162 research outputs found

    Il giuramento dell'infedele: stato di diritto, tolleranza e obbligazione politica

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    Numero monografico su La tolleranza: storia di una virtù difficil

    Miti filosofici e miraggi storiografici

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    The author addresses the nature and functions of some historiographical categories, showing their problematic application to philosophical currents and historical periodizations. By considering some cases of study in the history of modern philosophy, it is shown how the excessive expansion of such categories has made them useless as working tools, and has generated and nurtured philosophical mythologies and historiographical legends. Nonetheless, such mythologies and legends constitute an important field of investigation for historians of philosophy

    Rinascimento, rivoluzione scientifica e libertinismo erudito

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    The author examines an essay by Maurizio Torrini on the scientific revolution and libertinism. Studying the reception of Galileo’s discoveries in European philosophical culture, Torrini highlights the misunderstandings and instrumental uses that libertines made of Galilean astronomy. The scientific revolution and libertinism had independent paths and even when their paths crossed, no fusion emerged between the two components. Only at the end of the seventeenth century did apologetics unify libertinism and Galilean science into one doctrine to facilitate their condemnation. The essay shows the consequences that this interpretation produces on the historiographic categories with which modern philosophy is interpreted

    Moderni contro moderni: la strategia apologetica del cartesiano Gerdil

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    Follower of Malebranche, H.S. Gerdil is one of the greatest Eighteenth-Century Catholic philosophers. His Dissertation on the incompatibility of the principles of Descartes and Spinoza (1760), the subject of this article, highlights, thanks to an analytical examination of the texts, the irreconcilability between the two metaphysical perspectives. But the dissertation is not only a defense of Cartesianism against the accusation of closeness to Spinozism made by Leibniz and others – an accusation that for the author is denied by Spinoza's own Epistolary – it is also a recapitulation of Gerdil's philosophical apologetics. This is characterized by a skilful use of Descartes' philosophy to contrast all forms of materialistic monism, whether they derive from Spinoza, Locke or Leibniz. Against these philosophies Gerdil does not use the Aristotelian scholasticism but the philosophy of Descartes, considered the true champion of Modern philosophy. By clearly separating Descartes from Spinoza, Gerdil also constructs a narrative of philosophical modernity different from the one made by Voltaire in the Lettres philosophiques and accepted by the manifestos of the Lumières. The modèle anglais is replaced with the defense of the relevance of Cartesianism and its usefulness in contrasting the Eighteenth-Century materialistic and atheistic philosophies, presented as a corruption of Descartes' genuine principles, the only ones to guarantee the coexistence of philosophy with religion

    NEUROLOGICAL COMPLICATIONS IN ADULT ALLOGENEIC HEMATOPOIETIC STEM CELL TRANSPLANT PATIENTS: RESULTS FROM A RETROSPECTIVE MULTICENTRE STUDY

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    Patients undergoing allogeneic hematopoietic stem cell transplant (HSCT) are exposed to a number of neurological complications that may be related to drugs, infections, metabolic alterations, cerebrovascular events and immune-\uadmediated disorders including myositis, myasthenia gravis, Guillain-\uadBarr\ue8-\uadlike demyelinating polyneuropathy and central nervous system (CNS) manifestations of graft versus host disease (GVHD). The multifactorial etiology of neurological complications in HSCT patients makes diagnosis difficult. However a timely and rigorous characterization of such complications should be obtained in the attempt to avoid fatal outcomes or long-\uadterm effects. Data regarding neurological complications in HSCT patients derives from small series and varies largely in respect to incidence and severity. Aim of this study is to describe incidence, characteristics and outcome of neurological complications in a large series of consecutive HSCT patients

    Association of Variants in the SPTLC1 Gene With Juvenile Amyotrophic Lateral Sclerosis

