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Decompressive surgery has proven to be lifesaving in patients with a malignant anterior circulation ischemic stroke. Recently, some studies have shown a high frequency of epileptic seizures in patients undergoing this procedure. However, the quantification of this risk and its associated factors have not been extensively investigated.To determine the frequency of epileptic seizures and epilepsy in patients with an anterior circulation ischemic stroke admitted to our Stroke Unit from January 2006 to March 2019 that have been submitted to craniectomy and to study their associated demographic, clinical, imagiological and neurophysiological features.Retrospective observational study of 56 consecutive patients with an anterior circulation ischemic stroke that have undergone craniectomy. The frequency of seizures was both clinically and neurophysiologically assessed after reviewing clinical records, discharge or death reports and all EEGs performed during the hospital admission. Bivariate analysis was used to compare patients with and without seizures.Sixteen patients (28,6%) had epileptic seizures. Bivariate analysis showed an association between the occurrence of unprovoked seizures and the median ASPECTS from the first CT performed.In this study, the frequency of epileptic seizures after a malignant stroke submitted to craniectomy was high, albeit lower than that reported in previous studies. The size of infarction at hospital admission appears to be a risk factor for the occurrence of epilepsy in this group of patients.

Introduction and objectives: The coronavirus SARS-CoV-2 (COVID-19) pandemic has been an unmatched challenge to global healthcare. Although the majority of patients admitted with acute coronary syndrome (ACS) may not be infected with COVID-19, the quarantine and public health emergency measures may have affected this particular high risk group. The objective of this study is to assess the impact of the early period of the COVID-19 pandemic on ACS admissions and clinical course in a tertiary care hospital in Portugal's most affected region. Methods: This retrospective, case-control study included patients admitted with a diagnosis of ACS during March and April 2020 (pandemic group) and in the same period in 2019 (control group). Clinical course and complications were also assessed. Results: During the pandemic, there were fewer ACS admissions but presentation was more severe, with a larger proportion of acute ST-elevation myocardial infarctions (54.9% vs. 38.8%, p=0.047), higher maximum troponin levels and greater prevalence of left ventricular systolic dysfunction at discharge (58.0% vs. 35.0%, p=0.01). In this population, although not statistically significant, it was observed a delay between the onset of symptoms and percutaneous coronary intervention, which may traduce a deferred search for urgent medical care during the pandemic. Conclusion: The lockdown phase of COVID-19 pandemic was associated with fewer and more severe ACS in a Tertiary Care Hospital in Portugal's most affected region by the pandemic. Resumo: Introdução e objetivos: A pandemia pela COVID-19 colocou os sistemas de saúde em todo o mundo sob uma exigência sem precedentes. Apesar de a maioria dos doentes admitidos por síndrome coronária aguda não estar infetada com COVID-19, as medidas de saúde pública instituídas poderão afetar este grupo de risco particularmente elevado. O objetivo deste estudo passa por avaliar o impacto que a fase inicial da pandemia COVID-19 teve na apresentação e evolução clínica de doentes admitidos por síndrome coronária aguda num hospital terciário numa das regiões de Portugal mais afetadas pela pandemia. Métodos: Este estudo retrospetivo de caso-controlo incluiu doentes admitidos com o diagnóstico de síndrome coronária aguda entre março e abril de 2020 (grupo pandémico) e no mesmo período em 2019 (grupo controlo). Foram recolhidos dados em relação à evolução clínica em regime de internamento e após a alta. Resultados: Durante o período de pandemia, verificou-se uma diminuição do número de admissões por síndrome coronária aguda bem como apresentações mais graves, registando-se uma maior proporção de enfartes agudos do miocárdio com supradesnivelamento do segmento ST (54,9% versus 38,8%, p=0,047), níveis máximos de troponina I mais elevados, e maior prevalência de disfunção sistólica do ventrículo esquerdo à data de alta (58,0% versus 35,0%, p=0,01). Apesar de não se ter atingido valores estatisticamente significativos, observou-se um aumento do intervalo de tempo entre o início de sintomas e a realização de intervenção coronária percutânea, o que poderá traduzir um atraso na procura de cuidados de saúde. Conclusão: A fase inicial da pandemia COVID-19 associou-se a um menor número e uma maior gravidade de admissões por síndrome coronária aguda num hospital terciário numa das regiões de Portugal mais afetadas pela pandemia.

This case report describes the treatment of a 16-year-old female patient with a skeletal open bite and temporomandibular dysfunction. Clear aligners and miniscrews were used to control the occlusal plane and improve the skeletal problem. At the end of treatment, the mandible had rotated counterclockwise, allowing bite closure, upgrading dental and facial esthetics, and improving temporomandibular dysfunction.

