PubMed Journals Articles About "Intervention Used Neuromuscular Diseases" RSS

06:36 EST 22nd January 2020 | BioPortfolio

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Showing "intervention used Neuromuscular Diseases" PubMed Articles 1–25 of 15,000+

Presentation and management patterns of lower urinary tract symptoms in adults due to rare inherited neuromuscular diseases.

To describe the urologic sequalae of several rare congenital neuromuscular diseases.

Immunosuppressive and Immunomodulatory Therapies for Neuromuscular Diseases Part I: Traditional Agents.

Immunosuppressive and immunomodulatory therapies have had a major effect on the treatment of immune-mediated neuromuscular diseases. Following the landmark introduction of synthetic corticosteroids, other therapies have become available including plasma exchange (PLEX), immunoglobulin G (IgG), and steroid-sparing immunosuppressive drugs. More recently, novel biologically derived and antigen-specific pharmaceuticals have entered neuromuscular practice. Various levels of evidence guide the use of these medica...

Respiratory muscle training in children and adults with neuromuscular disease.

Neuromuscular diseases (NMDs) are a heterogeneous group of diseases affecting the anterior horn cell of spinal cord, neuromuscular junction, peripheral nerves and muscles. NMDs cause physical disability usually due to progressive loss of strength in limb muscles, and some NMDs also cause respiratory muscle weakness. Respiratory muscle training (RMT) might be expected to improve respiratory muscle weakness; however, the effects of RMT are still uncertain. This systematic review will synthesize the available ...

Guidelines for neuromuscular ultrasound training.

Neuromuscular ultrasound has become an essential tool in the diagnostic evaluation of various neuromuscular disorders, and as such, there is growing interest in neuromuscular ultrasound training. Effective training is critical in mastering this modality. Our aim was to develop consensus-based guidelines for neuromuscular ultrasound training courses. A total of 18 experts participated. Expert opinion was sought through the Delphi method using 4 consecutive electronic surveys. A high degree of consensus was a...

Water T Mapping in Fatty Infiltrated Thigh Muscles of Patients With Neuromuscular Diseases Using a T -Prepared 3D Turbo Spin Echo With SPAIR.

Muscle water T (T ) has been proposed as a biomarker to monitor disease activity and therapy effectiveness in patients with neuromuscular diseases (NMD). Multi-echo spin-echo (MESE) is known to be affected by fatty infiltration. A T -prepared 3D turbo spin echo (TSE) is an alternative method for T mapping, but has been only applied in healthy muscles.

Spinal Muscular Atrophy (SMA) Subtype Concordance in Siblings: Findings From the Cure SMA Cohort.

Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder caused by homozygous survival of motor neuron 1 (SMN1) gene disruption. Despite a genetic etiology, little is known about subtype concordance among siblings.

Effect of mechanical insufflation-exsufflation in children with neuromuscular weakness.

Children with neuromuscular diseases develop cough impairment. Airway clearance techniques (ACTs) may help to prevent recurrent respiratory tract infections (RTIs). A commonly used ACT is mechanical insufflation-exsufflation (MI-E), but evidence for efficacy is limited. We hypothesize that MI-E has beneficial effect on RTI related hospital admission rate.

Investigating the clinical use of structured light plethysmography to assess lung function in children with neuromuscular disorders.

Children and young people with neuromuscular disorders (NMD), such as Duchenne Muscular Dystrophy (DMD), develop progressive respiratory muscles weakness and pulmonary restriction. Pulmonary function monitoring of the decline in lung function allows for timely intervention with cough assist techniques and nocturnal non-invasive ventilation (NIV). NMD may find the measurement of lung function difficult using current techniques. Structured Light Plethysmography (SLP) has been proposed as a novel, non-contact,...

A step towards the future of seamless measurement with wearable sensors in pediatric populations with neuromuscular diseases.

Comparison of neostigmine vs. sugammadex for recovery of muscle function after neuromuscular block by means of diaphragm ultrasonography in microlaryngeal surgery: A randomised controlled trial.

Postoperative residual neuromuscular blockade or curarisation (PORC) is a risk directly related to the use of neuromuscular blocking agents during surgical procedures. Acceleromyography is distressing for conscious patients when assessing PORC. Diaphragm ultrasonography could be a valid alternative.

Gene therapy research for kidney diseases.

A resurgence in the development of newer gene therapy systems has led to recent successes in the treatment of B-cell cancers, retinal degeneration and neuromuscular atrophy. Gene therapy offers the ability to treat the patient at the root cause of their malady by restoring normal gene function and arresting the pathological progression of their genetic disease. The current standard of care for most genetic diseases is based upon the symptomatic treatment using polypharmacy while minimizing any potential adv...

Effects of neuromuscular electrical stimulation training on muscle size in collegiate track and field athletes.

The purpose of this study was to examine the effects of neuromuscular electrical stimulation training for 12 weeks on the abdominal muscle size in trained athletes. Male collegiate track and field athletes participated in the present study and were randomly allocated to either training or control groups. Eleven participants of the training group completed a 60-session training program over a 12-week period (23 min/session, 5 days/week) involving neuromuscular electrical stimulation (mostly 20 Hz) for the ab...

Adult neuromuscular disorders: a joint palliative/neuromuscular clinic.