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    Importance: Juvenile amyotrophic lateral sclerosis (ALS) is a rare form of ALS characterized by age of symptom onset less than 25 years and a variable presentation.Objective: To identify the genetic variants associated with juvenile ALS.Design, Setting, and Participants: In this multicenter family-based genetic study, trio whole-exome sequencing was performed to identify the disease-associated gene in a case series of unrelated patients diagnosed with juvenile ALS and severe growth retardation. The patients and their family members were enrolled at academic hospitals and a government research facility between March 1, 2016, and March 13, 2020, and were observed until October 1, 2020. Whole-exome sequencing was also performed in a series of patients with juvenile ALS. A total of 66 patients with juvenile ALS and 6258 adult patients with ALS participated in the study. Patients were selected for the study based on their diagnosis, and all eligible participants were enrolled in the study. None of the participants had a family history of neurological disorders, suggesting de novo variants as the underlying genetic mechanism.Main Outcomes and Measures: De novo variants present only in the index case and not in unaffected family members.Results: Trio whole-exome sequencing was performed in 3 patients diagnosed with juvenile ALS and their parents. An additional 63 patients with juvenile ALS and 6258 adult patients with ALS were subsequently screened for variants in the SPTLC1 gene. De novo variants in SPTLC1 (p.Ala20Ser in 2 patients and p.Ser331Tyr in 1 patient) were identified in 3 unrelated patients diagnosed with juvenile ALS and failure to thrive. A fourth variant (p.Leu39del) was identified in a patient with juvenile ALS where parental DNA was unavailable. Variants in this gene have been previously shown to be associated with autosomal-dominant hereditary sensory autonomic neuropathy, type 1A, by disrupting an essential enzyme complex in the sphingolipid synthesis pathway.Conclusions and Relevance: These data broaden the phenotype associated with SPTLC1 and suggest that patients presenting with juvenile ALS should be screened for variants in this gene.</p

    Genome-wide Analyses Identify KIF5A as a Novel ALS Gene

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    To identify novel genes associated with ALS, we undertook two lines of investigation. We carried out a genome-wide association study comparing 20,806 ALS cases and 59,804 controls. Independently, we performed a rare variant burden analysis comparing 1,138 index familial ALS cases and 19,494 controls. Through both approaches, we identified kinesin family member 5A (KIF5A) as a novel gene associated with ALS. Interestingly, mutations predominantly in the N-terminal motor domain of KIF5A are causative for two neurodegenerative diseases: hereditary spastic paraplegia (SPG10) and Charcot-Marie-Tooth type 2 (CMT2). In contrast, ALS-associated mutations are primarily located at the C-terminal cargo-binding tail domain and patients harboring loss-of-function mutations displayed an extended survival relative to typical ALS cases. Taken together, these results broaden the phenotype spectrum resulting from mutations in KIF5A and strengthen the role of cytoskeletal defects in the pathogenesis of ALS.Peer reviewe

    Introduction: Debates on Experience and Empiricism in Nineteenth Century France

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    The lasting effects of the debate over canon-formation during the 1980s affected the whole field of Humanities, which became increasingly engaged in interrogating the origin and function of the Western canon (Gorak 1991; Searle 1990). In philosophy, a great deal of criticism was, as a result, directed at the traditional narrative of seventeenth-and eighteenth-century philosophies—a critique informed by postcolonialism (Park 2013) as well as feminist historiography (Shapiro 2016). D. F. Norton (1981), L. Loeb (1981) and many others1 attempted to demonstrate the weaknesses of the tripartite division between rationalism, empiricism and critical philosophy.2 As time went on, symptoms of dissatisfaction with what has been called the “standard narrative” ( Vanzo 2013) and the “epistemological par-adigm” (Haakonssen 2004, 2006) only increased. Indeed, at present, a consensus has been reached that the narrative of the antagonism between “Continental rationalism” and “British empiricism”, and the consequent Aufhebung provided by “German critical philosophy,” has been unable to make sense of the complexity, variety and dynamics of early modern.Fil: Antoine-Mahut, Delphine. Ecole Normale Supérieure; FranciaFil: Manzo, Silvia Alejandra. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - La Plata. Instituto de Investigaciones en Humanidades y Ciencias Sociales. Universidad Nacional de La Plata. Facultad de Humanidades y Ciencias de la Educación. Instituto de Investigaciones en Humanidades y Ciencias Sociales; Argentin

    Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study.

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    We aimed to accurately estimate the frequency of a hexanucleotide repeat expansion in C9orf72 that has been associated with a large proportion of cases of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)
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