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A variety of hereditary spinocerebellar ataxia (SCA) develops a broad spectrum of both ataxia and non-ataxia symptoms. Cognitive and affective changes are one such non-ataxia symptoms, but have been described only in hereditary SCAs with exonic CAG gene expansion.
We newly found intronic hexanucleotide GGCCTG gene expansion in NOP56 gene as the causative mutation (=SCA36) in nine unrelated Japanese familial SCA originating from Asida river area in the western part of Japan, thus nicknamed Asidan for this mutation. These patients show unique clinical balance of cerebellar ataxia and motor neuron disease (MND), locating on the crossroad of these two diseases. We examined cognitive and affective analyses on 12 Asidan patients who agreed to join the examination.
The 12 Asidan patients demonstrated a significant decrease in their frontal executive functions measured by frontal assessment battery (FAB) and Montreal cognitive assessment (MoCA) compared with age- and gender-matched controls, whilst mini-mental state examination (MMSE) and Hasegawa dementia score-revised (HDS-R) were within normal range. the decline of frontal executive function was related to their disease duration and scale for the assessment and rating of ataxias (SARA). They also demonstrated mild depression and apathy. Single-photon emission tomography (SPECT) analysis showed that these Asidan patients showed decline of regional cerebral blood flow (rCBF) in a particular areas of cerebral cortices such as Brodmann areas 24 and 44-46.
These data suggest the patients with Asidan mutation show unique cognitive and affective characteristics different from other hereditary SCAs with exonal CAG expansion or MND.
Parkinson's disease (PD), PD dementia (PDD) and dementia with Lewy body (DLB), collectively termed Lewy body disease (LBD), characterize an array of emotional, neurobehavioral and cognitive symptoms. Pure psychiatric presentation (PPP) of LBD is the fourth subtype of LBD in which psychiatric symptoms lasted for many years in the absence of neurological disturbance.
The aim of this study is to localize the PPP and its clinical characteristics in subjects with low uptake of myocardial metaiodobenzylguanidine (MIBG) study.
Sixty MIBG-verified patients (28 women and 32 men) were grouped into three psychiatric pictures; depression (group D: 27 patients), isolated visual hallucinations (group V: 16 patients) and psychotic picture (group P: 17 patients). Fifty six cases were examined with cerebral single photon emission tomography study. Thirty-seven cases had hypoperfusion regions and 19 cases showed no abnormality. Final diagnoses of PD, PDD, DLB and PPP were evaluated with an aid of DSM-IV-TR, UPDRS part III, the Hoehn and Yahr Scale, MMSE and the movement disorder society task force diagnostic criteria.
Three clusters, PDD and Group P, DLB and Group V, and Group D and PPP-PD were found significantly with correspondence analysis. UPDRS average score showed negative correlation with average MMSE scores. All patients with PPP and majority of patients with PD featured depression.
PPP subjects are considered to be incidental LBD or depression of pre-motor PD. Psychotic parkinsonian features contributed highly to dementia of LBD, and visual hallucination was closely associated with DLB.
This study aims to create controlled fine space by electrospinning, and to develop the electrode materials for high-performance energy devices. With the popularization of mobile devices, household appliances, hybrid vehicles, electric vehicles, and the like, the use of power storage devices is expanding, and further performance improvements are required. In this study, a novel electrode material was developed by compositing Si with carbon nanofibers derived from polyacrylonitrile (PAN) by electrospinning and heat treatment. The texture and structure of the nanofibers were observed and analyzed by scanning electron microscopy (SEM), energy dispersive X-ray spectrometry (EDX) and transmission electron microscopy (TEM) combined with image processing. Nano spaces were created in the CNFs and Si particles were able to be contained in the CNFs. In the second and subsequent cycles of the charge/discharge experiments of lithium ion battery (LIB) electrode made from the materials, the capacity was more than twice the theoretical capacity using graphite, and good cycle performance was obtained.
The Keio Twin Research Center (KoTReC) was established in 2009 at Keio University to combine two longitudinal cohort projects — the Keio Twin Study (KTS) for adolescence and adulthood and the Tokyo Twin Cohort Project (ToTCoP) for infancy and childhood. KoTReC also conducted a two-time panel study of self-control and psychopathology in twin adolescence in 2012 and 2013 and three independent anonymous cross-sectional twin surveys (ToTcross) before 2012 — the ToTCross, the Junior and Senior High School Survey and the High School Survey. This article introduces the recent research designs of KoTReC and its publications.
