The Epidemiology of Stargardt Disease in the United Kingdom

The authors thank the British Ophthalmological Surveillance Unit (BOSU) for the support received, as well as Mr Barnaby Foot, research coordinator for BOSU, for his help and advice on this project. The authors thank the following ophthalmologists who assisted with data collection for this study: N. Acharya, S. Anwar, V. Bansal, P.N. Bishop, D. Byles, J.S. Chawla, A. Churchill, M. Clarke, B. Dhillon, M. Ekstein, S. George, J. Gillian, J.T. Gillow, D. Gilmour, R. Gray, P.T.S. Gregory, R. Gupta, S.P. Kelly, I.C. Lloyd, A. Lotery, M. McKibbin, R. MacLaren, G. Menon, A.T. Moore, A. Mulvihill, Y. Osoba, R. Pilling, H. Porooshani, A. Raghu Ram, T. Rimmer, I. Russell-Eggitt, M. Sarhan, R. Savides, S. Shafquat, A. Smith, A. Tekriwal, P. Tesha, P. Watts.Peer reviewedPublisher PD

Experiences in deploying metadata analysis tools for institutional repositories

Current institutional repository software provides few tools to help metadata librarians understand and analyze their collections. In this article, we compare and contrast metadata analysis tools that were developed simultaneously, but independently, at two New Zealand institutions during a period of national investment in research repositories: the Metadata Analysis Tool (MAT) at The University of Waikato, and the Kiwi Research Information Service (KRIS) at the National Library of New Zealand. The tools have many similarities: they are convenient, online, on-demand services that harvest metadata using OAI-PMH; they were developed in response to feedback from repository administrators; and they both help pinpoint specific metadata errors as well as generating summary statistics. They also have significant differences: one is a dedicated tool wheres the other is part of a wider access tool; one gives a holistic view of the metadata whereas the other looks for specific problems; one seeks patterns in the data values whereas the other checks that those values conform to metadata standards. Both tools work in a complementary manner to existing Web-based administration tools. We have observed that discovery and correction of metadata errors can be quickly achieved by switching Web browser views from the analysis tool to the repository interface, and back. We summarize the findings from both tools' deployment into a checklist of requirements for metadata analysis tools

Quantifying Aphantasia through drawing: Those without visual imagery show deficits in object but not spatial memory

Congenital aphantasia is a recently characterized variation of experience defined by the inability to form voluntary visual imagery, in individuals who are otherwise high performing. Because of this specific deficit to visual imagery, individuals with aphantasia serve as an ideal group for probing the nature of representations in visual memory, particularly the interplay of object, spatial, and symbolic information. Here, we conducted a large-scale online study of aphantasia and revealed a dissociation in object and spatial content in their memory representations. Sixty-one individuals with aphantasia and matched controls with typical imagery studied real-world scene images, and were asked to draw them from memory, and then later copy them during a matched perceptual condition. Drawings were objectively quantified by 2,795 online scorers for object and spatial details. Aphantasic participants recalled significantly fewer objects than controls, with less color in their drawings, and an increased reliance on verbal scaffolding. However, aphantasic participants showed high spatial accuracy equivalent to controls, and made significantly fewer memory errors. These differences between groups only manifested during recall, with no differences between groups during the matched perceptual condition. This object-specific memory impairment in individuals with aphantasia provides evidence for separate systems in memory that support object versus spatial information. The study also provides an important experimental validation for the existence of aphantasia as a variation in human imagery experience

The features underlying the memorability of objects

Despite decades of study of memory, it remains unclear what makes an image memorable. There is considerable debate surrounding the underlying determinants of memory, including the roles of semantic (e.g., animacy, utility) and visual features (e.g., brightness) as well as whether the most prototypical or most atypical items are best remembered. Prior studies have relied on constrained stimulus sets, limiting any generalized view of the features that may contribute to memory. Here, we collected over one million memory ratings (N=13,946) for THINGS (Hebart et al., 2019), a naturalistic dataset of 26,107 object images designed to comprehensively sample concrete objects. First, we establish a model of object features that is predictive of image memorability, capturing over half of the explainable variance. For this model, we find that semantic features have a stronger influence than visual features on what people will remember. Second, we examined whether memorability could be accounted for by the typicality of the objects, by comparing human behavioral data, object feature dimensions, and deep neural network features. While prototypical objects tend to be the most memorable, the relationship between memorability and typicality is more complex than a simple positive or negative association and typicality alone cannot account for memorability

To be or not to be an auctioneer: Some thoughts on the legal nature of online eBay auctions and the protection of consumers

This paper discusses the legal classification of online “eBay” auctions. The discussion has key implications on the scope of consumer protection law as sale by auctions are, for example, excluded from the scope of the Consumer Protection (Distance Selling) Regulations 2000. The paper uncovers that online “eBay” auctions cannot always be considered as traditional auctions and that eBay, as an intermediary, is not to be considered as an auctioneer. This creates difficulties associated with a distributive application of consumer protection laws such as the Consumer Protection (Distance Selling) Regulations 2000. Another set of difficulties is associated with a lenient legal regime applicable to the liability of eBay under the Electronic Commerce (EC Directive) Regulations 2002 . The paper concludes that there is an urgent need to clarify the legal classification of online auctions and to rethink the liability of online auction sites to better protect consumers

Alteration of rumen bacteria and protozoa through grazing regime as a tool to enhance the bioactive fatty acid content of bovine milk

