LONG-LASTING CRANIAL NERVE III PALSY AS A PRESENTING FEATURE OF CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY

We describe a patient with chronic inflammatory demyelinating polyneuropathy (CIDP) in which an adduction deficit and ptosis in the left eye presented several years before the polyneuropathy. A 52-year-old man presented with a 14-year history of unremitting diplopia, adduction deficit, and ptosis in the left eye. At the age of 45 a mild bilateral foot drop and impaired sensation in the four limbs appeared, with these symptoms showing a progressive course. The diagnostic workup included EMG/ENG which demonstrated reduced conduction velocity with bilateral and symmetrical sensory and motor involvement. Cerebrospinal fluid studies revealed a cytoalbuminologic dissociation.Aprolonged treatment with corticosteroids allowed a significant improvement of the limbweakness. Diplopia and ptosis remained unchanged.This unusual formof CIDP presented as a long-lasting isolated cranial nerve palsy. A diagnostic workup for CIDP should therefore be performed in those patients in which an isolated and unremitting cranial nerve palsy cannot be explained by common causes

The ubiquitin-proteasome pathway in cancer.

Degradation by the 26S proteasome of specific proteins that have been targeted by the ubiquitin pathway is the major intracellular non-lysosomal proteolytic mechanism and is involved in a broad range of processes, such as cell cycle progression, antigen presentation and control of gene expression. Recent work, reviewed here, has shown that this pathway is often the target of cancer-related deregulation and can underlie processes, such as oncogenic transformation, tumour progression, escape from immune surveillance and drug resistance

TRACHEOSTOMY MECHANICAL VENTILATION IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS: CLINICAL FEATURES AND SURVIVAL ANALYSIS.

Background: Tracheostomy mechanical ventilation (TMV) is performed in amyotrophic lateral sclerosis (ALS) patients with a respiratory failure or when the non-invasive ventilation (NIV) is no longer effective. We evaluated the clinical characteristics and survival of a cohort of tracheostomized ALS patients, followed in a single ALS Clinical Center. Methods: Between 2001 and 2010, 87 out of 279 ALS patients were submitted to TMV. Onset was spinal in 62 and bulbar in 25. After tracheostomy, most patients were followed up through telephone interviews to caregivers. A complete survival analysis could be performed in fifty-two TMV patients. Results: 31.3% ALS patients underwent tracheostomy, with a male prevalence (M/F=1.69) and a median age of 61 years (interquartile range=47–66). After tracheostomy, nearly all patients were under home care. TMV ALS patients were more likely than non-tracheostomized (NT) patients to be implanted with a PEG device, although the bulbar-/spinal-onset ratio did not differ between the two groups. Kaplan–Meyer analysis showed that tracheostomy increases median survival (TMV, 47 months vs NT, 31 months, p=0.008), with the greatest effect in patients younger than 60 at onset (TMV≤60 years, 57.5 months vs NT≤60 years, 38.5 months, p=0.002). Conclusions: TMV is increasingly performed in ALS patients. Nearly all TMV patients live at home and most of them are fed through a PEG device. Survival after tracheostomy is generally increased, with the stronger effect in patients younger than 60. This survival advantage is apparently lost when TMV is performed in patients older than 60. The results of this study might be useful for the decision-making process of patients and their families about this advanced palliative care

Materials and technological processes for High-Gradient accelerating structures: new results from mechanical tests of an innovative braze-free cavity

Pure oxygen-free high-conductivity copper is a widely used material for manufacturing accelerating cavities working at room temperature. Several studies attempted to explain limitations associated with the maximum allowed field gradients and the behaviour of vacuum RF breakdown in copper accelerating structures through generation and movement of dislocations under stresses associated with RF electric and magnetic fields. Pure copper and also copper alloys undergo mechanical and thermal treatments to be hardened and strengthened during manufacturing, although their mechanical properties significantly change after heating above 590ˆC. High temperature brazing and diffusion bonding are assembly methods widely used to manufacture ultra-high vacuum accelerating devices. However, these processes, occurring at about 800-1000ˆC, significantly affect the mechanical properties of copper and copper alloys. We present here a novel Tungsten Inert Gas welding procedure, which is fast and keeps the high-gradient surfaces of the cavity and other components well below the copper annealing temperature. This process may be successfully used to manufacture copper-based accelerating components. This technology preserves the hardness and cleanliness of copper in order to achieve the maximum accelerating gradient

Long-lasting positive effects of collaborative remembering on false assents to misleading questions

