30 resultados para first-ever seizure
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J Biol Inorg Chem (2006) 11: 548558 DOI 10.1007/s00775-006-0104-y
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RESUMO:Desde a declarao de Bethesda em 1983, a transplantao heptica considerada um processo vlido e aceite na prtica clnica para muitos doentes com doena heptica terminal, relativamente aos quais no houvesse outra alternativa teraputica. Em 1991, por proposta de Holmgren, professor de gentica, o cirurgio sueco Bo Ericzon realizou em Huntingdon (Estocolmo) o primeiro transplante heptico num doente PAF (Polineuropatia Amilloidtica Familiar), esperando que a substituio do fgado pudesse frenar a evoluo da doena. Nesta doena hereditria autossmica dominante, o fgado, apesar de estrutural e funcionalmente normal, produz uma protena anormal (TTR Met30) responsvel pela doena. A partir de ento, a transplantao heptica passou a ser a nica teraputica eficaz para estes doentes. Portugal o pas do mundo com mais doentes PAF, tendo sido o mdico neurologista portugus Corino de Andrade quem, em 1951, identificou e descreveu este tipo particular de polineuropatia hereditria, tambm conhecida por doena de Andrade. Com o incio da transplantao heptica programada em Setembro de 1992, o primeiro doente transplantado heptico em Portugal, no Hospital Curry Cabral, foi um doente PAF. Desde logo se percebeu que a competio nas listas de espera em Portugal, entre doentes hepticos crnicos e doentes PAF viria a ser um problema clnico e tico difcil de compatibilizar. Em 1995, Linhares Furtado, em Coimbra, realizou o primeiro transplante dum fgado dum doente PAF num doente com doena heptica metasttica, ficando este tipo de transplante conhecido como transplante sequencial ou em domin. F-lo no pressuposto de que o fgado PAF, funcional e estruturalmente normal, apesar de produzir a protena mutada causadora da doena neurolgica, pudesse garantir ao receptor um perodo razovel de vida livre de sintomas, tal como acontece na histria natural desta doena congnita, cujas manifestaes clnicas apenas se observam na idade adulta. A tcnica cirrgica mais adequada para transplantar o doente PAF a tcnica de piggyback, na qual a hepatectomia feita mantendo a veia cava do doente, podendo o transplante ser feito sem recorrer a bypass extracorporal. Antes de 2001, para fazerem o transplante sequencial, os diferentes centros alteraram a tcnica de hepatectomia no doente PAF, ressecando a cava com o fgado conforme a tcnica clssica, recorrendo ao bypass extracorporal. No nosso centro imaginmos e concebemos uma tcnica original, com recurso a enxertos venosos, que permitisse ao doente PAF submeter-se mesma tcnica de hepatectomia no transplante, quer ele viesse a ser ou no dador. Essa tcnica, por ns utilizada pela primeira vez a nvel mundial em 2001, ficou conhecida por Transplante Sequencial em Duplo Piggyback. Este trabalho teve como objectivo procurar saber se a tcnica por ns imaginada, concebida e utilizada era reprodutvel, se no prejudicava o doente PAF dador e se oferecia ao receptor heptico as mesmas garantias do fgado de cadver. A nossa srie de transplantes realizados em doentes PAF a maior a nvel mundial, assim como o o nmero de transplantes sequenciais de fgado. Recorrendo nossa base de dados desde Setembro de 1992 at Novembro de 2008 procedeu-se verificao das hipteses anteriormente enunciadas. Com base na experincia por ns introduzida, a tcnica foi reproduzida com xito em vrios centros internacionais de referncia, que por si provaram a sua reprodutibilidade. Este sucesso encontra-se publicado por diversos grupos de transplantao heptica a nvel mundial. Observmos na nossa srie que a sobrevivncia dos doentes PAF que foram dadores ligeiramente superior queles que o no foram, embora sem atingir significncia estatstica. Contudo, quando se analisaram, apenas, estes doentes aps a introduo do transplante sequencial no nosso centro, observa-se que existe uma melhor sobrevida nos doentes PAF dadores (sobrevida aos 5 anos de 87% versus 71%, p=0,047).Relativamente aos receptores observmos que existe um benefcio a curto prazo em termos de morbi-mortalidade (menor hemorragia peri-operatria) e a longo prazo alguns grupos de doentes apresentaram diferenas de sobrevida, embora sem atingir significncia estatstica, facto este que pode estar relacionado com a dimenso das amostras parcelares analisadas. Estes grupos so os doentes com cirrose a vrus da hepatite C e os doentes com doena heptica maligna primitiva dentro dos critrios de Milo. Fora do mbito deste trabalho ficou um aspecto relevante que a recidiva da doena PAF nos receptores de fgado sequencial e o seu impacto no longo prazo. Em concluso, o presente trabalho permite afirmar que a tcnica por ns introduzida pela primeira vez a nvel mundial exequvel e reprodutvel e segura para os doentes dadores de fgado PAF, que no vem a sua tcnica cirrgica alterada pelo facto de o serem. Os receptores no so, por sua vez, prejudicados por receberem um fgado PAF, havendo mesmo benefcios no ps-operatrio imediato e, eventualmente, alguns grupos especficos de doentes podem mesmo ser beneficiados.