67 research outputs found

    Compressive characterization of telecom photon pairs in the spatial and spectral degrees of freedom

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    In the past few years, physicists and engineers have demonstrated the possibility of utilizing multiple degrees of freedom of the photon to perform information processing tasks for a wide variety of applications. Furthermore, complex states of light offer the possibility of encoding and processing many bits of information in a single photon. However, the challenges involved in the process of extracting large amounts of information, encoded in photonic states, impose practical limitations to realistic quantum technologies. Here, we demonstrate characterization of quantum correlated photon pairs in the spatial and spectral degrees of freedom. Our technique utilizes a series of random projective measurements in the spatial basis that do not perturb the spectral properties of the photon. The sparsity in the spatial properties of downconverted photons allows us to exploit the potential of compressive sensing to reduce the number of measurements to reconstruct spatial and spectral properties of correlated photon pairs at telecom wavelength. We demonstrate characterization of a photonic state with 12 × 109 dimensions using only 20% of the measurements with respect to the conventional raster scan technique. Our characterization technique opens the possibility of increasing and exploiting the complexity and dimensionality of quantum protocols that utilize multiple degrees of freedom of light with high efficiency

    Electron-lattice kinetics of metals heated by ultrashort laser pulses

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    We propose a kinetic model of transient nonequilibrium phenomena in metals exposed to ultrashort laser pulses when heated electrons affect the lattice through direct electron-phonon interaction. This model describes the destruction of a metal under intense laser pumping. We derive the system of equations for the metal, which consists of hot electrons and a cold lattice. Hot electrons are described with the help of the Boltzmann equation and equation of thermoconductivity. We use the equations of motion for lattice displacements with the electron force included. The lattice deformation is estimated immediately after the laser pulse up to the time of electron temperature relaxation. An estimate shows that the ablation regime can be achieved.Comment: 7 pages; Revtex. to appear in JETP 88, #1 (1999

    Immunopathogenesis and proposed clinical score for identifying Kelch-like protein-11 encephalitis

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    In this study, we report the clinical features of Kelch-like protein 11 antibody-associated paraneoplastic neurological syndrome, design and validate a clinical score to facilitate the identification of patients that should be tested for Kelch-like protein 11 antibodies, and examine in detail the nature of the immune response in both the brain and the tumour samples for a better characterization of the immunopathogenesis of this condition. The presence of Kelch-like protein 11 antibodies was retrospectively assessed in patients referred to the French Reference Center for paraneoplastic neurological syndrome and autoimmune encephalitis with (i) antibody-negative paraneoplastic neurological syndrome [limbic encephalitis (n = 105), cerebellar degeneration (n = 33)] and (ii) antibody-positive paraneoplastic neurological syndrome [Ma2-Ab encephalitis (n = 34), antibodies targeting N-methyl-D-aspartate receptor encephalitis with teratoma (n = 49)]. Additionally, since 1 January 2020, patients were prospectively screened for Kelch-like protein 11 antibodies as new usual clinical practice. Overall, Kelch-like protein 11 antibodies were detected in 11 patients [11/11, 100% were male; their median (range) age was 44 (35-79) years], 9 of them from the antibody-negative paraneoplastic neurological syndrome cohort, 1 from the antibody-positive (Ma2-Ab) cohort and 1 additional prospectively detected patient. All patients manifested a cerebellar syndrome, either isolated (4/11, 36%) or part of a multi-system neurological disorder (7/11, 64%). Additional core syndromes were limbic encephalitis (5/11, 45%) and myelitis (2/11, 18%). Severe weight loss (7/11, 64%) and hearing loss/tinnitus (5/11, 45%) were common. Rarer neurologic manifestations included hypersomnia and seizures (2/11, 18%). Two patients presented phenotypes resembling primary neurodegenerative disorders (progressive supranuclear palsy and flail arm syndrome, respectively). An associated cancer was found in 9/11 (82%) patients; it was most commonly (7/9, 78%) a spontaneously regressed ('burned-out') testicular germ cell tumour. A newly designed clinical score (MATCH score: male, ataxia, testicular cancer, hearing alterations) with a cut-off ≥4 successfully identified patients with Kelch-like protein 11 antibodies (sensitivity 78%, specificity 99%). Pathological findings (three testicular tumours, three lymph node metastases of testicular tumours, one brain biopsy) showed the presence of a T-cell inflammation with resulting anti-tumour immunity in the testis and one chronic, exhausted immune response - demonstrated by immune checkpoint expression - in the metastases and the brain. In conclusion, these findings suggest that Kelch-like protein 11 antibody paraneoplastic neurological syndrome is a homogeneous clinical syndrome and its detection can be facilitated using the MATCH score. The pathogenesis is probably T-cell mediated, but the stages of inflammation are different in the testis, metastases and the brain

