Texture Analysis of T1-weighted Turbo Spin-Echo MRI for the Diagnosis and Follow-up of Collagen VI-related Myopathy

Muscle texture analysis in Magnetic Resonance Imaging (MRI) has revealed a good correlation with typical histological changes resulting from neuromuscular disorders. In this research, we assess the effectiveness of several features in describing intramuscular texture alterations in cases of Collagen VI-related myopathy. A T1-weighted Turbo Spin-Echo MRI dataset was used (Nsubj = 26), consisting of thigh scans from subjects diagnosed with Ullrich Congenital Muscular Dystrophy or Bethlem Myopathy, with different severity levels, as well as healthy subjects. A total of 355 texture features were studied, including attributes derived from the Gray-Level Co-occurrence Matrix, the Run-Length Matrix, Wavelet and Gabor filters. The extracted features were ranked using the Support Vector Machine Recursive Feature Elimination (SVM-RFE) algorithm with Correlation Bias Reduction, prior to cross-validated classification with a Gaussian kernel SVM.info:eu-repo/semantics/acceptedVersionhttps://ieeexplore.ieee.org/document/9433942

Orientation diagnostique d’un cas de «

La maladie de Pompe est une maladie lysosomale avec atteinte musculaire prédominante. On distingue la forme sévère du nourrisson avec troubles cardiaques des formes tardives de l’enfant, de l’adolescent et de l’adulte qui n’ont en général pas de manifestations cardiaques. L’association d’une maladie de Pompe et d’un « rigid spine » a été occasionnellement rapportée. Plusieurs profils ont été décrits. La présentation la plus fréquente est un déficit des muscles des ceintures et du diaphragme. Une scoliose est rapportée dans un tiers des patients, mais seulement 15 % associent une raideur spinale. L’atteinte extra-musculaire, en particulier des symptômes cardio-cérébrovasculaires sont observés dans un tiers des patients. L’observation rapportée ici présente un tableau de myopathie avec un phénotype typique de raideur spinale sélective, associé à une augmentation modérée des enzymes musculaires, des signes myopathiques à la biopsie, et une atteinte cardiaque

Contribution of muscle MRI for diagnosis of myopathy

International audienceInherited myopathies are a group of disease, which, although distinct from a genetic and prognostic point of view, can lead to non-specific clinical pictures due to phenotypic overlap. Acquired immuno-mediated myopathies may also pose the problem of clinically accurate etiological orientation. The assessment of fatty infiltration and pathological increase in water volume of the muscle contingent on whole-body muscle MRI is becoming increasingly important in aiding the initial diagnosis of inherited and acquired myopathies. MRI helps orientating the clinical diagnostic hypotheses thanks to the patterns of muscle involved (more or less specific according to the entities), which led to the development of decision-making algorithms proposed in the literature. The aim of this article is to specify the proper MRI protocol for the evaluation of myopathies and the basis of the interpretation and to provide a summary of the most frequently inherited and acquired myopathies described in the literature

Pre-test probability for SARS-Cov-2-related infection score: The PARIS score.

IntroductionIn numerous countries, large population testing is impossible due to the limited availability of RT-PCR kits and CT-scans. This study aimed to determine a pre-test probability score for SARS-CoV-2 infection.MethodsThis multicenter retrospective study (4 University Hospitals) included patients with clinical suspicion of SARS-CoV-2 infection. Demographic characteristics, clinical symptoms, and results of blood tests (complete white blood cell count, serum electrolytes and CRP) were collected. A pre-test probability score was derived from univariate analyses of clinical and biological variables between patients and controls, followed by multivariate binary logistic analysis to determine the independent variables associated with SARS-CoV-2 infection.Results605 patients were included between March 10th and April 30th, 2020 (200 patients for the training cohort, 405 consecutive patients for the validation cohort). In the multivariate analysis, lymphocyte (ConclusionsThe PARIS score has a good performance to categorize the pre-test probability of SARS-CoV-2 infection based on complete white blood cell count. It could help clinicians adapt testing and for rapid triage of patients before test results

Sex differences in mandibular repositioning device therapy effectiveness in patients with obstructive sleep apnea syndrome

International audiencePurpose: Mandibular repositioning devices (MRDs) are an effective treatment option for obstructive sleep apnea syndrome (OSAS), particularly in patients who refuse or cannot tolerate continuous positive airway pressure (CPAP). However, sex differences in the response to therapy and predictors of response are not clearly defined. This analysis of data from the long-term prospective ORCADES trial compared MRD efficacy in men and women with OSAS.Methods: The ORCADES study included patients with newly diagnosed mild-to-moderate or severe OSAS who refused or were non-compliant with CPAP. MRD therapy was titrated over 3-6 months. The primary endpoint was treatment success (≥ 50% decrease in apnea-hypopnea index (AHI)). Complete response was defined using a range of AHI cut-off values ( 30/h) were 100% and 68% (p = 0.0015). In women vs. men, overall complete response rates at AHI cut-off values of < 5/h, <10/h, and < 15/h were 49 vs. 34% (p = 0.0052), 78 vs. 62% (p = 0.016), and 92 vs. 76% (p = 0.0032). On multivariate analysis, significant predictors of MRD treatment success were overbite and baseline apnea index in men, and neck circumference and no previous CPAP therapy in women. There were sex differences in the occurrence of side effects. Temporomandibular joint pain was the most common reason for stopping MRD therapy.Conclusions: MRD therapy was effective in women with OSA of any severity, with significantly higher response rates compared with men especially in severe OSAS