132 research outputs found
Cortical pencil lining in neuroferritinopathy: A diagnostic clue
No full textNeurodegeneration with brain iron accumulation (NBIA) includes pantothenate kinase-associated neurodegeneration (PKAN, NBIA1), PLA2G6-associated neurodegeneration (PLAN, NBIA2), neuroferritinopathy, aceruloplasminemia, and MIN-associated neurodegeneration (MPAN).(1) Clinically, they can have similar presentation, with a combination of progressive extrapyramidal, cognitive, and bulbar features.(1) Since genetic testing is costly and not easily accessible, MRI clues, such as the eye of the tiger sign for PKAN,(2,3) are useful to guide the confirmatory genetic analyses. Herein, we describe a distinct imaging pattern of cortical iron deposition on susceptibility-weighted MRI (SWI) in genetically proven cases of neuroferritinopathy, which is not seen in genetically proven cases of PKAN or PLAN, the 2 most common forms of NBIA
Gigantic implements of war: images of Newton as a mathematician
No full texthttp://www.oup.com/uk/catalogue/?ci=9780199213122#author
Notes on meteorological balloon mission planning
No full textIn the 21st century the high altitude gas balloon remains an indispensable tool in atmospheric science, meteorology and other applications requiring stratospheric observations. A prerequisite of the effectiveness of many types of balloon operations is an accurate trajectory forecasting capability, complete with appropriate error estimates. This is particularly important in targeted flights, sample return missions or flights of expensive instruments, whose recovery is essential. The ASTRA (Atmospheric Science Through Robotic Aircraft) initiative led to the development of such a forecast model, which is at the centre of the present paper. A key source of error in such models is our incomplete understanding of the drag opposing the rise of balloons in the free atmosphere – here we propose a new, stochastic model based on empirical data derived from thousands of radiosonde flights. We also examine other sources of prediction error affecting the accuracy of the flight path forecast, such as uncertainties in the wind profile and balloon envelope manufacturing variability. A Monte Carlo framework is used to provide probabilistic touchdown point estimates taking these error sources into account. The above elements have been integrated into a web service, which can be used as a flight planning tool – here we review the key features of its architecture
Mare e laguna nelle fonti bizantine relative a Venezia e ai Veneziani
No full textThe Northern Adriatic lagoon environment and the settlements it encompassed in the Middle Ages, including Venice, presents some remarkable features that the Byzantine sources do not fail to acknowledge. The first part of this chapter examines the constellation of terms applied to the lagoon and to specific aspects of its landscape (beaches, salt marshes, islands, inlets, canals, etc.). It considers how these terms are used in Byzantine sources to portray the lagoon and its relationship with the city
of Venice (and other settlements such as Malamocco and Torcello). The second part of this chapter studies the relation between these descriptions of (or hints at) the lagoon and different stages in Byzantine and Venetian history that stimulated the interest of Byzantine authors in Venice. It also explores how the development of the relationship between Byzantium and
Venice influenced the perception of the lagoon. The author investigates texts covering a broad time span, from Procopius to Laonikos Chalkokondyles, with a special focus on middle Byzantine literature
Rasgos del exilio italiano de los jesuitas españoles
No full textThis essay tries to elucidate some aspects of the exile of Spanish Assistance in 18th century Italy. First of all, the author analyses the origins of their economic difficulties before and after the suppression. Secondly he links the cultural and editorial production developed by some Spanish Jesuits, in order to refute the ideological paradigm called «leyenda negra», with the press campaign sponsored by the count of Floridablanca.En este ensayo se esbozan algunas implicaciones del exilio de los jesuitas españoles en la Italia del siglo XVIII. En primer lugar el autor analiza las causas de los apuros económicos sufridos por los ignacianos antes y después de la supresión canónica. Secundariamente se relaciona la producción cultural y editorial desarrollada por los expulsos, particularmente en la crítica de la «leyenda negra», con la campaña de propaganda promovida por el conde de Floridablanca
PEMIKIRAN POLITIK NICCOLO MACHIAVELLI: STUDI KASUS DINASTI UMAYYAH
No full textThe Umayyad dynasty was an Islamic caliphate regime after the khulafa urrasydin which managed to maintain its power for 90 years before being overthrown by the Abbasids. This dynasty is famous for its political stability from the beginning of his reign to a systematized public administration and military. Nevertheless, this dynasty raised pros and cons in terms of morality which were considered to deviate from Islamic teachings at least during the political revolution and post-Caliph Mu\u27awiyah. The author tries to examine the practice of such government using the political theory of Niccolo Machiavelli in which there are also thoughts about the ethics of power. This study tries to answer several questions, namely what is the ideal government practice in the book "Il Principe" by Niccolo Machiavelli? and whether the practice of Umayyad dynasty government can be said to be ideal in terms of administration and ethics of power in Machiavelli\u27s perspective? This research is a qualitative research with primary sources in the form of historical literature about the Umayyad Dynasty and the books by Machiavelli mainly on "Il Principe". This study shows that in making political policies, for the sake of government stability, moral values do not need to be considered except in a pragmatic context. The stability of the state is also influenced by the ability and luck of the leader in managing the government. This study shows that the practice of the Umayyad dynasty has a correlation with the main goal of Machiavelli\u27s political theory, namely state stability
Developing and testing guidelines for designing surprising products
