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Mechanical characterisation of metallic materials at intermediate strain rates is essential to calibrate and validate computational models for industrial applications such as high-speed forming processes i.e. hammer forging, blanking, forming, etc. The most common devices that perform medium to high loading rate experiments are the servo-hydraulic universal testing machines and Split Hopkinson bar systems. Here we analyse the possibility of employing an in-house designed and constructed DirectImpact Drop Hammer (DIDH) for material mechanical characterisation at medium strain rates, ranging from 100 to 300 s-1. To show the suitability of the DIDH for mechanical characterisation, uniaxial compression experiments on S235JR structural steel are conducted and compared with finite element (FE) simulations performed with an elasticthermoviscoplastic material model previously calibrated with Split Hopkinson Pressure Bar (SHPB) tests.

A case of infantile metachromatic leucodystrophy is described in which symptoms started at 1 year of age with weakness and hypotonus in the lower extremities. The electrophysiological status was typical of a polyneuropathy, showing fibrillation and a reduction of the nerve conduction velocity to 30 percent of the average for normal children of the same age. Clinical signs of a central lesion and mental regression were not evident until a year later. Nerve biopsy showed metachromatic granules in the phagocytes and in the Schwann cells, confirming the diagnosis of metachromatic leucodystrophy. In peripheral neuropathy in infancy without obvious cause, a nerve biopsy is the most appropriate method for diagnosis of the metachromatic leucodystrophy. [ABSTRACT FROM AUTHOR]

Time-of-Flight Diffraction (TOFD) is a method of ultrasonic testing (UT) that is widely established as a non-destructive technique (NDT) mainly used for the inspection of welds. In contrast to other established UT techniques, TOFD is capable of identifying discontinuities regardless of their orientation. This paper proposes a redesign of the typical TOFD transducers, featuring an innovative curved sole aimed at enhancing their defect detection capabilities. This design is particularly beneficial for thick-walled samples, as it allows for deeper inspections without compromising the resolution near the surface area. During this research, an evaluation consisting in simulations of the ultrasonic beam distribution and experimental tests on a component with artificially manufactured defects at varying depths has been performed to validate the new design. The results demonstrate a 30 to 50% higher beam distribution area as well as an improvement in the signal-to-noise ratio (SNR) resulting in a 24% enhancement in the capability of defect detection compared to the traditional approach.

The final quality of sheet and tube metal-formed components strongly depends on the tribology and friction conditions between the tools and the material to be formed. Furthermore, it has been recently demonstrated that friction is the numerical input parameter that has the biggest effect in the numerical models used for feasibility studies and process design. For these reasons, industrial dedicated software packages have introduced friction laws which are dependent on sliding velocity, contact pressure and sometimes strain suffered by the sheet, and currently, temperature dependency is being implemented as it has also a major effect on friction. In this work, three lubricants having different viscosity have been characterized using the tube-sliding test. The final aim of the study is to fit friction laws that are contact pressure and sliding velocity dependent for their use in tube hydroforming modeling. The tests performed at various contact pressures and velocities have demonstrated that viscosity has a major effect on friction. Experimental hydroforming tests using the three different lubricants have corroborated the importance of the lubricant in the final forming of a triangular shape. The measurement of the axial forces and the final principal strains of the formed tubes have shown the importance of using advanced friction laws to properly model the hydroforming process using the finite element modeling.

Today's stamping simulations are realized by ignoring the elastic deformation of the press and tooling system through the assumption of a rigid behavior and a perfect press stroke. However, in reality, the press and tool components deform elastically and are one of the major error sources for the final adjustment and blue-spotting of the dies. In order to tackle this issue, a new approach is proposed in this study that substitutes the press stiffness by means of a substitutive model composed of cost-effective shell and beam elements. The substitute model was calibrated using full-scale measurements, in which a 20,000 kN trial press was experimentally characterized by measuring its deformation under static loads. To examine the robustness of the substitute model, a medium-size tool and a large-size tool were simulated together with the substitutive model. To this end, a B-pillar tool was re-machined based on the substitute-model results and a new cambering procedure was proposed and validated throughout the blue-painting procedure. The newly developed substitute model was able to replicate the global stiffness of the press with a high accuracy and affordable calculation time. The implementation of the findings can aid toolmakers in eliminating most of the reworking and home-line trials.

