Diffuse Sclerosis with Preserved Myelin Islands: A Pathological Report of a Case, with a Note on Cerebral Involvement in Raynaud's Disease

1939 ◽  
Vol 85 (354) ◽  
pp. 22-28 ◽  
Author(s):  
B. Horanyi-Hechst ◽  
A. Meyer
Keyword(s):  
Rapid Progression ◽  
Spastic Paraplegia ◽  
Clinical Symptomatology ◽  
Slowing Down ◽  
Familial Form ◽  
Intercurrent Disease ◽  
The One ◽  
Pathological Report ◽  
Disseminated Sclerosis ◽  
Diffuse Sclerosis

Pelizaeus (1885) described a family of which five members exhibited a rather uniform clinical symptomatology, consisting of tremor of the head, nystagmus, spastic paraplegia, etc. He thought he was dealing with an atypical familial form of disseminated sclerosis. Twenty-five years later Merzbacher (1910) investigated the same family and found the symptoms in twelve members, representing four generations. Symptomatology and clinical course of the condition was, in all patients, approximately identical: onset within the first months of life, rapid progression within the first six years, slowing down later considerably, death after twenty to thirty years due to intercurrent disease. The symptoms were tremor of the head, nystagmus; later paraplegia, ataxia, visual and auditory disturbances, involuntary movements, etc. The emotional and intellectual sphere remained comparatively unimpaired. The heredity was found to be recessive: the mothers, though themselves spared by the disease, pass it on to their sons (though among the twelve cases of Merzbacher there were also two females). In the one case investigated histopathologically by Merzbacher a diffuse demyelinization was found, particularly marked within the occipital region, distributed symmetrically in both the hemispheres and characterized by the persistence of myelin islands, often around vessels, which gave the condition the appearance of a tiger's skin. Merzbacher thought he was dealing with an hereditary aplasia of axis cylinders and myelin fibres, and called the condition aplasia axialis extra-corticalis congenita.

scholarly journals POS0083 USEFUL EXPERIENCE OF RHEUMATOLOGISTS FOR UNDERSTANDING OF THE DISEASE ORIGIN AND APPROACHES TO THERAPY IN FIBRODYSPLASIA OSSIFICANS PROGRESSIVA

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 249.2-250
Author(s):  
I. Nikishina ◽  
S. Arsenyeva ◽  
V. Matkava ◽  
A. Arefieva ◽  
M. Kaleda ◽  
...  
Keyword(s):  
Heterotopic Ossification ◽  
Kinase Inhibitor ◽  
Inflammatory Diseases ◽  
Clinical Manifestations ◽  
Fibrodysplasia Ossificans Progressiva ◽  
Rapid Progression ◽  
Time Duration ◽  
Long Term Follow Up ◽  
Vertebral Bodies ◽  
The One

