Crystal structures, microtopography, morphologies, elemental compositions, and ionic conductivity have been investigated for Li5-xLa3(Nb,Ta)O12-y using X-ray diffraction (XRD), field-emission analytical scanning and transmission electron microscopies (S/TEM), and electrochemical impedance spectroscopy. Using Rietveld refinements with powder XRD patterns, we determined that the number of Li atoms in the formula is less than 5 and that Li5-xLa3(NbTa)O12-y crystallizes in the cubic garnet structure with a space group Ia-3d. Sintering at varying temperatures (750–1000 °C) for 5 h in an ambient atmosphere produced distinct outcomes. Rietveld refinements disclosed that the sample sintered at 1000 °C (Li3.43(2)La3Nb1.07(2)Ta0.93(2)O12-y, a = 12.8361(7) Å, V = 2114.96(3) Å3) exhibited the highest ionic conductivity, while the 850 °C sample had the lowest conductivity, characterized by lower Li concentration and impurity phases (Li(Nb,Ta)3O88, Li2CO3). Analyses, including XRD and electron microscopy, confirmed the 1000 °C sample as a relatively phase pure with enhanced Li content (Li/La = 1.2), larger grains (15 μm), and uniform crystallinity. The 1000 °C sample introduced additional partially filled Li3 (96h) sites, promoting Li migration, and enhancing ionic conductivity. The resulting XRD pattern at 1000 °C has been submitted to the Powder Diffraction File as a reference.

Laser plasma accelerators (LPAs) enable the generation of intense and short proton bunches on a micrometre scale, thus offering new experimental capabilities to research fields such as ultra-high dose rate radiobiology or material analysis. Being spectrally broadband, laser-accelerated proton bunches allow for tailored volumetric dose deposition in a sample via single bunches to excite or probe specific sample properties. The rising number of such experiments indicates a need for diagnostics providing spatially resolved characterization of dose distributions with volumes of approximately 1 cm${}^3$ for single proton bunches to allow for fast online feedback. Here we present the scintillator-based miniSCIDOM detector for online single-bunch tomographic reconstruction of dose distributions in volumes of up to approximately 1 cm${}^3$. The detector achieves a spatial resolution below 500 $\unicode{x3bc}$m and a sensitivity of 100 mGy. The detector performance is tested at a proton therapy cyclotron and an LPA proton source. The experiments’ primary focus is the characterization of the scintillator’s ionization quenching behaviour.

Laser–plasma accelerated (LPA) proton bunches are now applied for research fields ranging from ultra-high-dose-rate radiobiology to material science. Yet, the capabilities to characterize the spectrally and angularly broad LPA bunches lag behind the rapidly evolving applications. The OCTOPOD translates the angularly resolved spectral characterization of LPA proton bunches into the spatially resolved detection of the volumetric dose distribution deposited in a liquid scintillator. Up to 24 multi-pinhole arrays record projections of the scintillation light distribution and allow for tomographic reconstruction of the volumetric dose deposition pattern, from which proton spectra may be retrieved. Applying the OCTOPOD at a cyclotron, we show the reliable retrieval of various spatial dose deposition patterns and detector sensitivity over a broad dose range. Moreover, the OCTOPOD was installed at an LPA proton source, providing real-time data on proton acceleration performance and attesting the system optimal performance in the harsh laser–plasma environment.

Background: The late-onset cerebellar ataxias (LOCAs) have until recently resisted molecular diagnosis. Contributing to this diagnostic gap is that non-coding structural variations, such as repeat expansions, are not fully accessible to standard short-read sequencing analysis. Methods: We combined bioinformatics analysis of whole-genome sequencing and long-read sequencing to search for repeat expansions in patients with LOCA. We enrolled 66 French-Canadian, 228 German, 20 Australian and 31 Indian patients. Pathogenic mechanisms were studied in post-mortem cerebellum and induced pluripotent stem cell (iPSC)-derived motor neurons from 2 patients. Results: We identified 128 patients who carried an autosomal dominant GAA repeat expansion in the first intron of the FGF14 gene. The expansion was present in 61%, 18%, 15% and 10% of patients in the French-Canadian, German, Australian and Indian cohorts, respectively. The pathogenic threshold was determined to be (GAA)≥250, although incomplete penetrance was observed in the (GAA)250-300 range. Patients developed a slowly progressive cerebellar syndrome at an average age of 59 years. Patient-derived post-mortem cerebellum and induced motor neurons both showed reduction in FGF14 RNA and protein expression compared to controls. Conclusions: This intronic, dominantly inherited GAA repeat expansion in FGF14 represents one of the most common genetic causes of LOCA uncovered to date.