---------ABSTRACT: Ever since Bethesda statement in 1983, Liver Transplantation has been accepted as a clinical therapeutic procedure for many patients with advanced hepatic failure Holmgren, professor of genetics, suggested that one could expect that transplanting a new liver could lead to improve progressive neurological symptoms of Familial Amyloidotic Polyneuropathy (PAF). Bo Ericzon, the transplant surgeon at Huddinge Hospital in Stockholm, Sweden, did in 1991 the first Liver Transplant on a FAP patient. FAP is an inherited autosomal dominant neurologic disease in which the liver, otherwise structural an functionally normal, produces more than 90% of an abnormal protein (TTR Met30) whose deposits are responsible for symptoms. Liver Transplantation is currently the only efficient therapy available for FAP patients. Portugal is the country in the world where FAP is most prevalent. The Portuguese neurologist Corino de Andrade was the first to recognize in 1951 this particular form of inherited polyneuropathy, which is also known by the name of Andrade disease. Liver Transplantation started as a program in Portugal in September 1992. The first patient transplanted in Lisbon, Hospital Curry Cabral, was a FAP patient. From the beginning we did realize that competition among waiting lists of FAP and Hepatic patients would come to be a complex problem we had to deal with, on clinical and ethical grounds. There was one possible way-out. FAP livers could be of some utility themselves as liver grafts. Anatomically and functionally normal, except for the inherited abnormal trace, those livers could possibly be transplanted in selected hepatic patients. Nevertheless the FAP liver carried with it the ability to produce the mutant TTR protein. One could expect, considering the natural history of the disease that several decades would lapse before the recipient could suffer symptomatic neurologic disease, if at all. In Coimbra, Portugal, Linhares Furtado performed in 1995 the first transplant of a FAP liver to a patient with metastatic malignant disease, as a sequential or domino transplant. FAP Liver Transplant patients, because of some dysautonomic labiality and unexpected reactions when they are subjected to surgery, take special advantage when piggyback technique is used for hepatectomy. This technique leaves the vena cava of the patient undisturbed, so that return of blood to the heart is affected minimally, so that veno-venous extracorporeal bypass will not be necessary. The advantages of piggyback technique could not be afforded to FAP patients who became donors for sequential liver transplantation, before we did introduce our liver reconstruction technique in 2001. The hepatectomy took the vena cava together with the liver, which is the classical technique, and the use of extracorporeal veno-venous bypass was of necessity in most cases. The reconstruction technique we developed in our center and used for the first time in the world in 2001 consists in applying venous grafts to the supra-hepatic ostia of piggyback resected FAP livers so that the organ could be grafted to a hepatic patient whose liver was itself resected with preservation of the vena cava. This is the double piggyback sequential transplant of the liver. It is the objective of this thesis to evaluate the results of this technique that we did introduce, first of all that it is reliable and reproducible, secondly that the FAP donor is not subjected to any additional harm during the procedure, and finally that the recipient has the same prospects of a successful transplant as if the liver was collected from a cadaver donor. Our series of liver transplantation on FAP patients and sequential liver transplants represent both the largest experience in the world. To achieve the analysis of the questions mentioned above, we did refer to our data-base from September 1992 to November 2008. The reconstructive technique that we did introduce is feasible: it could be done with success in every case ion our series. It is also reproducible. It has been adopted by many international centers of reference that did mention it in their own publications. We do refer to our data-base in what concerns the safety for the FAP donor.Five years survival of FAP transplanted patients that have been donors (n=190) has been slightly superior to those who were not (n=77), with no statistical significance. However, if we consider five year survival of FAP transplanted patients after the beginning of sequential transplant program in our center, survival is better among those patients whose liver was used as a transplant (87% survival versus 71%, p=0.047). In what concerns recipients of FAP livers: Some short-term benefit of less perioperative morbi-mortality mainly less hemorrhage. In some groups of particular pathologies, there is a strong suggestion of better survival, however the scarcity of numbers make the differences not statistically significant. Patients with cirrhosis HVC (83% versus73%) and patients with primitive hepatic cancer within Milan criteria (survival of 70% versus 58%) are good examples. There is one relevant problem we left beyond discussion in the present work: this is the long-term impact of possible recurrence of FAP symptoms among recipients of sequential transplants. In Conclusion: The reconstruction technique that we did develop and introduce is consistently workable and reproducible. It is safe for FAP donors with the advantage that removal of vena cava can be avoided. Hepatic patients transplanted with those livers suffer no disadvantages and have the benefit of less hemorrhage. There is also a suggestion that survival could be better in cirrhosis HVC and primary liver cancer patients.
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This paper summarises the results of the first systematic, detailed prosopographic study of the MPs the deputados of the Lower Chamber - of the First Portuguese Republic (1910-1926). Data are presented both by legislature and for the overall period. Two kinds of background variables are explored: sociodemographic (birthplace, age, education and profession) and political (previous experience in other elite positions). Regime change in 1910 resulted in the replacement of the former political elite by homines novi. Most MPs of the Republican regime were born in small towns and communities, had carried out higher educational studies (with prevalence for law training), were mainly drawn from the professions (practising lawyers and doctors) and the Army, and were elected for the first-time at relatively young ages. Parliamentary turnover was high (two-thirds of the representatives held just one mandate) and a large proportion of MPs had a consistent connection (birth, family ties, occupation) to the constituencies to which they had been elected.
