1,720,961 research outputs found
Two sisters with leukoencephalopathy, hearing loss and retinopathy: a familial case of Susac's syndrome?
No full textN.A
Paroxysmal dysarthria-ataxia in remitting-relapsing Bickerstaff's-like encephalitis.
No full textParoxysmal dysarthria-ataxia is a rare neurological condition due to ephaptic transmission, generally appearing in multiple sclerosis patients characterized by stereotyped attacks of slurred speech usually accompanied by ataxia, appearing many times a day. Here we describe a patient with an unusual remitting-relapsing form of Bickerstaff's-like brainstem encephalitis who manifested PDA after a relapse with the involvement of a peculiar region below the red nuclei and benefited from lamotrigine
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe 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
Two sisters with Leukoencephalopathy, hearing loss and retinopathy: a familial case of Susac's Syndrome?
No full textAims: Susac’s syndrome is a rare clinical entity characterized by encephalopathy, sensorineural hearing loss and retinopathy caused by immune-mediated arteriole occlusion in the brain, retina and inner ear. No familial cases have been reported. We describe here the cases of two sisters who were seen at our Department for subacute neurological symptoms suggestive of a multifocal central nervous system disorder associated to hearing impairment and clinical or subclinical involvement of visual function.
Presentation of Cases: The first case presented with a two-years history of progressive paraparesis, gait ataxia and cognitive dysfunction started at age 46; she also suffered from epileptic seizures since childhood and bilateral visual loss occurred between age 37 and 38. Her sister, aged 44, had a long-standing history of headache followed by sudden-onset bilateral hearing loss at age 35, which did not recover, and an acute episode of right-sided face paresthesias nine years later. Brain MRI showed multiple T2-hyperintense supratentorial lesions involving the corpus callosum in both sisters, with “snowball” appearance in the older one. Serum anti-endothelial cell antibodies assay was positive in the youngest patient.
Discussion and Conclusion: After exclusion of other possible options, a diagnosis of Susac's syndrome looked probable for both sisters. Further studies investigating the pathogenesis and the genetic background of the disease are needed
Immunoblot as a potential diagnostic tool for myofibrillar myopathies
No full textMyofibrillar myopathies (MFMs) are a group of inherited or sporadic neuromuscular disorders morphologically characterized by foci of myofibril dissolution, disintegration of the Z-disk and insoluble protein aggregates within the muscle fibers. The diagnosis is based on muscle biopsy. Light and electron microscopy has a central role in the diagnostic work-up and immunohistochemistry shows abnormal deposition of several proteins including αB-crystallin, desmin and myotilin. In contrast immunoblotting does not have any diagnostic value because it does not highlight differences in the amount of involved proteins. We investigated the pattern and level expression of desmin, αB-crystallin, myotilin and ZASP in muscle of seven patients with MFMs by immunoblotting after SDS-PAGE and 2D-PAGE using two different solubilizing solutions, one RIPA buffer and the other urea-containing buffer. Our data demonstrated that urea-containing buffer improves the solubilization and recovery of desmin, αB-crystallin, myotilin and ZASP as compared with RIPA buffer and that the total content of these proteins is increased in muscle of patients. The present results provide evidence that immunoblotting is an additional tool for confirming diagnosis of MFMs. This article is protected by copyright. All rights reserved
Variations on the Author
Full text link“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
Full text linkWe 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
Full text linkWe 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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