898 resultados para Neuromuscular manifestations


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The convergence of Internet marketplaces and service-oriented architectures has spurred the growth of Web service ecosystems. This paper articulates a vision for Web service ecosystems, discusses early manifestations of this vision, and presents a unifying architecture to support the emergence of larger and more sophisticated ecosystems

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The Time magazine ‘Person of theYear’ award is a venerable institution. Established by Time’s founder Henry Luce in 1927 as ‘Man of the Year’, it is an annual award given to ‘a person, couple, group, idea, place, or machine that ‘for better or for worse ... has done the most to influence the events of the year’ (Time 2002, p. 1). In 2010, the award was given to Mark Zuckerberg, the founder and CEO of the social networking site Facebook.There was, however, a strong campaign for the ‘People’s Choice’ award to be given to Julian Assange, the founder and editor-in-chief of Wikileaks, the online whistleblowing site. Earlier in the year Wikileaks had released more than 250 000 US government diplomatic cables through the internet, and the subsequent controver- sies around the actions of Wikileaks and Assange came to be known worldwide as ‘Cablegate’. The focus of this chapter is not on the implications of ‘Cablegate’ for international diplomacy, which continue to have great significance, but rather upon what the emergence of Wikileaks has meant for journalism, and whether it provides insights into the future of journalism. Both Facebook and Wikileaks, as well as social media platforms such as Twitter and YouTube, and independent media practices such as blogging, citizen journalism and crowdsourcing, are manifestations of the rise of social media, or what has also been termed web 2.0.The term ‘web 2.0’ was coined by Tim O’Reilly, and captures the rise of online social media platforms and services, that better realise the collaborative potential of digitally networked media. They do this by moving from the relatively static, top-down notions of interactivity that informed early internet development, towards more open and evolutionary models that better harness collective intelligence by enabling users to become the creators and collaborators in the development of online media content (Musser and O’Reilly 2007; Bruns 2008).

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Creativity plays an increasingly important role in our personal, social, educational, and community lives. For adolescents, creativity can enable self-expression, be a means of pushing boundaries, and assist learning, achievement, and completion of everyday tasks. Moreover, adolescents who demonstrate creativity can potentially enhance their capacity to face unknown future challenges, address mounting social and ecological issues in our global society, and improve their career opportunities and contribution to the economy. For these reasons, creativity is an essential capacity for young people in their present and future, and is highlighted as a priority in current educational policy nationally and internationally. Despite growing recognition of creativity’s importance and attention to creativity in research, the creative experience from the perspectives of the creators themselves and the creativity of adolescents are neglected fields of study. Hence, this research investigated adolescents’ self-reported experiences of creativity to improve understandings of their creative processes and manifestations, and how these can be supported or inhibited. Although some aspects of creativity have been extensively researched, there were no comprehensive, multidisciplinary theoretical frameworks of adolescent creativity to provide a foundation for this study. Therefore, a grounded theory methodology was adopted for the purpose of constructing a new theory to describe and explain adolescents’ creativity in a range of domains. The study’s constructivist-interpretivist perspective viewed the data and findings as interpretations of adolescents’ creative experiences, co-constructed by the participants and the researcher. The research was conducted in two academically selective high schools in Australia: one arts school, and one science, mathematics, and technology school. Twenty adolescent participants (10 from each school) were selected using theoretical sampling. Data were collected via focus groups, individual interviews, an online discussion forum, and email communications. Grounded theory methods informed a process of concurrent data collection and analysis; each iteration of analysis informed subsequent data collection. Findings portray creativity as it was perceived and experienced by participants, presented in a Grounded Theory of Adolescent Creativity. The Grounded Theory of Adolescent Creativity comprises a core category, Perceiving and Pursuing Novelty: Not the Norm, which linked all findings in the study. This core category explains how creativity involved adolescents perceiving stimuli and experiences differently, approaching tasks or life unconventionally, and pursuing novel ideas to create outcomes that are not the norm when compared with outcomes by peers. Elaboration of the core category is provided by the major categories of findings. That is, adolescent creativity entailed utilising a network of Sub-Processes of Creativity, using strategies for Managing Constraints and Challenges, and drawing on different Approaches to Creativity – adaptation, transfer, synthesis, and genesis – to apply the sub-processes and produce creative outcomes. Potentially, there were Effects of Creativity on Creators and Audiences, depending on the adolescent and the task. Three Types of Creativity were identified as the manifestations of the creative process: creative personal expression, creative boundary pushing, and creative task achievement. Interactions among adolescents’ dispositions and environments were influential in their creativity. Patterns and variations of these interactions revealed a framework of four Contexts for Creativity that offered different levels of support for creativity: high creative disposition–supportive environment; high creative disposition–inhibiting environment; low creative disposition–supportive environment; and low creative disposition–inhibiting environment. These contexts represent dimensional ranges of how dispositions and environments supported or inhibited creativity, and reveal that the optimal context for creativity differed depending on the adolescent, task, domain, and environment. This study makes four main contributions, which have methodological and theoretical implications for researchers, as well as practical implications for adolescents, parents, teachers, policy and curriculum developers, and other interested stakeholders who aim to foster the creativity of adolescents. First, this study contributes methodologically through its constructivist-interpretivist grounded theory methodology combining the grounded theory approaches of Corbin and Strauss (2008) and Charmaz (2006). Innovative data collection was also demonstrated through integration of data from online and face-to-face interactions with adolescents, within the grounded theory design. These methodological contributions have broad applicability to researchers examining complex constructs and processes, and with populations who integrate multimedia as a natural form of communication. Second, applicable to creativity in diverse domains, the Grounded Theory of Adolescent Creativity supports a hybrid view of creativity as both domain-general and domain-specific. A third major contribution was identification of a new form of creativity, educational creativity (ed-c), which categorises creativity for learning or achievement within the constraints of formal educational contexts. These theoretical contributions inform further research about creativity in different domains or multidisciplinary areas, and with populations engaged in formal education. However, the key contribution of this research is that it presents an original Theory and Model of Adolescent Creativity to explain the complex, multifaceted phenomenon of adolescents’ creative experiences.