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RESUMO: pela contraco involuntria de grupos musculares de extenso varivel, originando movimentos involuntrios e posturas anmalas, por vezes dolorosas. O tratamento convencional consiste em injeces localizadas de toxina botulnica, podendo, em casos refractrios, estar indicado o tratamento por estimulao cerebral profunda. A neurobiologia da distonia focal primria permanece incompletamente compreendida. Os estudos de neuro-imagem estrutural e funcional revelam alteraes subtis da anatomia e funcionamento do estriado e das vias cortico-basais, com destaque para o aumento do volume, da actividade metablica e da neuroplasticidade do putamen e de reas corticais motoras, pr-motoras e sensitivas. O conjunto destas alteraes aponta para uma disrupo da regulao inibitria de programas motores automticos sustentados pelo estriado e pelas vias ortico-subcorticais. Nos ltimos anos tem crescido o interesse pelas manifestaes psiquitricas e cognitivas da distonia (estas ltimas muito pouco estudadas). Tem despertado particular interesse a possvel associao entre distonia focal primria e perturbao obsessivo-compulsiva (POC), cuja neurobiologia parece notavelmente sobreponvel da distonia primria. Com efeito, os estudos de neuro-imagem estrutural e funcional na POC revelam consistentemente aumento do volume e actividade do estriado e do crtex rbito-frontal, apontando mais uma vez para uma disfuno do controlo inibitrio, no estriado, de programas comportamentais e cognitivos automticos. Objectivos: 1. Explorar a prevalncia e intensidade de psicopatologia em geral, e de psicopatologia obsessivo-compulsiva em particular, numa amostra de indivduos com distonia focal primria; 2. Explorar a ocorrncia, natureza e intensidade de alteraes do funcionamento cognitivo numa amostra de indivduos com distonia focal primria; 3. Investigar a associao entre a gravidade da distonia focal, a intensidade da psicopatologia, e a intensidade das alteraes cognitivas. Metodologia: Estudo de tipo transversal, caso-controlo, observacional e descritivo, com objectivos puramente exploratrios. Casos: 45 indivduos com distonia focal primria (15 casos de blefaroespasmo, 15 de cibra do escrivo, 15 de distonia cervical espasmdica), recrutados atravs da Associao Portuguesa de Distonia. Critrios de incluso: idade = 18; distonia focal primria pura (excluindo casos de distonia psicognica possvel ou provvel de acordo com os critrios de Fahn e Williams); Metabolismo do cobre e Ressonncia Magntica Nuclear sem alteraes. Controlos doentes: 46 casos consecutivos recrutados a partir da consulta externa do Hospital Egas Moniz: 15 doentes com espasmo hemifacial, 14 com espondilartropatia cervical, 17 com sndrome do canal crpico. Controlos saudveis: 30 voluntrios. Critrios de excluso para todos os grupos: Mini-Mental State Examination patolgico, tratamento actual com anti-colinrgicos, antipsicticos, inibidores selectivos da recaptao da serotonina, antidepressivos tri- ou tetracclicos. Avaliao: Avaliao neurolgica: histria e exame mdico e neurolgico completos. Cotao da gravidade da distonia com a Unified Dystonia Rating Scale. Avaliao psicopatolgica: Symptom Check-List-90-Revised; entrevista psiquitrica de 60 minutos incluindo a Mini-International Neuropsychiatric Interview (MINI), verso 4.4 (validada em Portugus), complementada com os mdulos da MINI Plus verso 5.0.0 para depresso ao longo da vida e dependncia/ abuso do lcool e outras substncias ao longo da vida; Yale-Brown Obsessive-Compulsive Symptom Checklist e a Yale-Brown Obsessive-Compulsive Scale (Y-BOCS). Avaliao neuropsicolgica: Wisconsin Card Sorting Test (WCST; flexibilidade cognitiva); Teste de Stroop (inibio de resposta); Block Assembly Test (capacidade visuo-construtiva); Teste de Reteno Visual de Benton (memria de trabalho visuo-espacial). Anlise estatstica:os dados foram analisados com a aplicao informtica SPSS for Windows, verso 13. Para a comparao de propores utilizaram-se o teste do Chi-quadrado e o teste de Fisher. Para a comparao de variveis quantitativas entre dois grupos utilizou-se o teste t de Student ou o teste U de Mann-Whitney (teste de Wilcoxon no caso de amostras emparelhadas). Para comparaes de mdias entre trs grupos recorreu-se Anlise de Varincia a um factor (variveis de intervalo e de rcio), ou ao teste de Kruskal-Wallis (variveis ordinais). Para o estudo da associao entre variveis foram utilizados os coeficientes de correlao de Pearson ou de Spearman, a anlise de correlaes cannicas, a anlise de trajectrias e a regresso logstica. Adoptou-se um Alpha de 0.05. Resultados: Os doentes com distonia focal primria apresentaram uma pontuao mdia na Y- -BOCS significativamente superior dos dois grupos de controlo. Em 24.4% dos doentes com distonia a pontuao na Y-BOCS foi superior a 16. Estes doentes eram predominantemente mulheres, tinham uma maior durao mdia da doena e referiam predominantemente sintomas obsessivo-compulsivos (SOC) de contaminao e lavagem. Os dois grupos com doena crnica apresentaram pontuaes mdias superiores s dos indivduos saudveis nas escalas de ansiedade, somatizao e psicopatologia geral. Os doentes com distonia tratados com toxina botulnica apresentaram pontuaes inferiores s dos doentes no tratados nas escalas de ansiedade generalizada, fobia, somatizao e depresso, mas no na Y-BOCS. Sessenta por cento dos doentes com distonia apresentavam pelo menos um diagnstico psiquitrico actual ou pregresso. O risco de apresentar um diagnstico psiquitrico actual