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Le syndrome de West (SW), communément appelé spasmes infantiles (SI), est un trouble épileptique généralement caractérisé par la triade de spasmes infantiles, un modèle d'électroencéphalogramme (EEG) pathognomonique appelé hypsarythmie, et la régression du développement. Alors que des études précédentes ont été en mesure d'obtenir une réponse relativement adéquate par rapport au contrôle des spasmes et la résolution d’hypsarythmie, elles n’ont pas réussi à fournir des options thérapeutiques décisives à l’égard des séquelles neurodéveloppementales souvent associées aux SI. Notre étude, sur laquelle est basée cette thèse, est la première à utiliser un traitement complémentaire aux médicaments antiépileptiques conventionnels, avec l'intention d'améliorer les résultats neurodéveloppementaux de cette population. Les patients recrutés dans notre essai clinique randomisé (ECR) original ont suivi un protocole de traitement standardisé composé de vigabatrin (VGB) comme traitement de première intention pendant deux semaines, suivi de l'hormone corticotrope (ACTH) chez les non-répondeurs pour une période de deux autres semaines, et le topiramate dans les cas réfractaires. En plus, les patients ont été randomisés pour recevoir soit le traitement expérimental, flunarizine, soit un placebo, pendant six mois. Notre ECR multicentrique consistait à recruter et évaluer 68 patients, la plupart suivis à 8 différentes visites sur une période de cinq ans afin de précisément évaluer leurs progrès neurodéveloppementaux. Notre essai clinique a généré trois études principales qui forment le coeur de cette thèse. Dans une première étude, les données cliniques et cognitives des deux premières années d’évaluation ont été analysées. Les résultats cliniques à court terme indiquent un taux élevé de cessation de spasmes et de l’hypsarythmie. De plus, cette étude rapporte les premiers résultats cognitifs mesurés par le Bayley Scales of Infant Development (BSID) et le Vineland Adaptive Behavior Scale (VABS). Notre deuxième étude a essentiellement fourni des données cognitives à plus long terme, 5 ans après le début de son initiation. Les réponses cognitives ont été mesurées par le BSID, le VABS, et aussi par le Stanford-Binet Intelligence Scale (SB5) chez les patients ayant un fonctionnement cognitif plus élevé. Une amélioration significative et progressive des fonctions cognitives a été observée, indépendamment de la thérapie adjuvante. Des facteurs de risque cognitifs à long terme ont également été révélés dans cette étude. Notre dernière étude a essayé d’élucider la relation entre les SI et les troubles du spectre autistique (TSA). Un test de dépistage avec le Checklist for Autism in Toddlers (CHAT) a été effectué à 24 mois, et un diagnostic a été obtenu par moyen du Autism Diagnostic Observation Schedule (ADOS) à 30 et 60 mois. L’ADOS a évalué 44 patients, dont 10 ont été diagnostiqués avec TSA. Une description des facteurs de risque associés aux TSA ont été présentés dans cet article. Enfin, basé sur nos résultats et les informations à ce sujet dans la littérature, nous avons tenté d'élucider les caractéristiques physiopathologiques de la maladie. Une description des mécanismes biologiques sous-jacents impliqués dans le syndrome de West et des traitements cibles associés ont été présentés. Bien que le traitement complémentaire, le flunarizine ne se soit pas avéré être avantageux pour notre cohorte, notre protocole de traitement a tout de même été en mesure de démontrer des résultats cliniques et cognitifs supérieurs dans le sous-groupe de patients avec SI dont l’étiologie est inconnue. Ces résultats, ainsi que l’identification de nouveaux facteurs de risque neurodéveloppementaux potentiels, pourraient être utilisés cliniquement afin d’améliorer