Congenital Myasthenic Syndromes (CMS) are a group of rare genetic disorders affecting the neuromuscular junction structure and function. They are characterised by the presence of fatigable muscle weakness, but the age of onset, presenting symptoms and distribution of weakness differ depending on the genotype and specific mutations affected. The severity of CMS is highly variable amongst individuals, fluctuating and worsening with physical effort, which makes assessments challenging. Currently, there are no validated outcome measures for use in CMS, with most clinicians using outcome measures validated in Myasthenia Gravis and other neuromuscular conditions. The need to establish robust natural history data and validated outcome measures in this rare condition will be increasingly important with emerging novel treatments already in development. This study looks to answer whether there is a relationship between an instrumented six-minute walking test (6MWT) and community physical activity levels through the u…
This chapter will discuss and demonstrate how it is possible to introduce young people to scientists’ life stories and draw on particular events or incidents that can inspire them to think more deeply about science. Noteworthy moments from historical scientific stories are dramatised in various ways to engage learners to consider these scientific happenings from different perspectives. The learning activities, adopted and adapted from established theatrical strategies, purposely oriented learners to think about the lives and work of scientists from varied viewpoints. Immersed and positioned differently in a range of historical contexts to work in-role enabled learners to consider science from alternate positions. This provided not only an historical dimension to learning about science, but many of the narratives the learners were introduced to offered insights about socio-cultural influences determining what and how scientists investigated in the past. Learners working in-role, in participatory way…
This chapter tracks the journey from teacher to materials writer, exploring the challenges, compromises and co-creation in making the leap from one to the other. The chapter explores some of the issues that confront the materials writer in a changing world, including the role of printed materials, the impact of digital media, and potential conflicts between teaching and publication. Multiple ‘real-world’ materials writing scenarios are shared to illustrate the challenges and compromises of developing professionalism in this shifting landscape. A notion of ‘informed preparedness’ is suggested as a way of navigating these challenges to arrive at principled and worthwhile materials.
The aim of this article is to critically review the scientific literature about the changes in travel behaviour and mobility amongst older adults caused by the COVID-19 pandemic across various countries, identify unmet travel needs and highlight patterns of inequalities in older adults' mobility. We have collected articles from four academic databases: PubMed, Scopus, Transportation Research International Documentation (TRID) and Web of Science. Papers were considered for inclusion if they were published online in 2020 or later, written in English, and referred to urban or rural changes in travel behaviour and mobility of older adults over 50 years old. We examined the pre-existing models developed before the outbreak and classified the articles based on Musselwhite and Haddad's hierarchy of older adults' travel needs. The synthesis of the selected 25 articles shows a general decline in literal mobility amongst older adults, an increased share of virtual travel and their decreased capacity to fulfil different…
This is a book about the book. Is this a book? is a question of wide appeal and interest. With the arrival of ebooks, digital narratives and audiobooks, the time is right for a fresh discussion of what is a book. Older definitions that rely solely on print no longer work, and as the boundaries of the book have been broken down, this volume offers a fresh and lively discussion of the form and purpose of the book. How does the audiobook fit into the book family? How is the role of reading changing in the light of digital developments? Does the book still deserve a privileged place in society? The authors present a dynamic model of the book and how it lives on in today's competitive media environment.
What is morally valuable—if anything at all—in entrepreneurship? Existing normative takes can be broadly categorized as belonging to two main views: a backward and a forward-looking approach. The former sees entrepreneurial activity as a permissible emergent product of individuals’ interactions within the boundaries of people’s existing rights; the latter looks at entrepreneurship in the broader context of market processes and emphasizes its role in generating Pareto-improvements in social welfare. In this paper, I suggest that certain instances of entrepreneurship can be intrinsically valuable when they constitute Millian Experiments in Living, that is when entrepreneurial ventures are the expression of an entrepreneur’s conception of the good. Engaging in entrepreneurial activity which reflects one’s conception of the good helps individuals in cultivating their individuality and originality by means of subjecting their normative beliefs to empirical scrutiny, thus allowing one to confirm, revise, or refine …
In this commentary, I make a case for recognizing and encouraging in human geography a sort of mundane kindness that is unremarkable in its ambition, but potentially profound in its impacts. Although it is important to continue to analyze the conditions that have left so many of us in need of acts of kindness that compensate for the failures and violence of the neoliberal academy, I argue that alongside this critical scholarship we could continue to make room for ‘ordinary’ kindness that does not necessarily need to justify itself through critical reflection.
Studies investigating competitive bodybuilding have primarily done so from a pathologizing perspective, and have often considered aspects of the competitive bodybuilding lifestyle in isolation, therefore overlooking the broader motivations underlying individuals’ engagement in the sport. The current study addressed these limitations by using a meta-ethnographic approach to review the existing competitive bodybuilding literature as a collective. Synthesis of 20 published studies relating to competitive bodybuilders’ motivations, behaviours, and experiences resulted in the construction of five third-order constructs: a journey of self-discovery and improvement, gaining a new identity, enacting control, conditional and unconditional social support, and decisional balance. Encapsulated as a ‘perfect storm’, the results offer novel conceptual understanding of how the interplay of personality traits, life experiences, and situational factors drive competitors to begin and maintain their participation in competitive…
If critical thought is to contribute to liberatory struggle, it arguably requires a general, even structural, theorisation of the nature and sources of power and oppression. This appears to be at odds with the critical project of questioning the immanence of truth to power, as famously framed by Michel Foucault. Yet Foucault’s philosophical project in fact hinged upon his own attempts to grapple with this tension. What is more, his ultimate failure to resolve it led to ambiguities that might be considered generative (especially in light of increased rapprochement between Foucauldian, Marxian and decolonial IR). Reading Indigenous and decolonial movement intellectuals in tandem with Foucault, alongside the philosophy of science of one of his major influences - Gaston Bachelard - we advocate attentiveness to the ‘experimental’ way in which struggles against capitalist extraction and (neo-)colonialism hold together dissonant theoretical - and ontological - commitments when putting forward structural accounts …
As tourism research has paid limited attention to children, this study investigates children’s reactions to tourism development, focusing on their unique viewpoints on the World Heritage Site of Dubrovnik, Croatia. It employed cognitive neuroscience methods with 397 participants, revealing that, despite their preference for sustainable tourism scenarios, children exhibit a notable fixation on images emblematic of overtourism and associated challenges, particularly overcrowding. When exposed to sustainable tourism photographs, there was an observable increase in physiological arousal, albeit not as pronounced as when confronted with an overtourism scenario. Intriguingly, regardless of the scenario, children predominantly expressed neutral emotions. Within the sustainable tourism context, gender differences manifest as girls exhibiting lower levels of place attachment. Furthermore, inner-city residents exhibit diminished levels of nature connectedness, and emotions are indirectly linked to nature connectedness,…