era menor nos doentes tratados com toxina botulnica, aumentando com a gravidade da doena. A prevalncia de POC foi 8,3% e a de depresso major 37,7%. No WCST e na Prova de Benton, os doentes com distonia focal primria demonstraram um desempenho inferior ao de ambos os grupos de controlo, cometendo sobretudo erros perseverativos. Os doentes com distonia e pontuao na Y-BOCS > 16 cometeram mais erros e respostas perseverativas no WCST do que os restantes doentes com distonia. As anlises de correlaes e de trajectrias revelaram que nos doentes com distonia a gravidade da distonia foi, juntamente com a idade e a escolaridade, o factor que mais interagiu com o desempenho cognitivo. Discusso: o nosso estudo o primeiro a descrever, nos mesmos doentes com distonia focal primria, SOC significativos e alteraes cognitivas. Os nossos resultados confirmam a hiptese de uma associao clnica especfica entre distonia focal primria e psicopatologia obsessivo-compulsiva. Confirmam igualmente que a distonia focal primria est associada a um maior risco de desenvolver morbilidade psiquitrica ansiosa e depressiva. O tratamento com toxina botulnica reduz este risco, mas no influencia os SOC. Entre os doentes com distonia, os que tm SOC significativos podero diconstituir um grupo particular com maior durao da doena (mas no uma maior gravidade), predomnio do sexo feminino e predomnio de SOC de contaminao e limpeza. Em termos cognitivos, os indivduos com distonia focal primria apresentam dfices significativos de flexibilidade cognitiva (particularmente acentuados nos doentes com SOC significativos) e de memria de trabalho visuo-espacial. Estes ltimos devem-se essencialmente a um dfice executivo e no a uma incapacidade visuo-construtiva ou visuo-perceptiva. A disfuno cognitiva no explicvel pela psicopatologia depressiva nem pela incapacidade motora, j que os controlos com doena perifrica crnica tiveram um desempenho superior ao dos doentes com distonia. No seu conjunto os nossos resultados sugerem que os SOC que ocorrem na distonia focal primria constituem uma das manifestaes clnicas da neurobiologia desta doena do movimento. O predomnio de sintomas relacionados com higiene e o perfil disexecutivo de alteraes cognitivasperseverao e dificuldades executivas de memria de trabalho visuo-espacial apontam para a via cortico-basal dorso-lateral e para as reas corticais que lhe esto associadas como estando implicadas na tripla associao entre sintomas motores, obsessivo-compulsivos e cognitivos. Concluses: A distonia focal primria um sndrome neuropsiquitrico complexo com importantes manifestaes no motoras, nomeadamente compromisso cognitivo do tipo disexecutivo e sintomas obsessivo-compulsivos. Clinicamente estas manifestaes representam necessidades de tratamento que vo muito para alm da simples incapacidade motora, devendo ser activamente exploradas e tratadas.-------------- ABSTRACT: Introduction: primary focal dystonia is an idiopathic movement disorder that manifests as involuntary, sustained contraction of muscular groups, leading to abnormal and often painful postures of the affected body part. Treatment is symptomatic, usually with local intramuscular injections of botulinum toxin. The neurobiology of primary focal dystonia remains unclear. Structural and functional neuroimaging studies have revealed subtle changes in striatal and cortical-basal pathway anatomy and function. The most consistent findings involve increased volume and metabolic activity of the putamen and of motor, pre-motor and somato-sensitive cortical areas. As a whole, these changes have been interpreted as reflecting a failure of striatal inhibitory control over automatic motor programs sustained by cortical-basal pathways. The last years have witnessed an increasing interest for the possible non-motor mainly psychiatric and cognitive manifestations of primary focal dystonia. The possible association of primary focal dystonia with obsessive-compulsive disorder (OCD) has raised particular interest. The neurobiology of the two disorders has indeed remarkable similarities: structural and functional neuroimaging studies in OCD have revealed increased volume and metabolic activity of the striatum and orbital-frontal cortex, again pointing to a disruption of inhibitory control of automatic cognitive and behavioural programs by the striatum. Objectives: 1. To explore the prevalence and severity of psychopathology with a special emphasis on obsessive-compulsive symptoms (OCS) in a sample of patients with primary focal dystonia;2. To explore the nature and severity of possible cognitive dysfunction in a sample of patients with primary focal dystonia; 3. To explore the possible association between dystonia severity, psychiatric symptom severity, and cognitive performance, in a sample of patients with primary focal dystonia. Methods: cross-sectional, case-control, descriptive study. Cases: forty-five consecutive, primary pure focal dystonia patients recruited from the Portuguese Dystonia Association case register (fifteen patients with blepharospasm, 15 with cervical dystonia and 15 with writers cramp). Inclusion criteria were: age = 18; primary pure focal, late-onset dystonia (excluding possible or probable psychogenic dystonia according to the Fahn & Williams criteria); normal copper metabolism and Magnetic Resonance Imaging. Diseased controls: forty-six consecutive subjects from our hospital case register (15 patients with hemi-facial spasm; 14 with cervical spondilarthropathy and cervical