    A review of the use of terrestrial laser scanning application for change detection and deformation monitoring of structures

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    Change detection and deformation monitoring is an active area of research within the field of engineering surveying as well as overlapping areas such as structural and civil engineering. The application of Terrestrial Laser Scanning (TLS) techniques for change detection and deformation monitoring of concrete structures has increased over the years as illustrated in the past studies. This paper presents a review of literature on TLS application in the monitoring of structures and discusses registration and georeferencing of TLS point cloud data as a critical issue in the process chain of accurate deformation analysis. Past TLS research work has shown some trends in addressing issues such as accurate registration and georeferencing of the scans and the need of a stable reference frame, TLS error modelling and reduction, point cloud processing techniques for deformation analysis, scanner calibration issues and assessing the potential of TLS in detecting sub-centimetre and millimetre deformations. However, several issues are still open to investigation as far as TLS is concerned in change detection and deformation monitoring studies such as rigorous and efficient workflow methodology of point cloud processing for change detection and deformation analysis, incorporation of measurement geometry in deformation measurements of high-rise structures, design of data acquisition and quality assessment for precise measurements and modelling the environmental effects on the performance of laser scanning. Even though some studies have attempted to address these issues, some gaps exist as information is still limited. Some methods reviewed in the case studies have been applied in landslide monitoring and they seem promising to be applied in engineering surveying to monitor structures. Hence the proposal of a three-stage process model for deformation analysis is presented. Furthermore, with technological advancements new TLS instruments with better accuracy are being developed necessitating more research for precise measurements in the monitoring of structures

    Natural History, Phenotypic Spectrum, and Discriminative Features of Multisystemic RFC1-disease

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    OBJECTIVE: To delineate the full phenotypic spectrum, discriminative features, piloting longitudinal progression data, and sample size calculations of RFC1-repeat expansions, recently identified as causing cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS). METHODS: Multimodal RFC1 repeat screening (PCR, southern blot, whole-exome/genome (WES/WGS)-based approaches) combined with cross-sectional and longitudinal deep-phenotyping in (i) cross-European cohort A (70 families) with ≥2 features of CANVAS and/or ataxia-with-chronic-cough (ACC); and (ii) Turkish cohort B (105 families) with unselected late-onset ataxia. RESULTS: Prevalence of RFC1-disease was 67% in cohort A, 14% in unselected cohort B, 68% in clinical CANVAS, and 100% in ACC. RFC1-disease was also identified in Western and Eastern Asians, and even by WES. Visual compensation, sensory symptoms, and cough were strong positive discriminative predictors (>90%) against RFC1-negative patients. The phenotype across 70 RFC1-positive patients was mostly multisystemic (69%), including dysautonomia (62%) and bradykinesia (28%) (=overlap with cerebellar-type multiple system atrophy [MSA-C]), postural instability (49%), slow vertical saccades (17%), and chorea and/or dystonia (11%). Ataxia progression was ∼1.3 SARA points/year (32 cross-sectional, 17 longitudinal assessments, follow-up ≤9 years [mean 3.1]), but also included early falls, variable non-linear phases of MSA-C-like progression (SARA 2.5-5.5/year), and premature death. Treatment trials require 330 (1-year-trial) and 132 (2-year-trial) patients in total to detect 50% reduced progression. CONCLUSIONS: RFC1-disease is frequent and occurs across continents, with CANVAS and ACC as highly diagnostic phenotypes, yet as variable, overlapping clusters along a continuous multisystemic disease spectrum, including MSA-C-overlap. Our natural history data help to inform future RFC1-treatment trials. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that RFC1-repeat expansions are associated with CANVAS and ACC