Background:Many monogenic genetic conditions, such as auto-inflammatory diseases (AIDs), have similar clinical manifestations and immunopathogenesis to “classic” rheumatic diseases (RD). Such cases may include Fibrodysplasia ossificans progressiva (FOP), an extremely rare genetic disease, which, according to our previous study and data from other authors1, may represent an example of AID with catastrophic heterotopic ossification due to a mutation in the ACVR1 gene. it seems that the experience of rheumatologists, especially children’s ones, will be useful in the treatment of FOP.Objectives:To analyzed the dynamics of clinical manifestations and to therapy approaches including target anti-inflammatory drug Tofacitinib (TOFA) in the one of the world’s largest groups of patients (pts) with FOP.Methods:The study was based on the analysis retrospective and prospective observation of the 35 pts (17 males and 18 females) with a verified diagnosis of FOP for the period from 1998 to 2020. In 9 pts with severe course of FOP TOFA administration were evaluated.Results:In all 35 pts the diagnosis was verified by “classic” FOP phenotype: malformed great toes in 33 pts (94,3%); short malformed thumbs-8 (22.8%); peripheral osteochondromas-20 (57.1%); abnormalities of the cervical spine-32 (91.4%), multiple heterotopic ossifications-32 (91,4%). Genetic tests were done in 26, it confirmed mutation in the ACVR1 gene in 100%. Long term follow-up detected a lot of spondyloarthritis-like signs similar to the manifestation of RD: ankylosis of the facet joints and vertebral bodies (by the type of syndesmophytes) in most pts, sacroiliitis, confirmed by radiological methods (X-ray, CT, MRI), gradual ankylosis in the peripheral joints in 18 (56.4%), synovitis in large joints in 8 (25%) pts (knee and hip mostly). In 9 pts with the most difficult course with rapid progression of ossification due to continuous flares despite the NSAIDs and steroids intake, we tried to use TOFA after the approval of the local Ethic Committee. We use the similar dose to randomized trial for JIA (up to 5 mg twice a day). The first patient was 16 y.o. at the time of TOFA administration in December 2019, the age of the other pts was from 2 to 12 y.o. By present time duration of TOFA therapy is from 6 to 15 mo. For the previous 6 months before TOFA initiation the number of flares was in average 8 per patient. After 6 months of TOFA treatment the number of new flares decreased to 0-1, except youngest patient of 2 y.o. in whom the number of flares decreased from 10 to 4 per the same period. In all 9 pts we minimize the dose or completely stop the steroids. New nodes formation stopped immediately in most pts and also the significant motion improvement of large (shoulder) joints were established. Drug tolerance was good in all pts, no AE were registered. But despite the good clinical effect without new heterotopic ossification in our first patient, we found continuous intraskeletal ossification between vertebral bodies, facet and sacroiliac joints in MRI.Conclusion:We are confident that the processes of heterotopic ossification in FOP are very similar to new born formation phenomenon in spondyloarthritis and reliable suppression of inflammation can interrupt the progression of the disease. We used similar justifications to our colleagues for the use of anti-cytokine drugs, but used a JAK-kinase inhibitor, it was extremely important the oral rout of drug administration and possibility to escape any injections in FOP. TOFA demonstrated positive effect and safety in children with severe course of FOP. It showed their advantages over the use of steroids and possibility to inhibit the rate of progression.References:[1]R.Haviv et al. Is fibrodysplasia ossificans progressiva aninterleukin-1 driven auto-inflammatory syndrome? Pediatric Rheumatology (2019) 17:84 //doi.org/10.1186/s12969-019-0386-6Disclosure of Interests:None declared.

scholarly journals POLITENESS/IMPOLITENESS IN CONFLICT DIALOGICAL DISCOURSE: PROSODY ASPECT

2021 ◽  
Author(s):  
O.K. Pelivan
Keyword(s):  
English Language ◽  
Pitch Range ◽  
Slowing Down ◽  
Conflict Communication ◽  
Syllable Duration ◽  
The One ◽  
Negative Feelings ◽  
Formal Communication ◽  
The Given ◽  
Speech Situation

The given article presents an experimental phonetic investigation, which deals with a comprehensive analysis of English-language formal and informal conflict dialogues. It focuses on the study of various types of the investigated conflict dialogues intonational structure.The basic criteria of the practical material classification are: 1) the degree of relations formality between the collocutors and 2) politeness/impoliteness. According to these criteria the investigated conflict dialogues were classified as formal polite, formal impolite, informal polite and informal impolite. The recorded speech was investigated with the help of computer and statistical analyses which allowed to reveal those elements of prosody which actualize politeness/impoliteness in various types of the investigated conflict dialogues. The prosodic means that most clearly differentiate the investigated dialogical units are the pitch and intensity  range, the pitch peaks, the average syllable duration, the average duration of the emphatic centre, the pause volume, types of pauses between the key remarks. The pitch and intensity peaks that coinside with the emphatic centre of a dialogical unity are greatly important for actualizing emphasis. The speech of collocutors who try to remain polite in a formal conflict situation is characterised by a normal tempo with the tendency to slowing down, a mid  loudness, a mid pitch range that is due to the formal and official speech situation that forces the interlocutors to follow status subordination. An impolite formal conflict is characterized by a greater degree of emotionality than a polite formal one, as collocutors do not always follow the rules and standards of a formal situation behaviour being rude and harsh with their speech partners. At the prosodic level it  is expressed by a faster tempo, a greater loudness and a wider pitch range  than in the polite formal communication. The polite informal conflict communication is more emotional and natural than the polite formal one, but less emotional and natural than the impolite informal one. On the one hand  the informal situation gives complete freedom to choose emotions and ways of  their realization but on the other hand the desire to be polite in order  not to offend the speech partner forces the collocutors to restrain their negative feelings in the polite formal conflict communication. The impolite informal conflict is the most emotional and uncontrolled. Expressiveness and uncontrollability in the impolite informal conflict are caused by the the speech situation informality, unwillingness and no need to restrain, complete freedom of choice of verbal and nonverbal means. At the prosodic level this type of conflict interaction is characterised by a high loudness, an accelerated tempo and a wide pitch range of the voice. The conducted  research enabled us to state that conflict dialogical discourse represents a peculiar type of speech with a specific prosodic structure.

