Miracle-dz/UniFAR-FTUs huggingface dataset:
Regional spreading pattern is associated with clinical phenotype in amyotrophic lateral sclerosis

Abstract Increasing evidence shows that disease spreading in amyotrophic lateral sclerosis (ALS) follows a preferential pattern with more frequent involvement of contiguous regions from the site of symptom onset. The aim of our study was to assess if: (i) the burden of upper (UMN) and lower motor neuron (LMN) involvement influences directionality of disease spreading; (ii) specific patterns of disease progression are associated with motor and neuropsychological features of different ALS subtypes (classic, bulbar, primary lateral sclerosis, UMN-predominant, progressive muscular atrophy, flail arm, flail leg); and (iii) specific clinical features may help identify ALS subtypes, which remain localized to the site of onset for a prolonged time (regionally entrenching ALS). A single-centre, retrospective cohort of 913 Italian ALS patients was evaluated to assess correlations between directionality of the disease process after symptom onset and motor/neuropsychological phenotype. All patients underwent an extensive evaluation including the following clinical scales: Penn Upper Motor Neuron Score (PUMNS), MRC Scale for Muscle Strength and the Edinburgh Cognitive and Behavioural ALS Screen (ECAS). The most frequent initial spreading pattern was that towards adjacent horizontal regions (77.3%), which occurred preferentially in patients with lower MRC scores (P = 0.038), while vertical diffusion (21.1%) was associated with higher PUMNS (P < 0.001) and with reduced survival (P < 0.001). Non-contiguous disease spreading was associated with more severe UMN impairment (P = 0.003), while contiguous disease pattern with lower MRC scores. Furthermore, non-contiguous disease spreading was associated with more severe cognitive impairment in both executive and visuospatial ECAS domains. Individuals with regionally entrenching ALS were more frequently female (45.6% versus 36.9%; P = 0.028) and had higher frequencies of symmetric disease onset (40.3% versus 19.7%; P < 0.001) and bulbar phenotype (38.5% versus 16.4%; P < 0.001). Our study suggests that motor phenotypes characterized by a predominant UMN involvement are associated with a vertical pattern of disease progression reflecting ipsilateral spreading within the motor cortex, while those with predominant LMN involvement display more frequently a horizontal spreading from one side of the spinal cord to the other. These observations raise the hypothesis that one of the mechanisms underlying disease spreading in ALS pathology is represented by diffusion of toxic factors in the neuron microenvironment. Finally, it is possible that in our cohort, regionally entrenching ALS forms are mainly observed in patients with atypical bulbar phenotypes, characterized by a slowly progressive course and relatively benign prognosis.
Antitussive efficacy of the current treatment protocol for refractory chronic cough: our real-world experience in a retrospective cohort study

Background: The management of refractory chronic cough (RCC) is a great challenge. Neuromodulators have long been used for RCC with imperfect efficacy. Objectives: We summarized the outcomes of the current treatments used at our specialist cough clinic, which provides a guideline-led service and real-world experience for the future management of RCC. Design: This is a single-centre retrospective observational cohort study. Methods: Consecutive RCC patients (the first clinic visit between January 2016 and May 2021) were included into this observational cohort study. Medical records in the Chronic Cough Clinical Research Database were fully reviewed using uniform criteria. The included subjects were followed-up for at least 6 months after the final clinic visit via instant messages with the link to self-scaled cough-associated questionnaires. Results: Overall, 369 RCC patients were analysed with a median age of 46.6 years and a cough duration of 24.0 months. A total of 10 different treatments were offered. However, 96.2% of patients had been prescribed at least one neuromodulator. One-third of patients had alternative treatments prescribed given the poor response to the initial therapy and 71.3% favourably responded to at least one of the treatments. Gabapentin, deanxit, and baclofen had comparable therapeutic efficacy (56.0%, 56.0%, and 62.5% respectively; p = 0.88) and overall incidences of adverse effects (28.3%, 22.0%, and 32.3% respectively; p = 0.76). However, 19.1 (7.7–41.8) months after the last clinic visit, 65.0% reported improvement (24.9%) or control of their cough (40.1%); 3.8% reported a spontaneous remission and 31.2% still had a severe cough. Both HARQ (n = 97; p < 0.001) and LCQ (n = 58; p < 0.001) demonstrated marked improvement. Conclusion: Trying different neuromodulators is a pragmatic strategy for RCC, which helped around two-thirds of patients. Relapse is common on withdrawal or reduction of dosage. Novel medication for RCC is an urgent clinical need. Plain language summary This is the first report that fully represented a guideline-led treatment protocol for refractory chronic cough (RCC) based on a large series of patients, which evaluated the short- and long-term effects of the currently available treatments for RCC. We found that the therapeutic trial of different neuromodulators is a pragmatic strategy, which helped around two-thirds of patients. Gabapentin, deanxit (flupentixol/melitracen), and baclofen had similar therapeutic outcomes. This study may offer real-world experience for the future management of RCC.
A Free-Field Strong Motion Network in Taiwan:TSMIP

