Visual signs and symptoms of Multiple System Atrophy

Multiple system atrophy (MSA) is a rare movement disorder and a member of a group of neurodegenerative diseases, which include Parkinson’s disease (PD) and progressive supranuclear palsy (PSP), and referred to as the ‘parkinsonian syndromes’. Although primarily a neurological disorder, patients with MSA may also develop visual signs and symptoms that could be useful in differential diagnosis. In addition, the eye-care practitioner may contribute to the management of visual problems of MSA patients and therefore, help to improve quality of life. This second article in the series considers the visual signs and symptoms of MSA with special reference to those features most useful in differential diagnosis of the parkinsonian syndromes.

Human performance modelling as an aid in the process of manufacturing system design

Manufacturing system design is an ongoing activity within industry. Modelling tools based on Discrete Event Simulation are often used by practitioners during this design cycle. However, such tools do not adequately model the behaviour of 'direct' workers in manufacturing environments. There is an important need to expand the capability of modelling to include the relationships between human centred factors (demography, attitudes, beliefs, etc), their working environment (physical and organizational), and their subsequent performance in terms of productive routines. Therefore, this paper describes research that has formed a pilot modelling methodology that is an important first step in providing such a capability.

A strategic activity model of Enterprise System implementation and use:scaffolding fluidity

This paper builds on a Strategic Activity Framework (Jarzabkowski, 2005) and activity based theories of development (Vygotsky, 1978) to model how Enterprise Systems are used to support emerging strategy. It makes three contributions. Firstly, it links fluidity and extensiveness of system use to patterns of strategising. Fluidity - the ability to change system use as needs change - is supported by interactive strategising, where top managers communicate directly with the organisation. Extensiveness requires procedural strategising, embedding system use in structures and routines. Secondly, it relates interactive and procedural strategising to the importance of the system - procedural strategising is more likely to occur if the system is strategically important. Thirdly, using a scaffolding metaphor it identifies patterns in the activities of top managers and Enterprise System custodians, who identify process champions within the organisational community, orient them towards system goals, provide guided support, and encourage fluidity through pacing implementation with learning.© 2013 Published by Elsevier B.V. All rights reserved.

Visual signs and symptoms of multiple system atrophy

Multiple system atrophy (MSA) is a rare movement disorder and a member of the 'parkinsonian syndromes', which also include Parkinson's disease (PD), progressive supranuclear palsy (PSP), dementia with Lewy bodies (DLB) and corticobasal degeneration (CBD). Multiple system atrophy is a complex syndrome, in which patients exhibit a variety of signs and symptoms, including parkinsonism, ataxia and autonomic dysfunction. It can be difficult to separate MSA from the other parkinsonian syndromes but if ocular signs and symptoms are present, they may aid differential diagnosis. Typical ocular features of MSA include blepharospasm, excessive square-wave jerks, mild to moderate hypometria of saccades, impaired vestibular-ocular reflex (VOR), nystagmus and impaired event-related evoked potentials. Less typical features include slowing of saccadic eye movements, the presence of vertical gaze palsy, visual hallucinations and an impaired electroretinogram (ERG). Aspects of primary vision such as visual acuity, colour vision or visual fields are usually unaffected. Management of the disease to deal with problems of walking, movement, daily tasks and speech problems is important in MSA. Optometrists can work in collaboration with the patient and health-care providers to identify and manage the patient's visual deficits. A more specific role for the optometrist is to correct vision to prevent falls and to monitor the anterior eye to prevent dry eye and control blepharospasm.

Self-organization of Functioning Process of Expert System with the Use of Subject Domain Ontology

Optimization of design, creation, functioning and accompaniment processes of expert system is the important problem of artificial intelligence theory and decisions making methods techniques. In this paper the approach to its solving with the use of technology, being based on methodology of systems analysis, ontology of subject domain, principles and methods of self-organisation, is offered. The aspects of such approach realization, being based on construction of accordance between the ontology hierarchical structure and sequence of questions in automated systems for examination, are expounded.

Quality of Service System Approximation in IP Networks

This paper is sponsored by the Ministry of Education and Research of the Republic of Bulgaria in the framework of project No 105 “Multimedia Packet Switching Networks Planning with Quality of Service and Traffic Management”.