Congenital tremor type A-II in piglets has been regarded as a transmissible disease since the 1970s, possibly caused by a very recently-described virus: atypical porcine pestivirus (APPV). Here, we describe several strains of APPV in piglets with clinical signs of congenital tremor (10 of 10 farms tested). Piglets on a farm with no history of congenital tremor were PCR-negative for the virus. To demonstrate a causal relationship between APPV and disease, three gilts were inoculated via intramuscular injection at day 32 of pregnancy. In two of the three litters, vertical transmission of the virus occurred. Clinical signs of congenital tremor were observed in APPV-infected newborns, yet also two asymptomatic carriers were among the offspring. Piglets of one litter were PCR-negative for the virus, and these piglets were all without congenital tremors. Long-term follow up of farm piglets born with congenital tremors showed that the initially high viremia in serum declines at five months of age, but shedding of the virus in feces continues, which explains why the virus remains present at affected farms and causes new outbreaks. We conclude that trans-placental transmission of APPV and subsequent infection of the fetuses is a very likely cause of congenital tremor type A-II in piglets., Competing Interests: The authors of this manuscript have the following competing interests: A.d.G., L.G., L.v.G., W.V., C.D., T.C., V.G., M.V., J.S., P.v.D. and C.S. are or have been employed at MSD Animal Health, a commercial company. A.d.G., M.D., L.v.d.H., L.G. and C.S. are inventors on a patent application on APPV. L.v.d.H. is a consultant for MSD Animal Health.

Introduction: The diagnosis of patients with cognitive deterioration or dementia requires a global approach in which the neuropsychological examination is a key piece. As part of the GERMCIDE study (Group for the Study and Multicenter Registry of Incident Cases of Dementia in Spain), a protocol was designed that included an assessment of the different cognitive functions that are most frequently altered in dementias (memory, orientation, speech, praxis, abstraction capacity and executive function)., Methods: In order to obtain data in normal subjects, this neuropsychological protocol was applied to a group of persons over 50 years without cognitive deterioration or dementia., Results: A total of 103 subjects whose ages ranged from 50 to 95 years (mean: 73.5; SD: 9.3 years); 39 (37.9%) men and 64 (62.1%) women were studied. The mean score on the Mini-Mental State Examination (MMSE) was 27/30 (SD: 2.0). In the speech and praxis tests, 90% of the subjects obtained the maximum value, while performances were more unequal in memory, reasoning and programming. Mean score, standard deviation and distribution in percentages for each subtest are presented., Conclusions: The values obtained in this sample of normal subjects and their distribution in percentages may be very helpful to facilitate the interpretation of the findings of the neuropsychological examination with the GERMCIDE protocol in the general neurology clinic visits and also in the specialized visits in dementia.

In 1995, a surveillance system for prion diseases was set up in the Basque Country, an autonomous region in northern Spain (2.1 million inhabitants). In the period from January 1993 to December 2003, we diagnosed 21 patients with familial prion diseases prospectively and another 4 patients retrospectively. They represent 35% of all the cases referred to the epidemiological registry. Two main possible explanations for this unusual high incidence of familial prion diseases are proposed: first, comprehensive case ascertainment by public health neurologists; second, a probable cluster of the D178N mutation within families of Basque origin related to a still unconfirmed common ancestor. Further genetic and genealogical studies should resolve this issue.

Although there is considerable evidence implicating apolipoprotein E (ApoE) epsilon4 in the development of the Alzheimer's disease (AD), additional factors are also known to be involved. Thus, an association has been described between C267T polymorphism of the 5-hydroxytryptamine 6 receptor (5-HT(6)) receptor gene and AD. This case-control study analyzes the ApoE and 5-HT(6) receptor polymorphisms in 173 cases and 102 age and sex matched controls from Araba and Bizkaia (The Basque Country, Spain). The analysis of ApoE showed the frequencies of epsilon4 allele to be significantly higher in AD patients (0.292) than in the controls (0.083). When 5-HT(6) receptor polymorphism was analyzed, a greater frequency of 267C allele was observed in AD patients than in controls, though the difference was not statistically significant. Likewise regarding ApoE epsilon4 status, no statistically significant difference was observed. In conclusion, the association of ApoE epsilon4 to AD in a sample of patients from the Basque Country is confirmed, though the association to C267T polymorphism of the 5-HT(6) receptor has not been observed.