Weak Pattern Research Articles

Examining the Effects of Environment, Geography, and Elevation on Patterns of DNA Methylation Across Populations of Two Widespread Bumble Bee Species.

Understanding the myriad avenues through which spatial and environmental factors shape evolution is a major focus in biological research. From a molecular perspective, much work has been focused on genomic sequence variation; however, recently there has been increased interest in how epigenetic variation may be shaped by different variables across the landscape. DNA methylation has been of particular interest given that it is dynamic and can alter gene expression, potentially offering a path for a rapid response to environmental change. We utilized whole genome enzymatic methyl sequencing to evaluate the distribution of CpG methylation across the genome and to analyze patterns of spatial and environmental association in the methylomes of two broadly distributed montane bumble bees (Bombus vancouverensis Cresson and Bombus vosnesenskii Radoszkowski) across elevational gradients in the western US. Methylation patterns in both species are similar at the genomic scale with ∼1% of CpGs being methylated and most methylation being found in exons. At the landscape scale, neither species exhibited strong spatial or population structuring in patterns of methylation, although some weak relationships between methylation and distance or environmental variables were detected. Differential methylation analysis suggests a stronger environment association in B. vancouverensis given the larger number of differentially methylated CpG's compared to B. vosnesenskii. We also observed only a handful of genes with both differentially methylated CpGs and previously detected environmentally associated outlier SNPs. Overall results reveal a weak but present pattern in variation in methylation over the landscape in both species.

Clinical Characteristics of Charcot-Marie-Tooth Disease Type 4J.

Charcot-Marie-Tooth disease type 4J (CMT4J) is caused by autosomal recessive variants in the Factor-Induced Gene 4 (FIG4) gene. Recent preclinical work has demonstrated the feasibility of adeno-associated virus serotype 9-FIG4 gene therapy. This study aimed to further characterize the CMT4J phenotype and evaluate feasibility of validated CMT-related outcome measures for future clinical trials. This cross-sectional study enrolled children and adults with genetically confirmed CMT4J, with 2 documented disease-causing variants in the FIG4 gene. Patients were recruited through the Inherited Neuropathy Consortium network. Disease severity was assessed using standardized CMT-specific outcome measures and exploratory biomarkers including muscle MRI fat fraction, electrophysiology, and neurofilament light chain levels. Descriptive statistics and correlation analyses were conducted to explore relationships between variables. We recruited a total of 19 patients, including 14 pediatric patients (mean age 10.9 ± 3.9 years) and 5 adults (mean age 40.0 ± 13.9 years). The most frequent symptoms were gross motor delay and distal more than proximal muscle weakness, which were observed in 14 of 19 patients. The most common non-neuromuscular symptoms were cognitive and respiratory deficits, each seen in 8 of 19 patients. We denoted asymmetric weakness in 2 patients and nonuniform slowing of conduction velocities in 6 patients. Charcot-Marie-Tooth Disease Pediatric Scale (CMTPedS), Pediatric Quality of Life Inventory, and Vineland Adaptive Behavior Scale scores were affected in most patients. We observed a significant positive correlation between neurofilament light chain levels and CMTPedS, but the study was underpowered to observe a correlation between CMTPedS and MRI fat fraction. We obtained baseline clinical and biomarker data in a broad cohort with CMT4J in pediatric and adult patients. Motor delay, muscle weakness, and respiratory and cognitive difficulties were the most common clinical manifestations of CMT4J. Many patients had nerve conduction studies with nonuniform slowing, and 2 had an asymmetric pattern of muscle weakness. We observed that the neurofilament light chain levels correlated with the CMTPedS in the pediatric population. This study showed feasibility of clinical outcomes including CMTPedS in assessment of disease severity in the pediatric patient population and provided baseline characteristics of exploratory biomarkers, neurofilament light chain levels, and muscle MRI fat fraction. The coronavirus disease 2019 pandemic affected some of the visits, resulting in a reduced number of some of the assessments.