scholarly journals Evolution of the Processed Tomato Sector in Portugal and Spain: Comparative Analysis

2019 ◽  
pp. 470
Author(s):  
Maria de Fátima Martins Lorena De Oliveira ◽  
Mariano Suárez de Cepeda ◽  
Rodolfo Bernabéu
Keyword(s):  
Comparative Analysis ◽  
Comparative Advantage ◽  
Industrial Development ◽  
Slowing Down ◽  
Sustainable Products ◽  
Tomato Paste ◽  
Primary Determinant ◽  
Reducing Costs ◽  
The One ◽  
Business Competitiveness

World production of processed tomato has shifted from the traditional producing countries to others that were previously importers, thereby increasing competition between companies and slowing down agro-industrial and trade growth. Portugal, one of the world’s leading producers of processed tomato has not been immune to this trend, as witnessed by its decline in international competitiveness over the period from 1981 to 2013. The aim of the present study is thus twofold. On the one hand, using various standardized indicators, the determinants of the business competitiveness of processed tomato are analyzed, and, on the other, we perform a comparative analysis between Spain and Portugal. The standardized indicators used in this study are the Revealed Comparative Advantage (RCA), the Revealed Symmetric Comparative Advantage (RSCA) and the Grubel-Lloyd indicator (GL). The results show that price continues to be the primary determinant in processed tomato exports. Specifically, while trade in tomato paste in Portugal is inter-industry, trade in other tomato products is horizontal intra-industry in nature. In conclusion, the relationship between agro-industrial development and technological innovation in Portugal and Spain is key to reducing costs as a strategy to bring competitive and sustainable products to the market.

Experiences of Time and Timelessness in Electroacoustic Music

2020 ◽  
Vol 25 (2) ◽  
pp. 232-247
Author(s):  
Jason Noble ◽  
Tanor Bonin ◽  
Stephen McAdams
Keyword(s):  
Auditory Perception ◽  
Music Perception ◽  
General Trend ◽  
Electroacoustic Music ◽  
Human Information Processing ◽  
Subjective Experiences ◽  
Slowing Down ◽  
The One ◽  
Musical Context ◽  
Historical Antecedents

Electroacoustic music and its historical antecedents open up new ways of thinking about musical time. Whereas music performed by humans is necessarily constrained by certain temporal limits that define human information processing and embodiment, machines are capable of producing sound with scales and structures of time that reach potentially very far outside of these human limitations. But even musics produced with superhuman means are still subject to human constraints in music perception and cognition. Focusing on five principles of auditory perception – segmentation, grouping, pulse, metre and repetition – we hypothesise that musics that exceed or subvert the thresholds that define ‘human time’ are likely to be recognised by listeners as expressing timelessness. To support this hypothesis, we report an experiment in which a listening panel reviewed excerpts of electroacoustic music selected for their temporally subversive or excessive properties, and rated them (1) for the pace of time they express (normative, speeding up, or slowing down), and (2) for whether or not the music expresses ‘timelessness’. We find that while the specific musical parameters associated with temporal phenomenology vary from one musical context to the next, a general trend obtains across musical contexts through the excess or subversion of a particular perceptual constraint by a given musical parameter on the one hand, and the subjective experiences of time and timelessness on the other.

