Stable Convergent Polyneuronal Innervation and Altered Synapse Elimination in Muscles from Patients with Blepharospasm Responding Poorly to Recurrent Botulinum Type-A Neurotoxin Injections.

Botulinum neurotoxin type-A (BoNT/A), which blocks quantal acetylcholine (ACh) release at the neuromuscular junction (NMJ), has demonstrated its efficacy in the symptomatic treatment of blepharospasm. In 3.89% of patients treated for blepharospasm at Tenon Hospital, BoNT/A was no longer effective in relieving the patient's symptoms, and a partial upper myectomy of the muscle was performed.

Lung ultrasound score and diaphragm ultrasound in weaning from mechanical ventilation: are they different in patients with and without COVID-19?

Objective: To compare pre-extubation physiological characteristics and ultrasound variables between patients intubated for COVID-19 compared to a clinical population and those intubated for other reasons.

Methods: This was a secondary analysis of a prospective cohort study of patients undergoing invasive mechanical ventilation (IMV) for more than 48 h. Patients were divided into two groups: those intubated for COVID-19-induced ARDS and those intubated for other clinical reasons.

Monoclonal antibodies that block Roundabout 1 and 2 signaling target pathological ocular neovascularization through myeloid cells.

Roundabout (ROBO) 1 and 2 are transmembrane receptors that bind secreted SLIT ligands through their extracellular domains (ECDs) and signal through their cytoplasmic domains to modulate the cytoskeleton and regulate cell migration, adhesion, and proliferation. SLIT-ROBO signaling regulates pathological ocular neovascularization, which is a major cause of vision loss worldwide, but pharmacological tools to prevent SLIT-ROBO signaling are lacking. Here, we developed human monoclonal antibodies (mAbs) against the ROBO1 and ROBO2 ECDs.

Impact of Fetal Umbilical Cord Blood CD34+ Cells on Breast Cancer Cell Lines: A Mechanism of Fetal Microchimerism.

Introduction: Fetal microchimerism could be involved in the regulation of breast cancer oncogenesis. CD34+ cells could be of a particular interest as up to 12% of the CD34+ population in maternal blood are of fetal origin. The aim of this research was to analyze the impact of umbilical cord blood (UCB) CD34+ on MCF-7 and MDA-MB-231 breast cancer cell lines, in order to uncover novel biological mechanisms and suggest novel treatment options for breast cancer.

-related myopathies: clinical, myopathological and genotypic spectrum in a multicentre French cohort.

Background: Myosin heavy chain 7 ()-related myopathies (-RMs) are a group of muscle disorders linked to pathogenic variants in the gene, encoding the slow/beta-cardiac myosin heavy chain, which is highly expressed in skeletal muscle and heart. The phenotype is heterogeneous including distal, predominantly axial or scapuloperoneal myopathies with variable cardiac involvement.

Methods: We retrospectively analysed the clinical, muscle MRI, genetic and myopathological features of 57 patients.

Magnetic Resonance Imaging Diagnosis of Spinal Longitudinal Epidural Collections in a Spontaneous Spinal Dural Breach and Successful Surgical Treatment.

Spontaneous intracranial hypotension is generally secondary to cerebrospinal fluid leaks. Spinal magnetic resonance imaging is an important part of the diagnostic workup, especially to display spinal longitudinal epidural collections. Other causes of leaks include nerve root sleeve tear and cerebrospinal fluid-venous fistula.

Environment-Stored Memory in Active Nematics and Extra-Cellular Matrix Remodeling.

Many active systems display nematic order, while interacting with their environment. In this Letter, we show theoretically how environment-stored memory acts an effective external field that aligns active nematics. The coupling to the environment leads to substantial modifications of the known phase diagram and dynamics of active nematics, including nematic order at arbitrarily low densities and arrested domain coarsening.

Cellular pattern and orbital fat involvement are possible risk factors for the failure of corticosteroids in patients with pure idiopathic orbital inflammation syndrome: lessons from the French prospective cohort study (part II).

Purpose: To better characterise the effects of corticosteroids on the course of pure idiopathic orbital inflammation syndrome (pIOIS).

Methods: This was a national, multicentre, prospective, non-interventional cohort study (). Among the 35 patients with histologically proven orbital inflammation who had previously been studied for their IgG4 immunostaining status, we selected those with a negative IgG4 status (ie, pIOIS) who received corticosteroids as single first-line treatment.

Stable pharmaceutical composition of cryo-protected non-pyrogenic isotonic chains of magnetosomes for efficient tumor cell destruction at 45 ± 1 °C under alternating magnetic field or ultrasound application.

