Exercises and Brain Stimulation to Preserve Function in Amyotrophic Lateral Sclerosis: A Systematic Review and Meta-Analysis.

Background And Objectives: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease leading to the loss of motor function and muscle strength. Nonpharmacologic neuromodulative therapeutic approaches such as active exercise may contribute to preserve motor functions in ALS, but this hypothesis remains debated. The present meta-analysis first aimed to evaluate the effect of active exercise on function and muscle strength preservation.

Quantitative spinal cord imaging: Early ALS diagnosis and monitoring of disease progression.

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by the progressive degeneration of motor neurons in the cortex, brainstem, and spinal cord. This degeneration leads to muscular weakness, progressively impairing motor functions and ultimately resulting in respiratory failure. The clinical, genetic, and pathological heterogeneity of ALS, combined with the absence of reliable biomarkers, significantly challenge the efficacy of therapeutic trials.

Fluid balance neutralization secured by hemodynamic monitoring versus protocolized standard of care in patients with acute circulatory failure requiring continuous renal replacement therapy: results of the GO NEUTRAL randomized controlled trial.

Purpose: Net ultrafiltration (UF) during continuous renal replacement therapy (CRRT) can control fluid balance (FB), but is usually 0 ml·h in patients with vasopressors due to the risk of hemodynamic instability associated with CRRT (HIRRT). We evaluated a UF strategy adjusted by functional hemodynamics to control the FB of patients with vasopressors, compared to the standard of care.

Methods: In this randomized, controlled, open-label, parallel-group, multicenter, proof-of-concept trial, adults receiving vasopressors, CRRT since ≤ 24 h and cardiac output monitoring were randomized (ratio 1:1) to receive during 72 h a UF ≥ 100 ml·h, adjusted using a functional hemodynamic protocol (intervention), or a UF ≤ 25 ml·h (control).

Most babies born at a French hospital before 26 weeks survived with good outcomes.

Aim: The aim of this French study was to determine the neonatal morbidity, mortality and neurodevelopmental outcomes when infants born at the limit of viability reached 2 years of corrected age. We then compared the results with national and international cohorts.

Methods: This study focused on 294 French infants born from 22 to 25 weeks of gestation in a single tertiary perinatal centre from January 2010 to December 2019.

Synaptic dynamics linked to widespread elevation of H-reflex before peripheral denervation in amyotrophic lateral sclerosis.

Changes in Hoffmann reflex (H-reflex) exhibit heterogeneity among patients with amyotrophic lateral sclerosis (ALS), likely due to phenotype diversity. Current knowledge primarily focuses on soleus H-reflex, which may demonstrate an initial increase before subsequent decline throughout the disease course. The main objective was to investigate other muscles, to determine whether H-reflex changes could be associated with patient phenotype (onset site, functional disabilities).

Subretinal fibrosis occurrence according to macular neovascularisation subtypes in neovascular age-related macular degeneration.

Purpose: To assess subretinal fibrosis (SF) occurrence in neovascular age-related macular degeneration (nAMD), according to macular neovascularisation (MNV) subtypes.

Methods: A Retrospective national multi centre cohort study included eyes with naive nAMD. Main outcome measures were, according to MNV subtypes, cumulative incidence for SF, risk factors, and best corrected visual acuity (BCVA) for 36 months.

Effect of baseline fluid localization on visual acuity and prognosis in type 1 macular neovascularization treated with anti-VEGF.

Purpose: To assess the prognostic value of subretinal (SRF) and intraretinal fluid (IRF) localizations in type 1 macular neovascularization (MNV) due to age-related macular degeneration (AMD).

Subjects: Eyes were prospectively treated with anti-vascular epithelial growth factor (anti-VEGF) intravitreal injections (IVT) according to a Pro-Re-Nata (PRN) or Treat and Extend (TAE) regimen during 24 months. A total of 211 eyes with treatment-naïve type 1 MNV secondary to AMD were consecutively included.

Assessment of the refractive outcomes of patients with regular corneal astigmatism implanted with high-power toric intraocular lenses.

Background: To describe the refractive outcomes of eyes with high regular corneal astigmatism undergoing age-related cataract surgery.

Methods: Astigmatic patients who underwent cataract surgery with implantation of high-power XY1AT HOYA toric lenses (IOLs) (≥ T5) between March 2020 and June 2022 were included. Patients were divided into 2 groups based on the type of Total Corneal Astigmatism (TCA) used for the toric IOL calculation: group 1 = TCA (TCA measured by the Iol Master 700) and group 2 = TCA (TCA estimated from the anterior keratometry of the Iol Master 700 and using the Abulafia-Koch regression).

Cost-effectiveness of a new multi-lumen infusion device to reduce central-venous-line-associated bloodstream infections in neonates.

Background: A new medical device was developed for multi-infusion in neonatal intensive care units (NICUs) with the aim of addressing issues related to drug incompatibilities and central-line-associated bloodstream infections (CLABSIs).

Aim: To assess the cost-effectiveness of implementing this new perfusion system in an NICU setting.

Methods: This single-centre, observational study was conducted in all infants admitted to the NICU within 3 days of birth, and who required a central venous line, to evaluate the cost and effectiveness before (2019) and after (2020) implementation of the new perfusion system.

