Journal of neuromuscular diseases最新文献

{"title":"Advancing the diagnosis of rare neuromuscular and neurological diseases through the collaborative Solve-RD research framework.","authors":"Lisa-Sophie Wüstner, Kornelia Ellwanger, Nika Schuermans, German Demidov, Kiran Polavarapu, Leslie Matalonga, Steven Laurie, Ana Töpf, Katja Lohmann, Holm Graessner","doi":"10.1177/22143602261460689","DOIUrl":"10.1177/22143602261460689","url":null,"abstract":"<p><p>Rare neuromuscular and neurological diseases (NMDs and RNDs) present diagnostic challenges due to their clinical heterogeneity and genetic complexity. Despite the advancements in next-generation sequencing (NGS) and other high-throughput genomic technologies, a significant proportion of patients with NMDs and RNDs remain undiagnosed. This is primarily due to genetic heterogeneity, the presence of novel or private variants, and incomplete variant detection by short-read sequencing platforms. The Solve-RD project, a pan-European initiative funded by the Horizon 2020 programme, established a robust interdisciplinary framework integrating expert clinical and bioinformatics teams through Data Interpretation Task Forces (DITFs) and Data Analysis Task Force (DATF). Focusing on previously undiagnosed NMD and RND patients, Solve-RD implemented a systematic reanalysis of exome/genome data. For specific cohorts, various omics approaches were added, including long-read genome sequencing, RNA sequencing, and optical genome mapping. This collaborative framework significantly improved diagnostic yield in RND and NMD cohorts and led to the identification of novel pathogenic variants and mechanisms. The Solve-RD model exemplifies how structured expert collaboration, data sharing and harmonisation, and cutting-edge multi-omics technologies can overcome current diagnostic limitations in rare disease research.</p>","PeriodicalId":16536,"journal":{"name":"Journal of neuromuscular diseases","volume":" ","pages":"22143602261460689"},"PeriodicalIF":3.5,"publicationDate":"2026-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13438582/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148447154","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Navigating sexual health, fertility, and adult wellness in individuals with Duchenne muscular dystrophy: Current standards of care and future directions.","authors":"Nat Nasomyont, Amanda Appel, Susan Apkon, Janet Hoskin, Prasanth N Surampudi, Natalie Truba, Gabriela Vargas, Aravind Veerapandiyan, Claire L Wood, Patrick Moeschen, Colin Werth, Alexandra Ahmet, Shipra Bansal, Lindsey Bratland, Janet L Crane, Melissa Fiscaletti, Rana Halloun, Kathi Kinnett, Carol Lam, Laura McAdam, Hugh J McMillan, Meilan M Rutter, Anne Marie Sbrocchi, Mena Scavina, Jaclyn Tamaroff, Cuixia Tian, Leanne M Ward, David R Weber, Sze Choong Wong, Nora E Renthal","doi":"10.1177/22143602261454423","DOIUrl":"10.1177/22143602261454423","url":null,"abstract":"<p><p>Many adolescents and young adults with Duchenne Muscular Dystrophy (DMD) face challenges related to sexuality, relationships, and fertility, which can negatively impact their quality of life. Current clinical guidelines emphasize the need for healthcare providers to address these aspects. Yet, there remains a lack of robust data regarding the implications of DMD on sexual and reproductive health. This article highlights the essential psychosocial topics that require attention. By reviewing existing literature and focusing on gaps in care, we aim to foster future research directions that will enhance the management and support strategies for individuals with DMD, ultimately improving their quality of life.</p>","PeriodicalId":16536,"journal":{"name":"Journal of neuromuscular diseases","volume":" ","pages":"22143602261454423"},"PeriodicalIF":3.5,"publicationDate":"2026-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13442938/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148430694","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"2025 update of the National French consensus on gene lists for the diagnosis of muscle diseases using high-throughput sequencing.","authors":"Emmanuelle Pion, Mireille Cossée, Valérie Biancalana, Cécile Acquaviva Bourdain, Céline Bouchet-Seraphin, Julien Fauré, Roseline Froissart, France Leturcq, Rita Menassa, Corinne Metay, Laurence Michel-Calemard, Juliette Nectoux, Francois Petit, John Rendu, Pascale Richard, Damien Sternberg, Sandrine Vuillaumier-Barrot, Charles Van Goethem, Corinne Thèze, Shahram Attarian, Martin Krahn, Svetlana Gorokhova","doi":"10.1177/22143602251400143","DOIUrl":"10.1177/22143602251400143","url":null,"abstract":"<p><p>Diagnosing rare muscle diseases can be challenging due to their genetic heterogeneity. The French National Network for Rare Neuromuscular Diseases (FILNEMUS) has previously established a pioneering nationwide strategy based on gene lists organized in 13 phenotype-specific gene panels. We now revise these lists and add recently described genes. Using data collected from all FILNEMUS diagnostic laboratories, we also establish a \"Major Muscle Genes\" panel that includes genes responsible for the most frequent genetic muscle diseases. The updated diagnostic strategy of the FILNEMUS network will help reduce the turn-around time for genetic results and facilitate rapid access to the French national genome sequencing platforms.