On the Operational Solution of a System of Fractional Differential Equations

MSC 2010: 26A33, 44A45, 44A40, 65J10

Optimization Problem in a Class of Linear System. Application to Multiple Drug Administration

2000 Mathematics Subject Classification: 62H15, 62P10.

Quantitative pathological changes in the cerebellum of multiple system atrophy

Multiple system atrophy (MSA) is a rare neurodegenerative disorder associated with parkinsonism, ataxia, and autonomic dysfunction. Its pathology is primarily subcortical comprising vacuolation, neuronal loss, gliosis, and α-synuclein-immunoreactive glial cytoplasmic inclusions (GO). To quantify cerebellar pathology in MSA, the density and spatial pattern of the pathological changes were studied in α-synuclein-immunolabelled sections of the cerebellar hemisphere in 10 MSA and 10 control cases. In MSA, densities of Purkinje cells (PC) were decreased and vacuoles in the granule cell layer (GL) increased compared with controls. In six MSA cases, GCI were present in cerebellar white matter. In the molecular layer (ML) and GL of MSA, vacuoles were clustered, the clusters exhibiting a regular distribution parallel to the edge of the folia. Purkinje cells were randomly or regularly distributed with large gaps between surviving cells. Densities of glial cells and surviving neurons in the ML and surviving cells and vacuoles in the GL were negatively correlated consistent with gliosis and vacuolation in response to neuronal loss. Principal components analysis (PCA) suggested vacuole densities in the ML and vacuole density and cell losses in the GL were the main source of neuropathological variation among cases. The data suggest that: (1) cell losses and vacuolation of the GCL and loss of PC were the most significant pathological changes in the cases studied, (2) pathological changes were topographically distributed, and (3) cerebellar pathology could influence cerebral function in MSA via the cerebello-dentato-thalamic tract.

Visszatérhet-e a "nagy elmélet" a közgazdaságtanban? Megjegyzések a rendszerparadigma elmélettörténetéhez (Can the "grand theory" return to economics? Notes on the theoretical history of the system paradigm)

Kornai János rendszerparadigma-fogalmából és az ott felsorolt közgazdászok névsorából kiindulva, a tanulmány kiemel néhány mozzanatot ennek eszmetörténeti előzményeiből. A hatvanas évek szociológiai irodalmában időszerűtlennek tartották a nagy elmélet létrehozását, a nyolcvanas évek társadalomtudományában viszont már a hagyományos nagy rendszeralkotás és elbeszélésmód visszatérését méltatták. Adam Smith közgazdaságtanát a társadalmi érintkezés átfogó elmélete részének szánta, amely tartalmazza a jog, politika és erkölcs elveit és történetét. Ezt szemlélteti a kereskedelmi társadalom paradoxonának elemzése és annak bemutatása, hogyan vezetett a fényűzés a szabadság helyreállításához és a rendszeres kormányzat létrejöttéhez. Marx és a német történeti iskola egyaránt törekedett a társadalom történeti fejlődéstörvényeinek feltárására, noha kölcsönösen elutasították egymás felfogásának többi elemét. Schumpeter életművét az a törekvés határozta meg, hogy összekapcsolja az elméleti analízist és a történeti leírást, hogy a gazdasági fejlődés elemzéséből kiindulva megalkossa a társadalmi élet különböző területeinek interdependenciáját bemutató elméletet. / === / The study starts from János Kornai's concept of a system paradigm and the list of economists there to lift certain elements from its antecedents in intellectual history. The sociological writers of the 1960s thought it was inopportune to create a grand theory, while the social scientists of the 1980s were busy celebrating the return of the great traditional system-creating process and its mode of narration. Adam Smith presented his economics as part of a comprehensive theory of social organization that would cover the principles and history of law, politics and morality. This is illustrated by his analysis of the paradox of commercial society and his demonstration of how wealth led to the restoration of liberty and the establishment of regular governance. Marx and the German historical school both strove to identify laws of the historical development of society, although they each rejected the other elements of the other's concept. Schumpeter's oeuvre was marked by an effort to combine theoretical analysis with historical description, so that from an analysis of economic development he could arrive at a theory presenting the interdependence of the various areas of social activity.

The existence of an inverse limit of inverse system of measure spaces - a purely measurable case

The existence of an inverse limit of an inverse system of (probability) measure spaces has been investigated since the very beginning of the birth of the modern probability theory. Results from Kolmogorov [10], Bochner [2], Choksi [5], Metivier [14], Bourbaki [3] among others have paved the way of the deep understanding of the problem under consideration. All the above results, however, call for some topological concepts, or at least ones which are closely related topological ones. In this paper we investigate purely measurable inverse systems of (probability) measure spaces, and give a sucient condition for the existence of a unique inverse limit. An example for the considered purely measurable inverse systems of (probability) measure spaces is also given.

