1,721,026 research outputs found
Epilessia del lobo temporale:considerazione a proposito di due casi a fenomenologia puramente affettiva
No full textDescrizione di due casi di Epilessia del lobo temporale:considerazione a proposito di fenomenologia puramente affettiv
Multiple sclerosis or Sjogren's syndrome: A clinical dilemma
No full textBackground: Multiple sclerosis (MS) like disease may be a feature of central nervous system (CNS) involvement in Sjogren''s syndrome prompting some difficulties in differential diagnosis with typical MS.
Objectives: To describe the clinical course, response to therapy and long term outcome of CNS Sjogren''s syndrome presenting as multiple sclerosis-like (MS) disease in a large series of primary Sjogren''s syndrome (pSS).
Methods: A cohort of 380 patients with pSS, consecutively observed at our unit from January 1990 to December 2002, was evaluated for CNS involvement with particular attention to MS-like disease. All the patients fulfilled the revised 2002 ECSG criteria for primary pSS. Clinical course, seroimmunological parameters and response to therapy of patients with MS-like clinical picture are described.
Results: Among 25 pts with CNS involvement, 4 pts (16%) had MS-like disease. All the pts were women, with a middle age of 41.5 years. In all cases, neurological symptoms preceded the diagnosis of pSS and occurred at a mean age of 33.5 years. Two patients had a monolateral visual loss due to retrobulbar optic neuritis. In two other cases there was also a spinal cord involvement, with a MRI imaging showing incomplete transverse myelitis. One patient developed brain stem involvement, with recurrent episodes of dizziness and sensorineural hearing loss. CSF analysis was performed in all patients showing mild lymphocytosis, raised IgG index and the presence of oligoclonal bands suggesting intyratechal immunological activation. In three pts brain MRI exhibited multiple areas of increased signal intensity on T2 weighted images, predominantly located in subcortical and periventricular white matter, with involvement of corpus callosum and cerebellum in one case. ANA were positive in 3 out 4 of pts, anti-Ro/SSA antibodies in 2 out of 4. Rheumatoid factor was positive in only one patient. No patient had anti-dsDNA or lupus anticoagulant. Only one patient presented a transient positivity for IgG anticardiolipin antibodies (low titer). All the patient had low levels of C4 (<15 mg/dl). Minor salivary glands biopsy was obtained in all patients showing a focal lymphocytic sialoadenytis with a focus score ≥ 1.
All the patient were treated with IV high doses of methylprednisolone during the acute phase of neurological manifestations; two patients received cyclosporine A 3 mg/kg/die, and one patient was treated with azathioprine 2 mg/kg/die. One patient with transverse myelitis had a good response to IV methylprednisolone and remained in remission with cyclosporine A, showing at instrumental follow-up evaluation a gradual disappearance of MRI lesions. Another patient suffered from a slowly primary progressive course with brainstem involvement following the first episode of optic neuritis. The remaining two pts had a relapsing-remitting course poorly influenced by immunosuppressive therapy (cyclosporine and azathioprine).
Conclusion: MS-like disease may be a rare but serious complication of pSS. Differential diagnosis from true MS is a troublesome challenge for the physician, expecially when the neurological syndrome represents the heralding picture as in the reported cases. Seroimmunological picture and salivary gland histopathology could be useful in the diagnostic interpretation permitting to avoid potentially dangerous immunomodulating therapies such as beta-IFN. In our opinion demyelinating disease should be considered in the CNS spectrum of pSS and should be carefully evaluated when dealing with MS disease with atypical presentation and/or in presence of warning signs of autoimmunity
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
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
Two novel mutations in the spastin gene (SPG4) found by DHPLC mutation analysis.
No full textThe most common form of autosomal dominant hereditary spastic paraplegia is caused by mutations in the gene encoding spastin (SPG4), a member of the AAA family of ATPases. In the current study, we designed a denaturing high-performance liquid chromatography based protocol for the analysis of the SPG4 gene. Using this method, we detected two novel missense mutations, 1375AOG (R459G) and 1378COT (R460C), one previously described five bases deletion (1215_1219del) and three polymorphic changes. This study suggests that denaturing high-performance liquid chromatography would be a fast and reliable tool in the investigation of the molecular defects in the SPG4 gene
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