Introduction: Dilated cardiomyopathy (DCM) is a disease of the heart muscle characterized by ventricular dilatation and impaired systolic function. Familial forms account for 30-50% of cases. Autosomal dominant inheritance is the predominant pattern of transmission. Causal genetic variants have been identified in several genes and molecular diagnosis has implications for genetic counseling and risk stratification. Objective: We aimed to estimate the frequency of genetic variants and the molecular basis of DCM in Portugal. Methods: We performed a multicenter study of unrelated patients, recruited between 2013 and 2014. Variants in 15 genes were screened using PCR with direct sequencing (next-generation sequencing with at least 30-fold coverage combined with Sanger sequencing). Results: A total of 107 patients were included, 64 (60%) men, mean age at diagnosis 38±13 years, with 48 (45%) familial cases. In total, 31 rare variants in eight genes (mainly in MYBPC3, TNNT2 and LMNA) were identified, in 28 patients (26%). Only four variants had been previously described in association with DCM, 11 with hypertrophic cardiomyopathy, and nine variants were novel. Four variants were likely pathogenic and the remainder were of uncertain significance. We found no major differences in the main clinical and imaging characteristics between patients with or without rare variants and patients with likely pathogenic variants. Conclusions: Our results reflect the complexity and diversity of DCM genetics. For better interpretation of the pathogenicity of the variants found and their causative roles in DCM, molecular cascade screening of families is imperative. Further insight into genotype-phenotype correlations and risk stratification is desirable. Resumo: Introdução: A miocardiopatia dilatada (MCD) é uma doença do músculo cardíaco caracterizada por dilatação ventricular e compromisso da função sistólica. As formas familiares são responsáveis por 30 a 50% dos casos. O padrão de hereditariedade predominante é o autossómico dominante. Variantes genéticas causais foram identificadas em vários genes e o diagnóstico molecular tem implicações para o aconselhamento genético e estratificação de risco. Objetivo: Avaliar a base molecular da MCD em Portugal. Métodos: Estudo multicêntrico de doentes não relacionados, recrutados entre 2013 e 2014. Foram analisados 15 genes, através da técnica de PCR com sequenciação direta (NGS com pelo menos uma cobertura de 30 vezes combinada com sequenciação de Sanger). Resultados: Foram incluídos 107 pacientes, 64 (60%) homens, idade média ao diagnóstico de 38 ± 13 anos, com 48 (45%) casos familiares. Foram identificadas 31 variantes raras, em oito genes, (principalmente MYBPC3, TNNT2 e LMNA) em 28 pacientes (26%). Apenas quatro variantes tinham sido previamente descritas em associação com MCD, 11 com miocardiopatia hipertrófica e nove variantes eram novas. Quatro variantes foram classificadas como provavelmente patogénicas e as restantes de significado incerto. Não encontrámos diferenças significativas nas principais características clínicas e imagiológicas entre doentes com ou sem variantes raras e doentes com variantes provavelmente patogénicas. Conclusões: Estes resultados refletem a complexidade e diversidade genética da MCD. Para uma melhor interpretação da patogenicidade das variantes e potencial causalidade, o rastreio molecular das famílias é imperativo. Uma visão mais aprofundada das correlações genótipo-fenótipo e da estratificação de risco é desejável. Keywords: Dilated cardiomyopathy, Genetic variant, Next-generation sequencing, Palavras-chave: Miocardiopatia dilatada, Variante genética, Sequenciação massiva paralela

Resumo: O uso dos inibidores da tirosina cínase (ITC) para o tratamento da leucemia mieloide crónica alterou significativamente o prognóstico dessa doença e permitiu uma esperança de vida praticamente normal. Apesar dos seus inegáveis benefícios, o uso dos ITC está associado a um aumento do risco de efeitos colaterais sobre o sistema cardiovascular, nomeadamente no risco de eventos aterotrombóticos. Torna‐se por isso necessário conhecer e prevenir os efeitos adversos desses fármacos de modo a permitir a continuação da terapêutica antileucémica e minimizar a toxidade para os doentes. Este documento multidisciplinar, elaborado através de uma colaboração entre hematologistas e cardiologistas de vários serviços hospitalares portugueses, tem por objetivo rever a toxidade cardiovascular associada aos vários ITCs e estabelecer sugestões para o seguimento desses doentes. São ainda propostas medidas para a avaliação e redução do risco cardiovascular desses doentes, critérios de referenciação e discutidas interações medicamentosas relevantes. Abstract: The use of tyrosine kinase inhibitors (TKIs) for the treatment of chronic myeloid leukemia has significantly altered the prognosis of this disease, enabling close to normal life expectancy.Despite their undeniable benefits, the use of TKIs is associated with an increased risk of side effects on the cardiovascular system, particularly of atherothrombotic events. It is therefore necessary to understand and prevent the adverse effects of these drugs, in order to enable antileukemic therapy to continue and to minimize patients’ toxic exposure.This multidisciplinary consensus document, developed through a collaboration between hematologists and cardiologists, aims to review the cardiovascular toxicity associated with various TKIs and to establish recommendations for the follow‐up of these patients. Measures are also proposed for the assessment and reduction of cardiovascular risk in these patients and referral criteria, and relevant drug interactions are discussed. Palavras chave: Cardio‐oncologia, Quimioterapia, Inibidores da tirosinacinase, Cardiotoxicidade, Leucemia mieloide crónica, Keywords: Cardio‐oncology, Chemotherapy, Tyrosine kinase inhibitors, Cardiotoxicity, Chronic myeloid leukemia

Intravenous thrombolysis (IV-rtPA) has been suggested as a potential cause of myocardial infarction (MI) after acute ischemic stroke (AIS), with randomized clinical trials showing a higher number of cardiac events within the thrombolysis group. We assessed the prevalence and MI mechanisms after IV-rtPA for AIS.Retrospective review of consecutive AIS patients admitted to six stroke units and systematic literature review searching for AIS patients who suffered a MI less than 24 h after IV-rtPA. In those with available coronary angiography, MI etiology was defined as atherosclerotic or embolic. Patients' characteristics were compared between groups.Fifty-two patients were included. Thirty-two patients (61.5%) derived from hospital cases, after reviewing 6958 patients treated with IV-rtPA [0.5% (95% CI 0.38-0.54) of total hospital cases]. After coronary angiography (n = 25, 48.1%), 14 (54%) patients were considered to have an atherosclerotic MI, and 11 (46%) due to coronary embolism. Patients with an embolic MI more frequently had a cardioembolic AIS (72.7% vs 28.6%; p-value = 0.047) and an intracardiac thrombus (27.3% vs 0.0%; p-value = 0.044). Although not statistically significant, patients with an embolic MI had apparent lower time intervals between starting IV-rtPA infusion and MI occurrence [2 h (0.2-3.0) vs 3 h (1.0-15.0); p-value = 0.134].MI within the first 24 h after IV-rtPA for AIS is an infrequent event, and more frequently non-embolic. However, the prevalence of embolic MI was superior to what is found in the general population with MI. There was an association between the pathophysiology of AIS and MI. The low number of events and publication bias may have limited our conclusions.

