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Early‐infantile Krabbe disease (EIKD) is an autosomal recessive, progressive, neurodegenerative disorder that usually leads to death in infancy. A study published in 2005 indicated that hematopoietic stem‐cell transplantation (HSCT) was effective in the treatment for EIKD when used before the onset of symptoms. This finding suggested that newborn screening for EIKD, which would allow earlier diagnosis, might lead to earlier treatment and better outcomes. In 2006, New York was the first state to implement newborn screening for Krabbe disease; however, the results were not as good as proponents had hoped. In this paper, we present the history of efforts to diagnose and treat EIKD. Based on our findings, we question the efficacy of newborn screening for Krabbe disease. We present two arguments. First, testing itself is too imprecise. Even with the most rigorous testing standards, such as those used in New York, many of the children who are identified as being ‘at risk’ for EIKD remain asymptomatic. It is unclear if they will remain asymptomatic forever and, thus, whether the tests should be considered ‘false positives’, or whether they will eventually develop the disease. Second, we question the efficacy of early HSCT. We recommend placing a moratorium on mandatory newborn screening for EIKD. What this paper adds Current tests to identify which children are likely to develop Krabbe diseased are inadequate. Many children identified as being ‘at risk’ for early infantile Krabbe disease remain asymptomatic. Psychosine appears to be more specific than low galactosylceramidase levels for diagnosing early infantile Krabbe disease.

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Executive functions (EFs) are higher-level cognitive functions that help keep an individual’s goal-oriented thoughts and actions aligned. While many studies have shown the importance of EFs in sport, a limitation in this literature is that female participants have been underrepresented. In this mixed-longitudinal study, we examined the development of EFs in a cohort of high performing female athletes. We collected data over five seasons in a large sample of 175 female soccer players (aged 12–29 years old) from the U14 - senior age groups of a professional German soccer club. Players undertook a large battery of cognitive tasks aimed at measuring higher-level cognitive functioning: a sustained attention task, a stop-signal task, a Go-No-go test, an N-Back Test, and both a 180°- and 360°-multiple-object tracking task. We used linear and non-linear mixed effect regressions to examine the relationship between age and EFs. Second order polynomial curves explained many of these relationships between age and EFs compared to their linear relationships. Negatively accelerated curves reveal that these players’ cognitive abilities mainly developed before players reached early adulthood, with a performance plateau evident at around 21 years of age. Age explained low to moderate proportions of the variance in EFs (<1–50%), while cognitive development across playing positions was not a strong contributor to this variance ( M = 2.1, SD = 2.1%). We concluded that age has a negatively accelerated relationship with EFs in female soccer players that does not differ between playing positions. These data support the idea that athletes require only a reasonable level of EF ability to perform at the highest level of their sport. Our research raises new questions regarding the validity of current EF measurement methods for inferring information about in-game use of these cognitive abilities.

GoalThis paper presents an immersive Virtual Reality (VR) system to analyze and train Executive Functions (EFs) of soccer players. EFs are important cognitive functions for athletes. They are a relevant quality that distinguishes amateurs from professionals.MethodThe system is based on immersive technology, hence, the user interacts naturally and experiences a training session in a virtual world. The proposed system has a modular design supporting the extension of various so-called game modes. Game modes combine selected game mechanics with specific simulation content to target particular training aspects. The system architecture decouples selection/parameterization and analysis of training sessions via a coaching app from an Unity3D-based VR simulation core. Monitoring of user performance and progress is recorded by a database that sends the necessary feedback to the coaching app for analysis.ResultsThe system is tested for VR-critical performance criteria to reveal the usefulness of a new interaction paradigm in the cognitive training and analysis of EFs. Subjective ratings for overall usability show that the design as VR application enhances the user experience compared to a traditional desktop app; whereas the new, unfamiliar interaction paradigm does not negatively impact the effort for using the application.ConclusionThe system can provide immersive training of EF in a fully virtual environment, eliminating potential distraction. It further provides an easy-to-use analyzes tool to compare user but also an automatic, adaptive training mode.