The famous Chapman-Ferraro problem for the determination of themagnetospheric cavity is elucidated for its two-dimensional solutions inmathematical detail.The general solution presented in this paper amountsto a complex variable extension of the well-known Poisson's integral formulafor the Dirichlet problem for a half-plane or a circle domain.It providesthe needed methodology for obtaining desirable solutions that avoidthe defects inherent in the Dungey-Hurley solution,which uses Ferraro'sapproximation of pressure balance as the boundary condition.
Sesquiterpenoids from the Resina Commiphora Promoting the Apoptotic Activity of PC‐3 Prostate Cancer Cells

Four new germacrane‐type sesquiterpenes commiphoranes M1‐M4 (1‐4) together with eighteen sesquiterpenes were isolated from the Resina Commiphora. The structures and relative configurations of new substances were determined by using spectroscopic methods. Biological activity investigation revealed that nine compounds including 7, 9, 14, 16, (+)‐17, (−)‐17, 18, 19, and 20 could induce the apoptosis of prostate cancer originated PC‐3 cells, through classic apoptosis signaling pathway, even using flow cytometry showed that the compound (+)‐17 caused apoptosis of PC‐3 cells more than 40 %, suggesting their potential therapeutic application in the development of novel drugs against prostate cancer.
Mismatch repair–deficient glioma with spatially distinct IDH-mutant and IDH-wild type components arising in the setting of Lynch syndrome

Pathogenic mutations in MLH1, MSH2, PMS2, and MSH6 compromise DNA mismatch repair mechanisms and in the heterozygous state result in Lynch syndrome, which is typified by a predisposition to endometrial, ovarian, colorectal, gastric, breast, hematologic, and soft tissue cancers. Rarely, germline pathogenic aberrations in these genes are associated with the development of primary central nervous system tumors. We present a report of an adult female with no prior cancer history who presented with a multicentric, infiltrative supratentorial glioma involving both the left anterior temporal horn and left precentral gyrus. Surgical treatment and neuropathological/molecular evaluation of these lesions revealed discordant isocitrate dehydrogenase (IDH) status and histologic grade at these spatially distinct disease sites. A frameshift alteration within the MLH1 gene (p.R217fs*12, c.648delT) was identified in both lesions and subsequently identified in germline testing of a blood sample, consistent with Lynch syndrome. Despite distinct histopathologic features and divergent IDH status of the patient's tumors, the molecular findings suggest that both sites of intracranial neoplasia may have developed as a consequence of underlying monoallelic germline mismatch repair deficiency. This case illustrates the importance of characterizing the genetic profile of multicentric gliomas and highlights the oncogenic potential of germline mismatch repair gene pathogenic alterations within central nervous system gliomas.
Length-biased Weighted Maxwell Distribution