Objectives: In this study, we compared the performance of a rapid polymerase chain reaction (PCR) method in detecting carbapenemase-producing organisms (CPOs) and its impact on infection prevention and control (IPC) measures compared with a culture PCR method. Methods: All patients requiring CPO screening were included. Rectal swabs were collected with double rayon swabs (Copan 139C). They were simultaneously analyzed for the presence of CPOs using rapid PCR assay (Xpert Carba-R assay, Cepheid, Sunnyvale, CA) and a culture–PCR method (ChromID CARBA-SMART, bioMerieux, Marcy-l’Etoile, France). For CARBA-SMART, only colored colonies (ie, Enterobacterales) were evaluated for CPOs according to the prevailing institutional protocol. We tracked time to CPO detection. Using CPO positivity from either the rapid PCR or the culture PCR method as the gold standard, we calculated the sensitivity and specificity of both tests. We calculated the number of epidemiologically linked contacts generated when the first test results were known. We prospectively followed the ward census to identify the putative additional number of contacts generated by the later known result. Contacts were patients who shared the same ward (with overlapping time) as the CPO patients. Results: Between April 2019 and June 2020, culture PCR method detected CPOs in 316 (1.3%) of 24,514 samples (blaOXA48, N = 211; blaNDM, N = 51; blaIMI, N = 21; blaIMP, N = 10; blaKPC, N = 9; mixed genotypes, N = 14). The rapid PCR test detected CPOs in 605(2.5%) of 24,514 samples (blaOXA48, N = 266; blaNDM, N = 161; blaIMP, N = 99; blaVIM, N = 29; blaKPC, N = 15; mixed genotypes, N = 35). The sensitivity of direct PCR and culture PCR methods were 94.2% (95% CI, 92.1%–95.8%) and 43.5% (95% CI, 39.6%–47.4%), respectively. Both tests had 100% specificity. The median times to detection for the rapid PCR and culture PCR methods were 3–4 hours and 4 days, respectively. Compared with rapid PCR, the culture PCR method generated additional 7,415 contacts when it also tested positive for CPOs and an additional 23,135 contacts when it tested negative for CPOs. Conclusions: In our study, the rapid PCR test was more sensitive, identified CPO faster, and generated fewer epidemiologically linked contacts than the culture PCR method.

We present WALLABY pilot data release 1, the first public release of H i pilot survey data from the Wide-field ASKAP L-band Legacy All-sky Blind Survey (WALLABY) on the Australian Square Kilometre Array Pathfinder. Phase 1 of the WALLABY pilot survey targeted three $60\,\mathrm{deg}^{2}$ regions on the sky in the direction of the Hydra and Norma galaxy clusters and the NGC 4636 galaxy group, covering the redshift range of $z \lesssim 0.08$ . The source catalogue, images and spectra of nearly 600 extragalactic H i detections and kinematic models for 109 spatially resolved galaxies are available. As the pilot survey targeted regions containing nearby group and cluster environments, the median redshift of the sample of $z \approx 0.014$ is relatively low compared to the full WALLABY survey. The median galaxy H i mass is $2.3 \times 10^{9}\,{\rm M}_{{\odot}}$ . The target noise level of $1.6\,\mathrm{mJy}$ per 30′′ beam and $18.5\,\mathrm{kHz}$ channel translates into a $5 \sigma$ H i mass sensitivity for point sources of about $5.2 \times 10^{8} \, (D_{\rm L} / \mathrm{100\,Mpc})^{2} \, {\rm M}_{{\odot}}$ across 50 spectral channels ( ${\approx} 200\,\mathrm{km \, s}^{-1}$ ) and a $5 \sigma$ H i column density sensitivity of about $8.6 \times 10^{19} \, (1 + z)^{4}\,\mathrm{cm}^{-2}$ across 5 channels ( ${\approx} 20\,\mathrm{km \, s}^{-1}$ ) for emission filling the 30′′ beam. As expected for a pilot survey, several technical issues and artefacts are still affecting the data quality. Most notably, there are systematic flux errors of up to several 10% caused by uncertainties about the exact size and shape of each of the primary beams as well as the presence of sidelobes due to the finite deconvolution threshold. In addition, artefacts such as residual continuum emission and bandpass ripples have affected some of the data. The pilot survey has been highly successful in uncovering such technical problems, most of which are expected to be addressed and rectified before the start of the full WALLABY survey.