le diagnostic et le suivi médical des patients atteints du syndrome de West. / West syndrome (WS), commonly referred to as infantile spasms (IS), is an epileptic disorder usually characterized by the triad of infantile spasms, a pathognomonic electroencephalogram (EEG) pattern called hypsarrhythmia, and developmental regression. While previous treatment studies were able to achieve relatively adequate spasm control and hypsarrhythmia resolution in this population of patients, they have failed to provide conclusive and definite therapeutic options aimed at improving the poor cognitive outcome often associated to IS. Our study, on which this thesis is based, was the first to use an add-on treatment to conventional antiepileptic drugs, with the intent to improve long-term cognitive outcome in this population. Patients recruited in our original randomized clinical trial (RCT) followed a standardized treatment protocol consisting of vigabatrin (VGB) as first-line treatment for two weeks, followed by adrenocorticotropic hormone (ACTH) in non-responders for another two-week period, and topiramate in refractory cases. In addition, patients were randomized to either receive placebo or flunarizine adjunct therapy for six months. Our multi-centric RCT recruited and evaluated 68 patients, most of which were followed at 8 different time points over a five-year period, to precisely evaluate their neurodevelopmental progress. Our clinical trial generated three main studies which comprise the core of this thesis. In a first study, clinical and cognitive data from the first two years were analyzed. Spasm arrest and hypsarrhythmia resolution were the short-term clinical endpoint measures, while the Vineland Adaptive Behavior Scale (VABS) and Bayley Scales of Infant Development (BSID) were used as cognitive outcome measures at 2 years. This first study most importantly reports on the superior short-term clinical response rate achieved in our study population. Preliminary cognitive results were also presented in this work. Our second study essentially presented long-term cognitive data 5 years after the start of the study. Cognitive outcome measures were similar to those used at two years with the addition of the Stanford-Binet Intelligence Scale, Fifth Edition (SB5) for higher functioning patients. Most IS patients, particularly those with no known etiology, displayed a significant and progressive improvement of cognitive functions, irrespective of adjunctive therapy. Risk factors of long term poor cognitive outcome were also revealed in this study. Our last study tried to understand the relationship between IS and autism spectrum disorders (ASD). Autism was initially screened by means of the Checklist for Autism in Toddlers (CHAT) at 24 months, and formally assessed at the 30-and 60-month follow-up visits using the Autism Diagnostic Observation Schedule (ADOS). ADOS was performed in 44 patients, 10 of which were diagnosed with ASD. A description of risk factors associated with an ASD outcome in the IS population were presented in this article. Finally, based on our study results and in conjunction with literature information on the topic, we attempted to elucidate the pathophysiological characteristics of the disorder. A conceivable description of the underlying biological mechanisms implicated in West syndrome and associated target treatments were presented. Although our complementary treatment, flunarizine, did not prove to be beneficial in our cohort, our treatment protocol was nonetheless able to demonstrate superior clinical and cognitive outcomes in patients with unknown etiologies. These findings, as well as the identification of new potential neurodevelopmental risk factors, could be used clinically to improve the diagnosis and medical follow-up of patients with West syndrome.