Increase in marginal sea alkalinity may impact air–sea carbon dioxide exchange and buffer acidification

AbstractTotal alkalinity (TA) has increased in the Baltic Sea, with implications for atmospheric CO2‐induced acidification and CO2 uptake. We compiled extensive data of TA in surface waters of the Baltic Sea, aiming to (i) identify new tendencies in the relationship between TA and salinity (TA–S relationship), (ii) update the TA trend analysis, (iii) investigate spatial–temporal patterns, and (iv) discuss potential drivers and implications. We observed a progressive decrease in the slopes and increase in the intercepts of the TA–S overtime due to the persistent process of TA enhancement. A weak seasonal pattern was identified, with warmer months presenting lower salinity and TA. Lower rates of TA increase were observed in high salinities (Skagerrak–Kattegat; +1.00 to +2.20 μmol kg−1 yr−1), intermediate trends in low salinities (Gulf of Bothnia; +3.28 to +3.57 μmol kg−1 yr−1), and maximal trends in the Central Baltic Sea (+3.70 to +4.57 μmol kg−1 yr−1) and Bornholm Basin (+4.82 to +5.32 μmol kg−1 yr−1). The increase in the intercept of the TA–S in the Gulf of Bothnia suggests a progressive increase in the external supply of TA, although lower than previously thought. The maximum trend in the Bornholm Basin suggests an increase in external supply from the Southern catchment and/or the accumulation of internal production. The positive TA–phosphorus correlations underscore a significant internal source. The TA increase amplifies the CO2 uptake by 1.8–7.8% during spring/summer and reduces the CO2 outgassing by 3.4–7.7% in autumn/winter. The TA enhancement has the potential to buffer CO2‐induced acidification by 39–60% by 2050.

Pattern recognition of control charts based on data feature enhancement and ensemble learning of classifiers for dimensional accuracy of products

Product quality in manufacturing is directly related to the reliability, durability and safety of the product. Product quality can be controlled by building a product control chart pattern recognition model. The complexity and temporal sequence of product quality data make it difficult to extract effective control chart features, and it is difficult for common classifiers to play an effective role for each control chart pattern category, thus affecting the accuracy of pattern recognition. Therefore, this paper proposes a data feature enhancement processing method that combines Smote algorithm and smooth shift processing. This method reveals data features by performing feature enhancement processing on raw data and extracts statistical and shape features for dimensionality reduction to improve recognition accuracy and efficiency. Based on this approach, this paper also proposes an ensemble learning model based on the Stacking method, which incorporates BP, ELM, PNN and SVM classifiers for control chart pattern recognition. Through extensive experimental validation, the data feature enhancement method improves the accuracy of the weak classifier normal pattern recognition by 30%, and effectively reduces the misclassification in the quality control process. The ensemble learning model is stronger than the weak classifier, and its recognition accuracy can reach 97.31%.

High O3 pollution initiated by cold front passage over Pearl River Estuary

The influence of cold fronts on the formation of ozone (O3) pollution, particularly the contributions of physical and chemical processes to O3 abundance during the passage of cold fronts in winter over the Pearl River Estuary (PRE) region was still unclear. This study provided an in-depth analysis on the occurrence of high O3 pollution based on data from field measurements and simulation of a chemical transport model (i.e., WRF-Chem) over the PRE region during winter 2021 when cold fronts were frequently observed. Totally 7 high O3 episode days were observed during pre- and post-frontal stages at DWS (i.e., Da Wan Shan island), a downwind island site of the inland Pearl River Delta (PRD) region, which were accompanied with the prevailing northerly wind patterns with insignificant variations of wind speeds in a stable boundary layer over the whole PRE region. The quantitative analysis based on simulation results indicated that horizontal transport and chemical processes accounted for the buildup of O3 abundance at DWS, with the highest mean rates of 7.3 and 5.6 ppbv/h at pre- and post-frontal stages, respectively. The high contributions from horizontal transport to O3 levels at DWS were associated with the persistent northerly winds with small variations, while the contributions of chemical processes were varied with the abundance of precursors and meteorological conditions. However, vertical transport made negative contributions to O3 abundance, with the most significant mean diffusion rate of −2.6 ppbv/h at the frontal stage because of the control of high-pressure system, while the less influence of vertical transport at pre- and post-frontal stages was associated with the weak pressure gradient forces and wind patterns. The sensitivity analysis further demonstrated that the emissions of precursors from the inland PRD region contributed 50–76% to O3 abundance over PRE at different stages. Overall, the above results quantitatively confirmed the dominant role of transport from inland PRD to the O3 pollution over the downwind PRE region and were helpful for better understanding of O3 pollution in winter over PRE.

