1,720,988 research outputs found

    Immunoglobuline nelle malattie neurologiche immunomediate del bambino

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    Le IVIG sono utilizzate in alcune malattie neurologiche immunomediate del bambino. Allo scopo di sintetizzare le loro attuali indicazioni e livelli di evidenza, abbiamo condotto una review sistematica di tutti gli studi clinici randomizzati controllati (RCT), studi retrospettivi e prospettici, case series e case reports disponibili sul database di Pubmed dal 1990 ad oggi. Alle IVIG può essere attribuita una classe di evidenza II ed un livello di raccomandazione A nella sindrome di Guillain-Barré (GBS) pediatrica (nonostante siano raccomandabili studi su un numero maggiore di pazienti). Le IVIG possono inoltre essere considerate terapia di prima linea nella poliradicolonevrite infiammatoria cronica demielinizzante (CIDP) pediatrica e nella crisi miastenica (Livello B). Le IVIG possono essere utili nell’encefalomielite acuta disseminata (ADEM), nella sclerosi multipla (SM), nell’Encefalite da anticorpi anti-NMDA e nella Dermatomiosite giovanile (Livello C). Anche se la bassa incidenza di malattie neurologiche immunomediate in età pediatrica, i costi elevati per condurre studi multicentrici, ed i problemi etici nel disegnare studi controllati verso placebo sono fattori limitanti, è auspicabile l’organizzazione di RCT multicentrici per valutare l’evidenza di efficacia delle IVIG in queste malattie

    Miopatie e malattie della giunzione neuromuscolare

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    Definizione, caratteristiche fisiopatologiche, morfologiche e cliniche delle malattie del muscolo e della giunzione neuromuscolareDefinition, pathophysiological, morphological and clinical characteristics of muscular and neuromuscular junction disorder

    Plasma-exchange responsive delayed pseudochoreoathetosis in a patient with anti-Hu neuronopathy

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    we report a patient with pseudochoreoathetosis that occurred several years after the onset of Hu anti-neuronal nuclear antibody (anti-Hu) sensory neuronopathy and improved after plasma exchange (PEX)

    Widening the phenotypical spectrum of EGR2-related CMT: unusual phenotype for R409W mutation

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    The Authors report a 46-year-old man who had presented, since his late thirties, a slowly progressive symmetric distal limb weakness and atrophy, associated with mild distal sensory loss. A heterozygous c.1225C > T (R409W) mutation on EGR2 gene was detecte

    Peripheral nerve ultrasound changes in CIDP and correlations with nerve conduction velocity

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    To evaluate the ultrasound (US) characteristics of peripheral nerves in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and their correlations with electrodiagnostic (EDX) characteristics. METHODS: Nineteen patients with CIDP and 19 healthy controls matched by age and body mass index were included in a blind case-control, observational study. All patients underwent a neurologic examination (including inflammatory neuropathy cause and treatment [INCAT] and Medical Research Council [MRC] sum score) and an EDX study. Each patient and each control underwent a US study of 14 nerve segments, yielding a total number of 266 segments scanned in each group. RESULTS: US changes, characterized by an increased nerve cross-sectional area (NCSA), were detected in 53% of the 266 patient nerve segments. Mean NCSA was higher in nerve segments of patients than in those of controls (p < 0.001). Nerve segments with abnormal US belonged to patients with longer disease duration, lower MRC sum score, higher INCAT score, and progressive disease form (all p < 0.0001). All the aforementioned variables were independently associated with the occurrence of US changes. Motor nerve conduction was significantly lower in nerve segments with increased NCSA than in those with normal NCSA (p < 0.0001). NCSA in segments with prevalent myelin damage was higher than that in segments with prevalent axonal damage (p = 0.001) or in segments with normal EDX characteristics (p < 0.0001). NCSA and motor nerve conduction velocity were inversely correlated in nerve segments with EDX evidence of myelin damage (R = 0.599; p < 0.0001). Conduction blocks were associated with increased NCSA (p = 0.001). CONCLUSIONS: US may, similar to MRI, have a supporting role in the diagnosis of CIDP. US and EDX changes are correlated

    Going Beyond Counting First Authors in Author Co-citation Analysis

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    The present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed

    Variations on the Author

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    “Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship

    Appropriate Similarity Measures for Author Cocitation Analysis

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    We provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis

    Dispelling the Myths Behind First-author Citation Counts

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    We conducted a full-scale evaluative citation analysis study of scholars in the XML research field to explore just how different from each other author rankings resulting from different citation counting methods actually are, and to demonstrate the capability of emerging data and tools on the Web in supporting more realistic citation counting methods. Our results contest some common arguments for the continued use of first-author citation counts in the evaluation of scholars, such as high correlations between author rankings by first-author citation counts and other citation counting methods, and high costs of using more realistic citation counting methods that are not well-supported by the ISI databases. It is argued that increasingly available digital full text research papers make it possible for citation analysis studies to go beyond what the ISI databases have directly supported and to employ more sophisticated methods
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