© 2018 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved., Objective: To identify the most accurate quantitative electroencephalographic (qEEG) predictor(s) of unfavorable post-ischemic stroke outcome, and its discriminative capacity compared to already known demographic, clinical and imaging prognostic markers. Methods: Prospective cohort of 151 consecutive anterior circulation ischemic stroke patients followed for 12 months. EEG was recorded within 72 h and at discharge or 7 days post-stroke. QEEG (global band power, symmetry, affected/unaffected hemisphere and time changes) indices were calculated from mean Fast Fourier Transform and analyzed as predictors of unfavorable outcome (mRS ≥ 3), at discharge and 12 months poststroke, before and after adjustment for age, admission NIHSS and ASPECTS. Results: Higher delta, lower alpha and beta relative powers (RP) predicted outcome. Indices with higher discriminative capacity were delta-theta to alpha-beta ratio (DTABR) and alpha RP. Outcome models including either of these and other clinical/imaging stroke outcome predictors were superior to models without qEEG data. In models with qEEG indices, infarct size was not a significant outcome predictor. Conclusions: DTAABR and alpha RP are the best qEEG indices and superior to ASPECTS in post-stroke outcome prediction. They improve the discriminative capacity of already known clinical and imaging stroke outcome predictors, both at discharge and 12 months after stroke. Significance: qEEG indices are independent predictors of stroke outcome.

This systematic review was performed to compare dental, skeletal, and aesthetic outcomes between orthodontic camouflage and surgical-orthodontic treatment, in patients with a skeletal class II malocclusion and a retrognathic mandible who have already finished their growth period. A literature search was conducted, and a modified Downs and Black checklist was used to assess methodological quality. The meta-analysis was conducted using the DerSimonian–Laird random-effects method to obtain summary estimates of the standardized mean differences and corresponding 95% confidence intervals. Nine articles were included in the qualitative synthesis and seven in the meta-analysis. The difference between treatments was not statistically significant regarding SNA angle, linear measurement of the lower lip to Ricketts’ aesthetic line, convexity of the skeletal profile, or the soft tissue profile excluding the nose. In contrast, surgical-orthodontic treatment was more effective with regard to ANB, SNB, and ML/NSL angles and the soft tissue profile including the nose. Different treatment effects on overjet and overbite were found according to the severity of the initial values. These results should be interpreted with caution, due to the limited number of studies included and because they were non-randomized clinical trials. Further studies with larger sample sizes and similar pre-treatment conditions are needed.

Resume Introduction Les troubles temporomandibulaires ou les desordres de l’appareil manducateur (DAM) comportent plusieurs risques et differents facteurs associes avec, par consequent, des approches therapeutiques variees. Il est important d’identifier les troubles caracteristiques du patient qui relevent d’orthodontie, de kinesitherapie, ou d’autres modalites therapeutiques plus ou moins combinees. Presentation des cas cliniques Nous presentons trois cas de patients presentant des signes et/ou symptomes communs de troubles temporomandibulaires avec des approches therapeutiques et des resultats differents ; et notre but est de comprendre ce qui pourrait expliquer les differences de resultats observees et reconnaitre les elements particuliers squelettiques, musculaires, faciaux et occlusaux qui pourraient justifier le benefice d’une intervention particuliere. Conclusion Il a ete demontre que l’orthodontie joue un role important pour resoudre des problemes occlusaux et modifier la dimension verticale. D’un autre cote, la kinesitherapie s’est averee efficace dans la gestion et l’amelioration de l’intensite de la douleur, lorsque les changements musculosquelettiques sont clairement constates. Enfin, il a egalement ete demontre qu’une approche pluridisciplinaire peut s’averer essentielle : le clinicien doit etre conscient de l’interet d’une evaluation globale, prenant en compte tous les facteurs associes, en particulier les facteurs psychologiques.

Pain is an unpleasant, emotional, and sensorial experience. Esthetics and pain express perceptions or sensations that are subjective and personal, but non-consensual, between professionals and patients. The Visual Analog Scale (VAS) and the Numerical Rating Scale (NRS) are simple methods to evaluate such subjective phenomena.To verify whether a concordance exists between VAS and NS in the perception of esthetics and pain.Regarding esthetics, an on-line questionnaire was prepared with extra- and intra-oral images (adapted). The order of the scales was alternated between interwoven and sequential. Regarding perception of pain, respondents personally classified its intensity at the present moment, or over the past 30 days.A statistically significant concordance (P0.001) - positive and moderate - was found between the scales, for the evaluation of both the extra- and intra-oral images, r=0.678 and 0.638, respectively. Regarding pain perception, taking formal education into account, no concordance was found between the two scales for participants who had "no formal education" (P0.05). The concordance between the scales increased proportionally with the level of education.There was a concordance between the scales with regard to esthetical perception and the evaluation of pain. When assessing esthetics, the order in which the two scales are applied does not affect the concordance between them. However, the strength of the concordance is influenced. Esthetic perception is not influenced by the differences between the panels of evaluators. The concordance between the two scales, for the evaluation of pain, increases proportionally with the level of formal education.