The concept of length-biased distribution can be employed in development of proper models for life-time data. In this paper, we develop the length-biased form of Weighted Maxwell distribution (WMD). We study the statistical properties of the derived distribution including moments, moment generating function, hazard rate, reverse hazard rate, Shannon entropy and estimation of parameters
Tests of a Theory of Human Image Understanding: Part II. The Perception of Degraded Objects

Two experiments tested the predictions of a recent theory of object recognition (Recognition-By-Components or RBC). The first experiment supports RBC's predictions that recognition is accomplished by ascertaining the object's 3D components in their specified relations by utilizing certain nonaccidental 2D image properties. When object components were rendered nonrecoverable by deleting their nonaccidental properties recognition in an object naming task was interfered with more than when an equal amount of contour was deleted without erasing such properties. The second experiment supported RBC's assumption that vertices constitute one of these important nonaccidental properties. In a naming task objects were recognized more accurately when contour was deleted from midsegment than when an equal amount was deleted from the objects vertices.
The Plasma Environment of Prominences – SOHO Observations

We describe the observational capabilities of the SOHO EIT and LASCO instruments for prominence research. A detailed comparison of EIT Hell A304 and BBSO Ha images of a quiescent prominence reveals differences in fine scale structure which are attributed to differences in ra diation emission and transport mechanisms. Absorption features present in coronal EIT images are attributed to dense prominence features lo cated between the source of the coronal emission and the observer. These features provide the locations of prominence material in coronal image se quences. Observations of a prominence eruption in both EIT Hell and LASCO CI FeXIV images show the heating of chromospheric material to coronal temperatures during the prominence eruption. In the last phase of the prominence eruption, a coronal mass ejection (CME) is initiated as a dark cavity forms over the prominence material and a bright loop-like structure forms over the dark cavity.
Toward the Meaning of Linguistic Signs: A Hierarchical Theory

The primary function of a linguistic sign is to express and convey meaning; thus meaning is a central issue in linguistic semiotics. Throughout the history of the humanities, the definition of “meaning” has remained controversial. From an ontological perspective, there have been varied approaches to the problem of meaning and these approaches have helped to generate different ways of understanding meaning. Hence the dyadic model, the triadic model, the semiotic triangle, the semantic trapezoid, and the semantic quadrangle. This essay proposes a hierarchical theory for the meaning of the linguistic sign, and argues that language is a system of signs with manifold layers, and the meaning of the linguistic sign is correspondingly characterized by homogeneity and complexity. In the process of meaning construction, the signifier and the signified are continuously combined, forming different layers: the sign of object (phonetic shell + reflected thing) in the deep layer, the sign of langue (phonetic word + generic thing) in the shallow layer, and the sign of parole (phonetic complex + actual thing) in the surface layer. These respectively resemble the associative name, the abstract name, and the concrete name of a thing, represent the signification, the denotation, and the referentiation of a concept, and reflect perceptual meaning, cognitive meaning, and contextual meaning. Within the theoretical framework this essay proposes, the sign, the thing, the concept, and the semiotic meaning are the core elements which together constitute the world of meaning where the real value of the linguistic sign lies.
Multi-fault localization based on knowledge graph combined with network topology in optical networks

When faults occur in optical networks, many derivative alarms are generated from the root alarms, because of the derived properties of optical network alarms. In a large and complex optical network, the management system will receive tremendous alarms even though there is only one actual fault. Therefore, accurate fault localization has significance for network operation and maintenance. The complex relationship between alarms and faults makes it difficult to locate faults, especially multiple faults. To solve this problem, this paper proposes a fault localization method based on knowledge graph and network topology information. Using the information association ability of the knowledge graph, the implicit relationship between the alarm data is mined, and the graph neural network is used to perform automatic fault reasoning on the alarm knowledge graph. Further, the network topology information is integrated into the fault localization, which increases the dimension of the knowledge graph. Simulation results show that the proposed method can achieve 97% accuracy in multiple fault localization scenarios.
Characteristic simulation and application of photonic crystal fiber

