safe use.

Patients And Methods: Descriptive, observational, retrospective study describing and characterizing the use of riluzole in ALS patients between July 2006 and December 2016 conducted in a Lisbon's Central Hospital.

Results: Over the course of the study period, 77 patients with different phenotypes of ALS received riluzole. The majority of patients (63%, n=49) were male. The median survival was 10.1 months, but 12 patients (16%) remained on therapy for more than 3 years; 65% of patients were lost to follow-up. The mean adherence rate was 91.2%, and the median adherence rate was 99.3%. One patient discontinued therapy due to gastrointestinal intolerance. Dyspnea and cough were the most common side effects, with roughly one third of patients experiencing each, followed by asthenia and hepatic effects.

Conclusion: Despite the extended enrollment period, only 77 patients met the criteria for study inclusion. Nonetheless, statistical data regarding our population is in accordance with reported international data. High adherence rates were observed, but 14% of patients discontinued riluzole. In such cases, assessment by a multidisciplinary team is warranted.
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http://dx.doi.org/10.2147/PPA.S167861DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6229141PMC
November 2018

Effect of patients' expectations on clinical response to fampridine treatment.

Neurol Sci 2019 Jan 29;40(1):175-180. Epub 2018 Oct 29.

Department of Neurology, Hospital Egas Moniz, Centro Hospitalar de Lisboa Ocidental, Serviço de Neurologia, Hospital Egas Moniz, Rua da Junqueira 126, 1349-019, Lisbon, Portugal.

Introduction: Patient expectation of treatment outcome is one of the primary mechanisms underlying the placebo effect. In multiple sclerosis trials with symptomatic treatments, a robust placebo effect is observed, which might be related to patient expectations. The aim of this study was to evaluate whether patient expectations regarding fampridine treatment influence the clinical response after 4 weeks and 6 months of treatment.

Materials And Methods: We designed and carried out a prospective study from June 2015 to August 2017. Before treatment, patients completed a questionnaire including a scale evaluating their expectations regarding the treatment. The effect of baseline positive expectancy on the response status after 4 weeks and 6 months of treatment was analyzed through univariable and, when applicable, multivariable analysis.

Results: A total of 47 consecutive patients were included in the study. At week 4, 37 (78.7%) patients were classified as responders; a one-point increase in the positive expectancy questionnaire was significantly associated with a fourfold increase in the likelihood of being a responder [OR = 4.020 (95% CI 1.082-14.933); p = 0.038]. At 6 months, 43 patients completed follow-up. The number of responders decreased to 28; at this point, positive expectancy at baseline was no longer associated with response status.

Conclusion: Baseline positive expectancy regarding fampridine was determinant of the clinical response after 4 weeks of treatment. However, in the long term, fampridine efficacy was not dependent on expectations prior to treatment.
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http://dx.doi.org/10.1007/s10072-018-3613-xDOI Listing
January 2019

Women Who Have Ischemic Strokes Have a Higher Burden of Left Atrial Fibrosis Than Men.

Stroke 2018 11;49(11):2584-2589

Department of Cardiology (A.G.A.), Hospital de Santa Maria, University of Lisboa, Portugal.

Background and Purpose- Women with atrial fibrillation (AF) have a higher risk of stroke than men who have AF. Atrial fibrosis is a marker of atrial disease that precedes the appearance of AF increasing the risk of ischemic stroke. We aimed to determine whether female sex is independently associated with left atrial fibrosis in stroke patients. Methods- We prospectively included a consecutive sample of ischemic stroke patients aged over 50 years of age. Late gadolinium enhancement cardiac magnetic resonance imaging was performed to quantify the severity of left atrial fibrosis and the wall pattern of its distribution. A multivariable linear regression analysis was performed to determine whether female sex was independently associated with left atrial fibrosis after adjusting for potential confounders namely AF and age. Results- One hundred twenty-four patients were deemed eligible; 117 patients were included (7 were excluded because of cardiomyopathy identified by cardiac magnetic resonance imaging). All had usable cardiac magnetic resonance imaging data. Fifty-three patients (45.3%) were women. Women were older and were less frequently treated with angiotensin-converting-enzyme inhibitors. Ninety-one patients had any degree of atrial fibrosis. Women had a higher percentage of atrial fibrosis than men-median (interquartile range)-18% (17) versus 10% (20). In a multivariable linear regression model adjusted for demographics, medications, AF, comorbidities, and cardiac parameters, female sex was found to be independently associated with left atrial fibrosis. Women were found to have more 4.70% of left atrial fibrosis than men (95% CI, 0.70-8.71%; P=0.02) after controlling for confounders. Conclusions- Female sex was found to be independently associated with left atrial fibrosis after controlling for confounders such as AF and age. Further studies are needed to understand if this contributes to the increased stroke risk related to AF in women compared with men.
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http://dx.doi.org/10.1161/STROKEAHA.118.022105DOI Listing
November 2018

Cholesterol is Inefficiently Converted to Cholesteryl Esters in the Blood of Cardiovascular Disease Patients.