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A 16 y.o. fully ambulant boy born to consanguineous Indian parents, presented for assessment of a fragility femoral neck fracture sustained against a background of autism and moderately severe intellectual disability. He had a past history of infantile eczema, and epilepsy treated with anticonvulsants from 2 to 10 years of age, with no further seizures following cessation of anticonvulsants. He had a thin body habitus (see Table 1) with long fingers and a high arched palate. He had no speech and negligible social interaction, but physical examination was otherwise unremarkable. Positive investigations revealed an undetectable serum creatinine and a urinary metabolic screen which showed an elevated GUA:Phe of 160 (< 36) and a decreased creatinine of 0.3 mmol/l (1.2–29.5) consistent with the diagnosis of guanidinoacetate methyltransferase(GAMT) deficiency. He was commenced on oral creatine 5 g three times daily. Despite improvement in physical activity, height and bone density, there was no discernable improvement in his intellectual functioning. Proton and phosphorous brain and leg magnetic resonance spectroscopy(MRS) was performed at baseline and showed an increased inorganic phosphorus peak and decreased phosphocreatine synthesis in brain and decreased creatine concentration in muscle. Following creatine treatment total brain creatine(1H-MRS) and phosphocreatine/ATP ratio (31P-MRS) content increased to 30% and 60% of control values, respectively. Brain GUA returned to normal levels.

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Summary:  Objective: We performed spike triggered functional MRI (fMRI) in a 12 year old girl with Benign Epilepsy with Centro-temporal Spikes (BECTS) and left-sided spikes. Our aim was to demonstrate the cerebral origin of her interictal spikes. Methods: EEG was recorded within the 3 Tesla MRI. Whole brain fMRI images were acquired, beginning 2–3 seconds after spikes. Baseline fMRI images were acquired when there were no spikes for 20 seconds. Image sets were compared with the Student's t-test. Results: Ten spike and 20 baseline brain volumes were analysed. Focal activiation was seen in the inferior left sensorimotor cortex near the face area. The anterior cingulate was more active during baseline than spikes. Conclusions: Left sided epileptiform activity in this patient with BECTS is associated with fMRI activation in the left face region of the somatosensory cortex, which would be consistent with the facial sensorimotor involvement in BECT seizures. The presence of BOLD signal change in other regions raises the possibility that the scalp recorded field of this patient with BECTs may reflect electrical change in more than one brain region.

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Purpose: This study used magnetic resonance spectroscopy (MRS) to examine metabolite abnormalities in the temporal and frontal lobe of patients with temporal lobe epilepsy (TLE) of differing severity. Methods: We investigated myoinositol in TLE by using short-echo MRS in 34 TLE patients [26 late onset (LO-TLE), eight hippocampal sclerosis (HS-TLE)], and 16 controls. Single-voxel short-echo (35 ms) MR spectra of temporal and frontal lobes were acquired at 1.5 T and analyzed by using LCModel. Results: The temporal lobe ipsilateral to seizure origin in HS-TLE, but not LO-TLE, had reduced N-acetylaspartate (NA) and elevated myoinositol (MI; HS-TLE NA, 7.8 ± 1.9 mM, control NA, 9.2 ± 1.3 mM; p < 0.05; HS-TLE MI, 6.1 ± 1.6 mM, control mI 4.9 ± 0.8 mM, p< 0.05). Frontal lobe MI was low in both patient groups (LO-TLE, 4.3 ± 0.8 mM; p < 0.05; HS-TLE, 3.6 ±.05 mM; p < 0.001; controls, 4.8 ± 0.5 mM). Ipsilateral frontal lobes had lower MI (3.8 ± 0.7 mM; p < 0.01) than contralateral frontal lobes (4.3 ± 0.8 mM; p < 0.05). Conclusions: MI changes may distinguish between the seizure focus, where MI is increased, and areas of seizure spread where MI is decreased.