This paper introduces a simulation analysis software COMSOL Multiphysics about the finite element method, and the application method of this software in the simulation analysis of photonic crystal fibers. The high frequency electromagnetic field module (RF Module) of the software is used to analyze the mode of photonic crystal fiber, and the characteristics of mode effective index, cladding effective index, normalized frequency and effective mode field area are further discussed. At the wavelength of 1. 55μm and the refractive index of the material of 1.45, the effects of structural parameters such as different air hole cladding layers, the same duty cycle and different duty cycles on the characteristics are analyzed.
Study of the effect of the low-pass filter time constant on the noise level of quartz-enhanced photoacoustic spectroscopy sensors

Quartz Enhanced Photoacoustic Spectroscopy (QEPAS) is a sensitive trace gas detection technique employing Quartz Tuning Forks (QTF) as sensitive elements. Usually, a transimpedance amplifier is used as QTF front-end electronic, with a 10 MΩ feedback resistor. With a low-pass filter time constant of 100 ms, the thermal noise of the QTF intrinsic resistance (~100 kΩ) represents the main noise contribution. For real-time applications, shorter time constants need to be employed. In this work, we studied different amplifier structures with the aim to reduce the QTF thermal noise and improve the signalto- noise ratio when the QTF is used in QEPAS sensors.
A type of fiber tag based on Bragg gratings array fabricated by femtosecond laser point-by-point inscription

Due to the exponential growth of data in the information society, there is a growing number of optical fibers as the main channels for data transmission. How to label the fiber channels clearly is one of the most critical issues to maintain the normal flow of work in communication systems. Conventional fiber channel labeling relies on physical numbering, which significantly increases the routine maintenance cost when dealing with numerous optical transmission links. This means that the existing methods can no longer meet the growing marking function of optical fiber transmission networks. As an important fiber device, fiber Bragg grating has shown great potential in optical fiber communication systems because of its good stability, anti-electromagnetic interference, and so on. In this work, we propose a fiber tag based on a fiber Bragg grating array fabricated by a highly efficient femtosecond-laser point-by-point inscription. Each Bragg grating in the fiber tag can be recognized by demodulating the time domain reflection signal characteristics of the fiber. By analyzing the spatial distribution information of the Bragg grating segment and demodulating the information carried by the fiber tag with the preset grating segment length, the feasibility of the information carried by the fiber tag is proved. Combined with the characteristics of Bragg grating with specific wavelength response, using different wavelengths of detection light to read the time-domain reflection signal of fiber tags, provides a method to improve the information storage capacity of fiber tags. This also implies that the wavelength range of the detection light will affect the acquired label information, which will bring interferences to the correct acquisition of the information carried by the tag. The proposed optical fiber tag is fully compatible with the existing fiber-optic communication network without additional physical labeling by using the physical characteristics as the basis of the coding mark, providing an innovative solution for the efficient labeling of numerous fiber transmission channels.
On-orbit autonomous navigation of geosynchronous satellite using novel infrared imaging Earth sensor

Novel Static Infrared Earth Sensor (SIES) was developed for high precision and strong adaptive autonomous optical navigation of Earth orbit satellites. The SIES was designed to obtain infrared image of the entire Earth through the focal plane detectors, which is conducive to obtaining more abundant boundary information, achieving higher measurement accuracy and rejecting irregular interference. By measuring the difference of infrared radiation between the earth and the space with SIES, the horizon circle formed by the earth’s atmosphere radiation circle was obtained, and the orientation of the earth in the inertial coordinate system was determined under integrated autonomous navigation framework. With high-precision Earth center vector measurement and autonomous on-orbit calibration abilities of SIES, the proposed autonomous navigation technology had been successfully verified on geosynchronous orbit, the position determination error was less than 9 km. The SIES is suitable for wide range types of satellites working on 17000 km to 70000 km orbit altitude by providing outstanding accuracy, large field of view, long-term high reliability, light weight and power efficiency.
Surface activation process of nickel-supported monolayer graphene