PASS Theory of Intelligence and Its Measurement Using the Cognitive Assessment System, 2nd Edition.

The goal of this paper was to describe the context within which the PASS theory of intelligence was conceived and the reasons why this theory was used to guide the construction of the Cognitive Assessment System and the several versions of the Cognitive Assessment System, 2nd Edition. We also discuss validity issues such as equitable assessment of intelligence, using PASS scores to examine a pattern of strengths and weaknesses related to academic variability and diagnosis, and the utility of PASS scores for intervention. We provide summaries of the research that informs our suggestions that intelligence testing should be theory-based, not constrained by the seminal work of test developers in the early 1900s, and neurocognitive processes should be measured based on brain function.

Species assemblages and their drivers differ between trees and lianas in a seasonal evergreen forest in Thailand

AbstractDespite a long tradition in ecology of studying tree species assembly and its potential drivers in tropical forest communities, little information exists with respect to lianas (woody climbers), the second most abundant life form of woody plants in tropical forests. Lianas influence forest diversity and stability and provide critical resources for forest fauna. Using a unique dataset of a 30‐ha plot in Thailand, where tree and liana individuals were fully mapped, we investigated the degree to which local species assemblages of trees and lianas of different size classes (i.e., seedlings, established individuals, and large individuals) are related to local environmental conditions. We asked (1) What are the spatial patterns and environmental drivers of local tree and liana species assemblages? (2) How do such patterns and drivers differ among size classes? (3) Which species associate with these assemblages? Local assemblages of established trees showed substantial structuring by environmental variables, whereas we found only weakly structured assemblages of tree seedlings, large trees, and lianas of all size classes. Our results indicated that the biotic and abiotic drivers of local species assemblages differed strongly between tree and liana communities and across size classes. Species assemblages of trees were mainly driven by soil nutrients, leading to patchy assemblages associated with high base saturation (Alfisols) and assemblages associated with lower levels of base saturation and higher aluminum (Ultisols), whereas tree seedling assemblages were only weakly structured by riparian zones. In contrast, species assemblages of established and large lianas were primarily associated with forest canopy structure, separating low‐canopy forests from high‐canopy forests, whereas soil nutrients were the only factors associated with liana seedling assemblages. The weak environmental structuring of tree seedlings and large trees suggests that other mechanisms, such as stochastic disturbances, competition for space, or animal seed dispersal, may play an important role in structuring tree communities in this seasonal tropical forest. The weak patterns observed in liana communities across all life stages raise questions about the underlying mechanisms of liana community assembly, and further research should focus on liana niches, their dispersal mechanisms, and host tree relations.

A CCG expansion in ABCD3 causes oculopharyngodistal myopathy in individuals of European ancestry

Oculopharyngodistal myopathy (OPDM) is an inherited myopathy manifesting with ptosis, dysphagia and distal weakness. Pathologically it is characterised by rimmed vacuoles and intranuclear inclusions on muscle biopsy. In recent years CGG • CCG repeat expansion in four different genes were identified in OPDM individuals in Asian populations. None of these have been found in affected individuals of non-Asian ancestry. In this study we describe the identification of CCG expansions in ABCD3, ranging from 118 to 694 repeats, in 35 affected individuals across eight unrelated OPDM families of European ancestry. ABCD3 transcript appears upregulated in fibroblasts and skeletal muscle from OPDM individuals, suggesting a potential role of over-expression of CCG repeat containing ABCD3 transcript in progressive skeletal muscle degeneration. The study provides further evidence of the role of non-coding repeat expansions in unsolved neuromuscular diseases and strengthens the association between the CGG • CCG repeat motif and a specific pattern of muscle weakness.