    Natural history, phenotypic spectrum, and discriminative features of multisystemic RFC1 disease

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    Objective To delineate the full phenotypic spectrum, discriminative features, piloting longitudinal progression data, and sample size calculations of replication factor complex subunit 1 (RFC1) repeat expansions, recently identified as causing cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS). Methods Multimodal RFC1 repeat screening (PCR, Southern blot, whole-exome/genome sequencing?based approaches) combined with cross-sectional and longitudinal deep phenotyping in (1) cross-European cohort A (70 families) with ?2 features of CANVAS or ataxia with chronic cough (ACC) and (2) Turkish cohort B (105 families) with unselected late-onset ataxia. Results Prevalence of RFC1 disease was 67% in cohort A, 14% in unselected cohort B, 68% in clinical CANVAS, and 100% in ACC. RFC1 disease was also identified in Western and Eastern Asian individuals and even by whole-exome sequencing. Visual compensation, sensory symptoms, and cough were strong positive discriminative predictors (>90%) against RFC1-negative patients. The phenotype across 70 RFC1-positive patients was mostly multisystemic (69%), including dysautonomia (62%) and bradykinesia (28%) (overlap with cerebellar-type multiple system atrophy [MSA-C]), postural instability (49%), slow vertical saccades (17%), and chorea or dystonia (11%). Ataxia progression was ?1.3 Scale for the Assessment and Rating of Ataxia points per year (32 cross-sectional, 17 longitudinal assessments, follow-up ?9 years [mean 3.1 years]) but also included early falls, variable nonlinear phases of MSA-C?like progression (SARA points 2.5?5.5 per year), and premature death. Treatment trials require 330 (1-year trial) and 132 (2-year trial) patients in total to detect 50% reduced progression. Conclusions RFC1 disease is frequent and occurs across continents, with CANVAS and ACC as highly diagnostic phenotypes yet as variable, overlapping clusters along a continuous multisystemic disease spectrum, including MSA-C-overlap. Our natural history data help to inform future RFC1 treatment trials. Classification of Evidence This study provides Class II evidence that RFC1 repeat expansions are associated with CANVAS and ACC.FUNDING: Study Funding This work was supported via the European Union’s Horizon 2020 research and innovation program by the BMBF under the frame of the E-Rare-3 network PREPARE (01GM1607; to M. Synofzik,M.A., H.P., B.P.v.d.W.), by the DFG under the frame of EJP-RD network PROSPAX (No. 441409627; M. Synofzik, B.P.v.d.W., A.N.B.), and grant 779257 “Solve-RD” (toM. Synofzik, B.P.v.d.W.). B.P.v.d.W. receives additional research support from ZonMW, Hersenstichting, Gossweiler Foundation, uniQure, and Radboud University Medical Centre. T.B.H. was supported by the DFG (No 418081722). A.T. receives funding from the University of T¨ubingen, medical faculty, for the Clinician Scientist Program grant 439-0-0. A.C. thanks Medical Research Council, MR/T001712/1) and Fondazione CARIPLO (2019-1836) for grant support. L.S., T.K., B.P.v.d.W., and M. Synofzik are members of the European Reference Network for Rare Neurological Diseases, project 739510. A.N.B. is supported by the Suna and Inan Kirac Foundation and Koç University School of Medicine

    Glucosinolates in three Brassicales of French Polynesia

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