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Neuromuscular therapy

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https://www.readbyqxmd.com/read/29666602/myasthenia-gravis-induced-by-ipilimumab-in-a-patient-with-metastatic-melanoma
#1
Vera Montes, Sandra Sousa, Fernando Pita, Rui Guerreiro, Cátia Carmona
In daily clinical practice, there is a growing number of patients receiving new biological agents used in the treatment of malignancies. Ipilimumab is a fully humanized monoclonal antibody approved for patients with melanoma. It acts as an immune checkpoint inhibitor, binding and blocking cytotoxic T-lymphocyte antigen-4 in order to increase the antitumor immune response. There are several reports of autoimmune responses after its use. A 74-year-old man developed a mild rash and pruritus a few hours after the second infusion of ipilimumab and 24 h after the third dose of ipilimumab, he presented with shortness of breath, proximal limb muscle weakness, and diplopia...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29662761/inflammation-and-age-associated-skeletal-muscle-deterioration-sarcopaenia
#2
REVIEW
Jinyu Wang, Kwok-Sui Leung, Simon Kwoon-Ho Chow, Wing-Hoi Cheung
Ageing is accompanied by chronic inflammatory responses due to elevated circulatory inflammatory cytokine production. Several inflammatory cytokines have been shown to be responsible for a decrease in muscle mass. However, little is known about the possible relationship between inflammation and sarcopaenia. This review aims to summarise the existing evidence about inflammation and sarcopaenia. Sarcopaenia is defined as an age-related decrease of muscle mass and/or muscle strength; it is caused by multiple factors, such as skeletal muscle atrophy, neuromuscular junction degeneration, hormone imbalance, cytokine imbalance, protein synthesis and proteolysis...
July 2017: Journal of Orthopaedic Translation
https://www.readbyqxmd.com/read/29660951/effects-of-neuromuscular-electrical-stimulation-in-patients-with-parkinson-s-disease-and-dysphagia-a-randomized-single-blind-placebo-controlled-trial
#3
Ji-Su Park, Dong-Hwan Oh, Na-Kyoung Hwang, Jung-Hoon Lee
BACKGROUND: Neuromuscular electrical stimulation has been used to improve swallowing function in neurologic patients with dysphagia, but its effect on patients with dysphagia and Parkinson's disease remains unclear. OBJECTIVES: This study aimed to identify the effect of effortful swallowing combined with neuromuscular electrical stimulation as a novel treatment approach in dysphagic patients with Parkinson's disease. METHODS: Participants were randomly allocated to an experimental group (n = 9) or a placebo group (n = 9)...
April 7, 2018: NeuroRehabilitation
https://www.readbyqxmd.com/read/29656576/repurposing-carbamazepine-for-the-treatment-of-amyotrophic-lateral-sclerosis-in-sod1-g93a-mouse-model
#4
Jing-Jing Zhang, Qin-Ming Zhou, Sheng Chen, Wei-Dong Le
AIMS: To investigate the effect and mechanisms of carbamazepine (CBZ) on the onset and progression of amyotrophic lateral sclerosis (ALS) in SOD1-G93A mouse model. METHODS: Starting from 64 days of age, SOD1-G93A mice were orally administered with CBZ at 200 mg/kg once daily until death. The disease onset and life span of SOD1-G93A mice were recorded. Motor neurons (MNs) in anterior horn of spinal cord were quantified by Nissl staining and SMI-32 immunostaining...
April 14, 2018: CNS Neuroscience & Therapeutics
https://www.readbyqxmd.com/read/29649521/modelling-motor-neuron-disease-in-fruit-flies-lessons-from-spinal-muscular-atrophy
#5
Beppe Aquilina, Ruben J Cauchi
Motor neuron disease (MND) is characterised by muscle weakness and paralysis downstream of motor neuron degeneration. Genetic factors play a major role in disease pathogenesis and progression. This is best underscored by spinal muscular atrophy (SMA), the most common MND affecting children. Although SMA is caused by homozygous mutations in the survival motor neuron 1 (SMN1) gene, partial compensation by the paralogous SMN2 gene and/or genetic modifiers influence age of onset and disease severity. SMA is also the first MND that is treatable thanks to the recent development of a molecular-based therapy...