spinal root compression; 17 with carpal tunnel syndrome). Healthy controls were 30 volunteers.Exclusion criteria for all groups: Mini-Mental State Examination score below the validated cut-off for the Portuguese population (<23 for education between 1 and 11 years; <28 for education >11 years); use of anti-cholinergics, neuroleptics, selective serotonin reuptake inhibitors, triciclic or tetraciclic antidepressants. Assessment: neurological assessment: complete medical and neurological history and physical examination; dystonia severity scoring with the Unified Dystonia Rating Scale. Psychiatric assessment:Symptom Check-List-90-Revised; 60 minute-long psychiatric interview, including Mini-International Neuropsychiatric Interview (MINI), version 4.4 (validated Portuguese version), extended with the sections for life-time major depressive disorder and life-time alcohol and substance abuse disorder from MINI-Plus version 5.0.0; Yale-Brown Obsessive-Compulsive Symptom Checklist and Yale-Brown Obsessive-Compulsive Scale (Y-BOCS). Cognitive assessment: Wisconsin Card Sorting Test (WCST; cognitive set-shifting ability); Stroop Test (response inhibition); Block Assembly Test(visual-constructive ability); Bentons Visual Retention Test (visual-spatial working memory). Statistic analysis: Data were analyzed with SPSS for Windows version 13. Proportions were compared using Chi-Square test, or Fishers exact test when appropriate. Students t-test or Mann-Whitneys U test (or Wilcoxons teste in the case of matched samples) were used for two-group comparisons. P-values were corrected for multiple comparisons. One-way ANOVA with Bonferroni post-hoc analysis (interval data), or the Kruskal-Wallis Test (ordinal data), were used for three-group comparisons. Associations were analysed with Pearsons or Spearmans correlation coefficients, canonical correlations, path analysis and logistic regression analysis. Alpha was set at 0.05. Results: Dystonia patients had higher Yale-Brown Obsessive-Compulsive Symptom scores than both control groups. 24.4% of primary dystonia patients had a Y-BOCS score > 16. These patients were predominantly women; they had longer disease duration, and showed a predominance of hygiene-related OCS. The two groups with chronic disease had higher anxiety, somatization and global psychopathology scores than healthy subjects. Primary dystonia patients undergoing treatment with botulinum toxin had lower anxiety, phobia, somatization and depression scores than their untreated counterparts, but similar Y-BOCS scores. Sixty percent of primary dystonia patients had at least one lifetime psychiatric diagnosis. The odds of having a currently active psychiatric diagnosis were lower in botulinum toxin treated patients, and increased with dystonia severity. The prevalence of OCD was 6.7%, and the lifetime prevalence of major depression was 37.7%. Primary dystonia patients had a lower performance than the two control groups in both the WCST and Bentons Visual Retention Test, mainly due to an excess of perseveration errors. Primary dystonia patients with Y-BOCS score > 16 had much higher perseveration error and perseveration response scores than dystonia patients with Y-BOCS = 16. Correlation and path analysis showed that, in the primary dystonia group, dystonia severity, along with age and education, was the main factor influencing cognitive performance. Discussion: our study is the first description ever of concomitant significant OCS and cognitive impairment in primary dystonia patients. Our results confirm that primary dystonia is specifically associated with obsessive-compulsive psychopathology. They also confirm that primary focal dystonia patients are at a higher risk of developing anxious and depressive psychiatric morbidity. Treatment with botulinum toxin decreases this risk, but does not influence OCS. Primary focal dystonia patients with significant OCS may constitute a particular subgroup. They are predominantly women, with higher disease duration (but not severity) and a predominance of hygiene related OCS.In terms of cognitive performance, primary focal dystonia patients have significant deficits involving set-shifting ability and visual-spatial working memory. The latter result from an essentially executive deficit, rather than from a primary visual-constructive apraxia or perceptual deficit. Furthermore, cognitive flexibility difficulties were more prominent in the subset of primary dystonia patients with significant OCS. The cognitive dysfunction found in dystonia patients is not attributable to depressive psychopathology or motor disability, as their performance was significantly lower than that of similarly impaired diseased controls. Our results suggest that OCS in primary focal dystonia are a direct, primary manifestation of the motor disorders neurobiology. The predominance of hygiene-related symptoms and the disexecutive pattern of cognitive impairment set-shifting and visual-spatial working memory deficits suggest that the dorsal-lateral cortical-basal pathway may play a decisive role in the triple association of motor dysfunction, OCS and cognitive impairment. Conclusions: primary focal dystonia is a complex neuropsychiatric syndrome with significant non- -motor manifestations, namely cognitive executive deficits and obsessive-compulsive symptoms.Clinically, our results show that PFD patients may have needs for care that extend far beyond a merely motor disability and must be actively searched for and treated.
