Introduction: Congenital myasthenic syndromes refer to a heterogenic group of neuromuscular transmission disorders. CMS-subtypes are diverse regarding exercise intolerance and muscular weakness, varying from mild symptoms to life-limiting forms with neonatal onset. Long-term follow-up studies on disease progression and treatment-response in pediatric patients are rare.Patients and Methods: We analyzed retrospective clinical and medication data in a cohort of 32 CMS-patients including the application of a standardized, not yet validated test to examine muscular strength and endurance in 21 patients at the (...) last follow-up. Findings obtained in our cohort were compared with long-term follow-up studies of CMS-cohorts from the literature by considering the underlying molecular mechanisms. Outcomes of CMS-ST were compared to results of normal clinical assessment.Results: Thirty-two pediatric patients with defects in eight different CMS-genes were followed by a median time of 12.8 years. Fifty-nine percentage of patients manifested with first symptoms as neonates, 35% as infants. While 53% of patients presented a reduced walking distance, 34% were wheelchair-bound. Even under adequate therapy with pyridostigmine and 3,4-diaminopyridine, CHAT-mutations led to the progression of muscular weakness partly in combination with persistent respiratory and bulbar symptoms. RAPSN, CHRND, and CHRNB1 patients with neonatal manifestation, early respiratory problems, and bulbar symptoms showed a good and maintained treatment response. CHAT and CHRNE patients required higher PS dosages, whereas RAPSN patients needed a lower mean dosage at the last follow-up. The benefits of short-term medication and long-term progression of symptoms were highly dependent on the specific genetic defect. CMS-ST was carried out in 17/21 patients, determined affected muscle groups including bulbar and ocular symptoms, some of which were not reported by the patients.Conclusions: Our findings and comparison with the literature- suggest a better treatment-response and less severe progression of symptoms present in patients suffering from mutations in CMS-genes directly associated with receptor deficiency, while patients with defects leading to synaptopathy and presynaptic defects tend to have worse outcomes. Assessment of affected muscular groups and clinical symptoms by CMS-ST may be a useful tool for optimal therapeutic management of the patients, especially for future clinical studies. (shrink)

Shawn Loht's ground-breaking Phenomenology of Film: A Heideggerian Account of the Film Experience (2017) offers a detailed account of film experience as rooted in Martin Heidegger's existential structure of Dasein. Adapting Being and Time for a phenomenology of cinematic experience, Loht accurately describes how various existential structures of Dasein are “fostered” by the projected world of film: in Loht's account, being-in-the-world is extended in the film experience. Loht's project offers fertile ground for developing the phenomenological basis of film experience. Yet, (...) Loht anchors this account of film experience primarily in Heidegger's early philosophy; this leaves his adaptation not without challenges. First, if the structure of film experience is mapped too closely onto the notion of Dasein projecting itself into the filmworld, this theory presents the risk of taking the early Heidegger's “hands-on” approach too literally. Moreover, in its concern for Dasein's extensions into the world of film, Phenomenology of Film says little about the distinctly mediated character of filmworlds; this leaves ambiguous where the film experience effectively takes place. (shrink)

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This article shows the philosophical kinship between Adam Smith and Mary Wollstonecraft on the subject of love. Though the two major 18th century thinkers are not traditionally brought into conversation with each other, Wollstonecraft and Smith share deep moral concerns about the emerging commercial society. As the new middle class continues to grow along with commerce, vanity becomes an ever more common vice among its members. But a vain person is preoccupied with appearance, status, and flattery—things that get in the (...) way of what Smith and Wollstonecraft regard as the deep human connection they variously describe as love, sympathy, and esteem. Commercial society encourages inequality, Smith argues, and Wollstonecraft points out that this inequality is particularly obvious in the relationships between men and women. Men are vain about their wealth, power and status; women about their appearance. Added to this is the fact that most middle class women are both uneducated and encouraged by the conduct literature of their day to be sentimental and irrational. The combined economic and moral considerations of Wollstonecraft and Smith show that there is very little room for love in commercial society as they conceived it. (shrink)