The search for life in the Universe is a fundamental problem of astrobiology and modern science. The current progress in the detection of terrestrial-type exoplanets has opened a new avenue in the characterization of exoplanetary atmospheres and in the search for biosignatures of life with the upcoming ground-based and space missions. To specify the conditions favourable for the origin, development and sustainment of life as we know it in other worlds, we need to understand the nature of global (astrospheric), and local (atmospheric and surface) environments of exoplanets in the habitable zones (HZs) around G-K-M dwarf stars including our young Sun. Global environment is formed by propagated disturbances from the planet-hosting stars in the form of stellar flares, coronal mass ejections, energetic particles and winds collectively known as astrospheric space weather. Its characterization will help in understanding how an exoplanetary ecosystem interacts with its host star, as well as in the specification of the physical, chemical and biochemical conditions that can create favourable and/or detrimental conditions for planetary climate and habitability along with evolution of planetary internal dynamics over geological timescales. A key linkage of (astro)physical, chemical and geological processes can only be understood in the framework of interdisciplinary studies with the incorporation of progress in heliophysics, astrophysics, planetary and Earth sciences. The assessment of the impacts of host stars on the climate and habitability of terrestrial (exo)planets will significantly expand the current definition of the HZ to the biogenic zone and provide new observational strategies for searching for signatures of life. The major goal of this paper is to describe and discuss the current status and recent progress in this interdisciplinary field in light of presentations and discussions during the NASA Nexus for Exoplanetary System Science funded workshop ‘Exoplanetary Space Weather, Climate and Habitability’ and to provide a new roadmap for the future development of the emerging field of exoplanetary science and astrobiology.
The purpose of this study was to clarify the association between hand, foot, and mouth disease (HFMD) epidemics and meteorological conditions. We used HFMD surveillance data of all 47 prefectures in Japan from January 2000 to December 2015. Spectral analysis was performed using the maximum entropy method (MEM) for temperature-, relative humidity-, and total rainfall-dependent incidence data. Using MEM-estimated periods, long-term oscillatory trends were calculated using the least squares fitting (LSF) method. The temperature and relative humidity thresholds of HFMD data were estimated from the LSF curves. The average temperature data indicated a lower threshold at 12 °C and a higher threshold at 30 °C for risk of HFMD infection. Maximum and minimum temperature data indicated a lower threshold at 6 °C and a higher threshold at 35 °C, suggesting a need for HFMD control measures at temperatures between 6 and 35 °C. Based on our findings, we recommend the use of maximum and minimum temperatures rather than the average temperature, to estimate the temperature threshold of HFMD infections. The results obtained might aid in the prediction of epidemics and preparation for the effect of climatic changes on HFMD epidemiology.
We report on the formation of shallow junctions with high activation in both n+/p and p+/n Ge junctions using ion implantation and Flash Lamp Annealing (FLA). The shallowest junction depths (Xj) formed for the n+/p and p+/n junctions were 7.6 nm and 6.1 nm with sheet resistances (Rs) of 860 ohms/sq. and 704 ohms/sq., respectively. By reducing knocked-on oxygen during ion implantation in the n+/p junctions, Rs was decreased by between 5% and 15%. The lowest Rs observed was 235 ohms/sq. with a junction depth of 21.5 nm. Hall measurements clearly revealed that knocked-on oxygen degraded phosphorus activation (carrier concentration). In the p+/n Ge junctions, we show that ion implantation damage induced high boron activation. Using this technique, Rs can be reduced from 475 ohms/sq. to 349 ohms/sq. These results indicate that the potential for forming ultra-shallow n+/p and p+/n junctions in the nanometer range in Ge devices using FLA is very high, leading to realistic monolithically-integrated Ge CMOS devices that can take us beyond Si technology.
Cyclone–anticyclone asymmetry in spontaneous gravity wave radiation from a co-rotating vortex pair is investigated in an
-plane shallow water system. The far field of gravity waves is derived analytically by analogy with the theory of aeroacoustic sound wave radiation (Lighthill theory). In the derived form, the Earth’s rotation affects not only the propagation of gravity waves but also their source. While the results correspond to the theory of vortex sound in the limit of
, there is an asymmetry in gravity wave radiation between cyclone pairs and anticyclone pairs for finite values of
. Anticyclone pairs radiate gravity waves more intensely than cyclone pairs due to the effect of the Earth’s rotation. In addition, there is a local maximum of intensity of gravity waves from anticyclone pairs at an intermediate
. To verify the analytical solution, a numerical simulation is also performed with a newly developed spectral method in an unbounded domain. The novelty of this method is the absence of wave reflection at the boundary due to a conformal mapping and a pseudo-hyperviscosity that acts like a sponge layer in the far field of waves. The numerical results are in excellent agreement with the analytical results even for finite values of
for both cyclone pairs and anticyclone pairs.
Four microseconds long Ar3+ beam with injection energy of 15 keV/u has been injected into the Digital Accelerator of the High-Energy Accelerator Research Organization. Beam production, transportation, and injection are described as well as machine properties. Results of a free running experiment under static magnetic field and longitudinal confinement and acceleration under a fast ramping magnetic field are presented in detail with a brief discussion on the beam lifetime.