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A Work Project, presented as part of the requirements for the Award of a Masters Degree in Management from the NOVA School of Business and Economics
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A Work Project, presented as part of the requirements for the Award of a Masters Degree in Management from the NOVA School of Business and Economics
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A Work Project, presented as part of the requirements for the Award of a Masters Degree in Management from the NOVA School of Business and Economics
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RESUMO: O Lbano um pequeno pas na costa leste do Mar Mediterrneo, com uma populao de aproximadamente 4.350.000 pessoas, incluindo 1,5 milhes de refugiados, 400 mil dos quais so palestinos atendidos pela UNRWA (Agncia das Naes Unidas de Socorro aos Refugiados da Palestina) (UNHCR, 2013; OMS, 2010a). Desde 2012, um excedente de 1.000.000 refugiados srios cruzaram a fronteira com o Lbano, representando um aumento populacional de aproximadamente 25%. Alm disso, entre 1975 e 1990, a violenta guerra civil pela qual o Lbano passou, destruiu grande parte da infra-estrutura do pas, incluindo os servios de sade. O sector da sade, mais especificamente os servios de sade mental, majoritariamente privado. Servios especializados em Sade Mental esto disponveis em trs hospitais psiquitricos privados, e em 4 unidades psiquitricas de hospitais gerais, que esto localizados centralmente em torno da capital, Beirute. O Lbano um dos dois nicos pases da regio que no tem uma Poltica de Sade Mental e um dos seis pases que no tm uma Legislao em Sade Mental. Nos ltimos anos, a Sade Mental est sendo colocada no topo da agenda nacional, apesar das contnuas questes polticas e de segurana. Baseando-se nas informaes acima, um projecto de estratgia em Sade Mental, conduzido pelo Ministrio da Sade e apoiado pela OMS, foi escrito para servir como um guia para trabalhar em diferentes aspectos relacionados tanto em sade mental quanto em organizao dos servios, reviso de legislao, financiamento e proteo dos direitos humanos bsicos dos usurios do servio. Esta tese descreve o processo pelo qual o projecto de estratgia nacional de Sade Mental foi desenvolvido, seus principais componentes, os prximos passos a serem tomados para a sua implementao, os desafios e as oportunidades para implement-lo e prope alguns passos iniciais a serem tomados em primeiro lugar.----------ABSTRACT: Lebanon is a small country on the eastern shore of the Mediterranean Sea with a population of approximately 4,350,000 including 1,500,000 refugees, 400,000 of whom are Palestinians served by UNRWA (the United Nations Relief and Works Agency for Palestine Refugees) (UNHCR, 2013; WHO, 2010a). Since 2012 an excess of 1,000,000 Syrian refugees have crossed the border into Lebanon accounting for approximately 25% increase in the population. In addition, from 1975 to 1990 Lebanon underwent a violent civil war that had also destroyed much of the country infrastructure including health services. The health sector, more so the mental health services, is mostly private. Specialized Mental Health services are available at three private mental hospitals, and 4 psychiatric units within general hospitals, which are located centrally around the capital, Beirut. Lebanon is one of only two countries of the region that does not have a Mental Health policy and one out of the six countries that does not have a Mental Health legislation. In recent years, Mental Health is getting placed higher on the national agenda despite the ever continuing political and security issues. Based on the above, A Mental Health strategy draft, lead by the Ministry of Health and supported by WHO, was written to serve as a guide to work on different aspects related to Mental Health from service organization, to the revision of legislation, financing and the protection of the basic human rights of service users. This thesis describes the process through which the national Mental Health draft strategy was developed, its main components, the next steps to be taken for its implementation, the challenges and the opportunities to implementing it and proposes a few initial steps to be taken first.
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MARQUES, B.P. (2014) From Strategic Planning to Development Initiatives: a first reflection on the situation of Lisbon and Barcelona, in 20th APDR Congress Proceddings, APDR and Uvora, vora, pp. 850-857, ISBN 978-989-8780-01-0.