生產者服務業其有創新與改變技術的功能，亦有調節勞力分工擴張、加強組織內部關聯的作用，由於該等功能與作用對於生產力的提昇、就業機會的創造與貿易出口量的增加均具有重要意義，因此它被視為現代生產系統中重要的部門。本研究首先以Gemmell產業成長理論探討各台北都會區產業結構的變遷，證明服務業已然成為現代生產系統中重要的部門；再以產業感應度(sensibility)、影響度(dispersion)分析生產者服務業的產業關聯，證明生產者服務業以服務生產部門為主；爾後再以組織理論(organization theory)中彈性生產(flexible production)及勞力分工(labor division)兩個向度分析生產者服務業的成長及原因，證實生產者服務業能自我創造就業及創新技術獲致成長。其後再以區位商數及成長商數交叉分析生產者服務業在台北都會區的成熟度，顯示台北縣生產者服務業呈顯著的成長然未具輸出性，成熟度較台北市弱，但因接收台北市的廠商而擴張。繼之假設生產者服務業於空間分布受到其所服務的產業影響而分布，利用兩產業間密度斜率(density gradient)的牽動關係分析生產者服務業在空間移動的現象，藉以瞭解我國在生產者服務業所應有的產業區位政策。所得的結論有二：其一，生產者服務業興製造業分布呈現互斥的情況。另外，生產者服務業與人口分布呈現移動一致的現象，亦即生產者服務業與人口的分布，呈互補性顯著的情形。最後以生產者服務業內的國際貿易業為例，分析其路網結構(network concept)後證明該業的集中與台北都會區通訊便利性及聚集經濟不可分。 / Producer Services Industres have the functions of innovation and reformation skills, and the functions to regulate the extension of division of labor and strengthen internal organizational correlation. Since these functions are important for upgrading productivity, creating job opportunities and increasing quantity of export, they are regarded as a vital sector of modern production system. 　　First, this research tries to make use of Gemmell's Theory of Industrial Development to study the changes in the structure of industries. It shows that Services Sector has become an important sector of the whole modern production system. 　　Second, this research attempts to use industrial sensbility and dispersion to analyze the correlation of Producer Services Industries. It has proved that Service Sector is a major part of Producer Services Industries. 　　Third, this research employs two kinds of vector, such as flexible production from Organization Theory, and division of labor to analyze the development and factors of emergence of Producer Services Industry. It has proved that Producer Services Inustries can bring to creation of new jobs, innovation and development of skills. 　　Fourth, this research uses Location Quotient and Growth Quotient to crossexamine the degree of maturity of Producer Services Industries in Taipei Urban District. It has shown that Producer Services Industries in Taipei Hsian bears a distinct development but not in an output manner. The degree of maturity in Taipei Hsian is lesser than that of Taipei City. And Taipei Hsian can be expanded because of absorbing or attracting the holders of factories from Taipei City. 　　Fifth, this research assumes that in terms of spatial distribution, Producer Services Industries are distributed according to the influence of Industries served by Producer Services Industries. By means of the linking and relations of density gradients among industries, the phenomenon spatial movement can be illustrated. It can be regarded as a proper industrial district policy of Producer Services Industries. 　　Two points of conclusion can be made: 1) there is a repulsion for distribution between Producer Services Industry and Manufacturing Industry; 2) there is a phenomenon of concurrent movement between Producer Services Industries and distribution of population. It means that they have a distinct condition of mutual compenstaion. 　　Sixth, this research tries to make International Trade as an example of Producer services Industries, to analyze the network concept and prove that there are no any separations among concentration of this Industry, convenience of communication in Taipei Urban District and aggregate economy.

Ce travail de thèse vise à évaluer le rôle des afférences cutanées plantaires dans le contrôle postural et oculomoteur en manipulant l’extéroception plantaire à l’aide de stimulations mécaniques plantaires fines. La 1ère étude a montré que des éléments médio-plantaires fins externes (EME) et surtout internes (EMI) améliorent la stabilité orthostatique et postériorisent le CPP de sujets jeunes et sains lors de mouvements des yeux. Ils ont aussi une action spécifique sur la vergence (pas sur les saccades) : les EMI augmentent l’amplitude phasique de divergence et diminuent la partie tonique, autrement dit agissent surtout sur la composante pré-programmée du mouvement ; alors que les EME agissent sur la convergence en augmentant son amplitude tonique, composante contrôlée sous influence de la rétroaction visuelle. Les inserts influent de manière directe sur le contrôle postural et oculomoteur par des voies spécifiques et indépendantes. La 2ème étude explique la variabilité des réponses des sujets de la 1ère. Nous avons mesuré leur degré d’utilisation des afférences extéroceptives plantaires par la méthode du Quotient Plantaire (QP = Surface CPP mousse / Surface CPP sol dur X 100). Un