Annual periodicities of reported chickenpox cases have been observed in several countries. Of these, Japan has reported a two-peaked, bimodal annual cycle of reported chickenpox cases. This study investigated the possible underlying association of the bimodal cycle observed in the surveillance data of reported chickenpox cases with the meteorological factors of temperature, relative humidity and rainfall. A time-series analysis consisting of the maximum entropy method spectral analysis and the least squares method was applied to the chickenpox data and meteorological data of 47 prefectures in Japan. In all of the power spectral densities for the 47 prefectures, the spectral lines were observed at the frequency positions corresponding to the 1-year and 6-month cycles. The optimum least squares fitting (LSF) curves calculated with the 1-year and 6-month cycles explained the underlying variation of the chickenpox data. The LSF curves reproduced the bimodal and unimodal cycles that were clearly observed in northern and southern Japan, respectively. The data suggest that the second peaks in the bimodal cycles in the reported chickenpox cases in Japan occurred at a temperature of approximately 8·5 °C.
Periodically arrayed rows of fine Fe2Hf Laves phase particles were found to form in 9Cr ferritic steel. Microstructural observation demonstrates that the particles were formed on cooling through the interphase precipitation on the phase transformation from the δ ferrite to the γ austenite along the eutectoid transformation route of δ → γ+Fe2Hf and subsequently a phase transformation from the austenite to the α ferrite took place. This eutectoid route is expected to be effectively used for improving the long term creep strength of ferritic steels with Laves phase.
Preparation of a sigma-CrFe single-phase specimen was achieved by arc melting of pure Fe and Cr, cold rolling, and subsequent annealing at 973 K or 1073 K in vacuum. Cold rolling before annealing is effective for the annealing-induced formation of sigma-CrFe from the bcc solid-solution phase. The phase stability and the structural change from sigma-CrFe to a bcc solid-solution phase under fast electron irradiation were investigated by in situ transmission electron microscope (TEM) observation in the temperature range between 22 K and 473 K by using an ultra-high voltage electron microscope (UHVEM). The phase transition of sigma-CrFe by fast electron irradiation was found to occur at a particular temperature.
The liver fluke, Opisthorchis viverrini, and the minute intestinal fluke, Haplorchis taichui, are prevalent in many Asian countries. This study analysed the patterns of infections of O. viverrini and H. taichui in Lahanam and Thakhamlien villages (Savannakhet Province, Lao PDR), in two cross-sectional investigations. Out of a total of 207 human participants, post-anthelmintic treatment positivity rates for expelled worms were 170 (82.1%) for H. taichui and 65 (31.4%) for O. viverrini. Both these species co-exist in the study villages. When each parasite was analysed separately, H. taichui infections reached a plateau among people aged >20 years. Opisthorchis viverrini infection rates were highest in the age group 21–30 years, with decreasing infection rates after the age of 30. Our findings indicated that fish-borne trematode infections were more prevalent among adults. Fish, common intermediate hosts, were acquired in the study area for analysis. The examination of 35 species of fish as intermediate hosts found O. viverrini metacercariae in only six species, and these were found mostly during the month of November. Many farmers who live on the rice fields obtain their food from their immediate environment, including these intermediate-host fish, potentially putting them at greater risk of O. viverrini infection. By contrast, H. taichui metacercariae were found in three species of fish obtained from the market, meaning that anyone could consume them and become infected. If people who work in rice fields limit the species of fish they consume, or avoid consuming raw fish during the month of November, they may reduce their risk of O. viverrini infection.
The correlation of stress in Silicon Carbide (SiC) crystal and frequency shift in micro- Raman spectroscopy was determined by an experimental method. We applied uniaxial stress to 4H- and 6H-SiC single crystal square bar specimen shaped with (0001) and (11-20) faces by four point bending test, under measuring the frequency shift in micro-Raman spectroscopy. The results revealed that the linearity coefficients between stress and Raman shift were -1.96 cm-1/GPa for FTO(2/4)E2 on 4H-SiC (0001) face, -2.08 cm-1/GPa for FTO(2/4)E2 on 4H-SiC (11-20) face and -2.70 cm-1/GPa for FTO(2/6)E2 on 6H-SiC (0001) face. Determination of these coefficients has made it possible to evaluate the residual stress in SiC crystal quantitatively by micro-Raman spectroscopy. We evaluated the residual stress in SiC substrate that was grown in our laboratory by utilizing the results obtained in this study. The result of estimation indicated that the SiC substrate with a diameter of 6 inch remained residual stress as low as ±15 MPa.
