206 research outputs found
Auditory object salience: human cortical processing of non-biological action sounds and their acoustic signal attributes
Whether viewed or heard, an object in action can be segmented as a distinct salient event based on a number of different sensory cues. In the visual system, several low-level attributes of an image are processed along parallel hierarchies, involving intermediate stages wherein gross-level object form and/or motion features are extracted prior to stages that show greater specificity for different object categories (e.g., people, buildings, or tools). In the auditory system, though relying on a rather different set of low-level signal attributes, meaningful real-world acoustic events and “auditory objects” can also be readily distinguished from background scenes. However, the nature of the acoustic signal attributes or gross-level perceptual features that may be explicitly processed along intermediate cortical processing stages remain poorly understood. Examining mechanical and environmental action sounds, representing two distinct non-biological categories of action sources, we had participants assess the degree to which each sound was perceived as object-like versus scene-like. We re-analyzed data from two of our earlier functional magnetic resonance imaging (fMRI) task paradigms (Engel et al., 2009) and found that scene-like action sounds preferentially led to activation along several midline cortical structures, but with strong dependence on listening task demands. In contrast, bilateral foci along the superior temporal gyri (STG) showed parametrically increasing activation to action sounds rated as more “object-like,” independent of sound category or task demands. Moreover, these STG regions also showed parametric sensitivity to spectral structure variations (SSVs) of the action sounds—a quantitative measure of change in entropy of the acoustic signals over time—and the right STG additionally showed parametric sensitivity to measures of mean entropy and harmonic content of the environmental sounds. Analogous to the visual system, intermediate stages of the auditory system appear to process or extract a number of quantifiable low-order signal attributes that are characteristic of action events perceived as being object-like, representing stages that may begin to dissociate different perceptual dimensions and categories of every-day, real-world action sounds
COST 733 - WG4: Applications of weather type classification
The main objective of the COST Action 733 is to achieve a general numerical method for
assessing, comparing and classifying typical weather situations in the European regions. To
accomplish this goal, different workgroups are established, each with their specific aims:
WG1: Existing methods and applications (finished); WG2: Implementation and development of
weather types classification methods; WG3: Comparison of selected weather types
classifications; WG4: Testing methods for various applications.
The main task of Workgroup 4 (WG4) in COST 733 implies the testing of the selected weather
type methods for various classifications. In more detail, WG4 focuses on the following topics:•
Selection of dedicated applications (using results from WG1),
• Performance of the selected applications using available weather types provided by WG2,
• Intercomparison of the application results as a results of different methods
• Final assessment of the results and uncertainties,
• Presentation and release of results to the other WGs and external interested
• Recommend specifications for a new (common) method WG2
Introduction
In order to address these specific aims, various applications are selected and WG4 is divided in
subgroups accordingly:
1.Air quality
2. Hydrology (& Climatological mapping)
3. Forest fires
4. Climate change and variability
5. Risks and hazards
Simultaneously, the special attention is paid to the several wide topics concerning some other
COST Actions such as: phenology (COST725), biometeorology (COST730), agriculture (COST 734)
and mesoscale modelling and air pollution (COST728).
Sub-groups are established to find advantages and disadvantages of different classification
methods for different applications. Focus is given to data requirements, spatial and temporal
scale, domain area, specifi
The impact of gender, puberty, and pregnancy in patients with POLG disease
OBJECTIVE: To study the impact of gender, puberty, and pregnancy on the expression of POLG disease, one of the most common mitochondrial diseases known. METHODS: Clinical, laboratory, and genetic data were collected retrospectively from 155 patients with genetically confirmed POLG disease recruited from seven European countries. We used the available data to study the impact of gender, puberty, and pregnancy on disease onset and deterioration. RESULTS: We found that disease onset early in life was common in both sexes but there was also a second peak in females around the time of puberty. Further, pregnancy had a negative impact with 10 of 14 women (71%) experiencing disease onset or deterioration during pregnancy. INTERPRETATION: Gender clearly influences the expression of POLG disease. While onset very early in life was common in both males and females, puberty in females appeared associated both with disease onset and increased disease activity. Further, both disease onset and deterioration, including seizure aggravation and status epilepticus, appeared to be associated with pregnancy. Thus, whereas disease activity appears maximal early in life with no subsequent peaks in males, both menarche and pregnancy appear associated with disease onset or worsening in females. This suggests that hormonal changes may be a modulating factor
Thiamine Diphosphate in Whole Blood, Thiamine and Thiamine Monophosphate in Breast-Milk in a Refugee Population