Effect of ultrasound-mediated blood-spinal cord barrier opening on survival and motor function in females in an amyotrophic lateral sclerosis mouse model.

Background: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by a progressive loss of motor neurons. The limited efficacy of recent therapies in clinical development may be linked to lack of drug penetration to the affected motor neurons due to the blood-brain barrier (BBB) and blood-spinal cord barrier (BSCB).

Methods: In this work, the safety and efficacy of repeated short transient opening of the BSCB by low intensity pulsed ultrasound (US, sonication) was studied in females of an ALS mouse model (B6.

Treatment continuity of amyotrophic lateral sclerosis with available riluzole formulations: state of the art and current challenges in a 'real-world' setting.

Amyotrophic lateral sclerosis (ALS) is a rare multisystem neurodegenerative disease leading to death due to respiratory failure. Riluzole was the first disease modifying treatment approved in ALS. Randomized clinical trials showed a significant benefit of riluzole on survival in the months following randomization, with a good safety profile.

Glucose breath test for the detection of small intestine bacterial overgrowth: Impact of diet prior to the test.

Background: Glucose breath test (GBT) is used for the diagnosis of small intestine bacterial overgrowth. A restrictive diet without fibers and/or fermentable food is recommended on the day before the test. The aim of our retrospective study was to evaluate the impact of two different restrictive diets on the results of GBT.

271st ENMC international workshop: Towards a unifying effort to fight Kennedy's disease. 20-22 October 2023, Hoofddorp, Netherlands.

The workshop held in the Netherlands from October 20-22, 2023, united 27 scientists from academia, healthcare, and industry representing 11 countries, alongside four patient and charity representatives. Focused on Kennedy's Disease (KD), also known as spinal and bulbar muscular atrophy (SBMA), the workshop aimed to consolidate knowledge, align on clinical trial designs, and promote participative medicine for effective treatments. Discussions emphasized KD's molecular mechanisms, highlighting its status as a neuromuscular disorder with motor neuron degeneration.

Cortical hyperexcitability in mouse models and patients with amyotrophic lateral sclerosis is linked to noradrenaline deficiency.

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease, characterized by the death of upper (UMN) and lower motor neurons (LMN) in the motor cortex, brainstem, and spinal cord. Despite decades of research, ALS remains incurable, challenging to diagnose, and of extremely rapid progression. A unifying feature of sporadic and familial forms of ALS is cortical hyperexcitability, which precedes symptom onset, negatively correlates with survival, and is sufficient to trigger neurodegeneration in rodents.

Long Intervals between Intravitreal Injections Using a Treat-and-Extend Protocol in a Real-Life Context in AMD: The LIRE Study.

Introduction: The aim of the study was to assess the outcome of long treat-and-extend (TE) anti-VEGF intravitreal injection (IVI) intervals (≥every 12 weeks [Q12W]) in neovascular age-related macular degeneration (nAMD). The aims of this retrospective study were to determine the proportion of nAMD eyes treated ≥ Q12W, to analyze their longitudinal, functional, and anatomical outcomes, and to compare functional and anatomical outcomes between eyes that rapidly versus slowly reached a Q12W regimen and between eyes directly treated with versus initiating lately the TE regimen.

Methods: All patients receiving IVIs for nAMD were screened.

Neurodevelopment at 24 months corrected age in extremely preterm infants treated with dexamethasone alternatives during the late postnatal period: a cohort study.

The administration of dexamethasone has been associated with suboptimal neurodevelopment. We aimed to compare the development of extremely premature infants treated or not with alternatives to dexamethasone: betamethasone, hydrocortisone hemisuccinate. This retrospective cohort study included infants born before 29 weeks of gestational age, treated or not with late (day ≥ 7) postnatal steroids (betamethasone, hydrocortisone hemisuccinate).

Quantitative brainstem and spinal MRI in amyotrophic lateral sclerosis: implications for predicting noninvasive ventilation needs.

Background: Respiratory complications resulting from motor neurons degeneration are the primary cause of death in amyotrophic lateral sclerosis (ALS). Predicting the need for non-invasive ventilation (NIV) in ALS is important for advance care planning and clinical trial design. The aim of this study was to assess the potential of quantitative MRI at the brainstem and spinal cord levels to predict the need for NIV during the first six months after diagnosis.

A new perfusion system to reduce the burden of central-venous-line-associated bloodstream infections in neonates.

Background: Central-venous-line-associated bloodstream infection (CLABSI) is a significant cause of morbidity and mortality in preterm infants. As there is large variation in the reported effect of multi-modal preventive strategies, it could be relevant to propose new additional strategies.

Aim: To assess the impact of a new perfusion system on CLABSI rate.

Willingness to use a drug consumption room among people who use drugs in Lyon, France, a city with no open scene of drug use (the TRABOUL survey).

Background: Drug consumption rooms (DCRs) have been developed in cities with open drug scenes, with the aim to reduce drug-related harm. In Lyon, France's second-largest city, there is no distinct drug use area, which raised doubts regarding the need for a DCR.

Methods: We conducted a face-to-face survey of 264 people who use drugs (PWUDs), recruited in harm reduction or addiction treatment centers, in the streets or in squats.