No full textLAUREA MAGISTRALELa sorpresa è diventata un argomento interessante nel mondo del design di prodotto; è stato dimostrato che la presenza di un elemento sorprendente all'interno di un prodotto aumenta l'interazione uomo-prodotto e l’apprezzamento in generale. Sono stati compiuti sforzi attraverso numerosi studi per capire cosa sia veramente il concetto di sorpresa e su cosa si basa, cosa rende sorprendenti alcuni prodotti e come si possono catturare i processi cognitivi coinvolti quando un utente valuta un oggetto probabilmente sorprendente. Tuttavia, mancava ancora una linea guida che permettesse a ingegneri meccanici e designer di esplorare sistematicamente la propria creatività per generare, infine, idee per prodotti sorprendenti. L'autore di questa tesi di laurea magistrale ha proposto delle linee guida per questo scopo, che vengono proposte attraverso una presentazione interattiva. Questa presentazione interattiva è stata testata per la sua usabilità come strumento metodologico da un set di ingegneri meccanici e designer industriali, ritenuti i destinatari delle linee guida. Inoltre, le sorpresa indotta dalle idee generate da detti ingegneri e designer è stata valutata da un insieme di partecipanti accuratamente filtrati. I risultati raggiunti durante entrambe le attività sperimentali hanno raggiunto gli obiettivi fissati e consentono alle linee guida proposte di fungere da solida base per ulteriori sviluppi, sulla base dei miglioramenti già ipotizzati dall'autore sia per incrementare l’efficacia delle linee guida proposte che la relativa procedura di verifica.Surprise has become an interesting topic in the world of product design; the presence of a surprising trigger within a product has been proved to increase human-product interaction and overall likeability. Efforts by numerous contributors have been made to understand what the concept of surprise truly is and what does it rely on, what makes some products surprising, and how can one capture the cognitive processes involved when a user assesses an arguably surprising object; however, there was yet to be a guideline, using which mechanical engineers and designers can systematically explore their own unique creativity to ultimately generate ideas for surprising products. The author of this master thesis has proposed guidelines that do exactly that in the form of an interactive presentation. This interactive presentation was tested for its usability as a methodological tool by a third party of mechanical engineers and industrial designers. Furthermore, the ideas generated by those said engineers and designers have been assessed whether if they are genuinely surprising or not by a set of carefully filtered participants. The results reached during both external tests met expectations and allow the proposed guidelines to serve as a firm base for further development; however, the author acknowledges some points where potential improvements are due in both the proposed guidelines and in the testing procedure
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Naming Genes for Dystonia: DYT-z or Ditzy?
No full textDystonias are a clinically and etiologically diverse group of disorders. Numerous genes have now been associated with different dystonia syndromes, and multiple strategies have been proposed for how these genes should be lumped and split into meaningful categories. The traditional approach has been based on the Human Genome Organization’s plan for naming genetic loci for all disorders. For dystonia this involves a DYT prefix followed by a number (e.g., DYT1, DYT2, DYT3, etc.). A more recently proposed approach involves assigning multiple prefixes according to the main elements of the phenotype (e.g., DYT, PARK, CHOR, TREM, etc.) followed by the name of the responsible gene. This article describes these nomenclature systems and summarizes some of their limitations. We focus on dystonia as an example, although the concepts may be applied to all movement disorders
Giorgio Metochites, Historia dogmatica, libro I. Edizione, traduzione e commento
No full textIl mio lavoro di tesi consiste nell’allestimento della prima edizione critica del libro I dell’Historia dogmatica di Giorgio Metochites, un autore bizantino vissuto fra il XIII e il XIV secolo. Tale studio si articola in tre parti. La prima si propone di analizzare la figura di Giorgio Metochites e il libro I dell’Historia dogmatica nel loro contesto storico-ecclesiale. Sono così brevemente presentati la biografia dell’autore, gli elementi che consentono la datazione dell’opera, le fonti utilizzate e le peculiarità del greco di Metochites. Seguono poi tre capitoli di analisi generale, ciascuno dedicato ad una sezione del testo, al fine di esaminare i principali temi affrontati dall’autore, offrire un’interpretazione storica dell’opera e metterne in luce il valore e l’originalità. La seconda parte riguarda l’edizione del testo, accompagnata dalla traduzione italiana a fronte, e introdotta da due capitoli, dedicati rispettivamente alla tradizione manoscritta e ai criteri di edizione adottati. La terza parte contiene il commento lineare al testo; l’analisi mira ad offrire uno studio completo, condotto su molteplici piani (filologico, linguistico, stilistico, storico ed ecclesiologico). La tesi si completa con l’indice dei nomi (Index nominum), un indice tematico relativo al tema dell’unione e dello scisma delle Chiese (Index verborum ad unionis quaestionem spectantium) e un Index locorum che racchiude le citazioni inserite dall’autore nella sua opera.My PhD disseration consists in the first critical edition of the first book of the Dogmatic History by George Metochites, a Byzantine author who lived between the 13th and 14th centuries. This study is divided into three parts. The first one analyzes the figure of George Metochites and the first book of the Dogmatic History in their historical and ecclesiastical context: the author’s biography, the elements that allow the dating of the work, the sources used and the peculiarities of the language of Metochites are thus briefly presented. The following three chapters are each one devoted to a specific section of the text: the aim is to examine the main themes addressed by the author, offer a historical interpretation of the work, and highlight its value and originality. The second part presents the edition of the text, accompanied by an Italian translation, and introduced by two chapters, dealing respectively with the manuscript tradition and the editorial criteria. The third part contains the commentary; the analysis offers a comprehensive study, conducted on multiple levels (philological, linguistic, stylistic, historical and ecclesiological). At the end of the thesis are the indices, namely, the index of names (Index nominum), a thematic index regarding the problem of union and schism of the Churches (Index verborum ad unionis quaestionem spectantium), and an Index locorum, which lists the quotations inserted by the author
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