Numerical study of a generic ship's airwake for understanding bi‐stability mechanism

A distinguishing feature of the bi-stable wake is that the wake persists in either of two preferred states for a sufficiently long time. Aiming to understand the persistence mechanism, this paper numerically investigates the airwake characteristics of the Chalmers ship model (CSM) using large eddy simulation with a wall-adapting local-eddy viscosity model and is complemented by experimental testings for validations. There are two cases of interest: (i) the baseline CSM with a sharp-edged superstructure front that induces massive boundary layer separation; (ii) the front-rounded (FR) CSM with suppressed flow separation. During a characteristic time ( $t^*$ ) of 1142 (26.5 s), the baseline case has a frequently switching wake, whereas the FR wake maintains a stable asymmetric structure with only one switch attempt. To understand the different wake behaviours, the study starts by analysing wake flow structures, vortex cores and the wake dynamics, followed by investigating the instantaneous flow physics. Results suggest that the baseline wake has a weak bi-stable pattern, whereas the FR wake behaves similarly to a reflectional symmetry breaking state of a potential bi-stable wake. The wake switching is found to be driven by the tilting of (vertical-oriented) $z$ -vorticity sheets from either side of the base toward the centre. This tilting behaviour is subjected to the high-magnitude vorticity that sheds from the upstream flow separation at the front sharp edges. With the sharp edges rounded in the FR case, the upstream vorticity is mitigated and the tilting effect is significantly reduced, leading to a more stable wake structure. The reasoning provided in the paper potentially explains the persistence mechanism of the bi-stable wake.

SPHERE RefPlanets: Search for ε Eridani b and warm dust

Context. Cold planets, including all habitable planets, produce only scattered light emission in the visual to near-infrared wavelength range. For this reason it is highly desirable to adapt the technique for the direct imaging of reflected light from extra-solar planets. Aims. For the nearby system ε Eri, we want to set much deeper detection limits for the expected scattered radiation from the radial velocity planet candidate (≈0.7 MJ) and the warm dust using the VLT/SPHERE adaptive optics (AO) instrument with the ZIMPOL imaging polarimeter. Methods. We carried out very deep imaging polarimetry of ε Eri based on 38.5 h of integration time with a broad-band filter (λc = 735 nm) for the search of the polarization signal from a planet or from circumstellar dust using AO, coronagraphy, high precision differential polarimetry, and angular differential imaging. The data were collected during 12 nights within four epochs distributed over 14 months and we searched for a signal in the individual epochs. We also combined the full data set to achieve an even higher contrast limit considering the Keplerian motion using the K-Stacker software. All data were also combined for the search of the scattering signal from extended dust clouds. We improved various data reduction and post-processing procedures and also developed new ones to enhance the sensitivity of SPHERE/ZIMPOL further. The final detection limits were quantified and we investigated the potential of SPHERE/ZIMPOL for deeper observations. Results. The data of ε Eridani provide unprecedented contrast limits but no significant detection of a point source or an extended signal from circumstellar dust. For each observing epoch, we achieved a 5 σ𝒩 point source contrast for the polarized intensity CP = Qϕ/I★ between 2 × 10−8 and 4 × 10−8 at a separation of ρ ≈ 1″, which is as expected for the proposed radial velocity planet at a quadrature phase. The polarimetric contrast limits are close to the photon noise limits for ρ > 0.6″ or about six times to 50 times better than the intensity limits because polarimetric imaging is much more efficient for speckle suppression. Combining the data for the search of a planet moving on a Keplerian orbit with the K-Stacker technique improves the contrast limits further by about a factor of two, when compared to an epoch, to about CP = 0.8 × 10−8 at ρ = 1″. This would allow the detection of a planet with a radius of about 2.5 RJ. Should future astrometry provide strong constraints on the position of the planet, then a 3 σ𝒩 detection at 1″ with CP ≈ 5 × 10−9 would be within reach of our data. The surface brightness contrast limits achieved for the polarized intensity from an extended scattering region is about 15 mag arcsec−2 at 1″ or up to 3 mag arcsec−2 deeper than previous limits. For ε Eri, these limits exclude the presence of a narrow dust ring and they constrain the dust properties. The photon statistics would allow deeper limits but we find a very weak systematic noise pattern probably introduced by polarimetric calibration errors. Conclusions. This ε Eri study shows that the polarimetric contrast limits for reflecting planets with SPHERE/ZIMPOL can be improved to a level below Cp < 10−8 by just collecting more data during many nights using software such as K-Stacker, which can combine all data considering the expected planet orbit. Contrast limits of Cp ≈ 10−9 are within reach for ε Eri if the search can be optimized for a planet with a well-known orbit. This limit is also attainable for other bright nearby stars, such as α Cen or Sirius A. Such data also provide unprecedented sensitivity for the search of extended polarized emission from warm circumstellar dust.

