Update on neurodegeneration with brain iron accumulation

Neurodegeneration with brain iron accumulation (NBIA) defines a heterogeneous group of progressive neurodegenerative disorders characterized by excessive iron accumulation in the brain, particularly affecting the basal ganglia. In the recent years considerable development in the field of neurodegenerative disorders has been observed. Novel genetic methods such as autozygosity mapping have recently identified several genetic causes of NBIA. Our knowledge about clinical spectrum has broadened and we are now more aware of an overlap between the different NBIA disorders as well as with other diseases. Neuropathologic point of view has also been changed. It has been postulated that pantothenate kinase-associated neurodegeneration (PKAN) is not synucleinopathy. However, exact pathologic mechanism of NBIA remains unknown. The situation implicates a development of new therapies, which still are symptomatic and often unsatisfactory. In the present review, some of the main clinical presentations, investigational findings and therapeutic results of the different NBIA disorders will be presented

On the recognition of the game type based on physiological signals and eye tracking

Automated interpretation of signals yields many impressive applications from the area of affective computing and human activity recognition (HAR). In this paper we ask the question about possibility of cognitive activity recognition on the base of particular set of signals. We use recognition of the game played by the participant as a playground for exploration of the problem. We build classifier of three different games (Space Invaders, Tetris, Tower Defence) and inter-game pause. We validate classifier in the player-independent and player-dependent scenario. We discuss the improvement in the player-dependent scenario in the context of biometric person recognition. On the base of the results obtained in game classification, we consider potential applications in smart surveillance and quantified self.Comment: 5 pages, 3 figures, extended version of ESM pape

Zespół twiddler jako rzadkie powikłanie po operacji implantacji elektrody do głębokiej stymulacji mózgu w chorobie Parkinsona

Zespół twiddler jest spowodowany bezwiednym bądź celowym zewnętrznym obracaniem zaimplantowanego stymulatora serca lub generatora impulsów do głębokiej stymulacji mózgu, powodującym skręcenie, przemieszczenie, a często również złamanie elektrody w kieszonce skórnej. Przedstawiono przypadek pacjentki z chorobą Parkinsona leczonej operacyjnie metodą obustronnej głębokiej stymulacji mózgu z implantacją elektrod do jądra nisko- wzgórzowego, u której wykryto skręcenie i przerwanie przewodu łączącego generator impulsów z elektrodą. Opisano diagnostykę obrazową oraz leczenie, które polegało na wymianie przerwanego łącznika

FLEXNET: Flexible Networks for IoT based services

Internet of Things is becoming one of the main triggers in designing and deploying new services aiming at fulfilling the wide demand imposed by end-users. Usually, concrete solutions addressing the optimization of the wireless segment are found in the literature. However, it is much less frequent to find end-to-end solutions to be easily adopted by the corresponding stakeholders. It is in this context that FLEXNET brings an integrated solution, relying on cutting-edge technologies, dealing with a wide set of technical requirements imposed by the different applications and services.This work was supported by FLEXNET Project: "Flexible IoT Networks for Value Creators" (Celtic 2016/3), in the Eureka Celtic-Next Cluster

External Factors Modulating Pain and Pain-Related Functional Impairment in Cervical Dystonia

Background Little is known about factors modulating pain and pain-related functional impairment in isolated cervical dystonia (CD). Objective The aim was to assess the prevalence and interrelationship between pain-modulating factors and pain-related determinants of functional impairment and quality of life in CD. Methods We analyzed pain-aggravating and pain-relieving external factors, the degree of pain-related functional impact on routine activities, and the relationship between these and pain severity, using cross-sectional data collected using the Pain in Dystonia Scale (PIDS) from 85 participants with CD. Pairwise correlation analyses and age- and sex-adjusted linear regression models estimated the relationship between pain-modulating factors and pain severity, and the impact of pain severity, dystonia severity, and psychiatric symptoms on pain-related functional impairment and disease-specific quality of life (measured using the Craniocervical Dystonia Questionnaire-24). Results Stress and prolonged fixed position were the most frequent and impacting pain triggers, with women reporting larger impact. The average impact of pain-relieving factors was lower than that of pain triggers. Physical exercise and social gatherings were the most impacted activities by pain in CD. The intensity of external modulating factors was a predictor of pain severity. Severity of pain, CD, and psychiatric symptoms independently predicted pain-related functional impairment, whereas quality of life was predicted by pain severity, pain-related functional impairment, and psychiatric symptom severity, but not dystonia severity. Conclusion The PIDS provides insight into external modulation and functional impact of pain in CD. The pattern of external modulation of pain in CD is in line with a multifactorial modulation and complex physiology

Intra-Company Crowdsensing: Datafication with Human-in-the-Loop

Every day employees learn about things happening in their company. This includes plain facts witnessed while on the job, related or not to one’s job responsibilities. Many of these facts, which we call “occurrence data”, are known by employees but remain unknown to the company. We suppose that some of them are valuable and may improve the company’s situational awareness. In the spirit of mobile crowdsensing, we propose intra-company crowdsensing (ICC), a method of “extracting” occurrence data from employees. In ICC, an employee occasionally responds to sensing requests, each about one plain fact. We elaborate the concept of ICC, proposing a model of human-system interaction, a system architecture, and an organizational process. We position ICC with respect to related concepts from information technology, and we look at it from selected organizational and managerial viewpoints. Finally, we conducted a survey, in which we presented the concept of ICC to employees of different companies and asked for their evaluation. Respondents positive about ICC outnumbered skeptics by a wide margin. The survey also revealed some concerns, mostly related to ICC being perceived as another employee surveillance tool. However, useful and acceptable sensing requests are likely to be found in every organization

Ontology-based Use Cases for Design-time and Runtime Composition of Mobile Services

This paper presents application of ontology-based modelling and reasoning related to the different phases of the lifecycle of mobile services. Ontology-based descriptions complement traditional design-time and runtime models allowing more complex reasoning. We present use cases for ontologies that may be applied at design time, deployment time and/or runtime. Some important characteristics of our approach are: 1. ontological descriptions define complex artefacts that are built from simpler ones defined in an ontology; 2. a single ontology can be used for specifying various artefacts and for reasoning on various aspects at different phases of the service lifecycle; 3. an artefact can be used for various purposes. This paper provides examples of ontological descriptions along with use cases, and discusses the applicability of the approach

Binding of the third Na+ ion to the cytoplasmic side of the Na,K-ATPase is electrogenic

AbstractA new experimental setup was constructed to allow parallel measurements of total internal reflection fluorescence and of capacitance changes in Na,K-ATPase-containing membranes. Effects correlated with cytoplasmic sodium binding to Na,K-ATPase were investigated. Ion binding-induced fluorescence changes of the electrochromic dye RH421 in membrane fragments adsorbed on a transparent capacitative electrode corresponded perfectly to capacitance increases detected by a lock-in technique. From these electric measurements it was possible to estimate a dielectric coefficient of about 0.25 for the electrogenic binding of the third Na+ ion. Binding of K+ to cytoplasmic sites was electroneutral