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Background: There is currently no accepted classification of recessive cerebellar ataxias, a group of disorders characterized by important genetic heterogeneity and complex phenotypes. The objective of this task force was to build a consensus and develop a clinical and pathophysiological classification for recessive ataxias. Methods: The work of this task force was based on a scoping systematic review of the literature that identified recessive disorders characterized primarily by a cerebellar motor syndrome and cerebellar degeneration. The task force regrouped 12 international ataxia experts who decided on general orientation and specific issues. Results: We identified 59 disorders that are classified as primary recessive ataxias. For each of these disorders, we present geographical and ethnical specificities along with distinctive clinical and imagery features. The primary recessive ataxias were organized in a clinical and a pathophysiological classification, and we present a general clinical approach to the patient presenting with ataxia. We also identified a list of 48 complex multisystem disorders in which ataxia is a secondary feature. Conclusions: This classification is based on a scoping systematic review of the literature and results from a sconsensus among a panel of international experts. It promotes a unified understanding of recessive cerebellar disorders for clinicians and researchers.
v sin i has been measured from high-resolution (λ/Δλ~50000) infrared spectra of ~30 M and L dwarfs. The spectral region observed covers approximately 0.01 μm centered at 2.312 μm in the CO 2-0 band. v sin i, measured from the CO lines, increases dramatically from the M to L dwarfs. In extreme cases the rotational periods are a few hours.
Organic thin-film transistors (OTFTs) are the most promising candidates for flexible electronics owing to their flexible structures, the simplicity of processing large-area devices, and excellent compatibility with flexible substrates. To date, many studies have been reported that have aimed at developing a wide range of plastic electronics such as flexible displays, sensors. In this paper, we discuss our recent work, focusing on OTFT arrays and their application to flexible display. An active-matrix (AM) backplane using a low-temperature cross-linkable olefin-type polymer as the gatedielectric and an air-stable DNTT as the organic semiconductor (OSC) was successfully fabricated on a plastic substrate. The short-channel TFT array exhibited a high hole mobility of over 0.5 cm2/Vs, a low subthreshold slope of 0.31, and excellent environmental and operational stability. A 5-inch flexible OLED display exhibited a high luminescence of over 300 cd/m2 by driving of the DNTT-based OTFTs. Solution-processed OTFTs are also attracting considerable attention owing to both their simple manufacturing process and excellent transistor performance. We present a simple patterning process for a solution-processable OSC that can be used to develop a high-mobility short-channel TFT array. The OSC film was directly patterned on the confined active channel region by a simple lamination coating technique and the resulting TFTs showed a high mobility of up to 1.3 cm2/Vs. In the final section, we report on eco-friendly paper-based organic TFT array. A transparent cellulose nanofibers paper was firstly applied to a flexible substrate for the TFT backplane. A solution-processed TFT on the transparent paper exhibited a high mobility exceeding 1 cm2/Vs, good air stability, and excellent mechanical stability.
The gullet worm (Gongylonema pulchrum) has been recorded from a variety of mammals worldwide, including monkeys and humans. Due to its wide host range, it has been suggested that the worm may be transmitted locally to any mammalian host by chance. To investigate this notion, the ribosomal RNA gene (rDNA), mainly regions of the internal transcribed spacers (ITS) 1 and 2, and a cytochrome c oxidase subunit I (COI) region of mitochondrial DNA of G. pulchrum were characterized using parasites from the following hosts located in Japan: cattle, sika deer, wild boars, Japanese macaques, a feral Reeves's muntjac and captive squirrel monkeys. The rDNA nucleotide sequences of G. pulchrum were generally well conserved regardless of their host origin. However, a few insertions/deletions of nucleotides along with a few base substitutions in the ITS1 and ITS2 regions were observed in G. pulchrum from sika deer, wild boars and Japanese macaques, and those differed from G. pulchrum in cattle, the feral Reeves's muntjac and captive squirrel monkeys. The COI sequences of G. pulchrum were further divided into multiple haplotypes and two groups of haplotypes, i.e. those from a majority of sika deer, wild boars and Japanese macaques and those from cattle and zoo animals, were clearly differentiated. Our findings indicate that domestic and sylvatic transmission cycles of the gullet worm are currently present, at least in Japan.
To report a case of multifocal fibrosclerosis with a nine-year follow up, and to discuss this disease's radiological appearance and management. The disease is a rare systemic disorder of unknown cause characterised by fibrous proliferation involving multiple anatomical sites.
A 50-year-old woman presented with histological findings characterised by similar inflammatory processes involving the meninges, pituitary gland, peritoneum, retroperitoneum and orbits, prompting a search for a common pathophysiology. A diagnosis of multifocal fibrosclerosis was postulated. Symptom improvement was noted after treatment with prednisone and azathioprine.