Sci Rep 2018 10 3;8(1):14764. Epub 2018 Oct 3.

CEDOC, NOVA Medical School, Faculdade de Ciências Médicas, Universidade NOVA de Lisboa, 1169-056, Lisboa, Portugal.

Shotgun lipidomic analysis of 203 lipids in 13 lipid classes performed on blood plasma of donors who had just suffered an acute coronary syndrome (ACS, n = 74), or an ischemic stroke (IS, n = 21), or who suffer from stable angina pectoris (SAP, n = 78), and an age-matched control cohort (n = 52), showed some of the highest inter-lipid class correlations between cholesteryl esters (CE) and phosphatidylcholines (PC) sharing a common fatty acid. The concentration of lysophospatidylcholine (LPC) and ratios of concentrations of CE to free cholesterol (Chol) were also lower in the CVD cohorts than in the control cohort, indicating a deficient conversion of Chol to CE in the blood plasma in the CVD subjects. A non-equilibrium reaction quotient, Q', describing the global homeostasis of cholesterol as manifested in the blood plasma was shown to have a value in the CVD cohorts (Q' = 0.217 ± 0.084; Q' = 0.201 ± 0.084; Q' = 0.220 ± 0.071) that was about one third less than in the control cohort (Q' = 0.320 ± 0.095, p < 1 × 10), suggesting its potential use as a rapid predictive/diagnostic measure of CVD-related irregularities in cholesterol homeostasis.
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http://dx.doi.org/10.1038/s41598-018-33116-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6170447PMC
October 2018

European expert consensus statement on therapeutic goals in Fabry disease.

Mol Genet Metab 2018 07 12;124(3):189-203. Epub 2018 Jun 12.

Department of Neurology, University of Erlangen-Nuremberg, Erlangen, Germany.

Background: Fabry disease, an inherited lysosomal storage disorder, causes multi-organ pathology resulting in substantial morbidity and a reduced life expectancy. Although Fabry disease is an X-linked disorder, both genders may be affected, but generally to a lesser extent in females. The disease spectrum ranges from classic early-onset disease to non-classic later-onset phenotypes, with complications occurring in multiple organs or being confined to a single organ system depending on the stage of the disease. The impact of therapy depends upon patient- and disease-specific factors and timing of initiation.

Methods: A European panel of experts collaborated to develop a set of organ-specific therapeutic goals for Fabry disease, based on evidence identified in a recent systematic literature review and consensus opinion.

Results: A series of organ-specific treatment goals were developed. For each organ system, optimal treatment strategies accounted for inter-patient differences in disease severity, natural history, and treatment responses as well as the negative burden of therapy and the importance of multidisciplinary care. The consensus therapeutic goals and proposed patient management algorithm take into account the need for early disease-specific therapy to delay or slow the progression of disease as well as non-specific adjunctive therapies that prevent or treat the effects of organ damage on quality of life and long-term prognosis.

Conclusions: These consensus recommendations help advance Fabry disease management by considering the balance between anticipated clinical benefits and potential therapy-related challenges in order to facilitate individualized treatment, optimize patient care and improve quality of life.
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http://dx.doi.org/10.1016/j.ymgme.2018.06.004DOI Listing
July 2018

Evidence of amyloid-β cerebral amyloid angiopathy transmission through neurosurgery.

Acta Neuropathol 2018 05 15;135(5):671-679. Epub 2018 Feb 15.

Division of Neuropathology, The National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square, London, WC1N 3BG, UK.

Amyloid-β (Aβ) is a peptide deposited in the brain parenchyma in Alzheimer's disease and in cerebral blood vessels, causing cerebral amyloid angiopathy (CAA). Aβ pathology is transmissible experimentally in animals and through medical procedures in humans, such as contaminated growth hormone or dura mater transplantation in the context of iatrogenic prion disease. Here, we present four patients who underwent neurosurgical procedures during childhood or teenage years and presented with intracerebral haemorrhage approximately three decades later, caused by severe CAA. None of these patients carried pathogenic mutations associated with early Aβ pathology development. In addition, we identified in the literature four patients with a history of neurosurgical intervention and subsequent development of CAA. These findings raise the possibility that Aβ pathology may be transmissible, as prion disease is, through neurosurgical procedures.
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http://dx.doi.org/10.1007/s00401-018-1822-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5904220PMC
May 2018

Portuguese Observational Study of Ischaemic Stroke in Patients Medicated with Non-Vitamin K Antagonist Oral Anticoagulants.

Eur Neurol 2018 8;79(1-2):108-112. Epub 2018 Feb 8.

Department of Neurology, Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal.

