310 resultados para joint hypermobility syndrome


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Background: From a young age the typical development of social functioning relies upon the allocation of attention to socially relevant information, which in turn allows experience at processing such information and thus enhances social cognition. As such, research has attempted to identify the developmental processes that are derailed in some neuro-developmental disorders that impact upon social functioning. Williams syndrome (WS) and Autism are disorders of development that are characterized by atypical yet divergent social phenotypes and atypicalities of attention to people.

Methods: We used eye tracking to explore how individuals with WS and Autism attended to, and subsequently interpreted, an actor’s eye gaze cue within a social scene. Images were presented for three seconds, initially with an instruction simply to look at the picture. The images were then shown again, with the participant asked to identify the object being looked at. Allocation of eye-gaze in each condition was analyzed by ANOVA and accuracy of identification was compared with t-tests.

Results: Participants with WS allocated more gaze time to face and eyes than their matched controls both with and without being asked to identify the item being looked at; while participants with Autism spent less time on face and eyes in both conditions. When cued to follow gaze, participants with WS increased gaze to the correct targets, while those with Autism looked more at the face and eyes but did not increase gaze to the correct targets, while continuing to look much more than their controls at implausible targets. Both groups identified fewer objects than their controls.

Conclusions: The atypicalities found are likely to be entwined with the deficits shown in interpreting social cognitive cues from the images. WS and Autism are characterised by atypicalities of social attention that impact upon socio-cognitive expertise but importantly the type of atypicality is syndrome-specific.

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Eighteen adolescents who had survived Reye syndrome (RS) in early childhood were assessed on cognitive, emotional, and behavioural variables in a second follow-up study tracking this group. Siblings were used as controls. The entire group with RS had survived with no obvious neurological damage at the first follow-up study. Indeed, current findings suggested that long-term cognitive, emotional, and behavioural functioning was comparable to siblings in approximately half of the group with RS. However, two factors were associated with a less favourable outcome. Cognitive, emotional, and behavioural functioning were significantly poorer in the subgroup of survivors whose illness had occurred in the first year of life. In addition, loss of consciousness, although the association with poor outcome was not as noticeable, was also associated with relative deficits on some scales of cognitive ability. Many of these deficits had not been obvious at the first follow-up and the importance of neurodevelopmental factors are considered. Finally, the implications of these findings for research and interventions in RS and other such encephalopathies are discussed.

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Objective: To describe plateau iris syndrome associated with multiple neuroepithelial cysts of the pars plicata. Methods: Case reports of 3 patients with plateau iris syndrome who were found to have multiple bilateral ciliary body cysts on ultrasound biomicroscopic examination. Results: Ultrasound biomicroscopy revealed classic features of plateau iris syndrome in each patient but also showed multiple neuroepithelial cysts of the ciliary body in each eye. Conclusion: Plateau iris syndrome may be associated with multiple ciliary body cysts.

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We describe a patient with Chandler's syndrome variant of the iridocorneal endothelial syndrome in whom ectopic Descemet's membrane was found intraoperatively on the anterior surface of the lens. Initially, the membrane was confused with the anterior lens capsule during extracapsular cataract extraction, leading to the performance of a pseudocapsulorrhexis. Electron microscopy disclosed that the epilenticular membrane was composed of multiple layers of abnormal basement membrane consistent with the iridocorneal endothelial syndrome.

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We consider a multiple femtocell deployment in a small area which shares spectrum with the underlaid macrocell. We design a joint energy and radio spectrum scheme which aims not only for co-existence with the macrocell, but also for an energy-efficient implementation of the multi-femtocells. Particularly, aggregate energy usage on dense femtocell channels is formulated taking into account the cost of both the spectrum and energy usage. We investigate an energy-and-spectral efficient approach to balance between the two costs by varying the number of active sub-channels and their energy. The proposed scheme is addressed by deriving closed-form expressions for the interference towards the macrocell and the outage capacity. Analytically, discrete regions under which the most promising outage capacity is achieved by the same size of active sub-channels are introduced. Through a joint optimization of the sub-channels and their energy, properties can be found for the maximum outage capacity under realistic constraints. Using asymptotic and numerical analysis, it can be noticed that in a dense femtocell deployment, the optimum utilization of the energy and the spectrum to maximize the outage capacity converges towards a round-robin scheduling approach for a very small outage threshold. This is the inverse of the traditional greedy approach. © 2012 IEEE.