Electron source can be considerably benefited from the application of photocathodes with long lifetime, broad spectral response and high quantum efficiency. As graphene has exhibited potential to serve as the passivation layer to improve electron emission behavior of photocathodes due to its unique structure and properties, exploring photoemission phenomenon of metal-supported graphene material can help to develop robust photocathodes. We report the photoemission performance of the nickel-supported monolayer graphene through various activation processes. To investigate the effects of different activation processes on this composite material, we compare the photoemission behavior of the nickel-supported monolayer graphene by cesium (Cs)-only activation at room temperature, Cs-only activation under heated condition and cesium-oxygen (Cs/O) activation under heated condition. The experimental results show that these activation methods are able to promote photoemission ability of the nickel-supported monolayer graphene. Among them, the Cs/O activation process under heated condition can improve the photocurrent most significantly. This process validation of activating the nickel-supported graphene material provides a new route to enhance photoemission performance of metal photocathodes, and the activation method could be applied to the surface modification of other metal-supported graphene materials.
Research on virtual reality fusion method based on spatial marker location

Virtual reality fusion based on augmented reality has become a research hotspot, which is widely used in cultural relics exhibition, medical care and other fields. Spatial projection mapping matrix is the basis for projection equipment to project the prefabricated image onto the target surface. However, in practical operation, it is necessary to determine the relative position relationship between the projection equipment and the actual scene based on complex spatial target calibration. This paper aims to solve the problem of projection information dislocation and realize real-time tracking projection. A high-precision center positioning method based on the invariable characteristic of concentric circle intersection ratio is designed, and the mapping matrix from projection equipment to target is calculated based on PNP method. Finally, the distortion parameters of the lens are used to generate a projection pattern that can offset the projection distortion, so as to optimize the coincidence between the projection pattern and the real object, and achieve efficient and high-precision virtual reality fusion projection.
Deep phenotyping of PROM1-associated retinal degeneration

Background/aims The purpose of this study was to investigate retinal structure in detail of subjects with autosomal-dominant (AD) and autosomal-recessive (AR) PROM1-associated retinal degeneration (PROM1-RD), study design: institutional, cross-sectional study. Methods Four eyes from four subjects (three with AD and one with AR) PROM1-RD were investigated by ophthalmic examination including best-corrected visual acuity (BCVA) and multimodal retinal imaging: fundus autofluorescence (FAF), spectral-domain optical coherence tomography (SD-OCT) and adaptive optics scanning light ophthalmoscopy. Quantitative assessment of atrophic lesions determined by FAF, thickness of individual retinal layers and cone photoreceptor quantification was performed. Results BCVA ranged from 20/16 to 20/200. Initial pathological changes included the presence of hyperautofluorescent spots on FAF imaging, while later stages demonstrated discrete areas of atrophy. In all patients, thinning of the outer retinal layers on SD-OCT with varying degrees of atrophy could be detected depending on disease-causing variants and age. Cone density was quantified both in central and/or at different eccentricities from the fovea. Longitudinal assessments were possible in two patients. Conclusions PROM1-RD comprises a wide range of clinical phenotypes. Depending on the stage of disease, the cone mosaic in PROM1-RD is relatively preserved and can potentially be targeted by cone-directed interventions.
Thickness and strain effects on electronic structures of transition metal dichalcogenides: 2H- M X 2 semiconductors ( M = Mo, W; X = S, Se, Te)

Using the first-principles calculations, we explore the electronic structures of 2H-$M{X}_{2}$ ($M$ $=$ Mo, W; $X$ $=$ S, Se, Te). When the number of layers reduces to a single layer, the indirect gap of bulk becomes a direct gap with larger gap and the band curvatures are found to lead to the drastic changes of effective masses. On the other hand, when the strain is applied on the single layer, the direct gap becomes an indirect gap and the effective masses vary. Especially, the tensile strain reduces the gap energy and effective masses while the compressive strain enhances them. Furthermore, the much larger tensile stress leads to become metallic.