Resume Contexte La douleur est une experience sensorielle et emotionnelle desagreable. L’esthetique et la douleur sont percues selon des sensations subjectives et personnelles, non consensuelles, entre les praticiens et les patients. L’Echelle Visuelle Analogue (EVA) et l’Echelle d’Evaluation Numerique (EEN) sont des methodes simples d’evaluation de tels phenomenes subjectifs. Objectif Verifier s’il existe une concordance entre l’EVA et l’EEN pour la perception de l’esthetique et de la douleur. Materiel et methodes Pour l’esthetique, un questionnaire en ligne a ete prepare comportant des images extra- et intra-orales. Des modifications ont ete apportees a l’ordre de presentation de l’echelle, qui pouvait etre entremele ou sequentiel. Pour la perception de la douleur, les repondants devaient classer leur evaluation personnelle de l’intensite de la douleur a l’instant « t », ou pendant les 30 derniers jours. Resultats Une association statistiquement significative (p Conclusion Aucune concordance n’a ete observee entre les echelles relatives a la perception esthetique et a l’evaluation de la douleur. Lors de l’evaluation de l’esthetique, l’ordre dans lequel les deux echelles sont appliquees n’influe pas sur la concordance entre les deux methodes. Cependant, le niveau de concordance est affecte. La perception esthetique n’est pas influencee par les differents panels d’evaluateurs. Pour l’evaluation de la douleur, la concordance entre les deux echelles augmente proportionnellement au niveau d’education.

Resume Les objectifs de cette etude etaient de comparer les modifications de dimension de la crete alveolaire avant et en fin de traitement orthodontique, ainsi qu’un an apres traitement dans des cas d’agenesie des incisives laterales maxillaires et de determiner la possibilite d’utiliser des modeles en plâtre et des radiographies panoramiques pour remplacer la tomographie volumique a faisceau conique, ou cone beam. Materiel et methodes En tout, 228 mesures ont ete effectuees sur des modeles en plâtre ou des radiographies panoramiques avant le traitement orthodontique (T1), ainsi qu’en fin de traitement (T2) et un an apres le traitement (T3). Au stade T2, les images cone beam ont egalement ete analysees. Des tests non parametriques ont ete realises. Resultats Aux trois stades, on n’a releve aucune difference statistique significative concernant l’epaisseur des modeles, la hauteur de l’os sur les radiographies panoramiques, la largeur de l’espace et la hauteur de l’os sur ces memes radiographies ou la largeur de l’espace sur les modeles et l’epaisseur sur les memes modeles en plâtre. Cependant, une difference statistique significative a ete observee concernant la largeur sur les radiographies panoramiques aux 3 stades. Le cone beam n’a pas presente de correlation statistiquement significative concernant la hauteur et la largeur de l’os, compare aux radiographies panoramiques, et concernant l’epaisseur comparee aux modeles en plâtre. Conclusion Il n’y avait pas de variation significative d’epaisseur, de hauteur ou de largeur de l’os avec le temps. Nous avons demontre que les modeles en plâtre et les radiographies ne semblent pas offrir une alternative efficace a la tomographie volumique a faisceau unique en ce qui concerne ces mesures specifiques.

We address an extension of the classical multi-period facility location problem in which customers are sensitive to delivery lead times. Accordingly, two customer segments are considered. The first segment comprises customers that require timely demand satisfaction, whereas customers accepting delayed deliveries make up the second segment. Each customer belonging to the latter segment specifies a maximum delivery time. A tardiness penalty is incurred to each unit of demand that is not satisfied on time. In the problem that we study, a network is already in place with a number of facilities being operated at fixed locations. The network can be expanded by establishing new facilities at a finite set of potential sites and selecting their capacity levels from a set of available discrete sizes. In addition, existing facilities may be closed over the time horizon. Two mixed-integer linear programming formulations are proposed to re-design the network at minimum cost and a theoretical comparison of their linear relaxations is provided. We also extend the mathematical models to the case in which each customer accepting delayed demand satisfaction requires late shipments to occur at most once over the delivery lead time. To gain insight into how challenging these problems are to solve, a computational study is performed with randomly generated instances and using a general-purpose solver. Useful insights are derived from analyzing the impact of different delivery lead time restrictions on the network structure and cost.

Resume L’objectif de cette etude etait d’evaluer la perception du sourire dans les cas d’agenesie des incisives laterales maxillaires (AILM) traites par mesialisation de canines. Materiel et methodes Neuf images ont ete modifiees numeriquement a partir de la meme photographie intraorale coronale afin de simuler les diverses options de traitement pour la fermeture d’espace en AILM. Un questionnaire a ete soumis a une cohorte de repondants comprenant des non-praticiens (303), des dentistes generalistes (215), des dentistes prothesistes (55) et des orthodontistes (81). Des tests statistiques avec un niveau de significativite de p Resultats Les vues du traitement AILM jugees les plus esthetiques etaient celles montrant un recontourage dentaire et gingival unilateral. Tous les groupes dans l’etude ont estime esthetique le simple recontourage dentaire du bord mesial de la canine. Dans l’analyse des images regroupees en AILM uni- ou bilaterale, la vue montrant le remodelage dentaire et gingival a ete jugee le resultat le plus esthetique alors que la mesialisation non modifiee etait consideree comme la moins attractive. Conclusion Concernant les traitements de fermeture d’espace, quoique tous les groupes aient considere le simple remodelage dentaire de la canine comme attractif, les praticiens dentaires ont juge le remodelage gingival et coronaire comme etant la solution la plus attractive. Par contre, les non-praticiens n’etaient pas significativement sensibles, dans les cas d’AILM, a cette modification dentaire et gingivale comparee au leger recontourage de la face mesiale de la cuspide de la canine mesialisee.

