SPICA, a new special environment powder neutron diffractometer was built at BL09 in the Material and Life science Facility (MLF) of the Japan Proton Accelerator Research Complex (J-PARC). This is the ...first instrument dedicated solely to the study of next-generation batteries in J-PARC and is optimized for in situ measurements to clarify structural changes of materials in batteries. The basic design and instrumentation of SPICA have been completed. The highest Δd/d resolution achieved at the commissioning stage was 0.09% at the back scattering bank of SPICA. The reliability of the diffraction data has achieved a sufficiently high level for the structural analysis of materials using the Rietveld method. The air scattering banks with the blades made of B4C for in situ measurements also function very well.
Aim To evaluate the usefulness of several parameters of 5 min compared to 10 min delayed contrast-enhanced CT in distinguishing adenomas from non-adenomas. Materials and methods The study population ...consisted of 94 patients (52 men and 42 women; mean age 62 years) with 103 adrenal lesions (75 adenomas and 28 non-adenomas). In each patient, unenhanced CT was followed by early, 5 and 10 min enhanced CT. Diagnostic parameters included delayed enhanced attenuation at 5 and 10 min, washout attenuation (WO) at 5 and 10 min, absolute percentage washout (APW) at 5 and 10 min, and relative percentage washout (RPW) at 5 and 10 min. The accuracy of each parameter for diagnosing adenomas from non-adenomas was calculated using receiver operating characteristic (ROC) analysis. Results Upon comparison between 5 and 10 min delayed contrast-enhanced CT for differentiating total adenomas or lipid-poor adenomas from non-adenomas, there was no significant difference in the area under the binomial ROC curve ( Az ) values of delayed enhanced attenuation (total adenomas versus non-adenomas, p = 0.164; lipid-poor adenomas versus non-adenomas, p = 0.178), WO (total adenomas versus non-adenomas, p = 0.216; lipid-poor adenomas versus non-adenomas, p = 0.230), APW (total adenomas versus non-adenomas, p = 0.401; lipid-poor adenomas versus non-adenomas, p = 0.870), or RPW (total adenomas versus non-adenomas, p = 0.160; lipid-poor adenomas versus non-adenomas, p = 0.780). Conclusion Five minute contrast-enhanced CT was as useful as 10 min contrast-enhanced CT for differentiation of adrenal adenomas from non-adenomas.
Super High Resolution Powder Diffractometer SuperHRPD 1 at J-PARC have suffered damage by the Great East Japan Earthquake on 11 March 2011. After temporarily restoration work, the general user ...program has been started since April 2012. Full-fledged restoration of the temporarily restored devices is being carried out in 2013. The absorber materials for two disk choppers were replaced to 10B4C which improved quality of incident spectrum. The new detector system of 8 mm diameter position sensitive detectors was developed, and the test experiments showed excellent results.
Background and purpose: Mutations in the valosin‐containing protein (VCP) gene are known to cause inclusion body myopathy with Paget’s disease of bone and frontotemporal dementia (IBMPFD) and ...familial amyotrophic lateral sclerosis (ALS). Despite an increasing number of clinical reports, only one Asian family with IBMPFD has been described.
Methods: To characterize patients with VCP mutations, we screened a total of 152 unrelated Asian families who were suspected to have rimmed vacuolar myopathy.
Results: We identified VCP mutations in seven patients from six unrelated Asian families. Five different missense mutations were found, including a novel p.Ala439Pro substitution. All patients had adult‐onset progressive muscle wasting with variable involvement of axial, proximal, and distal muscles. Two of seven patients were suggested to have mild brain involvement including cerebellar ataxia, and only one showed radiological findings indicating a change in bone. Findings from skeletal muscle indicated mixed neurogenic and myogenic changes, fibers with rimmed vacuoles, and the presence of cytoplasmic and nuclear inclusions. These inclusions were immunopositive for VCP, ubiquitin, transactivation response DNA‐binding protein 43, and also histone deacetylase 6 (HDAC6), of which function is regulated by VCP. Evidence of early nuclear and mitochondrial damage was also characteristic.
Conclusions: Valosin‐containing protein mutations are not rare in Asian patients, and gene analysis should be considered for patients with adult‐onset rimmed vacuolar myopathy with neurogenic changes. A wide variety of central and peripheral nervous system symptoms coupled with rare bone abnormalities may complicate diagnosis.
A pulsed neutron transmission coupled with a two-dimensional position sensitive neutron detector gives a time-of-flight spectrum at each pixel of the detector, which depends on the total ...cross-sections of materials. In order to extract quantitative information of the preferred orientation included in the Bragg scattering total cross-section data, a spectral analysis software for the 2D imaging has been developed, and the transmission data of an unbent iron plate were analyzed. The 2D images with respect to the preferred orientation were successfully obtained, and the effectiveness of spectroscopic neutron transmission imaging was indicated.
The residual strain and the total cross-section in Fe plate was investigated by a Bragg-edge transmission method. The experiments were performed at the position of the time-of-flight diffractometer ...at KENS, Sirius. The (Δ
d/
d) reached about 0.1%. The high counting rate and high efficiency pixel type 2d-detector with 8×8 pixels was used. The spatial resolution is about 2.1
mm. We clearly observed the image of the change on Bragg-edge shapes and the strain distribution. The total cross-section around the bending points was larger than other areas but there was no change in the Bragg-edge positions, which would correspond to the change of the microstructure. We obtained the image of residual strain at several points. The values of the residual strain at several lattice planes,
ε
110,
ε
200 and
ε
211, are −263 to 1707
με, −612 to 1665
με and −1205 to 327
με, respectively. It is indicated that the residual strain measurement by the Bragg-edge transmission is not inferior to the high-resolution Bragg diffraction.
The RITS code is a unique and powerful tool for a whole Bragg-edge transmission spectrum fitting analysis. However, it has had two major problems. Therefore, we have proposed methods to overcome ...these problems. The first issue is the difference in the crystallite size values between the diffraction and the Bragg-edge analyses. We found the reason was a different definition of the crystal structure factor. It affects the crystallite size because the crystallite size is deduced from the primary extinction effect which depends on the crystal structure factor. As a result of algorithm change, crystallite sizes obtained by RITS drastically approached to crystallite sizes obtained by Rietveld analyses of diffraction data; from 155% to 110%. The second issue is correction of the effect of background neutrons scattered from a specimen. Through neutron transport simulation studies, we found that the background components consist of forward Bragg scattering, double backward Bragg scattering, and thermal diffuse scattering. RITS with the background correction function which was developed through the simulation studies could well reconstruct various simulated and experimental transmission spectra, but refined crystalline microstructural parameters were often distorted. Finally, it was recommended to reduce the background by improving experimental conditions.