Towards Semantic Business Processes

2009 ◽  
pp. 244-274
Author(s):  
Muhammad Ahtisham Aslam ◽  
Sören Auer ◽  
Klaus-Peter Fähnrich
Keyword(s):  
Web Services ◽  
Process Model ◽  
Business Processes ◽  
Business Partners ◽  
Slowing Down ◽  
Business Process Execution ◽  
Automated Discovery ◽  
The One ◽  
Process Elements ◽  
Bpel Processes

The business process execution language for Web services (BPEL4WS, shortly BPEL) is one of the most popular languages and de facto standard for modelling business processes as Web services compositions. However, it only allows using hard-coded syntactical interfaces for partners and the process itself, i.e. semantic descriptions of services cannot be used within a process model. The lacks of an ontological description of the process elements cause limitations in the ways services are used within a process. A service providing the same functionality as the one referenced in the process model, but via a different syntactical interface, cannot be used instead. As a result, a process model cannot find an alternate service that performs the same functionality but exposes a different interface and can crash. Also, another drawback of such business processes is that they expose syntactical interfaces and cannot be discovered and composed dynamically by other semantic enabled systems slowing down the process of interaction between business partners. OWL-S on the other hand is suite of OWL ontologies and can be used to describe the compositions of Web services on the basis of matching semantics as well as to expose semantically enriched interfaces of business processes. Consequently, translating BPEL process descriptions to OWL-S suite of ontologies can overcome syntactical limitations of BPEL processes enabling them to 1) edit and model the composition of Web services on the basis of matching semantics 2) provide semantically enriched information of business processes. This semantically enriched information helps for dynamic and automated discovery, invocation and composition of business processes as Semantic Web services. Describing an approach and its implementation that can be used to enable business processes for semantic based dynamic discovery, invocation and composition by translating BPEL process descriptions to OWL-S suite of ontologies is the aim of this chapter.

Assessing the environmental impact of WWTP expansion: odour nuisance and its minimization

2002 ◽  
Vol 46 (4-5) ◽  
pp. 339-346 ◽  
Author(s):  
A.G. Capodaglio ◽  
F. Conti ◽  
L. Fortina ◽  
G. Pelosi ◽  
G. Urbini
Keyword(s):  
Air Quality Modelling ◽  
Drastic Reduction ◽  
Wastewater Disposal ◽  
Slowing Down ◽  
Pollutant Concentrations ◽  
Final Design ◽  
Modelling Techniques ◽  
Total Capacity ◽  
The One ◽  
The City

As part of the effort to provide proper wastewater disposal for the City of Milan, the expansion of the WWTP located in Peschiera Borromeo has been planned and is currently under completion. The plant, sized for a population of less than 300,000, will soon treat the wastes from the south-east areas of Milan for a total capacity of over 500,000 p.e. The paper describes the approach to find a satisfactory solution of an alleged odour problem that is slowing down the completion of the plant restructuring. This included a survey of the existing plant and analysis of its final design, identification of odour emission sources and their quantification, and the examination of different alternatives for odour abatement. These were carried out using air quality modelling techniques consisting of dynamic, continuous “puff” models capable of reproducing diffusion even at very low pollutant concentrations. Several intervention hypotheses were tested and compared with the few existing regulatory norms and guidelines for odour pollution. In all but the minimum and the no-action hypotheses, proposed actions would results in a drastic reduction of nuisance effects at the receptors. Under the strongest intervention hypothesis (the one adopted by the WWTP agency), odour impact would be virtually eliminated.

scholarly journals Two hundred days of COVID-19 in São Paulo State, Brazil

2020 ◽  
Vol 148 ◽  
Author(s):  
Gabriel Berg de Almeida ◽  
Micheli Pronunciate ◽  
Rejane Maria Tommasini Grotto ◽  
Edmur Azevedo Pugliesi ◽  
Raul Borges Guimarães ◽  
...  
Keyword(s):  
Reproduction Number ◽  
Health Department ◽  
Sao Paulo ◽  
Distance Measures ◽  
São Paulo ◽  
Health Security ◽  
Slowing Down ◽  
Paulo State ◽  
São Paulo State ◽  
The One

Abstract Two hundred days after the first confirmed case of COVID-19 in Brazil, the epidemic has rapidly spread in metropolitan areas and advanced throughout the countryside. We followed the temporal epidemic pattern at São Paulo State, the most populous of the country, the first to have a confirmed case of COVID-19, and the one with the most significant number of cases until now. We analysed the number of new cases per day in each regional health department and calculated the effective reproduction number (Rt) over time. Social distance measures, along with improvement in testing and isolating positive cases, general population mask-wearing and standard health security protocols for essential and non-essential activities, were adopted and impacted on slowing down epidemic velocity but were insufficient to stop transmission.