QP<100 suggère une Inefficience des Afférences Plantaires (IAP) : les sujets IAP ne présentent plus d’amélioration de leur stabilité ni aucune modification de la vergence avec les inserts plantaires. Nous proposons que cette situation soit non physiologique et relève d’un dysfonctionnement non douloureux latent des récepteurs plantaires, opposant les sujets IAP aux sujets ayant un QP normal. La 3ème étude a investigué les rapports entre l’utilisation des afférences plantaires et visuelles chez de jeunes adultes sains avec la méthode du QP et du Quotient du Romberg (QR). Elle mis en évidence l’existence d’une utilisation synergique des afférences visuelles et plantaires en vision de près, uniquement chez les sujets au QP normal. Les sujets IAP ont un QR plus bas que les sujets au QP normal, en vision de près et sur sol dur uniquement. Sur mousse, leur QR des sujets IAP augmente ; les yeux fermés, leur QP augmente, ce qui objective une asynergie visuo-podale. La 4ème étude, sur la même population, a montré qu’un EME bilatéral augmente l’esophorie, uniquement de loin et chez les sujets IAP. La 5ème étude a révélé l’influence de l’extéroception plantaire sur la perception de la Verticale Visuelle Subjective (VVS) sur la même population. Elle a montré une diminution de l’erreur vers la gauche de près chez les sujets au QP normal avec un EME droit, ainsi qu’une diminution de l’erreur absolue de loin chez les sujets IAP avec un EMI bilatéral. L’ensemble de ces travaux consolide au plan théorique l’importance des afférences plantaires aussi bien pour le contrôle orthostatique, le contrôle de la vergence, la perception de la VVS et l’alignement des yeux (phories). Ils démontrent que l’utilisation de ces afférences est variable selon les sujets et ont des implications cliniques pluridisciplinaires. / This thesis aims at assessing the role of plantar cutaneous afferents in postural and oculomotor control by manipulating plantar exteroception with thin mechanical plantar stimulations. The 1st study showed that lateral, and even more medial mid-foot plantar inserts (LAS / MAS) improve stability in quiet stance and induce a backward shift of the CoP of young healthy subjects during eye movements. They also have a specific action upon vergence (not saccades): MAS increase the phasic amplitude of divergence and decrease its tonic part, in other words they mainly act on the pre-programmed component of the movement; whereas LAS act upon convergence, increasing its tonic amplitude, which is under the influence of visual retroaction. The inserts have a direct influence upon postural and oculomotor control through specific and independent paths. The 2nd study explained the variability of the subjects’ answers of the former. We measured their degree of reliance upon plantar cutaneous afferents with the Plantar Quotient method (PQ = Surface CoP foam / Surface CoP firm gorund X 100). A PQ<100 suggest a Plantar Extercoeptive Inefficency (PEI): PEI subjects do not show any improvement of their stability or any modification of vergence with the inserts. We propose that this situation is non-physiological and results from a non-noxious latent dysfunction of the sole receptors, opposing the PEI subjects to those who have a normal PQ. The 3rd study investigated the relationship between the use of plantar and visual afferents in young healthy subjects with the method of the PQ and Romberg Quotient (RQ). It has evidenced the existence of a synergic use of visual and plantar afferents in close-distance vision, only among normal PQ subjects. The PEI subjects have a significantly lower RQ than the others only at close distance on firm ground. On foam, their RQ increases; eyes closed, their PQ increases, which objectifies a visual-podal asynergy. The 4th study, on the same population, showed that a bilateral LAS increase esophoria only at far distance and among the PEI subjects. The 5th study revealed the influence of plantar exteroception upon the perception of verticality on the same population. It has brought out a decrease of the leftward error at near distance among the normal PQ subjects with a right LAS, and a decrease of the absolute error at far distance among the PEI subjects with a bilateral MAS. Taken together, these studies support, from a theoretical point of view, the importance of plantar afferents for postural control as well as vergence control, SVV estimation and eyes alignment (phorias). They demonstrate that the use of these afferents depends on the subjects and have multidisciplinary clinical implications.