We report a method to prepare biocompatible, stable, and highly pure iron oxide nano-minerals by following the steps consisting of: (i) amplifying magnetotactic bacteria in non-toxic minimal growth media; (ii) extracting magnetosomes from magnetotactic bacteria under alkaline lysis; (iii) heating magnetosomes above 400 °C to yield sterile magnetosome minerals, M-uncoated, devoid of active non-denatured bacterial organic material; (iv) coating M-uncoated with biocompatible carboxymethyl-dextran (CMD) compounds to yield stable M-CMD; (v) adding 5% sorbitol to M-CMD; and (vi) lyophilizing these mixtures, resulting in formulated nano-minerals in powder forms, designated as (M-CMD). The long-term stability of the final products is demonstrated by re-suspending (M-CMD) in water after 12 months of storage, and by showing that these formulated magnetosomes have preserved their stability in suspension, chain arrangement, carbon content, surface charge, and surface composition. Furthermore, the formulation is optimized to yield an isotonic magnetosome suspension with an osmolality of between 275 and 290 mOsm kg HO upon reconstitution.

DORQ-seq: high-throughput quantification of femtomol tRNA pools by combination of cDNA hybridization and Deep sequencing.

Due to its high modification content tRNAs are notoriously hard to quantify by reverse transcription and RNAseq. Bypassing numerous biases resulting from concatenation of enzymatic treatments, we here report a hybrid approach that harnesses the advantages of hybridization-based and deep sequencing-based approaches. The method renders obsolete any RNAseq related workarounds and correction factors that affect accuracy, sensitivity, and turnaround time.

Author Correction: Cell-mediated exon skipping normalizes dystrophin expression and muscle function in a new mouse model of Duchenne Muscular Dystrophy.

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A developmental model of audio-visual attention (MAVA) for bimodal language learning in infants and robots.

A social individual needs to effectively manage the amount of complex information in his or her environment relative to his or her own purpose to obtain relevant information. This paper presents a neural architecture aiming to reproduce attention mechanisms (alerting/orienting/selecting) that are efficient in humans during audiovisual tasks in robots. We evaluated the system based on its ability to identify relevant sources of information on faces of subjects emitting vowels.

Meningeal lymphatic function promotes oligodendrocyte survival and brain myelination.

The precise neurophysiological changes prompted by meningeal lymphatic dysfunction remain unclear. Here, we showed that inducing meningeal lymphatic vessel ablation in adult mice led to gene expression changes in glial cells, followed by reductions in mature oligodendrocyte numbers and specific lipid species in the brain. These phenomena were accompanied by altered meningeal adaptive immunity and brain myeloid cell activation.

Prognostic impact of high-intensity lipid-lowering therapy under-prescription after acute myocardial infarction in women.

Aims: Women are less likely to receive lipid-lowering therapy (LLT) after acute myocardial infarction (AMI). We analysed whether this under-prescription currently persists and has an impact on long-term outcomes.

Methods And Results: The FAST-MI programme consists of nationwide registries including all patients admitted for AMI ≤ 48 h from onset over a 1 month period in 2005, 2010, and 2015, with long-term follow-up.

The EBMT-ADWP and the CIBMTR.

Hematopoietic stem cell transplantation (HSCT) has evolved over the last 25 years as a specific treatment of patients with severe neurologic autoimmune diseases (ADs), through eradication of the pathologic, immunologic memory, and profound immune "resetting." HSCT for ADs is recently facing a unique developmental phase across transplant centers. Data from patients undergoing HSCT and cellular therapies have been captured through the established major transplant registries, such as the European Society for Blood and Marrow Transplantation (EBMT) and the Center for International Blood and Marrow Transplant Research (CIBMTR).

Charting a course for global progress in PIDs by 2030 - proceedings from the IPOPI global multi-stakeholders' summit (September 2023).

The International Patient Organisation for Primary Immunodeficiencies (IPOPI) held its second Global Multi-Stakeholders' Summit, an annual stimulating and forward-thinking meeting uniting experts to anticipate pivotal upcoming challenges and opportunities in the field of primary immunodeficiency (PID). The 2023 summit focused on three key identified discussion points: (i) How can immunoglobulin (Ig) therapy meet future personalized patient needs? (ii) Pandemic preparedness: what's next for public health and potential challenges for the PID community? (iii) Diagnosing PIDs in 2030: what needs to happen to diagnose better and to diagnose more? Clinician-Scientists, patient representatives and other stakeholders explored avenues to improve Ig therapy through mechanistic insights and tailored Ig preparations/products according to patient-specific needs and local exposure to infectious agents, amongst others. Urgency for pandemic preparedness was discussed, as was the threat of shortage of antibiotics and increasing antimicrobial resistance, emphasizing the need for representation of PID patients and other vulnerable populations throughout crisis and care management.