Summary The objective of this study was to evaluate perception of the smile in maxillary lateral incisor agenesis (MLIA) cases treated by mesialization of a canine. Material and methods Nine images were digitally modified from the same frontal intraoral photograph to simulate various treatment options for space closure in MLIA. A questionnaire was submitted to laypersons (303), general dentists (215), prosthodontists (55) and orthodontists (81). Statistical tests with a significance level of P Results The views of MLIA treatment judged to be most attractive showed unilateral dental and gingival reshaping. All study groups considered the simple dental reshaping of the mesial edge of the canine to be attractive. In the analysis of the images grouped together for both unilateral and bilateral MLIA, the view showing dental and gingival reshaping was considered the most attractive whereas unmodified mesialization was considered the least attractive. Conclusion Regarding the space closure treatments, although all groups regarded simple dental reshaping of the canine to be attractive, the dental professionals considered gingival and crown reshaping to be more esthetic. In contrast, laypersons were not significantly responsive to this dental and gingival modification as compared to only slight reshaping of the mesial edge of the cusp of the mesialized canine in MLIA.

Resume L’objectif de cette etude etait de determiner quelles asymetries du sourire, dentaires ou gingivales etaient percues comme etant les moins esthetiques. Materiel et methodes Des non-professionnels (297), des omnipraticiens (223), des dentistes prothesistes (50) et des orthodontistes (49) ont evalue l’esthetique d’images numeriquement modifiees a partir de la meme photographie intraorale de face, en utilisant les memes levres et en simulant une deviation du milieu maxillaire superieur, une inclinaison du plan occlusal, une asymetrie du bord incisif et une migration gingivale asymetrique. Ulterieurement, les images ont ete presentees par paires de 3 groupes. Resultats Les seules images jugees esthetiques etaient : le bord incisif asymetrique de l’incisive centrale, plus courte de 0,5 mm et la migration gingivale asymetrique de 2 mm d’une incisive centrale superieure. Parmi les images appariees, la deviation du milieu du maxillaire superieur versus la migration gingivale asymetrique, celle-ci a ete jugee la moins esthetique alors que, entre le bord incisif asymetrique et la migration gingivale asymetrique, celle-ci a ete consideree comme la moins esthetique. Conclusion Les non-professionnels et les omnipraticiens ont attribue les scores plus favorables. Les seules images jugees esthetiques etaient le bord incisif asymetrique d’une incisive centrale plus courte de 0,5 mm et la migration gingivale asymetrique de 2 mm d’une incisive centrale superieure. Sur le plan horizontal (deviation du milieu maxillaire versus inclinaison du plan occlusal), les asymetries dentaires etaient jugees moins esthetiques que les asymetries gingivales. Cependant, sur le plan vertical (bord incisif asymetrique versus migration gingivale asymetrique) la situation inverse s’est produite.

Summary Open bite is related to various etiological factors and, in many cases, is difficult to diagnose. The present study is aimed at evaluating, through cephalometric analysis, the dimensions of the upper airways and dentoalveolar heights in open bite (OB) patients versus normal overbite patients. The relationship between the width of the upper airways and the lack of overbite is also studied, in order to differentiate between dental open bite (DOB) and skeletal open bite (SOB). Materials and methods Eighty X-rays were selected from files of orthodontic patients to form the control sample (n = 40) and open bite sample (n = 40). Dimensions of the upper airways and dentoalveolar heights were measured in both samples, using 16 linear measurements, two angle values and one ratio. Results In OB patients, anteroposterior narrowing of the upper airways, mainly in the nasopharynx and oropharynx, was observed, together with forward displacement of the hyoid bone and increased maxillary and mandibular dentoalveolar heights, and anterior facial height. In SOB, the overbite was more negative and facial growth was more clockwise-oriented than in DOB. Greater narrowing of the airways in the anteroposterior orientation was also noted. In DOB, there was evidence of muscular adaptation, as shown by increased values of the hyoid bone displacement to a more anterior and lower position, and increased values of the vertical dimensions of the airways. An increase in posterior facial height was also observed allowing anterior rotation of the mandible. Conclusion The results suggest that the airway's dimensions reflect a tendency to open bite. The variable vertical airway length (Val) and the position of the hyoid bone allow the adaptive potential of these individuals to be determined and make the treatment of open bites more predictable.

Resume Les beances sont associees a divers facteurs etiologiques et, dans de nombreux cas, sont difficiles a diagnostiquer. L’objectif de cette etude est d’evaluer, par analyse cephalometrique, les dimensions des voies aeriennes superieures et les hauteurs dentoalveolaires chez les patients presentant une beance compares a des patients avec une supraclusion normale. La relation entre la largeur des voies aeriennes superieures et l’absence de beance est egalement examinee afin de differencier les beances dentaires (BD) et les beances squelettiques (BS). Materiaux et methodes Quatre-vingts radiographies ont ete selectionnees parmi les dossiers de patients orthodontiques afin de constituer l’echantillon temoin ( n = 40) et un echantillon « beance » ( n = 40). Les dimensions des voies aeriennes superieures et les hauteurs dentoalveolaires ont ete mesurees pour les deux echantillons a l’aide de 16 mesures lineaires, de deux valeurs angulaires et d’un ratio. Resultats Chez les patients avec beance, il a ete observe un retrecissement anteroposterieur des voies superieures, surtout au niveau du rhinopharynx et de l’oropharynx, ainsi qu’un deplacement anterieur de l’os hyoidien et une augmentation des hauteurs dentoalveolaires maxillaire et mandibulaire et de la hauteur faciale anterieure. Chez les sujets BS, la supraclusion etait plus negative et la croissance faciale etait davantage caracterisee par une rotation posterieure que chez les sujets BD. Il a ete releve aussi un retrecissement plus important des voies aeriennes dans le sens anteroposterieur. Dans l’echantillon BD, nous avons note des signes d’une adaptation musculaire, indiquee par les valeurs augmentees du deplacement de l’os hyoidien dans le sens antero-inferieur, et des valeurs plus importantes des dimensions verticales des voies aeriennes. Une augmentation de la hauteur faciale posterieure a egalement ete observee permettant une rotation anterieure de la mandibule. Conclusion Les resultats suggerent que les dimensions des voies aeriennes refletent une tendance a la beance. La variable longueur verticale des voies aeriennes (Val) et la position de l’os hyoidien permettent de determiner le potentiel d’adaptation chez les sujets presentant une beance et de rendre plus previsible le traitement de ces patients.