Gender-specific analysis of social connection patterns and risk of dementia in community-dwelling older people.

Poor social connection is considered a risk factor for dementia. Since socializing behaviors may cluster together or act compensatorily, we aimed to investigate social connection patterns and their association with dementia, for men and women separately. A total of 12,896 community-dwelling older adults (mean±SD age: 75.2±4.3 years, 54% women) without major cognitive impairment were included. Latent class analysis was conducted using 24 baseline social connection indicators. Cox proportional hazards regression was used to estimate the association between latent classes and incident dementia over 12 (median: 8.4) years follow-up. Three distinct classes were identified in both genders: strong social connections with an intermediate friend-relative network (Class 1: men, 43.8%; women, 37.9%), weak social connections (Class 2: men, 29.6%; women, 27.4%), and strong social connections with a larger friend-relative network (Class 3: men, 26.6%; women, 34.7%). Compared to Class 1, men in Class 2 (HR: 1.38, 95% CI: 1.08-1.77) and women in Class 3 (HR: 1.27, 95% CI: 1.01-1.60) had an increased risk of dementia. Dementia risk varies with different social connection patterns among older men and women. Three distinct social connection patterns were identified based on 24 indicators. These patterns were related to dementia risk differently in men and women. In men, a weak social connection pattern was associated with a higher dementia risk. In women, a strong social connection with a relatively larger friend-relative network was associated with a greater dementia risk.

Mapping lower-limbs muscle vulnerability in patients with ALS: The role of upper and lower motor neurons

BackgroundSeveral studies have reported disproportionate wasting of the flexor muscles of the lower limbs (LL) compared to the extensors in patients with amyotrophic lateral sclerosis (ALS). However, these studies have involved small sample sizes (n 〈100), and their findings have been inconsistent. Thus, it remains uncertain whether a distinct pattern of LL muscle weakness is specific to ALS. AimsTo investigate the muscle weakness pattern in the LL at the knee, ankle, and toes in a large cohort of ALS patients and evaluate the relationship between the pattern of muscle strength and the extent of upper (UMN) and lower (LMN) motoneuron impairment. Material and methodsThe strength of flexor and extensor muscle was evaluated in 1250 legs of newly diagnosed ALS patients at the knee, ankle, and foot toes. UMN and LMN burden were assessed using validated scores. Within-subjects ANOVA considering the type of muscle (flexor/extensor) and anatomical sites (knee/ankle/toes) and mixed-factorial ANOVA were conducted to explore the impact of UMN and LMN impairments on the muscle weakness pattern. ResultsMuscle strength showed a significant decline from proximal to distal regions. Indeed both flexor and extensor muscles at the knee outperformed those at the ankle and toes. Within each site, extensor muscles exhibited less strength than flexor, except at the knee. Patients with heightened UMN impairment showed a more marked difference between flexors and extensors within each site, with extensor muscles being more compromised at the ankle and toes. Higher LMN impairment corresponded to a more pronounced weakness in flexor muscles at the ankle and toes compared to those at the knee. ConclusionsThe extensor muscle at the knee and the flexors at the foot and toes displayed relative resistance to ALS disease. UMN impairment amplified the differences between flexor and extensor muscles within each site, while LMN impairment demonstrated a clear distal-to-proximal vulnerability.