April 9, 2018: Journal of Neuroscience Methods
https://www.readbyqxmd.com/read/29649068/is-exercise-the-right-medicine-for-dystrophic-muscle
#6
Hannah R Spaulding, Joshua T Selsby
INTRODUCTION: Duchenne muscular dystrophy (DMD) is a neuromuscular disease caused by a dystrophin protein deficiency. Dystrophin functions to stabilize and protect the muscle fiber during muscle contraction, thus the absence of functional dystrophin protein leads to muscle injury. DMD patients experience progressive muscle necrosis, loss of function, and ultimately succumb to respiratory failure or cardiomyopathy. Exercise is known to improve muscle health and strength in healthy individuals as well as positively impact other systems...
April 11, 2018: Medicine and Science in Sports and Exercise
https://www.readbyqxmd.com/read/29643578/effect-of-mirror-therapy-and-electrical-stimulation-on-upper-extremity-function-in-stroke-with-hemiplegic-patient-a-pilot-study
#7
Young-Rim Paik, Jeong-Hoon Lee, Doo-Ho Lee, Hee-Su Park, Dong-Hwan Oh
[Purpose] This study investigated the effects of mirror therapy and neuromuscular electrical stimulation on upper extremity function in stroke patients. [Subjects and Methods] This study recruited 8 stroke patients. All patients were treated with mirror therapy and neuromuscular electrical stimulation five times per week for 4 weeks. Upper limb function evaluation was performed using upper extremity part of fugl meyer assessment. [Results] Before and after intervention, fugl meyer assessment showed significant improvement...
December 2017: Journal of Physical Therapy Science
https://www.readbyqxmd.com/read/29629201/anesthesia-and-fast-track-in-video-assisted-thoracic-surgery-vats-from-evidence-to-practice
#8
REVIEW
Marzia Umari, Stefano Falini, Matteo Segat, Michele Zuliani, Marco Crisman, Lucia Comuzzi, Francesco Pagos, Stefano Lovadina, Umberto Lucangelo
In thoracic surgery, the introduction of video-assisted thoracoscopic techniques has allowed the development of fast-track protocols, with shorter hospital lengths of stay and improved outcomes. The perioperative management needs to be optimized accordingly, with the goal of reducing postoperative complications and speeding recovery times. Premedication performed in the operative room should be wisely administered because often linked to late discharge from the post-anesthesia care unit (PACU). Inhalatory anesthesia, when possible, should be preferred based on protective effects on postoperative lung inflammation...
March 2018: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29626229/left-ventricular-hypertrabeculation-noncompaction-cardiac-phenotype-and-neuromuscular-disorders
#9
C Stöllberger, C Wegner, J Finsterer
BACKGROUND: The prognosis of patients with left ventricular hypertrabeculation/noncompaction (LVHT) and its association with neuromuscular disorders (NMDs) is a controversial topic. The aim of this study was to assess whether the prognosis of LVHT patients is dependent on cardiac phenotype and the presence of NMDs. METHODS: Consecutive patients who were diagnosed with LVHT between 1995 and 2016 were included in the study. Cardiac phenotype was classified according to the recommendations of the European Society of Cardiology as: "dilated" if the left ventricular end-diastolic diameter (LVEDD) was >57 mm and left ventricular fractional shortening (FS) was ≤25%; "hypertrophic" if LVEDD was ≤57 mm, FS > 25%, and left ventricular posterior wall (LVPWT) and interventricular septal thickness (IVST) were both >13 mm; "intermediate" if LVEDD was >57 mm and FS > 25% or if LVEDD was ≤57 mm and FS ≤ 25%; and "normal" if LVEDD was ≤57 mm, FS > 25%, and IVST and LVPWT ≤ 13 mm...
April 6, 2018: Herz
https://www.readbyqxmd.com/read/29626178/unique-clinical-and-neurophysiologic-profile-of-a-cohort-of-children-with-cmtx3
#10
Manoj Kanhangad, Kayla Cornett, Megan H Brewer, Garth A Nicholson, Monique M Ryan, Robert L Smith, Gopinath M Subramanian, Helen K Young, Stephan Züchner, Marina L Kennerson, Joshua Burns, Manoj P Menezes
OBJECTIVE: To describe in detail the clinical profile of Charcot-Marie-Tooth disease subtype 3 (CMTX3) to aid appropriate genetic testing and rehabilitative therapy. METHODS: We reviewed the clinical and neurophysiologic profile and CMT Pediatric Scale (CMTPedS) assessments of 11 children with CMTX3. RESULTS: Compared with the more common forms of CMT, CMT1A and CMTX, CMTX3 was characterized by early onset with early and progressive hand weakness...