"The Times They Are a-Changin'." In reply to El-Zaidy et al.: AVXS-101 (Onasemnogene Abeparvovec) for SMA1: Comparative Study with a Prospective Natural History Cohort.

Developmental neuromuscular synapse elimination: activity-dependence and potential downstream effector mechanisms.

Synaptic connections initially formed during nervous system development undergo a significant transformation during nervous system maturation. Such maturation is essential for the proper architecture and function of the nervous system. Developmental synaptic transformation includes "synapse elimination," a process in which multiple immature presynaptic inputs converge at and compete for control of a common postsynaptic target. This developmental synaptic remodeling is best understood at mammalian neuromuscu...

Neuromuscular Development in Neonates and Postnatal Infants: Implications for Neuromuscular Electrical Stimulation Therapy for Dysphagia.

Purpose The aim of the current study was to review neuromuscular development, summarize the current body of evidence describing the use of neuromuscular electrical stimulation (NMES) therapy in infants, and identify possible contraindications for the use of NMES in the neonate and young infant. Method After a review of the literature describing neuromuscular development, we created a timeline of the developmental processes. Key milestones were determined, and a literature search was conducted to identify po...

Overground exoskeletons may boost neuroplasticity in myotonic dystrophy type 1 rehabilitation: A case report.

Myotonic dystrophy type 1 (DM1) is a slowly progressive multisystem neuromuscular disease characterized by myotonia and muscle weakness and wasting of distal and axial muscles. People with DM1, due to the disease progression, are often concerned about their ability to carry out and participate in the activities of daily living. Rehabilitation approaches in DM1, including moderate-to-intense strength training, have shown not univocal efficacy to face such difficulties. Aim of this case-study was to demonstra...

In vitro models of neuromuscular junctions and their potential for novel drug discovery and development.

: Neuromuscular Junctions (NMJs) are the synapses between motor neurons and skeletal muscle fibers, and they are responsible for voluntary motor function. NMJs are affected at early stages of numerous neurodegenerative and neuroimmunological diseases. Due to the difficulty of systematically studying and manipulating NMJs in live subjects, systems with human tissue models would provide a powerful complement to simple cell cultures and animal models for mechanistic and drug development studies.: The authors ...

Early Enteral Nutrition in Patients Undergoing Sustained Neuromuscular Blockade: A Propensity-Matched Analysis Using a Nationwide Inpatient Database.

Whether enteral nutrition should be postponed in patients undergoing sustained treatment with neuromuscular blocking agents remains unclear. We evaluated the association between enteral nutrition initiated within 2 days of sustained neuromuscular blocking agent treatment and in-hospital mortality.

Cardiac and Neuromuscular Features of Patients with LMNA-Related Cardiomyopathy.

Mutations in the LMNA (lamin A/C) gene have been associated with neuromuscular and cardiac manifestations, but the clinical implications of these signs are not well understood.

Salbutamol modifies the neuromuscular junction in a mouse model of ColQ myasthenic syndrome.

The β-adrenergic agonists salbutamol and ephedrine have proven to be effective as therapies for human disorders of the neuromuscular junction, in particular many subsets of congenital myasthenic syndromes. However, the mechanisms underlying this clinical benefit are unknown and improved understanding of the effect of adrenergic signalling on the neuromuscular junction is essential to facilitate the development of more targeted therapies. Here, we investigated the effect of salbutamol treatment on the neuro...

Seasonal variation in neuromuscular control in young male soccer players.

Determine how lower limb neuromuscular control changes over the course of a competitive soccer season.

Four Weeks of Neuromuscular Training Improve Static and Dynamic Postural Control in Overweight and Obese Children: A Randomized Controlled Trial.

Thirty-two children with overweight or obesity were randomly divided into a neuromuscular training group (NTG) (n = 16) and a control group (CG) (n = 16). All individuals participated in the measurement of static postural control under two conditions: the double-leg stance with eyes open (EO) and eyes closed (EC). The center of pressure variables was obtained. mSEBT was used for dynamic postural control. Neuromuscular training was performed twice per week and lasted 4 weeks. The results of this study indica...

Oculopharyngeal Muscular Dystrophy, an Often Misdiagnosed Neuromuscular Disorder: A Southern California Experience.

Oculopharyngeal muscular dystrophy (OPMD) is a rare neuromuscular disorder characterized by late-onset development of bilateral eyelid ptosis, ophthalmoparesis and dysphagia with further progression to proximal limb muscle weakness that is an under recognized condition. The mode of inheritance is usually autosomal dominant, but a recessive form has been reported. OPMD is caused by a short expansion of the alanine repeat (GCN trinucleotide) in the poly(adenylate)-binding protein nuclear1 (PABPN1) gene.

Retrospective Investigation of Grafted Kidney Function After Reversal of Neuromuscular Blockade Using Neostigmine or Sugammadex.

Sugammadex has the steroid-encapsulating effect that reverses neuromuscular block induced by aminosteroid neuromuscular-blocking agents. Sugammadex can interact with other drugs that have the same steroidal structure with rocuronium, such as corticosteroids. Corticosteroids play a crucial role in the immunosuppression of kidney transplantation. The purpose of this study was to determine if there are any differences in grafted kidney function in recipients of kidney transplantation when sugammadex or neostig...

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