Andrea Cesalpino is an important figure in the history of science. He demonstrated that blood circulates into heart from veins and from the heart to arteries, paving the way to Harvey’s complete description of blood circulation. Moreover, he was the founder of botany as a systematic discipline, which he based, rather than on the observation of accidental similarities of plants, on the discovery of their vegetative-generative principle. In philosophy, he attempted to conciliate the immortality of the soul (i.e., the form (...) of the body) with the existence of a unique immaterial intellect, which is individuated and multiplied in the singular souls that participate to it. (shrink)

On 6 September 1966, the prime minister of South Africa, Dr HF Verwoerd was killed by Mr Tsafendas, a Portuguese national of Greek descent, in parliament by stabbing him in the chest. Mr Tsafendas was a messenger in parliament. At the enquiry trial of Mr Tsafendas, he was found unfit to stand trial on the ground that he suffered from schizophrenia. The psychiatric evidence during the enquiry trial was reviewed and discussed under the following headings: Diagnosis of schizophrenia; Consideration of (...) cultural factors in forensic psychiatric settings; Delusional infestation v. extreme overvalued beliefs; Simulation of psychosis; Ethical considerations in criminal capacity and trial competency assessments. Lessons from the Mr Tsafendas enquiry trial for forensic psychiatrists where a defendant previously diagnosed with schizophrenia commits a prominent political murder, are summarised. It is emphasised that the personhood of an accused referred for forensic observation should be respected and protected, instead of focusing exclusively on a specific diagnosis. (shrink)

Au-delà d’une bataille écologique tenace pour l’abandon du projet d’aéroport, le bocage de Notre-Dame des Landes a constitué un terrain d’expérimentation pour transformer l’occupation militante en droit pérenne d’usage des terres. Les « zadistes » se sont battus pour devenir agriculteurs et exploiter collectivement la terre au service d’une agriculture « écolo et non Monsanto ». Ils ont parié sur le fait de mener une bataille sur le droit face à l’administration et, en échange de fiches de projets, ont obtenu (...) des baux ruraux individuels de neuf ans, comme les paysans résistants et les paysans cumulards, qui exploitaient les terres avant la préemption. Le rêve éveillé est devenu réalité, même si l’un des principes de base initiaux, « la terre en commun », a subi des altérations. (shrink)

Martin Folkes (1690–1754) was Newton's protégé, an English antiquary, mathematician, numismatist and astronomer who would in the latter part of his career become simultaneously president of the Royal Society and of the Society of Antiquaries. Folkes took a Grand Tour from March 1733 to September 1735, recording the Italian leg of his journey from Padua to Rome in his journal. This paper examines Folkes's travel diary to analyse his Freemasonry, his intellectual development as a Newtonian and his scientific peregrination. It (...) shows how, in this latter area, how he used metrology to understand not only the aesthetics but also the engineering principles of antique buildings and artefacts, as well as their context and place in the Italian landscape. Using Folkes's diary, his account book of his journey in the Norwich archives, and his correspondence with other natural philosophers such as Francesco Algarotti (1712–1764), Anders Celsius (1701–1744) and Abbé Antonio Schinella Conti (1667–1749), this paper will also demonstrate to what extent Folkes's journey established his reputation as an international broker of Newtonianism, as well as the overall primacy of English scientific instrumentation to Italian virtuosi. (shrink)

In chapter seven ‘Self Identification’ of his challenging book The Varieties of Reference, Gareth Evans attempts to give an account of how it is that one is able to think about oneself self‐consciously. On Evans’ view, when one attempts to think of oneself self‐consciously that person is having what he calls an ‘I’ thought. Since these ‘I’ thoughts are a case of reference, more specifically self‐reference, Evans thinks that these thoughts can be explained by employing the same theoretical framework that (...) he uses to explain other kinds of reference. Evans thinks all thoughts are essentially structured, and this means that they must fall under his ‘generality constraint’. Since ‘I’ thoughts are also ‘thoughts’ they are essentially structured as well, and they too must be subject to the generality constraint. The radical implication of this is that Evans thinks that if ‘I’ thoughts are subject to the generality constraint, then he can show that self‐reference must be reference to a thing which we can locate on a spatio‐temporal map. In this article I hope to accomplish three things. First, I will spell out in detail the argument Evans uses to arrive at his claim that self‐reference must be reference to something located on a spatio‐temporal map. Second, I will raise an objection, which states that Evans’ conclusion that self‐reference must involve spatio‐temporal location is not a consequence of the generality constraint. Finally I will argue that Evans’ conclusion that self‐reference must involve spatio‐temporal location is in fact in tension with the generality constraint, rather than being an implication of it. (shrink)