Background: Degenerative cervical myelopathy is a spinal disorder resulting in progressive spinal cord compression and consequent neurological deficits that can be assessed and tracked using the modified Japanese Orthopedic Association (mJOA) questionnaire. However, it is difficult to predict which patients will recover neurological function after surgery, making it difficult for clinicians to set reliable postoperative patient expectations. Methods: Sixty-eight operative myelopathy patients (50 male, 14 female) consented to complete the mJOA questionnaire both preoperatively and 6-months postoperatively. Fifteen of these patients had mild, twenty-three had moderate, and thirty had severe preoperative disease. Results: We found that in mild myelopathy, sensation and strength recover in similar proportions. In moderate myelopathy, a greater proportion of patients recover in each domain except for sensation. Recovery in severe myelopathy was comparable to moderate disease, but showed more dramatic recovery in sensation and sphincter function. Conclusions: This study shows that the severity of myelopathic disease influences the pattern of postoperative recovery. Though limited in sample size, the recovery patterns identified above are an important first step in recognizing myelopathy as a disease that patients experience heterogeneously both pre- and post-operatively. Our results will aid clinicians in goals-of-surgery discussions and assist with managing postoperative patient expectations.

Background: Predictors of hospitalization and reasons for admissions can inform healthcare planning and prevention. We sought to characterize the hospitalization pattern and risk factors for admission of children with cerebral palsy (CP). Methods: Data from the Registre de paralysie cérébrale du Québec and provincial administrative databases were linked. The CP cohort contained children born between 1999 and 2002. Data related to admissions were captured in 2012. Relative risks (RR) were calculated to identify factors increasing hospitalization risk. Peers without CP were matched from administrative databases in a 20:1 ratio. Chi-square tests and Student’s T-tests were used to compare cohorts. Results: 301 children with CP and 6040 peer controls were selected. Hospitalizations were increased in children with CP (raw mean difference (RMD) 5.0 95% CI 4.7 to 5.2), with significantly longer lengths of stay (RMD 2.8 95% CI 1.8 to 3.8) and number of diagnoses per hospitalization (RMD 1.6 95% CI 1.4 to 1.8). Increased risk of any hospitalization was observed in children with a more complex profile. Conclusions: Children with a more severe profile of CP and greater health care complexity face more frequent and longer hospital stays. Coordinated interdisciplinary care is needed in school-aged children with CP and medical complexity.

Background: Degenerative cervical myelopathy is characterized by progressive compression of the spinal cord resulting in debilitating loss of dexterity, independent ambulation, and sphincter control. Diffusion tensor imaging (DTI) has shown that, compared to healthy controls, myelopathy patients have decreased integrity of the corticospinal tracts and corpus callosum (Bernabeu-Sanz et al, 2020). Methods: Twenty-six myelopathy patients consented to cerebral diffusion tensor imaging (3 Tesla, 32 directions, b=1000) preoperatively, as well as 6-weeks, 12-weeks, and 6-months postoperatively. Average mean diffusivity (MD), fractional anisotropy (FA), radial diffusivity (RD), and axial diffusivity (AD) were measured in the corticospinal tracts, forceps major, and forceps minor. Results: Both MD and RD decreased from 6-12 weeks postoperatively in the right corticospinal tract. The forceps major of the corpus callosum showed an initial postoperative increase in MD followed by a subsequent increase in FA and decrease in RD 3-6 months postoperatively. The AD of the forceps major increased both immediately and 3-6 months postoperatively. Conclusions: Changes in microstructural integrity of the corticospinal tract and forceps major over the postoperative recovery period suggest a pattern of recovery in myelopathy patients. This study is the first to report postoperative DTI changes in myelopathy-relevant white matter tracts in the brain.