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A total histological grade does not necessarily distinguish between different manifestations of cartilage damage or degeneration. An accurate and reliable histological assessment method is required to separate normal and pathological tissue within a joint during treatment of degenerative joint conditions and to sub-classify the latter in meaningful ways. The Modified Mankin method may be adaptable for this purpose. We investigated how much detail may be lost by assigning one composite score/grade to represent different degenerative components of the osteoarthritic condition. We used four ovine injury models (sham surgery, anterior cruciate ligament/medial collateral ligament instability, simulated anatomic anterior cruciate ligament reconstruction and meniscal removal) to induce different degrees and potentially 'types' (mechanisms) of osteoarthritis. Articular cartilage was systematically harvested, prepared for histological examination and graded in a blinded fashion using a Modified Mankin grading method. Results showed that the possible permutations of cartilage damage were significant and far more varied than the current intended use that histological grading systems allow. Of 1352 cartilage specimens graded, 234 different manifestations of potential histological damage were observed across 23 potential individual grades of the Modified Mankin grading method. The results presented here show that current composite histological grading may contain additional information that could potentially discern different stages or mechanisms of cartilage damage and degeneration in a sheep model. This approach may be applicable to other grading systems.

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Background Type 1 Neurofibromatosis (NF1) is a genetic disorder linked to mutations of the NF1 gene. Clinical symptoms are varied, but hallmark features of the disease include skin pigmentation anomalies (café au lait macules, skinfold freckling) and dermal neurofibromas. Method These dermal manifestations of NF1 have previously been reported in a mouse model where Nf1+/− mice are topically treated with dimethylbenz[a]anthracene (DMBA) and 12-O-tetradecanoylphorbol-13-acetate (TPA). We adopted this mouse model to test the protective effects of a nitroxide antioxidant, 5-carboxy-1,1,3,3-tetramethylisoindolin-2-yloxyl (CTMIO). Antioxidants have previously been shown to increase longevity in nf1-deficient fruitflies. Doses of 4 μM and 40 μM CTMIO provided ad libitum in drinking water were given to Nf1-deficient mice. Results Consistent with previous reports, Nf1-deficient mice showed a 4.7-fold increase in papilloma formation (P < 0.036). However, neither dose of CTMIO had any significant affect on papilloma formation. A non-significant decrease in skin pigmentation abnormalities was seen with 4 μM but not 40 μM CTMIO. Subsequent analysis of genomic DNA isolated from papillomas indicated that DMBA/TPA induced tumors did not exhibit a local loss of heterozygosity (LOH) at the Nf1 locus. Conclusion These data reveal that oral antioxidant therapy with CTMIO does not reduce tumor formation in a multistage cancer model, but also that this model does not feature LOH for Nf1.

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Description ‘The second volume of the Handbook on the Knowledge Economy is a worthy companion to the highly successful original volume published in 2005, extending its theoretical depth and developing its coverage. Together the two volumes provide the single best work and reference point for knowledge economy studies. The second volume with fifteen original essays by renowned scholars in the field, provides insightful and robust analyses of the development potential of the knowledge economy in all its aspects, forms and manifestations.’ – Michael A. Peters, University of Illinois, US

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Abstract: This study explores the contradictions and ambivalences experienced by a working artist at a time when her age, her gender, and broader cultural shifts are all potential obstacles or liabilities to creative flourishing. It is the product of practice-led research into the creative process from the perspective of the female "late bloomer". In this phrase, I have in mind the mature-aged woman who is, in mid-life, suddenly seized with inspiration and fired with creative energy. At its heart is the question: If an Elizabeth Jolley were in our midst today, would we hear from her? The result is a full-length libretto and accompanying exegetical binoculars in the form of a Preface and an Afterword. The creative work, Things That Fall Over (TTFO) is conceived in two parts: a libretto and oratorio for performance. It begins as a play, but over three acts and into a coda, the work becomes something entirely other - an (anti-) musical. The work grew from a personal interest in the nexus between women, ageing and creative practice, via investigation into the oeuvre of two Australian artists, Elizabeth Jolley, author, first published at age 53, and Rosalie Gascoigne, sculptor, first exhibited at 58. A second strand of the research grew from a fascination for the stage musical, especially in its more alternative modes as in the hands of Stephen Sondheim, or in more provocative manifestations as witnessed in recent Tony Award winners Avenue Q and The Book of Mormon. Contextually, this research is conducted at a time when anecdotal evidence suggests that women’s work in the performing arts and in literature is being pushed to the margins after a late twentieth century Golden Age on page and stage. Using hybrid practice-led methodologies - bricolage, log-keeping - and working within queer and feminist paradigms, this study seeks to counter that push with a new work that is all-female, part-pantomime, part monstrous allegory. In illuminating the creative process of a mature-aged playwright it concludes that hybrid and interstitial forms still offer an inclusive and democratic space in which voices that may otherwise be muted will continue to be heard.