<p>Experiment One: The Effect of Noise on Autonomic Arousal</p><p><br></p><p>In response to the growing demand for research that helps us understand the complex interactions between Autonomic Arousal (AA) on behavior and performance there is an increasing need for robust techniques to efficiently utilize stimuli, such as sound, to vary the level of AA within a study. The goal of this study was to look at the impact of several factors, including sound intensity, order of presentation, and direction of presentation on skin conductance level, a widely utilized technique for approximating levels of AA. To do this we had 34 young adults ages 18- 34 listen to a series of 2-minute blocks of a sound stimuli based off a heating, ventilation, and air conditioning system (HVAC). Blocks included 5 single intensity conditions each block differing in 10 dBA steps ranging from 35-75 dBA. We presented blocks in both rising and falling level of intensity, with half the participants hearing them in a rising order first and half in a falling order first. The evidence found by this study suggests that increasing the sound level plays an important role in increasing AA and habituation is an extremely important factor that must be accounted for as it, in the case of typical young adults, quickly dampens the response to stimuli and subsequent stimuli. These findings suggest that researchers can best efficiently maximize the range of AA they can use while keeping their participants comfortable by starting out with the most intense stimuli and proceeding to the less intense stimuli, working with habitation instead of against it.</p><p> </p><p><br></p><p> Experiment Two: The Effect of Autonomic Arousal on Visual Attention</p><p><br></p><p>The goal of this study was to better understand how various levels of autonomic arousal impact different components of attentional control and if ASD-related traits indexed by Autism Quotient scores (AQ) might relate to alterations in this relationship. This study had 41 young adult participants (23 women, 17 men, 1 prefer not to say), ages ranging from 18 to 38 years old. Participants listened to varying levels of noise to induce changes in AA, which were recorded as changes in skin conductance level (SCL). To evaluate attentional control, participants preformed pro and anti-saccade visual gap–overlap paradigm tasks as measures of attentional control. The findings of this study suggest that increased levels of autonomic arousal are helpful for improving performance on anti-saccade tasks, which are heavily dependent on top-down attentional control. Additionally, increases in AQ scores were related to having less of a benefit from increasing levels of arousal on anti-saccade tasks. Additional interactions were also found and are discussed in this paper.</p>

The criterion groups validity of emotional intelligence according to Mayer & Salovey (1997), ability model of emotional intelligence was investigated. Specifically, psychology (n+207) and engineering (n=195) students were compared on the Mayer, Salovey and Caruso Emotional Intelligence Test version 2 (MSCEIT). The primary factor structure of the MSCEIT was found to be valid with some revisions needed in terms of the reliability and content of the MSCEIT. The second-order factor structure of the MSCEIT was partially confirmed. In terms of the criterion groups validity of emotional intelligence, psychology students were found to exhibit higher levels of the ability to manage emotions in relationships, the ability to understand emotion as well as the ability to facilitate emotions. Engineering and Psychology students exhibited the same level of general emotional management and the ability to accurately identify emotion. Therefore the construct validity of emotional intelligence was partially confirmed. / Psychology / M.A. (Psychology)

Made available in DSpace on 2016-04-26T20:27:20Z (GMT). No. of bitstreams: 1 Mauricio Bellucci.pdf: 1288370 bytes, checksum: 3a6e5c17c472eef0d2e84aa497db9439 (MD5) Previous issue date: 2008-05-28 / This work is aimed at examining scientifically if the indemnifications that one receives fit to the legal rule (regra-matriz) of income tax incidence. It justifies itself because the solution and foundation presented by the doctrine and jurisprudence is not uniform. To do so, it presents an income conception based on the legislation and doctrine and confronts it with the theme of moral and material damages repairing. It shows that such a question must be solved according to superior rules presented by the Brazilian Constitution. In the light of law general theory, of