This is the first documented case of involvement of the cochleovestibular nerve in a patient with multifocal fibrosclerosis. The rare association between fibrotic diseases and masses showing various clinical patterns should be kept in mind by otolaryngologists, and imaging performed to investigate for multifocal fibrosclerosis. However, diagnosis can only be confirmed with tissue biopsy and histopathological examination.
The silicon carbide thin film formation process, completely performed at room temperature, was developed by argon plasma and a chemical vapor deposition using monomethylsilane gas. Time-of-flight secondary ion mass spectrometry showed that siliconcarbon bonds existed in the obtained film, the surface of which could remain specular after the exposure to hydrogen chloride gas at 800 °C. The silicon dangling bonds formed at the silicon surface by the argon plasma are considered to easily accept the monomethylsilane molecules at room temperature to produce the amorphous silicon carbide film.
Effects of Al-concentration on growth of antiphase domains (APDs) in Ti3Al crystals have been investigated using crystals with stoichiometric (Ti-25at.%Al) and Al-rich (Ti-33at.%Al) compositions in the temperature range from 973K to 1173K. The growth rate of APDs in the Al-rich crystal is several times higher than that in the stoichiometric crystals at all the temperatures investigated. While the time dependence of APD size obeys the parabolic-growth-law in the stoichiometric crystal, negative deviations from the law takes place at the late stage of the APD growth in the Al-rich crystal owing to the pinning effect of low-energy APB boundaries. APD boundaries lying on prism planes are formed in the Al-rich crystal annealed at 973K.
The growth of the D03-type antiphase domain (APD) in Fe3Al was investigated focusing on the effect of excess vacancies that were introduced during the quenching process from the disordered state. The variation in the APD size exhibited considerable deviation from the conventional “parabolic growth law” in the early stage of APD growth. This variation was numerically calculated on the assumption that the migration of the APD boundaries was enhanced by non-equilibrium excess vacancies and the vacancy concentration decreased during the isothermal annealing for the APD growth. The calculated variations in the APD size could be successfully fitted to the experimental results in cases with quenching temperatures (Tq) of 873 K or 1073 K, but not when Tq was 1273 K. The APD growth in the latter case was much slower than the expected growth derived from the calculation. This discrepancy was attributed to the rapid decrease in the vacancy concentration due to vacancy clustering since a significant amount of dotted contrasts were observed in TEM image of only the specimen quenched from 1273K.
We studied antiphase domain (APD) growth and lamellar structure formation during isothermal annealing of Ti-39at%Al single crystals at α2+γ dual phase temperature after quenching from α single phase state, intending to obtain a APD/lamellae mixed microstructure and to examine whether such a microstructure provides a strength higher than that obtained only by refining lamellar structure. The effect of plastic deformation prior to the annealing was also examined expecting a acceleration of γ lamellae formation through a preferential nucleation of γ-plates at dislocations. The lower was the annealing temperature, the smaller both the APD size and the lamellar spacing at the moment of a homogeneous lamellar structure formation tended to be, although naturally both the APD growth and the γ lamellae formation were slower. However, the APD size in the homogeneous lamellar structure was no smaller than 400 nm. A structure with finer APDs and finer lamellar structure was obtained by deforming the crystal before annealing since the lamellar structure formation was accelerated and the time for APD growth before the lamellar structure formation was shortened. For instance, a structure with an average lamellar spacing of 88 nm and an average APD size of 214 nm was obtained by deforming the crystals to 10 % plastic strain and subsequently annealing at 1073 K for 1×104 s, while no γ plate was obtained only by such an annealing without deformation.
SiO2 films were prepared at a substrate temperature of 100°C by the simultaneous use of a microwave ion source and an ICB system. Transparent and good insulating SiO2 films could be obtained by using 02 gas ions, and they were thermally and chemically stable. Furthermore, both the ionization energy and the incident energy of the 02 gas ions were found to enhance the chemical reaction between SiO and 02 molecules, resulting in the Si02 film formation at a low substrate temperature.
Light-induced changes in current-voltage characteristics of amorphous single-junction solar cells made of silicon alloys, a-SiC, a-Si, and a-SiGe have been studied systematically. The effect of the light intensity and the bias voltage on the light-induced degradation in the conversion efficiency and other photovoltaic parameters has been clarified quantitatively, and it has been shown that the light-induced degradation characteristics of the photovoltaic parameters can be described by a single function of some normalized parameters of exposure condition. Theoretical background for the experimental results is also examined and discussed by applying a defect creation model modified to an active layer of the a-Si alloy solar cells. Utilizing these analytical formula, we propose an accelerated test method by solar simulator indoor measurement instead of natural sunlight outdoor testing.