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Drawing on the work of Ian Hunter the authors argue that literary education continues a tradition of circularity of argument derived from the humanities. They propose that the school subject, English in all of its apparently different historical manifestations focuses on the ideals of self-discovery and freedom of expression through literary study. The idea that literary interpretation or the production of specific readings is a skill that is taught in English classrooms challenges traditional understandings of literary study as a means for uncovering or revealing that which is hidden – be it the secrets of the text (or society or culture) or the secrets of the self – in order to come to a fuller realisation of culture and the self. Using examples from their previous work in developing activities for use with students in English classrooms the authors explore what it means to produce one’s ‘own reading’ of a text.

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Binge-like patterns of excessive drinking during young adulthood increase the propensity for alcohol use disorders (AUDs) later in adult life; however, the mechanisms that drive this are not completely understood. Previous studies showed that the δ-opioid peptide receptor (DOP-R) is dynamically regulated by exposure to ethanol and that the DOP-R plays a role in ethanol-mediated behaviors. The aim of this study was to determine the role of the DOP-R in high ethanol consumption from young adulthood through to late adulthood by measuring DOP-R-mediated [(35)S]GTPγS binding in brain membranes and DOP-R-mediated analgesia using a rat model of high ethanol consumption in Long Evans rats. We show that DOP-R activity in the dorsal striatum and DOP-R-mediated analgesia changes during development, being highest during early adulthood and reduced in late adulthood. Intermittent access to ethanol but not continuous ethanol or water from young adulthood leads to an increase in DOP-R activity in the dorsal striatum and DOP-R-mediated analgesia into late adulthood. Multiple microinfusions of naltrindole into the dorsal striatum or multiple systemic administration of naltrindole reduces ethanol consumption, and following termination of treatment, DOP-R activity in the dorsal striatum is attenuated. These findings suggest that DOP-R activity in the dorsal striatum plays a role in high levels of ethanol consumption and suggest that targeting the DOP-R is an alternative strategy for the treatment of AUDs.

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Objective: To assess the relationship between Bayesian MUNE and histological motor neuron counts in wild-type mice and in an animal model of ALS. Methods: We performed Bayesian MUNE paired with histological counts of motor neurons in the lumbar spinal cord of wild-type mice and transgenic SOD1 G93A mice that show progressive weakness over time. We evaluated the number of acetylcholine endplates that were innervated by a presynaptic nerve. Results: In wild-type mice, the motor unit number in the gastrocnemius muscle estimated by Bayesian MUNE was approximately half the number of motor neurons in the region of the spinal cord that contains the cell bodies of the motor neurons supplying the hindlimb crural flexor muscles. In SOD1 G93A mice, motor neuron numbers declined over time. This was associated with motor endplate denervation at the end-stage of disease. Conclusion: The number of motor neurons in the spinal cord of wild-type mice is proportional to the number of motor units estimated by Bayesian MUNE. In SOD1 G93A mice, there is a lower number of estimated motor units compared to the number of spinal cord motor neurons at the end-stage of disease, and this is associated with disruption of the neuromuscular junction. Significance: Our finding that the Bayesian MUNE method gives estimates of motor unit numbers that are proportional to the numbers of motor neurons in the spinal cord supports the clinical use of Bayesian MUNE in monitoring motor unit loss in ALS patients. © 2012 International Federation of Clinical Neurophysiology.

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To develop a rapid optimized technique of wide-field imaging of the human corneal subbasal nerve plexus. A dynamic fixation target was developed and, coupled with semiautomated tiling software, a rapid method of capturing and montaging multiple corneal confocal microscopy images was created. To illustrate the utility of this technique, wide-field maps of the subbasal nerve plexus were produced in 2 participants with diabetes, 1 with and 1 without neuropathy. The technique produced montages of the central 3 mm of the subbasal corneal nerve plexus. The maps seem to show a general reduction in the number of nerve fibers and branches in the diabetic participant with neuropathy compared with the individual without neuropathy. This novel technique will allow more routine and widespread use of subbasal nerve plexus mapping in clinical and research situations. The significant reduction in the time to image the corneal subbasal nerve plexus should expedite studies of larger groups of diabetic patients and those with other conditions affecting nerve fibers. The inferior whorl and the surrounding areas may show the greatest loss of nerve fibers in individuals with diabetic neuropathy, but this should be further investigated in a larger cohort.