</p>","PeriodicalId":16536,"journal":{"name":"Journal of neuromuscular diseases","volume":" ","pages":"22143602251400143"},"PeriodicalIF":3.5,"publicationDate":"2026-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13438939/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148411574","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"IMU-based workspace area as a promising complementary tool to assess upper limb function in Neuromuscular diseases: A one-year follow-up.","authors":"Alessandra Favata, Jesica Expósito-Escudero, Roger Gallart-Agut, Julita Medina-Cantillo, Carme Torras, Josep M Font-Llagunes, Rosa Pàmies-Vilà, Daniel Natera-de Benito","doi":"10.1177/22143602261464735","DOIUrl":"10.1177/22143602261464735","url":null,"abstract":"<p><p>AimTo evaluate the longitudinal performance of inertial measurement unit (IMU)-based workspace metrics as a complementary tool to standard clinical scales for monitoring upper limb function in Duchenne muscular dystrophy (DMD) and spinal muscular atrophy (SMA).MethodsEighteen children and adolescents (10 DMD, 8 SMA) previously assessed at baseline underwent a one-year follow-up evaluation. Functional status was measured using disease-specific clinical scales (Performance of Upper Limb [PUL] for DMD; Revised Upper Limb Module [RULM] for SMA) combined with IMU-based kinematic analysis. Longitudinal changes in IMU-derived variables were compared with the evolution of clinical scores.ResultsOverall, IMU-based metrics showed longitudinal trends broadly comparable to those observed with clinical scales, with most participants remaining stable over 12 months. Minor individual discrepancies occurred in a few cases, and only two individuals exhibited a clear divergence between IMU-derived measures and clinical scale scores.InterpretationIMU-based workspace analysis is feasible and showed meaningful associations with established functional assessments in pediatric neuromuscular disorders. These findings support its further evaluation as a complementary tool for longitudinal monitoring and highlight its potential future role in both clinical trials and routine follow-up.</p>","PeriodicalId":16536,"journal":{"name":"Journal of neuromuscular diseases","volume":" ","pages":"22143602261464735"},"PeriodicalIF":3.5,"publicationDate":"2026-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13438583/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148391115","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Myotonic dystrophy family registry. The patient experience.","authors":"Sofia Olmos, Danny Kuei, Kleed Cumming, Tanya Stevenson, Andreas Rohrwasser","doi":"10.1177/22143602261466409","DOIUrl":"10.1177/22143602261466409","url":null,"abstract":"<p><p>IntroductionThe Myotonic Dystrophy Family Registry (MDFR) is a disease-specific, patient-reported online registry designed to capture real-world data on disease burden and lived experience in myotonic dystrophy (DM). By integrating patient-reported data with mechanisms for participant engagement, the MDFR provides a platform to characterize disease heterogeneity and support clinical research and trial readiness.AimsTo characterize patient-reported disease burden and registry participation in DM, and to explore associations between disease subtype, age-related variables, and repeat expansion length.MethodsDe-identified MDFR data collected between 2013 and 2024 were aggregated and analyzed. Patient demographics and symptom prevalence were tested for associations between DM subtype, age, and repeat expansion length.ResultsThe analysis demonstrated substantial, age-dependent disease burden in both DM1 and DM2, with multisystem involvement across central nervous system, cardiac, pulmonary, visual, and neuromuscular domains. Daytime sleepiness, myotonia, and fatigue were among the most frequently reported manifestations. Significant genotype-phenotype associations were identified in DM1, with CTG repeat length associated with increased prevalence of motor, cardiac, and central nervous system manifestations.ConclusionsPatient-reported data from the MDFR provide complementary insights into disease burden and heterogeneity in DM, including domains that may be underrepresented in clinically derived datasets. Going forward, these findings support the integration of patient-reported, clinical, and genetic data to improve disease characterization, inform clinical trial design, and advance therapeutic development. Continued efforts to harmonize and expand longitudinal data collection and standardize data elements will be important to enhance the utility of registries.