Brain magnetic resonance imaging (MRI) with diffusion-weighted imaging (DWI) sequences and correlative apparent diffusion coefficient (ADC) maps is a very sensitive way to detect acute ischemic stroke. Cases of negative MRI-DWI on acute phase of ischemic stroke are uncommon, and most of them are reported in single small-sized lesions, which in most cases are below the technical spatial resolution and in patients imaged shortly after the symptoms start. The few published cases of territorial ischemic stroke with negative DWI affect exclusively one vascular territory. We report the case of an ischemic stroke involving 2 different arteries of the posterior circulation, with a negative DWI/ADC brain MRI 18 hours after time-last-seen-well. We also suggest a possible explanation regarding the mechanism of false-negative diffusion MRI on ischemic stroke.

Resume L’objectif de cette etude etait de determiner la perception esthetique de certaines composantes du sourire telles que l’exposition gingivale, la longueur coronaire, la mediane maxillaire et les diastemes interincisifs par des profanes, des etudiants en dentaire et des dentistes. Materiaux et methodes Six cent trente-quatre sujets portugais (292 profanes, 241 etudiants en dentaire et 101 dentistes) ont evalue l’esthetique de 13 images modifiees du meme sourire presentees de facon randomisee. Les composantes modifiees (exposition gingivale, niveau des collets gingivaux, longueur coronaire, ligne mediane maxillaire et diastemes interincisifs) ont ete manipulees a l’aide du logiciel Adobe Photoshop® CS6. La classification des images a ete effectuee au moyen de l’echelle visuelle analogique (EVA) avec des scores de 1 a 10. Les reponses ont ete ensuite analysees et traitees par le SPSS® version 21.0 en utilisant des tests d’egalite de moyennes et de correlation. Resultats Le sourire moyen etait le sourire le plus apprecie alors que le sourire gingival et les sourires avec diasteme ont ete consideres les moins esthetiques. Parmi tous les parametres, le changement de la ligne mediane a ete le moins perceptible. La preference en faveur de l’asymetrie en rapport avec le collet gingival des incisives laterales maxillaires (ILM) et de la symetrie de la longueur coronaire des incisives centrales maxillaires (ICM) temoigne de l’importance accordee aux ICM au niveau du sourire. Le sexe des evaluateurs n’a pas influe sur les scores attribues, sauf en ce qui concerne le sourire gingival. Ce sont les participants jeunes qui ont donne les scores les plus eleves. Concernant la formation universitaire ou professionnelle, il y avait une concordance intragroupe des opinions ; les profanes avaient tendance a donner des scores plus eleves et les professionnels les scores les plus bas sans aucune correlation entre les variables. Le fait que les profanes aient recu, ou non, un traitement orthodontique n’a pas eu d’impact sur leurs opinions. Conclusion Lors d’une evaluation de divers elements d’un sourire modifie, les profanes, les etudiants en dentaire et les praticiens avaient des perceptions differentes de leur attractivite, a l’exception des sourires avec diasteme ; il n’y avait pourtant pas de differences significatives entre les groupes. On a observe une correlation entre le sexe des evaluateurs et les sourires tres gingivaux. Il n’y avait pas de correlation entre leur âge et leurs opinions. Il n’y avait aucune difference entre les perceptions des profanes, qu’ils aient recu, ou non, un traitement orthodontique.

Resume Objectif Le but de cette etude etait d’evaluer, chez un echantillon portugais, la prevalence et la distribution des agenesies, des dents surnumeraires, des dents incluses et des transpositions dentaires, aussi bien que la relation entre ces differentes anomalies. Materiaux et methodes L’echantillon comprenait 2888 patients, observes entre 2005 et 2009 a la clinique de dentisterie de l’Instituto Superior de Ciencias da Saude-Norte (ISCSN, Portugal). L’etude comprenait l’evaluation des parametres suivants : agenesie de toutes les dents, dents surnumeraires, dents permanentes incluses et transpositions de dents. L’âge des sujets s’echelonnait de sept a 21 ans. Pour l’etude portant sur l’absence de la troisieme molaire, les sujets âges de moins de 14 ans ont ete exclus. L’analyse statistique a ete realisee a l’aide de SPSS ® . Resultats En excluant les troisiemes molaires, la prevalence de l’agenesie dentaire, des dents surnumeraires, des dents incluses et des transpositions etait, respectivement, de 6,1 %, 0,8 %, 1,8 % et 0,2 % dans cet echantillon de la population portugaise. La prevalence de dents surnumeraires etait significativement plus elevee chez les hommes que chez les femmes ( p p Conclusions L’agenesie est l’anomalie dentaire la plus frequente. Il n’existe pas de differences entre les sexes, a l’exception des dents surnumeraires, que l’on observe plus frequemment chez les hommes. Une relation entre l’agenesie de la troisieme molaire et les canines incluses a ete observee.