The methanation of CO2 has been studied by using reduced Ni(II)-bearing ferrite (NixFe3-xO4-δ; x=0.39, δ=0.1∼0.2) in an H2/CO2 mixed gas flow system. A yield of 31% and a high selectivity of 89% were obtained in the methanation for 6h using the H2-reduced Ni(II)-bearing ferrite. The X-ray diffractometry showed that the reduced Ni(II)-bearing ferrite during the methanation retained the spinel-type structure whose lattice constant increased from 0.8375 to 0.8379 nm. The chemical analysis corroborated an increase in the mole ratio of Fe2+/Fetotal in the Ni(II)-bearing ferrite. These results suggest that oxygen-deficient sites were formed in the spinel structure of the reduced Ni(II)-bearing ferrite. It has been found from the analysis using the Langmuir isotherm for dissociative adsorption that CO2 is adsorbed onto oxygen-deficient sites to be decomposed to an elemental carbon and two oxygen-deficient ions. The methanation of CO2 is considered to proceed owing to the oxygen deficient sites. It is considered that the methanation consists of three elementary reaction steps: 1) Formation of oxygen-deficient sites by H2-reduction, 2) Reduction of CO2 to carbon and Incorporation of two oxygen ions of the CO2 into the oxygen-deficient sites, and 3) Hydrogenation of the deposited carbon to CH4.
Oxygen-deficient Zn(II)-bearing ferrites (ZnxFe3-xO4-δ, 0≤×≤l, δ>0) have been synthesized and studied for their reactivity in the decomposition of CO2 to carbon at 300 °C. They were prepared by reducing Zn(II)-bearing ferrites with H2 gas at 300 °C. The oxygen-deficient Zn(II)-bearing ferrites consisted of a single phase of a spinel-type structure which was oxygendeficient compared with their stoichiometric compositions. Their lattice constants were larger than those of the corresponding stoichiometric spinels. Decomposition of CO2 to carbon proceeded accompanied by an oxidation of the oxygen-deficient Zn(II)-bearing ferrite. The amount of carbon deposited on the ferrite decreased when Zn content in Zn(II)-bearing ferrite increased. The decrease in the amount of carbon deposited is due to changes in the electron conductivity according to the Zn content in Zn(II)-bearing ferrite. These changes contribute to their reactivity for decomposition of CO2 to carbon.
An oxygen-deficient magnetite (Fe3O4–8) has been found to form by passing H2 gas through magnetite powder at 300°C with its spinel structure retained. The oxygen-deficient magnetite is a metastable phase in the transformation of magnetite into α-Fe. The lattice constant of the oxygen-deficient magnetite enlarged to 0.8407nm in the maximum value which is substantially larger than that of the stoichiometric magnetite (a0=0.8396nm). The formation mechanism of the oxygen-deficient magnetite was studied at 300°C using two specimens of magnetite powders with developed orientations on the (111) and (100) planes. These are referred to as (111)- and (100)-magnetite, respectively. The formation of α-Fe was suppressed over the crystal of (111)-magnetite, where wUstite and oxygen-deficient magnetite were formed while keeping the f.c.c. arrangement of the oxygen ions in the solid. On the other hand, over the (100)-magnetite, the formation of a-Fe was enhanced. It is considered that, on the (111)- magnetite, the Fe2+ ion formed by H2-reduction on the surface can move into interstices of the f.c.c. lattice of bulk to form the oxygen deficient state. This will come from the fact that the Fe2+ ion on the surface is so strongly supported with three Fe-O bondings that the Fe2+ ions cannot readily diffuse on the surface when the surface oxygen ions are removed on the (111)- magnetite.
Effects of ruthenium (Ru) substitution on constituent phases, phase transformation temperatures and mechanical properties were investigated for Ti-Ni shape memory alloys. Ti50Ni50-XRuX alloys with Ru contents (X) from 0mol% (binary TiNi) to 50mol% (binary TiRu) were systematically prepared by Ar arc-melting followed by hot-forging at temperatures from 1173K to 1673K depending on chemical composition. Phase stability was assessed by DSC (differential scanning calorimetry), XRD (X-ray diffractometry) and TEM (transmission electron microscopy). Mechanical properties were investigated using hardness and tensile tests at room temperature. With increasing Ru content, it was found that the lattice parameter of B2 phase increases, the martensitic transformation temperature slightly decreases, and the melting temperature increases monotonously. Besides, R-phase appears for Ti-Ni alloys containing 3mol% and 20mol%Ru but no diffusionless phase transformation is seen in Ti-Ni alloy containing 5mol%Ru. Vickers hardness shows the maximum at an intermediate composition (HV1030 at 30mol%Ru); this suggests that large solid solution hardening is caused by Ru substitution for the Ni-sites in TiNi.