Although much of the growing literature on organizational identity implicitly recognizes the normative nature of identity, the ethical implications of organizational identity work and talk have not yet been explored in depth. Working from a meta-ethical perspective, we claim that the dynamic, processual, and temporal activities recently associated with organizational identity always have an ethical dimension, whether "good" or "bad." In order to describe the ethical dimensions of organizational identity, we introduce the balance theory of practical wisdom as a theoretical (...) framework, and connect this theory to existing organizational identity concepts. We present an empirical case focused on an international paint company to illustrate the relevance of this theory for empirical organizational identity research. Our intention is to expand existing theory by bringing an aspect of organizational identity that has been tangentially acknowledged to the forefront, and by identifying it as a fruitful avenue for future theory development as well as empirical research. (shrink)

Le territoire (les territoires), objet politique s’il en est, a surgi dans la campagne des présidentielles comme enjeu électoral. Cette Mineure s’est fixée pour tâche de « refroidir » le débat, de regarder en dessous des idéologies charriées par le mot, de décomposer la polysémie territoriale. L’auteure a voulu ici proposer un point de vue sur les controverses qui agitent les milieux scientifiques autour des formes et des dynamiques territoriales. Par là-même, ces disputations confrontent l’action publique aux agirs spatiaux, en (...) mêlant objectivités et subjectivités. En cela, le territoire est prise de position politique. (shrink)

This paper begins from one of the most commonly found questions in phenomenology, “What is Phenomenlogy?”, to argue that phenomenology is a trans-disciplinary approach to engaging with the products of human culture. This approach is characterized by paying particular attention to the distinction between facticity and transcendence within “lived experience” so as to help us better articulate and evaluate the promises that animate every human institution. Such a task necessarily requires inter-disciplinary input and helps us engage in our lives—in our (...) shared cultural life—differently. (shrink)

This book brings together leading scholars in the history of science, history of universities, intellectual history, and the history of the Royal Society, to honor Professor Mordechai Feingold. The essays collected here reflect the impact Feingold's scholarship has had on a range of fields and address several topics, including: the dynamic pedagogical techniques employed in early modern universities, networks of communication through which scientific knowledge was shared, experimental techniques and knowledge production, the life and times of Isaac Newton, Newton's reception, (...) and the scientific culture of the Royal Society. Modeling the interdisciplinary approaches championed by Feingold as well as the essential role of archival studies, the volume attests to the enduring value of his scholarship and sets a benchmark for future work in the history of science and its allied fields. (shrink)

Over the past decade, Wolfgang Streeck has emerged as one of the most prominent voices in the debate on the crisis of democratic capitalism. This article provides a critical appraisal of Streeck’s recent writings in light of his wider intellectual trajectory, tracing the evolutions and continuities in his work over time; highlighting its important contributions to our understanding of the present crisis; and presenting a fourfold critique of his latest book on the end of capitalism. The main argument is that (...) Streeck’s work, while very valuable for its elucidation of the dynamics behind the demise of social democracy, ultimately remains plagued by a corporatist residue that keeps him from drawing his increasingly radical critique of capitalism to its logical conclusions. As a result, Streeck’s embrace of an exceedingly catastrophist worldview, devoid of any emancipatory potential, has tempted him to veer dangerously close to the welfare chauvinism of the nationalist right. (shrink)