The aim of this study was to evaluate the prevalence and pattern of agenesis, supernumerary teeth, impacted teeth and transpositions, as well as the relation between them, in a Portuguese sample.The study sample consisted of 2888 patients, observed between 2005 and 2009 at the Dentistry Clinic of the Instituto Superior de Ciências da Saúde-Norte (ISCSN, Portugal). The study included evaluation of the following parameters: agenesis of all teeth, supernumerary teeth, impacted permanent teeth and tooth transposition. The age range varied from 7 to 21 years. In order to study the absence of the third molar, subjects aged below 14 years were excluded. Statistical analysis was performed using SPSS(®).Excluding third molars, the prevalence of tooth agenesis, supernumerary teeth, impacted teeth and transpositions was 6.1%, 0.8%, 1.8% and 0.2%, respectively, for this Portuguese population. There was a significantly higher prevalence of supernumerary teeth in males than in females (P0.05). The mesiodens was the most frequent supernumerary tooth, the upper canine was the most frequent impacted tooth, and the upper canine and upper lateral were the two most frequently transposed teeth. There was a significantly higher prevalence of missing third molars in the impacted canine group than in the non-impacted canine group (P0.05).Agenesis is the most frequent dental anomaly. There are no differences between genders, except for supernumerary teeth, which are found more frequently in men. A relation between third molar agenesis and impacted canines was found.

This article describes the complex dental treatment of an adult patient with multiple missing teeth, mild chronic periodontitis, and a malocclusion with a cant of the occlusal plane. After periodontal treatment, titanium implants and a miniscrew were placed to correct the occlusal plane canting with orthodontic treatment. Prosthodontic treatment was completed by using osseointegrated implants to replace the missing teeth.

Resume Objectif Cette etude a cherche a evaluer la prevalence et la distribution de l’hypodontie dans la dentition permanente, avec et sans les troisiemes molaires, dans un echantillon de population portugaise. Patients et methodes Le groupe d’etude comprenait 2888 patients, suivis entre 2005 et 2009 a la clinique dentaire de l’Instituto Superior de Ciencias de Saude-Norte (ISCSN, Portugal). Les patients ont ete examines pour l’hypodontie, et pour la presence ou l’absence de dents temporaires chez ceux qui presentaient une agenesie. L’âge des sujets s’etalait de sept a 21 ans. Pour l’etude sur l’absence de troisiemes molaires, nous avons exclu les patients de moins de 14 ans. L’analyse statistique a ete realisee en utilisant SPSS®. Resultats Sans tenir compte des troisiemes molaires, la prevalence d’agenesie dentaire etait de 6,1 % pour cette population. L’agenesie dentaire a ete observee plus frequemment chez les femmes que chez les hommes, quoique cette difference ne fut pas statistiquement significative (p > 0,05). L’absence de dent la plus frequente etait la deuxieme premolaire mandibulaire, suivie de l’incisive laterale maxillaire et de la deuxieme premolaire maxillaire. Nous avons releve une prevalence significativement plus elevee de troisiemes molaires absentes dans le groupe avec agenesie que dans le groupe sans agenesie. Il y avait une correlation entre l’agenesie des deuxiemes premolaires et des incisives laterales superieures avec la presence des dents temporaires correspondantes. Conclusions La prevalence de l’agenesie dentaire a ete retrouvee chez 6,1 % de cette population portugaise ainsi qu’une correlation entre l’agenesie et la presence de dents temporaires, et entre l’agenesie et l’absence de troisiemes molaires.

Resume Objectif Evaluer l’impact de l’agenesie des incisives laterales maxillaires (AILM) sur la morphogenese du visage. Methodes Evaluation des dimensions squelettiques du maxillaire et du segment des incisives superieures. L’analyse a ete realisee aupres de trois groupes : individus avec une AILM, leurs parents proches et la population normale. Dans ces groupes, une comparaison a ete faite entre les adultes et les individus en pleine croissance. Resultats L’AILM peut influer sur la longueur du maxillaire et sur la hauteur anterosuperieure de la face, ce qui peut avoir un impact negatif sur sa croissance potentielle. Il n’y a aucune correlation entre l’AILM et les modifications de l’inclinaison du plan palatin, la hauteur du maxillaire, l’evolution des dimensions de l’epine nasale anterieure ou de l’epine nasale posterieure par rapport au plan de Francfort pendant la phase de croissance observee. L’inclinaison coronopalatine des incisives chez les enfants avec une agenesie bilaterale des incisives laterales semble se modifier pendant la vie d’adulte. Conclusions L’AILM est associee a un raccourcissement du maxillaire et semble avoir un impact significatif sur la hauteur anterieure du visage en reduisant ses dimensions potentielles.

Summary Objectives Assessment of the influence of maxillary lateral incisor agenesis (MLIA) on the facial morphogenesis. Methods Evaluation of the skeletal dimensions of the upper maxilla and its incisor region. Analysis was performed among three groups: individuals with MLIA, its relatives and the normal population. Among these a comparison between adults and growing individuals was performed. Results MLIA may interfere with the maxillary length and the anterosuperior facial height, negatively conditioning on its potential growth. MLIA was not correlated with changes of the palatine plane inclination, maxillary height nor with adjustment of the anterior nasal spine dimensions related to the Frankfurt plan or to the posterior nasal spine–Frankfurt plan during the observed development stage; the backward angle of the incisors in children with bilateral agenesis of lateral incisors appear to have been corrected in adulthood. Conclusions MLIA is associated with an upper maxilla shortening, and appear to interfere significantly with the anterior facial height, reducing its potential size.

This case report describes the treatment of a patient with a horizontally impacted maxillary central incisor, a canine in the same quadrant, and an inclusion tendency. Due to severe crowing in the maxilla and the Class II molar relationship on the impaction side, a 2-stage treatment plan was developed. In the first stage, the right first premolar and deciduous canine were extracted; this allowed enough space for the eruption of the maxillary right permanent canine. The second stage included surgical exposure and traction of the impacted central incisor with a fixed orthodontic appliance. An excisional uncovering technique was needed to expose the impacted incisor. After it erupted, an apically positioned partial-thickness flap was used to add keratinized attached gingiva in the area surrounding the crown, initially located in an area of unattached gingiva. The patient finished treatment with a normal and stable occlusion between the maxillary and mandibular arches and an adequate width of attached gingiva.