Effect of antiphase domain boundaries (APDBs) on yielding and dislocation structure were investigated in Ti3Al single crystals oriented for prism slip. The yield stress greatly depended on the size of antiphase domains (APDs). The yield stress of Ti3Al with the average APD size of 35nm was about six times higher than that of Ti3Al without APDB. Single dislocations (isolated superpartial dislocations) were observed in the deformed Ti3Al single crystal with APD sizes smaller than 100nm, while superdislocation pairs were observed in those with larger APDs. The mechanism of the interaction between the prism dislocations and APDBs is discussed.
Since the maximum shape recovery temperature of the binary Ti-Ni alloys is limited to be around 400K, the increase in martensitic transformation temperature (Ms) of Ti-Ni should be done by alloying for the demand of high temperature applications. Although most of additional elements are known to decrease Ms of Ti-Ni, substitutional elements having large atomic size are expected to increase Ms. In this study, phase constitution, phase transformation temperature, lattice parameter of B2 phase and Vickers hardness were investigated for Ti-Ni alloys containing several platinum-group metals (PGM). The alloy systems investigated were the pseudobinary systems of TiNi-TiRh, TiNi-TiIr and TiNi-TiPt where the PGM atoms substitute for the Ni-sites of TiNi. The phase transformation and phase constitution were assessed by differential scanning calorimetry (DSC), X-ray diffraction analysis (XRD) and transmission electron microscopy (TEM). It was found by XRD that TiNi can contain a large amount of the PGMs as Ti(Ni, Rh), Ti(Ni, Ir) and Ti(Ni, Pt). Lattice parameters monotonously increase with increasing amount of PGMs. With increasing Pt content, Ms slightly decreases when less than 10mol%Pt while continuously increases as the rate of 26K/mol%Pt when more than 10mol%Pt. On the other hand, Ms decreases and then disappears with increasing Rh or Ir content. Hardness ranges from HV180 to HV570 and the maximum values in the pseudobinary systems lie around 20–30mol%PGM, suggesting solid solution hardening caused by the substitution of PGMs.
The recent attention paid to stress migration of aluminum (Al) electrodes in thin-film transistor liquid crystal display (TFT-LCD) applications indicates that wiring materials with low electrical resistivities are of considerable interest for their potential use in higher-resolution displays. In this paper, we firstly describe how as-grown Al whiskers on Al electrodes fabricated on a LCD-grade glass substrate can be characterized by means of a high-voltage transmission electron microscope (HV-TEM) operated at 1 MV. The whiskers ranging from 300 to 400 nm in diameter are sufficient to be transparent to high-voltage electrons. This allows detailed observation of whisker characteristics such as its morphology and crystallography. In most cases, the as-grown Al whiskers in our study had straight rod shapes, and could be regarded as single crystals. Secondly, we report on the in-situ fabrication and observation of Al whiskers at elevated temperature with the HV-TEM. Since relatively thick TEM samples (up to about 1 mm) can be set on a sample holder in the HV-TEM, various growth stages of Al whiskers can be investigated under various heating conditions. Finally, we demonstrate a TEM sample preparation method for the cross-section of an individual Al whisker, using focused ion beam (FIB) etching. This technique makes it possible to reduce the thickness of an Al whisker close to the root. Both bright- and dark-field TEM images provide nanostructural information on the whisker/Al thin-film interface.
Since cementitious materials used in repositories will be in contact with water, one of the most important tasks is to assess their long-term performance while they are being degraded very slowly by leaching. The authors have proposed an electrochemical acceleration test method and clarified its applicability. In this method, a specimen is placed between two glass vessels containing water. An anode and a cathode connected to a DC power source provide a potential gradient across the specimen. Ca2+ ions in the pore solution move rapidly to the cathode side, and thus hasten cement hydrate dissolution.
In this study, we obtained two types of almost homogeneously degraded specimens by controlling cumulative quantity of dissolved Ca2+ ions. In the first type, there was no Ca(OH)2and the C-S-H phases underwent insignificant alteration, whereas in the second type, degradation of the C-S-H phases occurred.
As a preliminary evaluation of cementitious materials, diffusion coefficients of tritiated water were measured for nondegraded and degraded specimens. Diffusion coefficients were increased by degradation and closely corresponded to changes in porosity.
The relation between the nanostructure of pure Al and Al-0.2 wt.% Cu thin films on glass substrates and anti-stress migration properties were investigated. These films were deposited on liquid-crystal display (LCD) grade glass substrate (550 x 650 mm) by means of two types of dc magnetron multi-chamber sputtering apparatus.
We developed the nanoindentation techniques to accelerate the characterization time for stress migration test. By AFM and cross-sectional TEM observations, we found an unusual three-layer structure in a Al-Cu thin film with strong anti-stress migration property.