Ethical considerations The study was conducted according to the principles of Declaration of Helsinki, and was approved by the Norwegian Social Science Data Services. Objective To describe patients’ experiences of staying in multiple- and single-bed rooms. Patients and methods This qualitative study employed a descriptive and exploratory approach, and systematic text condensation was used to analyze the material. Data were collected in a hospital trust in Norway. A total of 39 in-depth interviews were performed with patients discharged from the medical, (...) surgical, and maternity departments. Results Patients had ambiguous views on whether multiple-bed rooms or single-bed rooms were to be preferred. Main results include how patients cherished “the importance of others” but at the same time valued “the importance of privacy.” Being hospitalized in multiple-bed rooms was for many patients a very positive experience in terms of social interaction. Patients in single-bed rooms reported being more dependent on nurses to maintain social contact and obtain safety. Conclusion This research provides new knowledge on how the need for privacy can be in contradiction with the need for socializing with other patients. When hospitalized, the physical structure of a hospital impacts with whom patients interact and to what extent they depend on the nursing staff to have their social needs met. (shrink)

Living in the digital world, we have little choice but to share our personal data with big tech companies. These companies use our data to direct our attention so that they can make money from advertising. In a so-called surveillance society, we stand in an asymmetrical power relationship to these companies (and sometimes governments) and have little to no control over what happens to our data. Our dependence on these asymmetrical relationships is increasing as “big tech” uses the information it (...) gathers about us to cater to our personal habits and desires. (shrink)

Cette étude fournit une analyse comparative des politiques liées à la position des personnes handicapées sur le marché du travail en Finlande et en Italie, deux pays où l’écart d’emploi entre les personnes handicapées et non handicapées est relativement faible. Cette étude examine les politiques visant à améliorer la position des personnes handicapées sur le marché du travail et se base sur la littérature existante et des entretiens avec des experts. L’analyse comparative montre que ces deux pays emploient des approches (...) politiques différentes. En Finlande, les compensations sont relativement élevées et les politiques mettent l’accent sur la prévention. Cependant, les mesures d’activation pour les bénéficiaires de compensations d’invalidité font défaut. En Italie, les politiques se concentrent sur l’activation. La loi italienne sur les quotas oblige les entreprises à embaucher des personnes handicapées. En outre, les coopératives sociales offrent des emplois aux personnes handicapées. Les résultats suggèrent que les efforts visant à garantir des mesures de compensation et d’activation adéquates, combinés à des processus créés pour construire une société inclusive, peuvent contribuer à améliorer la situation du marché du travail pour les personnes handicapées. (shrink)

The essential tension in Nehemiah Grew's working methods in his Anatomy of Plants (1682) resulted in a flowering of scientific creativity. On the one hand, he utilised his intuition about the plants he studied in order to understand them in their own right, and indeed to idealise them visually as structures of emotional sympathy and great geometric beauty. On the other hand, Grew was a secretary of the Royal Society, a physician, and a museum cataloguer, as well as a Baconian (...) empirical inductivist, and he believed his work should have practical, applied, and economic uses. Although he shared several of these impulses with other early modern English virtuosi, Grew's commitment to understanding the whole organism, from the observation of its smallest bits of matter to an emotional identification with his experimental subjects as sentient beings, shows us the power of cultivating “a feeling for the organism” in scientific work. (shrink)

Automated technologies and robots make decisions that cannot always be fully controlled or predicted. In addition to that, they cannot respond to punishment and blame in the ways humans do. Therefore, when automated cars harm or kill people, for example, this gives rise to concerns about responsibility-gaps and retribution-gaps. According to Sven Nyholm, however, automated cars do not pose a challenge on human responsibility, as long as humans can control them and update them. He argues that the agency exercised in (...) automated cars should be understood in terms of human–robot collaborations. This brief note focuses on the problem that arises when there are multiple people involved, but there is no obvious shared collaboration among them. Building on John Danaher’s discussion of command responsibility, it is argued that, although Nyholm might be right that autonomous cars cannot be regarded as acting on their own, independently of any human beings, worries about responsibility-gaps and retribution-gaps are still justified, because it often remains unclear how to allocate or distribute responsibility satisfactorily among the key humans involved after they have been successfully identified. (shrink)