April 6, 2018: Neurology
https://www.readbyqxmd.com/read/29625589/intramuscular-transplantation-of-bone-marrow-cells-prolongs-the-lifespan-of-sod1-g93a-mice-and-modulates-expression-of-prognosis-biomarkers-of-the-disease
#11
Amaya Rando, Diego Pastor, Mari Carmen Viso-León, Anna Martínez, Raquel Manzano, Xavier Navarro, Rosario Osta, Salvador Martínez
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive muscle weakness, paralysis and death. There is no effective treatment for ALS and stem cell therapy has arisen as a potential therapeutic approach. METHODS: SOD1 mutant mice were used to study the potential neurotrophic effect of bone marrow cells grafted into quadriceps femoris muscle. RESULTS: Bone marrow intramuscular transplants resulted in increased longevity with improved motor function and decreased motoneuron degeneration in the spinal cord...
April 6, 2018: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/29623241/collective-statement-regarding-patient-access-to-approved-therapies-from-the-center-directors-of-parent-project-muscular-dystrophy-s-certified-duchenne-care-centers
#12
Cristian Ionita, Kathi Kinnett, Katherine Mathews
The dystrophinopathies (Duchenne [DMD] and Becker muscular dystrophy) are progressive diseases that until recently had no specific treatments. New FDA pathways to drug approval in rare diseases have resulted in a dramatic increase in the number of treatment trials for DMD and recently, two approved drugs. Health insurance policies for DMD products have been constructed with limited input from neuromuscular specialists directly involved in patient care and without patient input. These policies often reflect a lack of understanding of the disease, clinical population or the treatment...
March 15, 2018: PLoS Currents
https://www.readbyqxmd.com/read/29610664/hypomagnesemia-in-critically-ill-patients
#13
REVIEW
Bent-Are Hansen, Øyvind Bruserud
Background: Magnesium (Mg) is essential for life and plays a crucial role in several biochemical and physiological processes in the human body. Hypomagnesemia is common in all hospitalized patients, especially in critically ill patients with coexisting electrolyte abnormalities. Hypomagnesemia may cause severe and potential fatal complications if not timely diagnosed and properly treated, and associate with increased mortality. Main body: Mg deficiency in critically ill patients is mainly caused by gastrointestinal and/or renal disorders and may lead to secondary hypokalemia and hypocalcemia, and severe neuromuscular and cardiovascular clinical manifestations...
2018: Journal of Intensive Care
https://www.readbyqxmd.com/read/29609909/-why-is-the-morbidly-obese-patient-at-high-risk-of-anesthetic-complications
#14
Audrey De Jong, Daniel Verzilli, Marie Geniez, Gérald Chanques, David Nocca, Samir Jaber
Obesity is often associated with obstructive sleep apnea (OSA), which increases the risk of intraoperative and postoperative complications. The role of preoperative screening of OSA is crucial, with adequate management based on continuous positive pressure before, during and after surgery. The obese patient is at risk of postoperative complications: difficult airway management, acute respiratory failure following extubation due to atelectasis and airway obstruction, added to morphine overdosing. Optimal management of difficult mask ventilation and intubation, protective ventilation, combined to the reduction of sedatives and analgesics and the sitting position as soon as possible with a postoperative monitoring should decrease the occurrence of complications...
March 30, 2018: La Presse Médicale
https://www.readbyqxmd.com/read/29608040/muscle-restricted-nuclear-receptor-interaction-protein-knockout-causes-motor-neuron-degeneration-through-down-regulation-of-myogenin-at-the-neuromuscular-junction
#15
Hsin-Hsiung Chen, Li-Kai Tsai, Kuan-Yu Liao, Tung-Chien Wu, Yun-Hsin Huang, Yuan-Chun Huang, Szu-Wei Chang, Pei-Yu Wang, Yeou-Ping Tsao, Show-Li Chen
BACKGROUND: Nuclear receptor interaction protein (NRIP) is a calcium/calmodulin (CaM) binding protein. Nuclear receptor interaction protein interacts with CaM to activate calcineurin and CaMKII signalling. The conventional NRIP knockout mice (global knockout) showed muscular abnormality with reduction of muscle oxidative functions and motor function defects. METHODS: To investigate the role of NRIP on neuromuscular system, we generated muscle-restricted NRIP knockout mice [conditional knockout (cKO)]...