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A producer of 5.4 M bbl/d, totalling almost half of the consumption of the entire European Union, the Gulf of Guinea is a fundamental lifeline and maritime link between Europe, the Americas and Africa. Geographically positioned as a staging post for transit originating in Latin America and coupled with its relatively porous borders, the region is also the perfect stepping stone for contraband heading to European shores. While blessed with an enviable wealth of marine and mineral resources, the region is also plagued by an ever-increasing spectre of maritime piracy; accounting for around 30% of incidents in African waters from 2003 to 2011. It is for these reasons that this research centres around the issues of maritime security in the Gulf of Guinea, with a particular focus on the first two decades of the 21st century. This research looks to examine the overall picture of the present state of play in the area, before going on to provide an analysis of potential regional developments in maritime security. This research begins with the analysis of concepts/phenomena that have played a notable role in the shaping of the field of maritime security, namely Globalisation and security issues in the post-Cold War era. The ensuing chapter then focuses in on the Gulf of Guinea and the issues dominating the field of maritime security in the region. The penultimate chapter presents a SWOT analysis, undertaken as part of this research with the aim of correlating opinions from a variety of sectors/professions regarding maritime security in the Gulf of Guinea. The final chapter builds upon the results obtained from the abovementioned SWOT analysis, presenting a series of potential proposals/strategies that can contribute to the field of maritime security in the region over the coming years. This research draws to a close with the presentation of conclusions taken from this particular investigation, as well as a final overview of the earlier presented proposals applicable to the field of maritime security during the second decade of the 21st century.
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A Work Project, presented as part of the requirements for the Award of a Double Degree in Economics from NOVA School of Business and Economics and Maastricht School of Business and Economics
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RESUMO: Introduo e objetivos: No existia um estudo multicntrico que descrevesse as caractersticas dos doentes com EM, da doena em si, ou do seu tratamento, em Portugal.Mtodos: Doentes McDonald 2010 positivos foram sequencialmente recrutados em 7 centros entre Maio e Novembro 2014. Aplicou-se um Caderno de Recolha de Dados incidindo na demografia, doena, educao e emprego (estudo PORT-MS). Resultados: 561 doentes includos. Primeiros sintomas aos 30,210,5 anos (RRMS 29,210, PPMS 39,411,7, p<0,001); diagnstico 3,25,3 anos depois (RRMS 3,05,1, PPMS 4,92,5, p=0,002); tempo de doena aps diagnstico 9,47,2 anos (semelhante RRMS no diagnstico e PPMS); idade atual 42,912,4 anos (grupo RRMS no diagnstico 42,012,1, PPMS 52,511,3, p<0,001); EDSS atual 2,5 (RRMS 2.0, PPMS 6.0); proporo feminino:masculino 2,5:1 (RRMS semelhante, PPMS 1,1:1, p<0,05); no diagnstico RRMS 90,6%, SPMS 0,9%, PPMS 8,6%; 9,5% dos RRMS encontravam-se em SP na incluso (nomeadamente os com mais idade no diagnstico e/ou atualidade ou tempo de doena mais prolongado). PPMS mais frequente em doentes diagnosticados mais tardiamente (p<0,001), onde aumenta tambm ligeiramente a proporo de mulheres na PPMS. Nas ltimas dcadas: novos casos mostram estabilidade na proporo de gneros e tipos de doena; idade nos primeiros sintomas e no diagnstico aumentou ligeiramente, tempo entre eles diminuiu ligeiramente. Proporo sob DMT (Maio 2014): global 84,5%; atualmente RRMS 90,4%; SPMS 70,8%; PPMS 36,8%; progressivas agregadas 48%. Tipo de DMT, amostra global: interferes 56,5%, GA 18,4%, Natalizumab 11,6%, Fingolimod 9,7%. Global: economicamente ativos 61,5%, desemprego 13,5%, 74,1% dos no activos esto reformados por doena. Gravidezes aps diagnstico em 15% mulheres. Casos com histria familiar positiva 7,8%. Discusso e concluses: Includa cerca de 10% da populao portuguesa. Resultados congruentes com dados internacionais. Elevada proporo sob DMT, mesmo EDSS alto e formas progressivas. Teraputicas de segunda linha sub representadas. Doentes jovens e com doena ligeira com vida econmica ativa; restantes essencialmente reformados por doena.---------------- ABSTRACT : Background/aims: In Portugal, there wasnt a multicentric study on the general characteristics (demography, disease milestones, DMT, socioeconomic status) of Multiple Sclerosis patients. Methods: Patients fulfilling McDonald 2010 criteria were sequentially recruited from May to November 2014 in 7 centers and data was systematically collected. Results: 561 patients included. First symptoms occurred at 30,210,5 years-old (RRMS 29,210, PPMS 39,411,7, p<0,001); diagnosis 3,25,3 years later (RRMS 3,05,1, PPMS 4,92,5, p=0,002); 9,47,2 years elapsed since diagnosis (similar for those is RRMS at diagnosis and PPMS); current age 42,912,4 years-old (group RRMS at diagnosis 42,012,1, PPMS 52,511,3, p<0,001); current EDSS 2,5 (RRMS 2.0, PPMS 6.0); females to males 2,5:1 (RRMS similar, PPMS 1,1:1, p<0,05); at diagnosis RRMS 90,6%, SPMS 0,9%, PPMS 8,6%; 9,5% of RRMS reached SP at inclusion (those older at diagnosis, in actuality, or with longer follow-up). PPMS more frequente in patients diagnosed at older ages (p<0,001), also slight increase in females. Along the last decades: new cases have showed stable proportions of gender and disease types; age at first symptoms and diagnosis slightly increased, time between them slightly decreased. Proportion on DMT (May 2014): 84,5% of all; 90,4% of currently in RRMS; 70,8% of SPMS; 36,8% of PPMS; 48% of progressive forms together. Type of DMT, all patients: interferons 56,5%, Glatiramer Acetate 18,4%, Natalizumab 11,6%, Fingolimod 9,7%. Economically active 61,5% of all, unemployment 13,5%, 74,1% of non-active are retired due to disease. Females pregnant after diagnosis 15%. Positive family cases in 7,8%. Discussion/Conclusions: 10% of the national MS population collected. Data generally consistente with international reports. Proportion under DMT relatively high in all disease types, but second line therapies underrepresented. Young patients with mild disease have an active economic life. Those not active are essentially retired due to disease.