</p>","PeriodicalId":16536,"journal":{"name":"Journal of neuromuscular diseases","volume":" ","pages":"22143602261466409"},"PeriodicalIF":3.5,"publicationDate":"2026-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13438913/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148391028","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Disease progression and economic burden of duchenne muscular dystrophy: A retrospective study using Swedish register data.","authors":"Thomas Sejersen, Anna-Karin Kroksmark, Aina Törnblom, Samira Toghanian, Gustaf Ortsäter, Anne-Berit Ekström","doi":"10.1177/22143602261458431","DOIUrl":"10.1177/22143602261458431","url":null,"abstract":"<p><p>BackgroundDuchenne muscular dystrophy (DMD) is a severe X-linked neuromuscular disorder causing progressive muscle loss and early death. Despite global evidence on its burden, data from Sweden remain limited.ObjectiveThis study described the natural history of DMD, treatment patterns, healthcare resource utilization, and direct medical and non-medical costs among patients with DMD and their caregivers in Sweden.MethodsA retrospective, observational matched cohort study was conducted using data from the Swedish National Registry for Neuromuscular Disorders linked to national health and social registers. Patients diagnosed with DMD and their caregivers were compared to matched population controls. Disease progression was classified into four stages based on the HERCULES model.ResultsA total of 211 DMD patients (mean age at diagnosis: 4.9 years) and 877 comparators were included. Patients experienced progressive loss of function, spending on average 3.8, 4.8, 4.8, and 7.4 years in stages 1-4, respectively. Glucocorticoid use was high (84% on either prednisolone or deflazacort). Annual direct medical costs were significantly higher for DMD patients (€14,590 vs. €660). Direct non-medical costs, predominantly for personal assistance, increased substantially with disease stage, representing 89% of total DMD-related costs (€135,671 annually).ConclusionsDMD is a severe disorder marked by prolonged time in the later stages of the disease and increasing need for support. DMD imposes a significant economic burden in Sweden, with direct non-medical costs far exceeding direct medical costs. These findings highlight the need for and value of early and effective interventions to delay disease progression and alleviate societal burden.</p>","PeriodicalId":16536,"journal":{"name":"Journal of neuromuscular diseases","volume":" ","pages":"22143602261458431"},"PeriodicalIF":3.5,"publicationDate":"2026-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13438996/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148382095","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Functional disease progression in children with inherited peripheral neuropathies: A prospective cohort study.","authors":"Katharina Vill, Moritz Tacke, Anna König, Jan Senderek, Iris Hannibal, Maggie C Walter, Beate Schlotter-Weigel, Simone Thiele, Johanna Wagner, Moritz Netzer, Wolfgang Müller-Felber, Elke Hobbiebrunken, Astrid Blaschek","doi":"10.1177/22143602261450041","DOIUrl":"10.1177/22143602261450041","url":null,"abstract":"<p><p>ObjectiveCharcot-Marie-Tooth disease (CMT) is the most common inherited peripheral neuropathy and usually manifests in the first two decades of life. However, prospective data on the natural course during childhood remain scarce.MethodsThis two-year, prospective, observational cohort study was conducted at two German pediatric neuromuscular centers. Annual standardized clinical assessments were performed. Sixty-eight pediatric patients with CMT (age range: 4-18 years; 35 females) were included. The primary outcome was the CMTPedS score, assessed at baseline and after a mean interval of 2.03 ± 0.23 years. Changes in the 11 individual CMTPedS subitems were also analyzed.ResultsThe mean baseline CMTPedS score for the total cohort (n=68) was 20.5 ± 8.8. Stratified by genotype, mean scores were: CMT1A (n=31), 16.5 ± 7.6; CMT1B (n=6), 21.5 ± 9.4; CMT2A (n=4), 35.8 ± 10.0; CMT4C (n=4), 27.0 ± 3.7; CMTX (n=4), 24.3 ± 10.1); others (n=13), 24.6 ± 7.7; genetically unsolved (n=6), 19.8 ± 8.6. Over a two-year observation period, the mean change in CMTPedS was 0.0 ± 2.7, indicating overall no significant progression.ConclusionIn this well-characterized cohort, overall disease course was non-progressive over two years-most notably in CMT1A, the most prevalent subtype. CMT2A and CMT1B showed numerically greater changes, yet none reached statistical significance. These results emphasize the slow-progressive nature of pediatric CMT and highlight the limitations of using short-term clinical progression as an outcome measure in therapeutic trials.</p>","PeriodicalId":16536,"journal":{"name":"Journal of neuromuscular diseases","volume":" ","pages":"22143602261450041"},"PeriodicalIF":3.5,"publicationDate":"2026-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13438984/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148376004","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Trach and treat: Safety and motor outcomes following onasemnogene abeparvovec in patients with spinal muscular atrophy and tracheostomies in the RESTORE registry.","authors":"Yasemin Erbaş, Laurent Servais, Perry B Shieh, Natalie L Goedeker, Megan A Waldrop, Ryosuke Bo, Dheeraj Raju, Kamal Benguerba, Sandra P Reyna, David Wolff, Richard S Finkel","doi":"10.1177/22143602251395173","DOIUrl":"10.1177/22143602251395173","url":null,"abstract":"<p><p>We evaluated the safety and effectiveness of onasemnogene abeparvovec (OA) for 34 patients with spinal muscular atrophy (SMA) and tracheostomies enrolled in the RESTORE registry. Most patients (76.5%) received other SMA treatments before and/or after OA. Safety findings were consistent with OA's established profile. There were four fatalities, three caused by respiratory events unrelated to OA, one of unknown cause. Overall, patients exhibited positive motor outcomes, even in cases where the need for tracheostomy emerged after treatment. These real-world data support OA treatment for patients with SMA and tracheostomies and can inform future access, treatment, and care decisions.