Rumen microorganisms are the origin of many bioactive fatty acids (FA) found in ruminant-derived food products. Differences in plant leaf anatomy and chemical composition between cool- and warm-season pastures may alter rumen microorganisms, potentially enhancing the quantity/profile of bioactive FA available for incorporation into milk. The objective of this study was to identify rumen bacteria and protozoa and their cellular FA when cows grazed a warm-season annual, pearl millet (PM), in comparison to a diverse cool-season pasture (CSP). Individual rumen digesta samples were obtained from five Holstein cows in a repeated measures design with 28-day periods. The treatment sequence was PM, CSP, then PM. Microbial DNA was extracted from rumen digesta and sequence reads were produced with Illumina MiSeq. Fatty acids (FA) were identified in rumen bacteria and protozoa using gas-liquid chromatography/mass spectroscopy. Microbial communities shifted in response to grazing regime. Bacteria of the phylum Bacteroidetes were more abundant during PM than CSP (P \u3c 0.05), while protozoa of the genus Eudiplodinium were more abundant during CSP than PM (P \u3c 0.05). Microbial cellular FA profiles differed between treatments. Bacteria and protozoa from cows grazing CSP contained more n-3 FA (P \u3c 0.001) and vaccenic acid (P \u3c 0.01), but lower proportions of branched-chain FA (P \u3c 0.05). Microbial FA correlated with microbial taxa and levels of vaccenic acid, rumenic acid, and a-linolenic acid in milk. In conclusion, grazing regime can potentially be used to alter microbial communities shifting the FA profile of microbial cells, and subsequently, alter the milk FA profile

Association of metabolic syndrome and change in Unified Parkinson\u27s Disease Rating Scale scores.

OBJECTIVE: To explore the association between metabolic syndrome and the Unified Parkinson\u27s Disease Rating Scale (UPDRS) scores and, secondarily, the Symbol Digit Modalities Test (SDMT). METHODS: This is a secondary analysis of data from 1,022 of 1,741 participants of the National Institute of Neurological Disorders and Stroke Exploratory Clinical Trials in Parkinson Disease Long-Term Study 1, a randomized, placebo-controlled trial of creatine. Participants were categorized as having or not having metabolic syndrome on the basis of modified criteria from the National Cholesterol Education Program Adult Treatment Panel III. Those who had the same metabolic syndrome status at consecutive annual visits were included. The change in UPDRS and SDMT scores from randomization to 3 years was compared in participants with and without metabolic syndrome. RESULTS: Participants with metabolic syndrome (n = 396) compared to those without (n = 626) were older (mean [SD] 63.9 [8.1] vs 59.9 [9.4] years; p \u3c 0.0001), were more likely to be male (75.3% vs 57.0%; p \u3c 0.0001), and had a higher mean uric acid level (men 5.7 [1.3] vs 5.3 [1.1] mg/dL, women 4.9 [1.3] vs 3.9 [0.9] mg/dL, p \u3c 0.0001). Participants with metabolic syndrome experienced an additional 0.6- (0.2) unit annual increase in total UPDRS (p = 0.02) and 0.5- (0.2) unit increase in motor UPDRS (p = 0.01) scores compared with participants without metabolic syndrome. There was no difference in the change in SDMT scores. CONCLUSIONS: Persons with Parkinson disease meeting modified criteria for metabolic syndrome experienced a greater increase in total UPDRS scores over time, mainly as a result of increases in motor scores, compared to those who did not. Further studies are needed to confirm this finding. CLINICALTRIALSGOV IDENTIFIER: NCT00449865

‘Video Replay: Families, films and fantasy’ as a transformational text: Commentary on Valerie Walkerdine's ‘Video Replay’.

In this commentary I explore the significance of Valerie Walkerdine's paper ‘Video Replay: Families, Films and Fantasy’. I review its impact in 1986 and then discuss how some of its ideas about subjectivity and popular culture – specifically film - can be developed in the contemporary context. A recurring fantasy of Rocky II and its reception is that of social and psychological transformation. I address this theme by drawing on the work of Christopher Bollas to argue that Walkerdine's psychosocial analysis continues to facilitate, across a range of contexts, some of the transformational processes described in her article

The Paranormal is (Still) Normal: The Sociological Implications of a Survey of Paranormal Experiences in Great Britain

Historically, there has been limited sociological interest in the paranormal and no systematic study of reported paranormal experiences. There are also few medium-to-large-scale survey results with nationally representative populations focusing on paranormal experiences. This paper provides details of an exploratory survey conducted in 2009 with a nationally representative sample of 4,096 adults aged 16 years and over across Great Britain. Our findings show that 37% of British adults report at least one paranormal experience and that women, those who are middle-aged or individuals resident in the South West are more likely to report such experiences. These results establish incidence levels of reported paranormal experiences in contemporary Britain. We argue also that they merit a more sustained sociological consideration of the paranormal. In this respect we renew and update the robust justification and call for serious research positioning the paranormal as a social phenomenon, originally proposed well over thirty years ago by Greeley (1975)

The CMS Tracker Readout Front End Driver

The Front End Driver, FED, is a 9U 400mm VME64x card designed for reading out the Compact Muon Solenoid, CMS, silicon tracker signals transmitted by the APV25 analogue pipeline Application Specific Integrated Circuits. The FED receives the signals via 96 optical fibers at a total input rate of 3.4 GB/sec. The signals are digitized and processed by applying algorithms for pedestal and common mode noise subtraction. Algorithms that search for clusters of hits are used to further reduce the input rate. Only the cluster data along with trigger information of the event are transmitted to the CMS data acquisition system using the S-LINK64 protocol at a maximum rate of 400 MB/sec. All data processing algorithms on the FED are executed in large on-board Field Programmable Gate Arrays. Results on the design, performance, testing and quality control of the FED are presented and discussed