Procedural generation of geometric patterns for thin shell fabrication

This paper addresses the design of surface shells as assemblies of tileable, flat geometric patterns with predictable performance in response to mechanical stimuli. We design a family of tileable and fabricable patterns represented as triangle meshes, which can be assembled for creating surface tessellations. First, a regular recursive subdivision of the planar space generates different geometric configurations for candidate patterns, having interesting and varied aesthetic properties. Then, a refinement step addresses manufacturability by solving for non-manifold configurations and sharp angles that would produce disconnected or weak patterns. We devise a strategy for creating continuous variations on the geometry of individual patterns, in both aesthetics and behavior, to enrich the catalog of available designs. Finally, we simulate our patterns to evaluate their mechanical response when loaded in different scenarios targeting out-of-plane bending. Through a simple browsing interface, we show that our patterns span a variety of different bending behaviors. The result is a catalog of patterns with varied aesthetics and predefined mechanical behavior, to use for the direct design of mechanical metamaterials. To assess the feasibility of our design-to-fabricate approach, we show fabricated 3D objects with different curvatures, and compare physical and simulated experiments.

Immunohistochemistry study of PHOX2B expression in Hirschsprung's disease allied disorders

ObjectiveThis study aimed to investigate the significance of PHOX2B expression in Hirschsprung's disease allied disorders (HAD). MethodsEight specimens of HAD were surgically resected and collected at Beijing Children's Hospital Affiliated to Capital Medical University Heilongjiang Hospital in recent years. Additionally, three specimens from patients with immature ganglion development in the intestinal wall and six control specimens from normal colons were also collected. PHOX2B, S-100, and CR antibodies were used for immunohistochemical staining to observe their expression at the mucosal, submucosal, and intrinsic muscular layer ganglia. ResultsIn the control group,the nuclei of the submucosal and myenteric ganglion cells showed strong or weak PHOX2B staining pattern,the mature ganglion cells showed weak PHOX2B staining,and the immature ganglion cells showed strong staining;S-100 only stained nerve fibers but not ganglion cells; CR stained nerve fibers in the mucosal layer and submucosal layer and also stained ganglion cells. These three antibodies showed different expression patterns in poorly developed enteric ganglia and immature enteric ganglion cells. ConclusionPHOX2B is only expressed in the nucleus of ganglion cells, has discriminatory significance in identifying mature and immature ganglion cells, and is an essential marker for diagnosing HAD.

Distinct Central and Eastern Pacific El Niño Influence on Antarctic Surface Mass Balance

AbstractThe El Niño‐Southern Oscillation causes anomalous atmospheric circulation, temperature and precipitation across southern polar latitudes, but the influence of Central and Eastern Pacific El Niño events on Antarctic surface mass balance and snow accumulation has not yet been assessed. Here, we use reanalysis and reanalysis‐forced regional climate model output and find that Central Pacific El Niño results in significantly increased snow accumulation in the western Ross Sea sector and significantly decreased snow accumulation in the Amundsen Sea sector. Eastern Pacific El Niño is associated with similar but weaker patterns, with some regional exceptions. In some areas, like Dronning Maud Land, or the Wilkes Subglacial Basin, the effect of El Niño on snow accumulation changes from increased to reduced accumulation depending on the type of El Niño. Our results show that projecting El Niño types is important for constraining future changes in Antarctic surface mass balance.