<b>Trial registration</b>: Clinicaltrials.gov NCT identifier NCT04174157, registration date September 12, 2019.</p>","PeriodicalId":16536,"journal":{"name":"Journal of neuromuscular diseases","volume":" ","pages":"788-794"},"PeriodicalIF":3.5,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13434910/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145495896","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Dermatomyositis masking late onset Pompe disease in a patient with proximal muscle weakness.","authors":"Omar Keritam, Philipp Haas, Sigrid Klotz, Kastriot Kastrati, Martin Krenn, Matias Wagner, Timothy Hasenoehrl, Rosa Weng, Gudrun Zulehner, Gregor Kasprian, Günther Regelsberger, Hans Kiener, Ellen Gelpi, Fritz Zimprich, Hakan Cetin, Thomas Scherer, Suren Jengojan","doi":"10.1177/22143602251385048","DOIUrl":"10.1177/22143602251385048","url":null,"abstract":"<p><p>Slowly progressive proximal muscle weakness in an otherwise healthy male posed particular challenges for the treating physicians, considering the wide range of possible differentials. Here we present a case of a 52-year-old male with paraparesis, elevated creatine kinase-levels, antibodies against the Mi-2 antigen and subtle skin lesions, leading to subsequent treatment for dermatomyositis. Beyond that, exome sequencing revealed biallelic variants in the gene encoding acid alpha-glucosidase with concordant reduced enzymatic activity in fibroblasts, indicating late onset Pompe disease. Subsequently performed magnetic resonance imaging revealed a pattern of involvement typical for LOPD, but histological workup from the vastus lateralis muscle was more indicative of an immune-mediated myopathy. After treatment for dermatomyositis and Pompe disease the patient showed an improvement in skin changes and a halt in muscular weakness. In conclusion, both entities could be seen in the patient. However, early and prolonged subclinical hyper-CK-emia hinted at Pompe disease as the primary entity.</p>","PeriodicalId":16536,"journal":{"name":"Journal of neuromuscular diseases","volume":" ","pages":"824-831"},"PeriodicalIF":3.5,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13434907/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145345687","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Limitations in activities of daily living in individuals with spinal muscular atrophy: A scoping review and multidisciplinary recommendations for clinical practice.","authors":"Marta Ruggiero, Gabriele Giannotta, Giulia Morleo, Maria Carmela Oliva, Camilla Ferrante, Antonio Trabacca","doi":"10.1177/22143602251397438","DOIUrl":"10.1177/22143602251397438","url":null,"abstract":"<p><strong>Objective: </strong>This scoping review aims to explore and map the most frequently reported limitations in activities of daily living (ADLs) among individuals with spinal muscular atrophy (SMA), with the goal of informing clinical assessment and multidisciplinary care strategies.</p><p><strong>Methods: </strong>The review followed the Joanna Briggs Institute (JBI) methodology for scoping reviews and adhered to the PRISMA-ScR reporting guidelines. A comprehensive search was conducted across PubMed, Scopus, Web of Science, and Embase up to December 2024. Eligible studies included qualitative, quantitative, and mixed-methods designs that reported ADL limitations in individuals with any SMA subtype. Data were extracted and descriptively synthesized, with additional input from a multidisciplinary rehabilitation team to provide practical clinical recommendations.</p><p><strong>Results: </strong>Sixteen studies were included, encompassing a wide range of methodologies and SMA types. The most frequently reported ADL limitations included mobility (e.g., walking, transferring, climbing stairs) and self-care tasks (e.g., dressing, toileting, feeding). Upper limb function and instrumental ADLs, such as cooking, writing, and technology use, were less frequently assessed but emerged as important in maintaining autonomy, particularly in adults. Recent studies post-gene therapy demonstrates a broader focus on daily functioning and patient-reported outcomes. A multidisciplinary framework outlining domain-specific interventions and professional roles was developed based on the extracted data.</p><p><strong>Conclusions: </strong>While mobility remains central to SMA-related disability, this review highlights the substantial burden of self-care and upper limb limitations. Findings support a shift toward more comprehensive, patient-centered assessment and rehabilitation approaches in SMA care.</p>","PeriodicalId":16536,"journal":{"name":"Journal of neuromuscular diseases","volume":" ","pages":"602-612"},"PeriodicalIF":3.5,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13434903/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145604545","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}