philosophy of law, and of semiotics, this work suggests some criteria to be followed by the agent that applies tax law when evaluates economic events related to damage repairing. It concludes that indemnifications do not fit to the materiality of income tax / Este trabalho objetiva analisar cientificamente o enquadramento ou não das indenizações recebidas na regra-matriz de incidência tributária do imposto sobre a renda. Justifica-se porque na doutrina e na jurisprudência não há uniformidade na solução e fundamentação apresentadas para se definir a questão. Para cumprir esse objetivo, constrói-se, com base na legislação e na doutrina, o conceito de renda, confrontando-o com o tema das reparações de danos materiais e morais. Demonstra-se que a questão deve ser resolvida por normas superiores presentes na Constituição Federal. Neste contexto e à luz da Teoria Geral do Direito, da Filosofia do Direito e da Semiótica, apresentam-se critérios que devem ser seguidos pelo aplicador da lei tributária ao avaliar os eventos econômicos inerentes às reparações de danos. Conclui-se pelo não-enquadramento das indenizações na materialidade do imposto sobre a renda

Made available in DSpace on 2016-04-27T16:57:06Z (GMT). No. of bitstreams: 1 Diana Mazo Malheiro.pdf: 6282691 bytes, checksum: a46fef7e4a9774bb3feb0dedf10ab1f7 (MD5) Previous issue date: 2011-05-19 / Secretaria da Educação do Estado de São Paulo / This study aimed to draw up a supplementary material for teachers in state schools of São Paulo, making suggestions for the teaching of fractional numbers. To prepare this material analyzed the organization of the notebook of teacher and student, developed and proposed by the Ministry of Education of São Paulo, for the teaching of fractional numbers in the 5th and 6th grade (6 and 7 years) Elementary School Second Cycle, in use since 2008. This analysis relied on studies and research in mathematics education and has a theoretical semiotic representation of the records of Raymond Duval (2003) and the meanings of fractional numbers: part-whole, measure, quotient, ratio and operator / Este estudo teve por objetivo analisar o material sugerido para professores da rede estadual de ensino do Estado de São Paulo, apresentando de possíveis sugestões para o ensino de números fracionários. Para elaboração deste material analisamos a organização do caderno do professor e do aluno, desenvolvido e proposto pela Secretaria da Educação de São Paulo, para o ensino dos números fracionários, na 5ª e 6º série (6º e 7º ano) do Ensino Fundamental ciclo II, em uso desde 2008. Esta análise contou com estudos e pesquisas em Educação Matemática e teve como referencial teórico os registros de representação semiótica de Raymond Duval (2003) e os significados dos números fracionários: parte-todo, medida, quociente, razão e operador

O objetivo central desta dissertação é apresentar as folheações infinitesimalmente polares, fornecendo uma demonstração para o teorema que as caracteriza. Seguimos a abordagem original encontrada em Lytchak e Thorbergsson [25], de 2010. Diretamente da definição e do teorema principal obtem-se dois exemplos: folheações polares e folheações riemannianas singulares de codimensão 1 ou 2. Dedicamos especial atenção a um terceiro exemplo: folheações sem pontos horizontalmente conjugados. A demonstração deste resultado utiliza resultados obtidos anteriormente pelos mesmos autores em 2007, Lytchak e Thorbergsson [24]. Abordamos também, brevemente, as implicações do teorema caracterizador (que é um resultado local) sobre o quociente global de uma folheação infinitesimalmente polar. Variedades com folheações infinitesimalmente polares podem ser encaradas como um objeto que apresenta aspectos clássicos do teorema do toro maximal para grupos de Lie compactos, em um contexto mais amplo. / The present work aims at introducing infinitesimally polar foliations -- as defined by Lytchak and Thorbergsson [25] -- providing a proof for the classification theorem. Polar foliations and low codimension singular Riemannian foliations are two immediate examples. A third example is given by foliations without horizontally conjugate points. The proof of this assertion relies on previous results established by the same authors in Lytchak and Thorbergsson [24]. The classification theorem for infinitesimally polar foliations is a local result; we also derive from it some global consequences on the quotient space of such foliations. Infinitesimally polar foliations may be regarded as a generalised setting where one can find characteristic features from the maximal torus theorem for compact Lie groups.