Background The Hepatitis C virus (HCV) infection is associated with various extrahepatic manifestations, being the Central Nervous System (CNS) rarely involved. Case report We report a case of a 54 year-old black man with arterial hypertension who presented with progressively worsening headaches, apathy, somnolence and left hemiparesis. Brain MRI showed an acute ischemic lesion in the left anterior cerebral artery (ACA) and an old ischemic infarct in the right ACA territory. Brain MRI with gadolinium revealed mural thickening and contrast enhancement of the A1 and A2 segments of the ACAs, of the middle and distal basilar artery and of the P1 segment of the left posterior cerebral artery, suggesting active vasculitis. Digital angiography confirmed those irregularities and stenosis. Laboratory evaluation revealed ESR (73 mm/h), transaminase elevation, elevated HCV viral load genotype 2, positive IGRA, negative cryoglobulins, CSF protein elevation with oligoclonal bands (mirror pattern) and no pleocytosis; investigation excluded other infectious causes. Pegylated interferon alpha-2a and ribavirin, corticotherapy and tuberculosis prophylaxis were started with clinical and imagiological improvement. Conclusion The typical inflammation signs of the vascular wall demonstrated by the gadolinium-enhanced MRI strengthened the hypothesis of CNS vasculitis. The association with HCV infection is rare but should be investigated once specific therapeutic is required.

Familial dilated cardiomyopathy (FDCM) is characterized by genetic heterogeneity, incomplete age-dependent penetrance, and a multifactorial pathogenesis ([1][1]). Diagnosis is still dependent on clinical criteria and familial investigation ([2][2]). On the other hand, several abnormalities have been

A 32-year-old woman with infantile nephropathic cystinosis presented with cystinosis and recurrent ischemic stroke. The neuropathological description demonstrates that recurrent stroke was caused by intracranial stenosis and showed evidence of cystinosis brain involvement. There are few reports of cerebrovascular disease in patients with longstanding nephropathic cystinosis. This case reinforces that cerebrovascular disease can be a cause of neurological impairment and disability in patients with longstanding nephropathic cystinosis, with implications on primary stroke prevention strategies in these patients.

Information about the security of intravenous recombinant tissue type plasminogen activator (rtPA) in immunoglobulins related stroke can only be ascertained from singular cases as it was not assessed in clinical trials. Only two cases of stroke associated with IVIg treated with rtPA were described in literature. We report the outcome of two patients with IVIg associated stroke that were treated with rtPA. In our patients there were hemorrhagic complications — remote cerebral haemorrhage and diffuse cutaneous hematomas.

Fabry disease is a rare X-linked lysosomal storage disorder caused by mutations in the alpha-galactosidase gene. The most frequent cardiac presentation of Fabry disease is cardiomyopathy characterized by left ventricular (LV) hypertrophy, usually concentric.Heart disease in affected females tends to be clinically recognized later than in males and cardiac complications are the most frequently reported cause of death in females with Fabry disease. There are few data regarding the association between Fabry disease and LV noncompaction. We report a case of a 30-year-old asymptomatic woman, heterozygous for a nonsense alpha-galactosidase gene mutation (p.R220X), who presented LV noncompaction on cardiac magnetic resonance imaging, without LV wall hypertrophy. Histopathological examination of myocardial fragments showed marked deposition of glycosphingolipids in cardiomyocytes, confirming the diagnosis of Fabry cardiomyopathy. Based on this finding, the patient was proposed for enzyme replacement therapy. This case illustrates the role of endomyocardial biopsy in the clarification of doubtful or atypical findings related to cardiac Fabry disease, even in heterozygous women, and corroborates the contention that Fabry disease should be included in the differential diagnosis of LV hypertrabeculation/noncompaction. Resumo: A doença de Fabry é uma doença rara de armazenamento lisossómico, ligada ao cromossoma X, causada por mutações no gene da α – galactosidase. A apresentação cardíaca mais frequente é uma miocardiopatia caracterizada por hipertrofia ventricular esquerda geralmente concêntrica.Nas mulheres afetadas a doença cardíaca tende a ser clinicamente reconhecida mais tardiamente do que em homens e as complicações cardíacas são a causa mais frequente de morte reportada em mulheres com doença de Fabry. Existem poucos dados sobre a associação entre a doença de Fabry e a não compactação do ventrículo esquerdo (VE). Reportamos o caso de uma mulher assintomática, com 30 anos de idade, heterozigota para uma mutação nonsense do gene da α–galactosidase gene (p.R220X) que apresentava critérios de VE não compactado na ressonância magnética cardíaca, sem hipertrofia das restantes paredes ventriculares. O exame histopatológico de fragmentos do miocárdio mostrou deposição acentuada de glicoesfingolípidos nos cardiomiócitos, corroborando o diagnóstico de miocardiopatia de Fabry. Com base nestes achados, foi proposto o início de terapia de substituição enzimática. Este caso ilustra o papel da biópsia endomiocárdica no esclarecimento de achados duvidosos ou atípicos relacionados com a doença de Fabry cardíaca, mesmo em mulheres heterozigotas, e corrobora a afirmação de que a doença de Fabry deve ser incluída no diagnóstico diferencial da hipertrabeculação/não compactação do VE. Keywords: Fabry disease, Hypertrabeculation/noncompaction, Endomyocardial biopsy, Palavras-chave: Doença de Fabry, Hipertrabeculação/não compactação, Biópsia endomiocárdica

Stroke has been rarely associated with carbon monoxide (CO) intoxication. We report a symptomatic internal carotid artery (ICA) thrombosis in a patient with acute CO intoxication.