April 2, 2018: Journal of Cachexia, Sarcopenia and Muscle
https://www.readbyqxmd.com/read/29604977/neuromuscular-complications-of-hiv-infection
#16
Michelle Kaku, David M Simpson
With the introduction of combination antiretroviral therapy, human immunodeficiency virus (HIV)-infected individuals are living longer, and are commonly confronted with chronic neuromuscular complications. The spectrum of neuromuscular disorders in patients living with HIV infection is wide, and is caused by HIV per se and its products, particular antiretroviral drugs, or a combination of both. The purpose of this chapter is to review peripheral nervous system disorders in the setting of HIV infection, and to provide a general approach to diagnosis and management of these disorders...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29589203/anabolic-therapy-for-the-treatment-of-osteoporosis-in-childhood
#17
REVIEW
Leanne M Ward, Frank Rauch
PURPOSE OF REVIEW: Numerous forms of osteoporosis in childhood are characterized by low bone turnover (for example, osteoporosis due to neuromuscular disorders and glucocorticoid exposure). Anti-resorptive therapy, traditionally used to treat osteoporosis in the young, is associated with further reductions in bone turnover, raising concerns about the long-term safety and efficacy of such therapy. These observations have led to increasing interest in the role of anabolic therapy to treat pediatric osteoporosis...
March 27, 2018: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/29584523/brain-behavior-mechanisms-for-the-transfer-of-neuromuscular-training-adaptions-to-simulated-sport-initial-findings-from-the-train-the-brain-project
#18
Dustin R Grooms, Adam W Kiefer, Michael A Riley, Jonathan D Ellis, Staci Thomas, Katie Kitchen, Christopher DiCesare, Scott Bonnette, Brooke Gadd, Kim D Barber Foss, Weihong Yuan, Paula Silva, Ryan Galloway, Jed Diekfuss, James Leach, Kate Berz, Gregory D Myer
CONTEXT: A limiting factor for reducing anterior cruciate ligament (ACL) injury risk is ensuring that the movement adaptions made during the prevention program transfer to sport-specific activity. Virtual reality provides a mechanism to assess transferability and neuroimaging provides a means to assay the neural processes allowing for such skill transfer. OBJECTIVE: To determine the neural mechanisms for injury risk reducing biomechanics transfer to sport after ACL injury prevention training...
March 27, 2018: Journal of Sport Rehabilitation
https://www.readbyqxmd.com/read/29582188/immune-mediated-necrotizing-myopathy
#19
REVIEW
Iago Pinal-Fernandez, Maria Casal-Dominguez, Andrew L Mammen
PURPOSE OF REVIEW: Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune myopathy characterized by relatively severe proximal weakness, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy, and infrequent extra-muscular involvement. Here, we will review the characteristics of patients with IMNM. RECENT FINDINGS: Anti-signal recognition particle (SRP) and anti-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) autoantibodies are closely associated with IMNM and define unique subtypes of patients...
March 26, 2018: Current Rheumatology Reports
https://www.readbyqxmd.com/read/29579078/a-novel-high-throughput-immunofluorescence-analysis-method-for-quantifying-dystrophin-intensity-in-entire-transverse-sections-of-duchenne-muscular-dystrophy-muscle-biopsy-samples
#20
Valentina Sardone, Matthew Ellis, Silvia Torelli, Lucy Feng, Darren Chambers, Deborah Eastwood, Caroline Sewry, Rahul Phadke, Jennifer E Morgan, Francesco Muntoni
Clinical trials using strategies aimed at inducing dystrophin expression in Duchenne muscular dystrophy (DMD) are underway or at advanced planning stage, including splice switching antisense oligonucleotides (AON), drugs to induce read-through of nonsense mutations and viral mediated gene therapy. In all these strategies, different dystrophin proteins, often internally deleted, are produced, similar to those found in patients with the milder DMD allelic variant, Becker muscular dystrophy (BMD). The primary biological endpoint of these trials is to induce functional dystrophin expression...
2018: PloS One
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