Examining the elements of culturally relevant pedagogy captured and missed in a measure of high-quality mathematics instruction

Mathematics instruction is not race or culture neutral. For students who have been historically marginalized in mathematics classrooms, high-quality mathematics instruction, instruction that helps students build conceptual understanding, on its own might not be enough to disrupt inequities. These students might also need instruction that is culturally relevant, with teachers who demonstrate cultural competence, build critical consciousness, and support student learning. Our goal in this study was to understand which components of culturally relevant pedagogy (CRP) are captured and which are missed in a typical U.S. framework of high-quality mathematics instruction. To find the overlaps and gaps, we analyzed the mathematics lessons of three elementary teachers through both the lens of CRP and the Mathematics-Scan, a mathematics observation tool. We found the strongest overlap between the two frameworks in the patterns of strengths and weaknesses in the teachers’ lessons. When the teachers were delivering high-quality instruction, they were also often supporting students’ learning or showing cultural competence. When the teachers were delivering lower quality instruction, they were also often missing opportunities to enact CRP. At the same time, key elements of CRP including linguistic support for students, high expectations, critical consciousness, and nuances within cultural competence, were missed by the high-quality instruction framework. High-quality instruction was the foundation for CRP in the teachers’ classrooms, but CRP was more than just high-quality instruction. We conclude with recommendations for increasing the alignment between the frameworks and implications for international educators also grappling with equity in their own frameworks of mathematics instruction.

Towards understanding fermion masses and mixings

The Standard Model does not constrain the form of the Yukawa matrices and thus the origin of fermion mass hierarchies and mixing pattern remains puzzling. On the other hand, there are intriguing relations between the quark masses and their weak mixing angles, such as the well-known relationship [Formula: see text] for the Cabibbo angle, which may point towards specific textures of Yukawa matrices hypothesized by Harald Fritzsch at the end of the 70s. Though the original ansatz of Fritzsch is excluded by the experimental data, one can consider its minimal modification which consists in introducing an asymmetry between the 23 and 32 entries in the down-quark Yukawa matrix. We show that this structure is perfectly compatible with the present precision data on quark masses and CKM mixing matrix, and theoretically it can be obtained in the context of [Formula: see text] model with inter-family [Formula: see text] symmetry. We also discuss some alternative approaches which could give a natural description of the fermion mass spectrum and weak mixing pattern.

Recognition Algorithm of AE Signal of Rock Fracture Based on Multiscale 1DCNN-BLSTM

Acoustic emission (AE) signals produced by different types of rocks have different characteristics of information. Determining the brittle mineral content of rock according to the acoustic emission characteristics of rock is helpful to understand the mechanical behavior of rock in field monitoring. This article constructs a deep learning algorithm model to identify acoustic emission signals released from rock fractures with different brittle mineral contents. In response to the interference characteristics of acoustic emission signal data, a multiscale one-dimensional convolutional neural network embedded with efficient channel attention (ECA) module was incorporated into the model, and multiscale convolutional kernels were used to extract features of different levels of precision. In the latter half of the model, the BLSTM network was incorporated to extract time series-related features, local spatial uncorrelated features, and weak periodic pattern features from the acoustic emission signal data. To solve the problem that the recognition accuracy of minority samples decreases, this study replaces ReLU activation function with SELU. The results show that the multiscale 1DCNN-BLSTM model embedded in ECA module has a good antinoise performance, and the recognition accuracy can reach over 90%. The discovery of this work provides a new idea for exploring the mechanism of rock mass instability.