We investigated the seasonality of tuberculosis (TB) in Wuhan, China, to evaluate the increased risk of disease transmission during each season and to develop an effective TB control strategy. We applied spectral analysis to the weekly prevalence data of sputum smear positive (SSP) and sputum smear negative (SSN) pulmonary TB reported from 2006 to 2010. Cases of both SSP and SSN feature 1·0- and 0·5-year periodic modes. The least squares method was used to fit curves to the two periodic modes for SSP and SSN data. The curves demonstrated dominant peaks in spring similar to cases reported previously for other locations. Notably for SSP, dominant peaks were also observed in summer. The spring peaks of SSP and SSN were explained in terms of poorly ventilated and humid rooms and vitamin D deficiency. For the summer peaks of SSP, summer influenza epidemics in Wuhan may contribute to the increase in TB prevalence.
To describe a case of giant cell reparative granuloma of the temporal bone which extended into the middle-ear cavity, and which was successfully treated surgically via a transmastoid approach, with hearing preservation.
A 37-year-old man presented with a one-year history of right-sided hearing loss, complicated by a three-month history of otalgia and a sensation of aural fullness. Computed tomography and magnetic resonance imaging demonstrated an osteolytic tumour lesion in the right temporal bone. The diagnosis was confirmed by biopsy from the mastoid lesion.
Investigation and intervention:
Pure-tone audiometry, computed tomography and magnetic resonance imaging were conducted, followed by total resection.
The giant cell reparative granuloma of the temporal bone was completely resected, with preservation of hearing.
Although this patient's giant cell reparative granuloma of the temporal bone extended into the middle-ear cavity, total resection was achieved, with preservation of hearing. To the best of our knowledge, hearing preservation following resection of giant cell reparative granuloma of the temporal bone has not previously been reported.
During the installation of the buffer in a deposition hole of an HLW repository, it is necessary to control water flow from the fractured rock into the deposition hole. Water flow with inflow rate greater than 0.001 l/min may cause piping and erosion of the buffer, and may trigger mass redistribution of the buffer, sedimentation and material separation of bentonite materials. This paper describes the condition of parameters which cause piping and erosion; revised conditions which keep advection, inflow rate, buffer component, gap between buffer materials, gap between outside wall and buffer block, and type of water. The results from the experiment show the condition of the self-sealing function of bentonite materials, formation of piping, allowable limit of inflow rate in the case of an Na type bentonite block of 70 wt.% Kunigel V1 and 30 wt.% silica sand, or a pellet of 100 wt.% Kunigel V1. Piping and erosion continue until the engineered barrier (EB) is filled with water, and then the hydraulic gradient becomes small. Piping may lead to erosion and redistribution of material which needs to be taken into account in the long-term performance assessment.
In order to evaluate the long-term behaviour of the engineered barriers in geological disposal sites for transuranic element-bearing (TRU) waste, an evaluation by numerical analysis is required. Although chemical and hydraulic/mechanical analyses have been conducted independently until now, essentially both type of phenomena occur simultaneously and produce synergistic effects. Therefore, we focused attention on the buffer (bentonite) engineered barrier and conducted a study of which involved incorporating hydraulic/mechanical phenomena into the chemical analysis of bentonite alteration. The simulations employed weakly-coupled chemical and hydraulic/mechanical effects to study the behaviour in one dimension.
The results showed that the dissolution of the montmorillonite is suppressed in the buffer section nearest the cement material. Moreover, in order to achieve a fully coupled analysis in future, the present study also identifies issues that need to be resolved.
Rhino-sinus mucosal involvement is well documented in untreated lepromatous leprosy, but less understood in ex-leprosy patients (i.e. leprosy patients who have been treated and cured) with atrophic rhinitis.
Materials and methods:
Rhino-sinus abnormalities were investigated in 13 ex-lepromatous leprosy patients with atrophic rhinitis, using interviews enquiring about sinonasal symptoms, nasal endoscopy, nasal swab culture and computed tomography. Endoscopic sinus surgery had been performed in three patients. The clinical course, computed tomography findings and nasal biopsy results of these three patients were evaluated.
All patients had turbinate atrophy and 6 of the 13 (46.2 per cent) had septal perforation. Paranasal sinus involvement was noted in 9 of 12 examined patients (75 per cent). The most commonly affected sinus was the maxillary sinus (in 8 of 12; 66.7 per cent). All three patients treated by endoscopic sinus surgery experienced relapse and required further surgery. Maxillary sinus irrigation was effective for reduction of persistent symptoms such as postnasal discharge and crusts.
Ex-lepromatous leprosy patients with atrophic rhinitis had various rhino-sinus abnormalities and persistent symptoms. These patients had chronic rhinosinusitis because of underlying atrophic rhinitis. These patients required repeated otolaryngological observations together with combined surgery and conservative treatment.