scholarly journals Yiddish and Judeo-Spanish as Determinants of Identity: As Illustrated in the Jewish Press of the First Half of the Twentieth Century

2016 ◽  
pp. 79-103
Author(s):  
Izabela Olszewska ◽  
Aleksandra Twardowska
Keyword(s):  
Twentieth Century ◽  
World War Ii ◽  
Jewish Identity ◽  
The Other ◽  
World War ◽  
Slowing Down ◽  
Jewish Diaspora ◽  
Other Hand ◽  
The One

Yiddish and Judeo-Spanish as Determinants of Identity: As Illustrated in the Jewish Press of the First Half of the Twentieth CenturyThe paper shows an image and functions of Yiddish and Judeo-Spanish languages among Jewish Diaspora groups – the Balkan Sephardim and the Ashkenazim (the Ostjuden group) – in the period from the beginning of the twentieth century until the outbreak of World War II. The study is based on the articles from Jewish weeklies, magazines and newspapers from pre-war Bosnia and Hercegovina and from Germany/Poland. It demonstrates a double-sided attitude towards the languages. On the one hand – an image of the languages as determinants of Jewish identity. Touching on this theme, the authors of the paper also try to highlight the images of Yiddish and Judeo-Spanish and as determinants in a narrower sense – of the Sephardi/Ashkenazi identity in that period. On the other hand, the paper shows a tendency to treat the languages as “corrupted” and “dying” languages, and as factors slowing down the assimilation of Jewish groups and also as an obstacle for Zionist ideologies. Języki jidysz i żydowsko-hiszpański jako wskaźniki tożsamości – na przykładzie żydowskich tekstów prasowych pierwszej połowy XX wiekuArtykuł ukazuje obraz i funkcje języków jidysz i żydowsko-hiszpańskiego wśród żydowskich grup diasporowych – bałkańskich Sefardyjczyków oraz Aszkenazyjczyków (Ostjuden) – w okresie od początków wieku XX do wybuchu II wojny światowej. Opis oparty jest na artykułach z żydowskich magazynów, tygodników, prasy codziennej z przedwojennej Bośni i Hercegowiny oraz Niemiec/Polski. Ukazany jest ambiwalentny stosunek wobec języków. Z jednej strony – obraz języków jako wskaźników żydowskiej tożsamości, jak również obraz jidysz i żydowsko-hiszpańskiego jako wskaźników tożsamości w węższym ujęciu: tożsamości sefardyjskiej/aszkenazyjskiej w omawianym okresie. Z drugiej strony zaś – artykuł zwraca uwagę także na to, że oba języki były traktowane jako „zepsute”, „umierające” i stanowiące czynniki spowalniające asymilację grup żydowskich oraz przeszkodę dla idei syjonistycznych.

scholarly journals System Reliability at the Crossroads

2012 ◽  
Vol 2012 ◽  
pp. 1-36 ◽  
Author(s):  
Vitali Volovoi
Keyword(s):  
System Reliability ◽  
Technology Development ◽  
Applied Mathematics ◽  
System Level ◽  
Component Level ◽  
Slowing Down ◽  
Current State ◽  
State Of Research ◽  
The One ◽  
The Cold War

This paper surveys the current state of research related to the modeling and prediction of failures of engineering systems. It is argued that while greater understanding of the physics of failure has led to significant progress at the component level, there are significant challenges remaining at the system level. System reliability, a field of applied mathematics that addresses the latter challenges, is at a juncture where fundamental changes are likely. On the one hand, the traditional part of the field entered a phase of diminishing returns, largely having followed the trajectory of the Cold-War era technology development: golden years of rapid growth in the 1950s and 1960s, followed by maturation and slowing down in the ensuing decades. On the other hand, the convergence of several technologies related to data collection and processing, combined with important changes in engineering business and government priorities, has created the potential for a perfect storm that can revive and fundamentally transform the field; however, for this transformation to occur, some serious obstacles need to be overcome. The paper examines these obstacles along with several key areas of research that can provide enabling tools for this transformation.

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