P.064 A case of late onset Pompe Disease presenting in 6th decade

Background: Late onset Pompe disease (LOPD), rare autosomal recessive lysosomal storage disease, resulting from mutation in alpha glucosidase enzyme (GAA) can present even in 6th decade of life. Slowly progressive, subtle, limb girdle pattern of weakness (LGPW), with auxiliary features such as ptosis, enlarged tongue, axial rigidity, facial diplegia, variable degree of respiratory weakness is not uncommon. Hypertrophic and electrical cardiac abnormalities are well described in LOPD. Methods: We present a case of 67-year-old male presenting with proximal weakness, subtle ptosis, bilateral quadriceps and shoulder girdle atrophy, and left toe numbness. PMHx: CABG, NSTEMI. Statin use. FMHx: noncontributory. Results: EMG: L5 radiculopathy, with unexpected myopathic units in hip/pelvic/ shoulder girdle muscles with active denervation and muscle irritability. CK, CRP, SPEP, ANA, LFTs, HMG-CoA reductase: normal. GAA enzymatic activity=0.96µmol/L/hr (low), genetics: pathogenic variants in GAA gene: c.-32-13T>G and c.1194+3G>C. ECHO: severe diastolic dysfunction, restrictive left ventricular filling. PFTs: normal. Diagnosed with LOPD, started on therapy. Conclusions: LOPD remains a differential for LGPW especially in older patient population with history of cardiopulmonary features. Age-appropriate conconminant pathologies may confound the diagnostic process.Symptoms may preceed diagnosis for years.GAA enzymatic activity followed by genetic testing remains readily available and can confirm diagnosis, preventing delay of approved therapy.

MR Imaging Features of Critical Spinal Demyelinating Lesions Associated with Progressive Motor Impairment.

Progressive MS is typically heralded by a myelopathic pattern of asymmetric progressive motor weakness. Focal individual "critical" demyelinating spinal cord lesions anatomically associated with progressive motor impairment may be a compelling explanation for this clinical presentation as described in progressive solitary sclerosis (single CNS demyelinating lesion), progressive demyelination with highly restricted MR imaging lesion burden (2-5 total CNS demyelinating lesions; progressive paucisclerotic MS), and progressive, exclusively unilateral hemi- or monoparetic MS (>5 CNS demyelinating progressive unilateral hemi- or monoparetic MS [PUHMS] lesions). Critical demyelinating lesions appear strikingly similar across these cohorts, and we describe their specific spinal cord MR imaging characteristics. We performed a retrospective, observational MR imaging study comparing spinal cord critical demyelinating lesions anatomically associated with progressive motor impairment with any additional "noncritical" (not anatomically associated with progressive motor impairment) spinal cord demyelinating lesions. All spinal cord MR images (302 cervical and 91 thoracic) were reviewed by an experienced neuroradiologist with final radiologic assessment on the most recent MR imaging. Anatomic association with clinical progressive motor impairment was confirmed independently by MS subspecialists. Ninety-one individuals (PUHMS, 37 [41%], progressive paucisclerosis 35 [38%], progressive solitary sclerosis 19 [21%]) with 91 critical and 98 noncritical spinal cord MR imaging demyelinating lesions were evaluated. MR imaging characteristics that favored critical spinal cord demyelinating lesions over noncritical lesions included moderate-to-severe, focal, lesion-associated spinal cord atrophy: 41/91 (45%) versus 0/98 (0%) (OR, 161.91; 9.43 to >999.9); lateral column axial location (OR, 10.43; 3.88-28.07); central region (OR, 3.23; 1.78-5.88); ventral column (OR, 2.98; 1.55-5.72); and larger lesion size of the axial width (OR, 2.01;1.49-2.72), transverse axial size (OR, 1.66; 1.36-2.01), or lesion area (OR, 1.14; 1.08-1.2). Multiple regression analysis revealed focal atrophy and lateral axial location as having the strongest association with critical demyelinating lesions. Focal, lesion-associated atrophy, lateral column axial location, and larger lesion size are spinal cord MR imaging characteristics of critical demyelinating lesions. The presence of critical demyelinating lesions should be sought as these features may be associated with the development of progressive motor impairment in MS.