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RESUMO - As mudanas na sade so cada vez mais rpidas e os servios de sade tm cada vez mais dificuldade em dar resposta aos problemas de sade dos portugueses. Responsveis por grande parte da despesa em sade, os idosos so a populao que mais utiliza os servios de sade e as respetivas unidades hospitalares e servios de urgncia. Estes tm estadias mais prolongadas e consomem mais recursos durante essas permanncias nas instituies de sade. Sabendo isto revelou-se oportuno encontrar as principais causas de internamento hospitalar, os principais diagnsticos secundrios, demoras mdias e a sua relao com as principais causas de morte na populao portuguesa com mais de 65 anos no perodo de 2003-2012. Para tal, optou-se por uma anlise descritiva de 3375817 episdios de internamento referentes a dez anos. Daqui retirou-se que os diagnsticos principais mais frequentes para todos os anos e todas as faixas etrias so o acidente vascular cerebral isqumico e a pneumonia, sendo que o primeiro o mais frequente at 2006, passando depois a ser a pneumonia o mais frequente. A demora mdia maior quanto mais diagnsticos secundrios associados houver e aumenta com a idade. Os diagnsticos secundrios mais frequentes so a hipertenso essencial e a diabetes mellitus. Estes dados so relevantes para o conhecimento da sade em Portugal, podendo-se alterar e uniformizar e melhorar prticas hospitalares e com isso progredir na qualidade dos tratamentos e aumentar a qualidade de vida com hiptese de diminuio da demora mdia.
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This study analyses the principle of presumption of innocence in the preliminary stages of the Portuguese criminal process, its procedural aspect related with the principle of in dubio pro reo and its material aspect concerning the treatment of the defendant during the proceedings. The consequences and manifestations of the principle of presumption of innocence are analysed in the decisions of the closing stages of the preliminary criminal procedure and the application of the principle of in dubio pro reo is analysed in the judgement of sufficiency of evidence for the procedure to continue. It addresses the question of circumstantial evidence, its particular relevance in economic and financial crime, highly organized crime, the grounds for the indictment in general and when the sufficiency of evidence criteria is based on that evidence. It analyses the scope of the principle of presumption of innocence in the application of coercive measures, with reference to the arrest, first interrogation of the accused under detention and reasons for the subsequent dispatch about the measures. The asset assurance measures of preventive seizure and the preventive seizure to ensure confiscation are analysed and principle of presumption of innocence is considered non applicable to those measures.
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In broad sense, Project Financing1 as a mean of financing large scale infrastructural projects worldwide has had a steady growth in popularity for the last 20 years. This growth has been relatively unscathed from most economic cycles. However in the wake of the 2007 systemic Financial Crisis, Project Financing was also in trouble. The liquidity freeze and credit crunch that ensued affected all parties involved. Traditional Lenders, of this type of financial instrument, locked-in long-term contractual obligations, were severely hit with scarcity of funding compounded by rapidly increasing cost of funding. All the while, Banks were rescued by the concerted actions of Central Banks and other Multi-Lateral Agencies around the world but at the same time stressed by upcoming regulatory effort (Basel Committee). This impact resulted in specific changes to this type of long-term financing. Changes such as Commercial Banks increased risk aversion; pricing increase and maturities decrease of credit facilities; enforcement of Market Disruption Event clauses; partial responsibility for project risk by Multilateral Agencies; and adoption of utility-like availability payments in other industrial sectors such as transportation and even social infrastructure. To the extent possible, this report is then divided in three parts. First, it begins with a more instructional part, touching academic literature (theory) and giving the Banks perspective (practice), but mostly as an overview of Project Finance for awareness sake. The renowned Harvard Business School professor Benjamin Esty, states2 that Project Finance is a relatively unexplored territory for both empirical and theoretical research which means that academic research efforts are lagging the practice of Project Finance. Second, the report presents a practical case regarding the first Road Concession in Portugal in 1998 ending with the lessons learned 10 years after Financial Close. Lastly, the report concludes with the analysis of the current trends and changes to the industry post Financial Crisis of the late 2000s. To achieve this Ill reference relevant papers, books on the subject, online articles and my own experience in the Project Finance Department at a major Portuguese Investment Bank. Regarding the latter, with the signing of a confidentiality agreement, Im duly omitting sensitive and proprietary bank information.