BACKGROUND: The provision of high doses of thiamine may prevent thiamine deficiency in the post-partum period of displaced persons. METHODOLOGY/PRINCIPAL FINDINGS: The study aimed to evaluate a supplementation regimen of thiamine mononitrate (100 mg daily) at the antenatal clinics in Maela refugee camp. Women were enrolled during antenatal care and followed after delivery. Samples were collected at 12 weeks post partum. Thiamine diphosphate (TDP) in whole blood and thiamine in breast-milk of 636 lactating women were measured. Thiamine in breast-milk consisted of thiamine monophosphate (TMP) in addition to thiamine, with a mean TMP to total thiamine ratio of 63%. Mean whole blood TDP (130 nmol/L) and total thiamine in breast-milk (755 nmol/L) were within the upper range reported for well-nourished women. The prevalence of women with low whole blood TDP (<65 nmol/L) was 5% and with deficient breast-milk total thiamine (<300 nmol/L) was 4%. Whole blood TDP predicted both breast-milk thiamine and TMP (R(2) = 0.36 and 0.10, p<0.001). A ratio of TMP to total thiamine ≥63% was associated with a 7.5 and 4-fold higher risk of low whole blood TDP and deficient total breast-milk thiamine, respectively. Routine provision of daily 100 mg of thiamine mononitrate post-partum compared to the previous weekly 10 mg of thiamine hydrochloride resulted in significantly higher total thiamine in breast-milk. CONCLUSIONS/SIGNIFICANCE: Thiamine supplementation for lactating women in Maela refugee camp is effective and should be continued. TMP and its ratio to total thiamine in breast-milk, reported for the first time in this study, provided useful information on thiamine status and should be included in future studies of breast-milk thiamine
Simplifying the clinical classification of polymerase gamma (POLG) disease based on age of onset; studies using a cohort of 155 cases
Background: Variants in POLG are one of the most common causes of inherited mitochondrial disease. Phenotypic classification of POLG disease has evolved haphazardly making it complicated and difficult to implement in everyday clinical practise. The aim of our study was to simplify the classification and facilitate better clinical recognition. /
Methods: A multinational, retrospective study using data from 155 patients with POLG variants recruited from seven European countries. /
Results: We describe the spectrum of clinical features associated with POLG variants in the largest known cohort of patients. While clinical features clearly form a continuum, stratifying patients simply according to age of onset—onset prior to age 12 years; onset between 12 and 40 years and onset after the age of 40 years, permitted us to identify clear phenotypic and prognostic differences. Prior to 12 years of age, liver involvement (87%), seizures (84%), and feeding difficulties (84%) were the major features. For those with onset between 12 and 40 years, ataxia (90%), peripheral neuropathy (84%), and seizures (71%) predominated, while for those with onset over 40 years, ptosis (95%), progressive external ophthalmoplegia (89%), and ataxia (58%) were the major clinical features. The earlier the onset the worse the prognosis. Patients with epilepsy and those with compound heterozygous variants carried significantly worse prognosis. /
Conclusion: Based on our data, we propose a simplified POLG disease classification, which can be used to guide diagnostic investigations and predict disease course
EFNS guidelines on the molecular diagnosis of ataxias and spastic paraplegias
Background and purpose: These EFNS guidelines on the molecular diagnosis of neurogenetic disorders are designed to provide practical help for the general neurologist to make appropriate use of molecular genetics in diagnosing neurogenetic disorders.Methods: Literature searches were performed before expert members of the task force wrote proposals, which were discussed in detail until final consensus had been reached among all task force members.Results and conclusion: This paper provides updated guidelines for molecular diagnosis of two particularly complex groups of disorders, the ataxias and spastic paraplegias. Possibilities and limitations of molecular genetic diagnosis of these disorders are evaluated and recommendations are provided
The European 2015 drought from a hydrological perspective
In 2015 large parts of Europe were affected by drought. In this paper, we analyze the hydrological footprint (dynamic development over space and time) of the drought of 2015 in terms of both severity (magnitude) and spatial extent and compare it to the extreme drought of 2003. Analyses are based on a range of low flow and hydrological drought indices derived for about 800 streamflow records across Europe, collected in a community effort based on a common protocol. We compare the hydrological footprints of both events with the meteorological footprints, in order to learn from similarities and differences of both perspectives and to draw conclusions for drought management. The region affected by hydrological drought in 2015 differed somewhat from the drought of 2003, with its center located more towards eastern Europe. In terms of low flow magnitude, a region surrounding the Czech Republic was the most affected, with summer low flows that exhibited return intervals of 100 years and more. In terms of deficit volumes, the geographical center of the event was in southern Germany, where the drought lasted a particularly long time. A detailed spatial and temporal assessment of the 2015 event showed that the particular behavior in these regions was partly a result of diverging wetness preconditions in the studied catchments. Extreme droughts emerged where preconditions were particularly dry. In regions with wet preconditions, low flow events developed later and tended to be less severe. For both the 2003 and 2015 events, the onset of the hydrological drought was well correlated with the lowest flow recorded during the event (low flow magnitude), pointing towards a potential for early warning of the severity of streamflow drought. Time series of monthly drought indices (both streamflow- and climate-based indices) showed that meteorological and hydrological events developed differently in space and time, both in terms of extent and severity (magnitude). These results emphasize that drought is a hazard which leaves different footprints on the various components of the water cycle at different spatial and temporal scales. The difference in the dynamic development of meteorological and hydrological drought also implies that impacts on various water-use sectors and river ecology cannot be informed by climate indices alone. Thus, an assessment of drought impacts on water resources requires hydrological data in addition to drought indices based solely on climate data. The transboundary scale of the event also suggests that additional efforts need to be undertaken to make timely pan-European hydrological assessments more operational in the future
COST 733 – WG4: Applications of weather type classifications
Presentación realizada para: European Geosciences Union General Assembly celebrado del 19-24 de abril de 2009 en Viena
Unique spectrum of SPAST variants in Estonian HSP patients: presence of benign missense changes but lack of exonic rearrangements
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