Az SAP-rendszer fejlesztése egy műanyag-feldolgozó vállalatnál (Development of SAP system in a plastic manufacturing company)

Az SAP-rendszernek a kontrollingszervezetek működésére gyakorolt pozitív hatása nem titok. A visszacsatolás lehetőségének biztosításával alkalom nyílik a szervezetek tevékenységének követésére, ellenőrzésére, felülbírálására. A logisztika mint az egész vállalatot átszövő rendszer, működésének nyomon követése is létfontosságúvá vált, hiszen összetettségéből kifolyólag jellegzetességei, színvonala az egész rendszerre hatást gyakorol. A logisztikai rendszer és folyamatainak figyelemmel kísérésére a logisztikai kontrollingrendszer nyújt megoldást, visszacsatolási pontjain keresztül. A műanyag-feldolgozó vállalat esetében a szervezeti SAP-rendszer logisztikai kontrollingterületének fejlesztésétől várják a szervezeti hatékonyság emelkedését és a jobb színvonal elérését. _____ Positive effect of the SAP system on the operation of controlling organisations has not been a secret. Opportunity of the feedback will be possible to track, control, override operation of the organisations. The logistic controlling system provides a solution through feedback points for monitoring the logistic system and processes. In the case of plastics manufacturing company the increasing of organisational efficiency and achievement of a better standard is anticipated from development of logistic controlling area of the organisational SAP system.

Physics-based modeling of power system components for the evaluation of low-frequency radiated electromagnetic fields

The low-frequency electromagnetic compatibility (EMC) is an increasingly important aspect in the design of practical systems to ensure the functional safety and reliability of complex products. The opportunities for using numerical techniques to predict and analyze system's EMC are therefore of considerable interest in many industries. As the first phase of study, a proper model, including all the details of the component, was required. Therefore, the advances in EMC modeling were studied with classifying analytical and numerical models. The selected model was finite element (FE) modeling, coupled with the distributed network method, to generate the model of the converter's components and obtain the frequency behavioral model of the converter. The method has the ability to reveal the behavior of parasitic elements and higher resonances, which have critical impacts in studying EMI problems. For the EMC and signature studies of the machine drives, the equivalent source modeling was studied. Considering the details of the multi-machine environment, including actual models, some innovation in equivalent source modeling was performed to decrease the simulation time dramatically. Several models were designed in this study and the voltage current cube model and wire model have the best result. The GA-based PSO method is used as the optimization process. Superposition and suppression of the fields in coupling the components were also studied and verified. The simulation time of the equivalent model is 80-100 times lower than the detailed model. All tests were verified experimentally. As the application of EMC and signature study, the fault diagnosis and condition monitoring of an induction motor drive was developed using radiated fields. In addition to experimental tests, the 3DFE analysis was coupled with circuit-based software to implement the incipient fault cases. The identification was implemented using ANN for seventy various faulty cases. The simulation results were verified experimentally. Finally, the identification of the types of power components were implemented. The results show that it is possible to identify the type of components, as well as the faulty components, by comparing the amplitudes of their stray field harmonics. The identification using the stray fields is nondestructive and can be used for the setups that cannot go offline and be dismantled

Genomic consequences of mating system evolution in the Pacific coastal dune endemic, Abronia umbellata (Nyctaginaceae)

The advent of next-generation sequencing has significantly reduced the cost of obtaining large-scale genetic resources, opening the door for genomic studies of non-model but ecologically interesting species. The shift in mating system, from outcrossing to selfing, has occurred thousands of times in angiosperms and is accompanied by profound changes in the population genetics and ecology of a species. A large body of work has been devoted to understanding why the shift occurs and the impact of the shift on the genetics of the resulting selfing populations, however, the causes and consequences of the transition to selfing involve a complicated interaction of genetic and demographic factors which are difficult to untangle. Abronia umbellata is a Pacific coastal dune endemic which displays a striking shift in mating system across its geographic range, with large-flowered outcrossing populations south of San Francisco and small-flowered selfing populations to the north. Abronia umbellata is an attractive model system for the study of mating system transitions because the shift appears to be recent and therefore less obscured by post-shift processes, it has a near one-dimensional geographic range which simplifies analysis and interpretation, and demographic data has been collected for many of the populations. In this study, we generated transcriptome-level data for 12 plants including individuals from both subspecies, along with a resequencing study of 48 individuals from populations across the range. The genetic analysis revealed a recent transition to selfing involving a drastic reduction in genetic diversity in the selfing lineage, potentially indicative of a recent population bottleneck and a transition to selfing due to reproductive assurance. Interestingly, the genetic structure of the populations was not coincident with the current subspecies demarcation, and two large-flowered populations were classified with the selfing subspecies, suggesting a potential need for re-evaluation of the current subspecies classification. Our finding of low diversity in selfing populations may also have implications for the conservation value of the threatened selfing subspecies.