Introduction: Clinical trials and subsequent meta-analyses showed advantages of non-vitamin K antagonists oral anticoagulants (NOACs) over vitamin K antagonists (VKAs) in patients with non-valvular atrial fibrillation. The impact of preadmission anticoagulation in acute ischaemic stroke (AIS) has not been established.

Objective: To compare functional outcome of patients with AIS with preadmission NOACs vs. VKAs.

Methods: A retrospective analysis was conducted on consecutive AIS patients under oral anticoagulation (VKAs or NOACs) admitted in 4 Portuguese hospitals within a period of 30 months. Two primary outcomes were defined and compared between VKA and NOAC groups: symptomatic intracerebral hemorrhage transformation (sICH) and modified Rankin Scale (mRS) at 3 months.

Results: Four hundred sixty-nine patients were included, of whom 332 (70.8%) were treated with VKA and 137 (29.2%) with NOAC. Patients' median age was 78.0 and 234 (49.9%) were male. NOAC-treated patients had a higher median CHA2DS2-VASc score than those under VKA (5.0 vs. 4.0, p = 0.023). The two primary outcomes showed no statistical differences between the VKAs' group and the NOACs' group (sICH: 5.4 vs. 5.4% [p = 0.911]; mRS at 3 months: 3.0 vs. 3.0 [p = 0.646], respectively).

Conclusion: Preadmission anticoagulation with NOACs in AIS has a functional impact similar to that of VKAs.
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http://dx.doi.org/10.1159/000486755DOI Listing
January 2019

Patients With Undetermined Stroke Have Increased Atrial Fibrosis: A Cardiac Magnetic Resonance Imaging Study.

Stroke 2018 03 25;49(3):734-737. Epub 2018 Jan 25.

From the Department of Neurology (A.C.F., P.A., T.P.-e.-M., J.M.F.) and Department of Cardiology (A.G.A.), Hospital de Santa Maria, University of Lisbon, Portugal; Department of Neurology, Hospital Beatriz Ângelo, Loures, Portugal (N.I.); and Department of Neurology, Hospital Egas Moniz, Lisboa, Portugal (J.P.M., M.V.-B.).

Background And Purpose: Some patients with ischemic strokes that are currently classified as having an undetermined cause may have structural or functional changes of the left atrium (LA) and left atrial appendage, which increase their risk of thromboembolism. We compared the LA and left atrial appendage of patients with different ischemic stroke causes using cardiac magnetic resonance imaging.

Methods: We prospectively included a consecutive sample of ischemic stroke patients. Patients with structural changes on echocardiography currently considered as causal for stroke in the Trial of ORG 10172 in Acute Stroke Treatment (TOAST) classification were excluded. A 3-T cardiac magnetic resonance imaging was performed.

Results: One hundred and eleven patients were evaluated. Patients with an undetermined cause had a higher percentage of LA fibrosis (=0.03) than patients with other stroke causes and lower, although not statistically significant, values of LA ejection fraction. Patients with atrial fibrillation and undetermined stroke cause showed a similar value of atrial fibrosis.

Conclusions: The LA phenotype that was found in patients with undetermined cause supports the hypothesis that an atrial disease may be associated with stroke.
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http://dx.doi.org/10.1161/STROKEAHA.117.019641DOI Listing
March 2018

Cerebral amyloid angiopathy - The modified Boston criteria in clinical practice.

J Neurol Sci 2018 Jan 20;384:55-57. Epub 2017 Nov 20.

Department of Neurology, Hospital Egas Moniz, Centro Hospitalar de Lisboa Ocidental, Rua da Junqueira, 126, Lisbon 1349-019, Portugal; CEDOC - Chronic Diseases Research Center, Nova Medical School/Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisbon, Portugal.

Background: Early identification of patients with cerebral amyloid angiopathy (CAA) is relevant considering the increased risk for cerebral hemorrhage. A new set of diagnostic criteria for CAA was recently proposed, which include the presence of superficial siderosis. We aimed to assess the impact of applying these criteria regarding use of antithrombotic therapy.

Methods: Review of consecutive patients admitted to a Neurology Department from 2014 to 2016, with acute parenchymal or subarachnoid hemorrhage and/or atypical transient focal neurological episodes. Patients with a possible or probable CAA according to the original and modified Boston criteria were included. Information was collected regarding presentation, imaging findings and concomitant therapy.

Results: Among a total of 1436 admitted patients, 52 with acute hemorrhagic lesions or atypical TFNE were screened: 22 met criteria for CAA; 4 were deemed too young; 21 had other causes for hemorrhagic parenchymal lesions; and 5 had uncertain diagnosis. Using the modified Boston criteria, 8 patients fulfilled criteria for probable CAA and 14 for possible CAA. When we applied the original Boston criteria to the same patients, only 7 fulfilled criteria for probable CAA and 8 for possible CAA. Among the additional patients identified with the modified Boston criteria, 4 were using antithrombotic therapy.