Background: Spinal cord compression from degenerative cervical myelopathy is characterized by progressive loss of hand dexterity, alongside changes in the metabolite profiles in the brain and spinal cord. Correlating the changing metabolite profile with measures of dexterity following decompression surgery may assist in identifying which patients may benefit most from surgery. Methods: Thirty operative myelopathy patients consented to receive spectroscopy and GRASSP-M dexterity assessments both preoperatively and 6-weeks postoperatively. Magnetic resonance spectroscopy (TE=135) was performed in the motor cortex using a 3 Tesla Siemens MRI scanner at Robarts Research Institute. Spearman correlations were used to evaluate associations between metabolite levels and dexterity (p<0.05 was considered significant). Paired two-tailed Student t-tests were used to assess for postoperative changes in metabolite levels. Results: Postoperatively, we observed a statistically significant (p<0.05) negative correlation (r=-0.44) between the N-acetylaspartate-to-creatine ratio (NAA/Cr) and GRASSP-M dexterity scores. There was no significant difference in NAA, Cr, or NAA/Cr postoperatively. Conclusions: These findings demonstrate that patients with lower postoperative NAA/Cr usually have better recovery of dexterity. This link between the myelopathic metabolite profile and clinically meaningful dexterity values requires further investigation to understand the role of both NAA and Cr in mechanisms of postoperative recovery from myelopathy.

Background: Improved understanding of factors predictive of emergency department (ED) visits in children with cerebral palsy (CP) can help optimize healthcare use. We sought to identify the pattern of ED consultations in these children. Methods: Data from the Registre de paralysie cérébrale du Québec and provincial administrative databases were linked. The CP cohort was comprised of children born between 1999 and 2002. Data pertaining to ED presentations between 1999 and 2012 were obtained. Relative risks were calculated to identify factors associated with increased ED visits. Peers without CP were selected from administrative databases and matched in a 20:1 ratio. Chi-square tests and Student’s T-tests were used to compare the two cohorts. Results: 301 children with CP and 6040 peer controls were selected. Ninety-two percent (92%) of the CP cohort had at least one ED visit, compared to 74% amongst controls. Children with CP had an increased risk of high ED use compared to peers (RR 1.40 95% CI 1.30-1.52). Factors predictive of high ED use were comorbid epilepsy, severe motor impairment and low socioeconomic status. Conclusions: Children with CP have a higher need for urgent health assessments than their peers, resulting in increased use of ED services. System factors and barriers should be investigated.

Background: Degenerative cervical myelopathy is a debilitating condition of the spinal column resulting in a progressive, clinically measurable loss of motor and sensory function secondary to spinal cord compression. We sought to correlate the patient’s subjective experience of specific myelopathic impairments with components of the objective clinical exam, to determine if the latter provides any clinically-relevant information postoperatively. Methods: Thirty-eight myelopathy patients consented to complete the mJOA questionnaire and receive a physical exam preoperatively, and 6-weeks and 6-months postoperatively. mJOA components were correlated with the physical exam using Spearman correlations with an alpha of 0.05. Results: mJOA scores for sensation and lower limb motor function correlated with the sensory and lower limb motor exams respectively, both preoperatively and 6-weeks postoperatively. mJOA scores for upper limb motor function did not correlate with the upper limb motor exam at either timepoint. Conclusions: At baseline and immediately postoperatively, patients self-report sensation and lower limb motor function accurately. However, the patients’ subjective experience of upper limb motor function does not align with clinical exam findings, suggesting either a continued need for this component of the physical exam or a need for tools that better correlate with the patient’s experience of upper limb motor impairment.

The Variables and Slow Transients Survey (VAST) on the Australian Square Kilometre Array Pathfinder (ASKAP) is designed to detect highly variable and transient radio sources on timescales from 5 s to $\sim\!5$ yr. In this paper, we present the survey description, observation strategy and initial results from the VAST Phase I Pilot Survey. This pilot survey consists of $\sim\!162$ h of observations conducted at a central frequency of 888 MHz between 2019 August and 2020 August, with a typical rms sensitivity of $0.24\ \mathrm{mJy\ beam}^{-1}$ and angular resolution of $12-20$ arcseconds. There are 113 fields, each of which was observed for 12 min integration time, with between 5 and 13 repeats, with cadences between 1 day and 8 months. The total area of the pilot survey footprint is 5 131 square degrees, covering six distinct regions of the sky. An initial search of two of these regions, totalling 1 646 square degrees, revealed 28 highly variable and/or transient sources. Seven of these are known pulsars, including the millisecond pulsar J2039–5617. Another seven are stars, four of which have no previously reported radio detection (SCR J0533–4257, LEHPM 2-783, UCAC3 89–412162 and 2MASS J22414436–6119311). Of the remaining 14 sources, two are active galactic nuclei, six are associated with galaxies and the other six have no multi-wavelength counterparts and are yet to be identified.