Previous studies showed that collaborative remembering can reduce false memories through a process of mutual error checking, although conclusions were limited by the nature of the memory tasks (very few errors). The present experiments extend these findings to eyewitness memory by using a paradigm designed to increase the frequency of memory errors. Collaborative and nominal pairs viewed a video-clip illustrating a bank robbery, provided an immediate free recall, were forced to confabulate answers to false-event questions, and, after a short- (1 h: Experiment 1) or a long-term delay (1 week: Experiment 2), were administered a yes/no recognition task in which the misleading statements either matched the questions presented in the confabulation phase (answered questions) or not (control questions). Collaborative pairs recalled fewer correct details in the immediate free recall task, replicating the negative effects of collaborative inhibition. Most importantly, in the final recognition test, collaborative pairs were less likely to provide false assents to misleading statements, regardless of whether they had provided a response to the related false-event questions 1 h or 1 week earlier. Our results suggest that collaboration can increase the eyewitnesses' tendency to check the accuracy of others' responses and reject false memories through discussion

From gridmap-file to VOMS: managing authorization in a Grid environment

Grids are potentially composed of several thousands of users from different institutions sharing their computing resources (or using resources provided by third parties). Controlling access to these resources is a difficult problem, as it depends on the policies of the organizations the users belong to and of the resource owners. Moreover, a simple authorization implementation, based on a direct user registration on the resources, is not applicable to a large scale environment. In this paper, we describe the solution to this problem developed in the framework of the European DataGrid [M. Draoli, G. Mascari, R. Piccinelli, Project Presentation, DataGrid-11-NOT-0103-_1] and DataTAG [http://www.datatag.org/] projects: the Virtual Organization Membership Service (VOMS) [R. Alfieri, et al., Managing Dynamic User Communities in a Grid of Autonomous Resources, TUBT005, in: Proceedings of the CHEP 2003, 2003]. VOMS allows a fine grained control of the use of the resources both to the users' organizations and to the resource owners

Molybdenum sputtering film characterization for high gradient accelerating structures

Technological advancements are strongly required to fulfill the demands of new accelerator devices with the highest accelerating gradients and operation reliability for the future colliders. To this purpose an extensive R&D regarding molybdenum coatings on copper is in progress. In this contribution we describe chemical composition, deposition quality and resistivity properties of different molybdenum coatings obtained via sputtering. The deposited films are thick metallic disorder layers with different resistivity values above and below the molibdenum dioxide reference value. Chemical and electrical properties of these sputtered coatings have been characterized by Rutherford backscattering, XANES and photoemission spectroscopy. We will also present a three cells standing wave section coated by a molybdenum layer $\sim$ 500 nm thick designed to improve the performance of X-Band accelerating systems.Comment: manuscript has been submitted and accepted by Chinese Physics C (2012

Complete locked-in and locked-in patients: Command following assessment and communication with vibro-tactile P300 and motor imagery brain-computer interface tools

Many patients with locked-in syndrome (LIS) or complete locked-in syndrome (CLIS) also need brain-computer interface (BCI) platforms that do not rely on visual stimuli and are easy to use. We investigate command following and communication functions of mindBEAGLE with 9 LIS, 3 CLIS patients and three healthy controls. This tests were done with vibro-tactile stimulation with 2 or 3 stimulators (VT2 and VT3 mode) and with motor imagery (MI) paradigms. In VT2 the stimulators are fixed on the left and right wrist and the participant has the task to count the stimuli on the target hand in order to elicit a P300 response. In VT3 mode an additional stimulator is placed as a distractor on the shoulder and the participant is counting stimuli either on the right or left hand. In motor imagery mode the participant is instructed to imagine left or right hand movement. VT3 and MI also allow the participant to answer yes and no questions. Healthy controls achieved a mean assessment accuracy of 100% in VT2, 93% in VT3, and 73% in MI modes. They were able to communicate with VT3 (86.7%) and MI (83.3%) after 2 training runs. The patients achieved a mean accuracy of 76.6% in VT2, 63.1% in VT3, and 58.2% in MI modes after 1-2 training runs. 9 out of 12 LIS patients could communicate by using the vibro-tactile P300 paradigms (answered on average 8 out of 10 questions correctly) and 3 out of 12 could communicate with the motor imagery paradigm(answered correctly 4,7 out of 5 questions). 2 out of the 3 CLIS patients could use the system to communicate with VT3 (90 and 70% accuracy). The results show that paradigms based on non-visual evoked potentials and motor imagery can be effective for these users. It is also the first study that showed EEG-based BCI communication with CLIS patients and was able to bring 9 out of 12 patients to communicate with higher accuracies than reported before. More importantly this was achieved within less than 15-20 min

Predicting the impact of lava flows at Mount Etna (Italy)

Forecasting the time, nature and impact of future eruptions is difficult at volcanoes such as Mount Etna, in Italy, where eruptions occur from the summit and on the flanks, affecting areas distant from each other. Nonetheless, the identification and quantification of areas at risk from new eruptions is fundamental for mitigating potential human casualties and material damage. Here, we present new results from the application of a methodology to define flexible high-resolution lava invasion susceptibility maps based on a reliable computational model for simulating lava flows at Etna and on a validation procedure for assessing the correctness of susceptibility mapping in the study area. Furthermore, specific scenarios can be extracted at any time from the simulation database, for land-use and civil defence planning in the long-term, to quantify, in real-time, the impact of an imminent eruption, and to assess the efficiency of protective measures