Conclusions: The use of the modified Boston criteria allowed for the identification of 7 additional patients, more than half of which were taking antithrombotic therapy. Systematic use of these criteria could have an important impact in clinical practice. Raising awareness on the different presentations of CAA among clinicians is of the utmost importance.
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http://dx.doi.org/10.1016/j.jns.2017.11.021DOI Listing
January 2018

Letter to the Editor: Medication-Overuse Headache is Common in Portuguese Migraineurs.

Acta Med Port 2017 09 29;30(9):664-665. Epub 2017 Sep 29.

Headache Study Group. Centro Hospitalar de Lisboa Ocidental. Lisboa. Portugal.

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http://dx.doi.org/10.20344/amp.9076DOI Listing
September 2017

Capsular warning syndrome: The role of blood pressure.

J Neurol Sci 2017 10 9;381:20-21. Epub 2017 Aug 9.

Neurology Department, Hospital de Egas Moniz, Centro Hospitalar de Lisboa Ocidental, Portugal; CEDOC, Chronic Diseases Research Center, Nova Medical School, Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisbon, Portugal.

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http://dx.doi.org/10.1016/j.jns.2017.08.008DOI Listing
October 2017

Prediabetes Is Associated with Unfavorable Outcome in Young Adult Patients with Ischemic Stroke.

J Stroke Cerebrovasc Dis 2018 Feb 28;27(2):352-356. Epub 2017 Sep 28.

Department of Neurology, Hospital Egas Moniz, Lisbon, Portugal.

Background: Prediabetes has been associated with unfavorable short-term outcome in patients with ischemic stroke (IS). However, its effect in the subset of young adult patients has not been fully assessed. Our aim was to study the association between prediabetes and 3-month outcome in young adult patients with IS.

Methods: This is a retrospective analysis of consecutive patients aged 18-55 years with a clinical diagnosis of acute IS between January 2010 and December 2016. According to their glucose profile, patients were divided in 3 groups: normal glucose metabolism, prediabetes, and diabetes. The outcome at 3 months was assessed by the modified Rankin Scale (mRS) and dichotomized as good (mRS score ≤2) and poor (mRS score >2) outcomes.

Results: A total of 247 patients were included, the median age was 49 years (interquartile range 42-53), and 144 (58.3%) were men. Prediabetes was diagnosed in 79 patients (32.0%) and diabetes was diagnosed in 45 patients (18.2%). Prediabetic (adjusted odds ratio [OR] 2.4, 95% confidence interval [CI] 1.1-5.1, P = .031) and diabetic (adjusted OR 2.8, 95% CI 1.3-6.1, P = .020) patients had a worse prognosis at 3 months. A statistical significant shift in the distribution of the mRS score at 3 months was found in prediabetic (adjusted OR 2.5, 95% CI .3-1.5, P = .002) and diabetic (adjusted OR 3.74, 95% CI .5-2.2, P = .002) patients.

Conclusion: In young adults with IS, prediabetes and diabetes increase the risk of unfavorable outcome at 3 months.
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http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2017.09.005DOI Listing
February 2018

Stroke in adults with Down syndrome.

J Neurol Sci 2017 04 8;375:279-280. Epub 2017 Feb 8.

Serviço de Neurologia, Hospital Egas Moniz, Centro Hospitalar Lisbon Ocidental, Lisbon, Portugal; CEDOC, Nova Medical School, Universidade Nova de Lisbon, Lisbon, Portugal. Electronic address:

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http://dx.doi.org/10.1016/j.jns.2017.02.014DOI Listing
April 2017

[Ischemic Stroke: Increasing the Number of Patients Submitted to Acute Specific Therapy and Estimating the Individual Benefits].

Acta Med Port 2016 11 30;29(11):683. Epub 2016 Nov 30.

Serviço de Neurologia. Hospital Egas Moniz. Centro Hospitalar de Lisboa Ocidental. Lisboa. Portugal; CEDOC - iNOVA4Health. Faculdade de Ciências Médicas. Universidade Nova de Lisboa. Lisboa. Portugal.

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http://dx.doi.org/10.20344/amp.8367DOI Listing
November 2016

Teaching Video NeuroImages: Candy sign: The clue to the diagnosis of neurosyphilis.

Neurology 2017 01;88(4):e35

From the Department of Neurology (J.P.M., C.B., L.A., M.V.-B.), Hospital Egas Moniz, Centro Hospitalar Lisboa Ocidental, Lisboa; Department of Neurology (T.L.), Hospital Beatriz Ângelo, Loures; and CEDOC (L.A., M.V.-B.), Nova Medical School, Universidade Nova de Lisboa, Portugal.

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http://dx.doi.org/10.1212/WNL.0000000000003544DOI Listing
January 2017
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