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Neuromuscular weakness

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https://www.readbyqxmd.com/read/29770120/multiparametric-analysis-of-sniff-nasal-inspiratory-pressure-test-in-middle-stage-amyotrophic-lateral-sclerosis
#1
Antonio Sarmento, Andrea Aliverti, Layana Marques, Francesca Pennati, Mario Emílio Dourado-Júnior, Guilherme Fregonezi, Vanessa Resqueti
The relaxation rates and contractile properties of inspiratory muscles are altered with inspiratory muscle weakness and fatigue. This fact plays an important role in neuromuscular disorders patients and had never been extensively studied in amyotrophic lateral sclerosis (ALS). In this cross-sectional study, these parameters were investigated non-invasively through nasal inspiratory sniff pressure test (SNIP) in 39 middle stage spinal onset ALS subjects and compared with 39 healthy controls. ALS patients were also divided into three subgroups according to a decline in their percentage of predicted forced vital capacity (FVC%pred ) as well as a decline in the ALS functional rating scale score and its respiratory subscore (R-subscore) in order to determine the best parameter linked to early respiratory muscle weakness...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29765992/serological-immunoglobulin-free-light-chain-profile-in-myasthenia-gravis-patients
#2
Umberto Basile, Mariapaola Marino, Cecilia Napodano, Krizia Pocino, Paolo Emilio Alboini, Francesca Gulli, Amelia Evoli, Carlo Provenzano, Emanuela Bartoccioni
Background: Serological levels of free immunoglobulin light chains (FLCs), produced in excess of heavy chains during synthesis of immunoglobulins by plasma cells, can be considered a direct marker of B cell activity in different systemic inflammatory-autoimmune conditions and may represent a useful predictor of rituximab (RTX) therapeutic efficacy, as reported for rheumatoid arthritis and systemic lupus erythematosus. Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction with antibodies (abs) targeting the acetylcholine receptor (AChR) or the muscle-specific tyrosine kinase (MuSK), inducing muscle weakness and excessive fatigability...
2018: Journal of Immunology Research
https://www.readbyqxmd.com/read/29764215/myotonic-dystrophytype-1-report-of-non-24-h-sleep-wake-disorder-with-excessive-daytime-sleepiness
#3
Lucio Huebra Pimentel Filho, Ana Carolina Dias Gomes, Bruno Gonçalves, Sergio Tufik, Fernando Morgadinho Coelho
Myotonic dystrophy (MD) is a neuromuscular disease with myotonia, progressive weakness, and involvement of CNS, heart, and gastrointestinal system. Excessive daytime sleepiness (EDS) in myotonic dystrophy type 1 (MD1) is related to sleep breathing diseases, restless leg syndrome, periodic limb movements during sleep and narcoleptic-like phenotype. However, authors highlight a central dysfunction of sleep regulation. We describe a 26-year-old, female, MD1 patient with EDS. Sleep diary/actigraphy evidenced two different circadian periods with values of 1442 and 1522 min...
May 15, 2018: Chronobiology International
https://www.readbyqxmd.com/read/29722155/cold-adaptation-does-not-alter-atp-homeostasis-during-cold-exposure-in-drosophila-melanogaster
#4
Caroline M Williams, James R Rocca, Arthur S Edison, David B Allison, Theodore J Morgan, Daniel A Hahn
In insects and other ectotherms, cold temperatures cause a coma resulting from loss of neuromuscular function, during which ionic and metabolic homeostasis are progressively lost. Cold adaptation improves homeostasis during cold exposure, but the ultimate targets of selection are still an open question. Cold acclimation and adaptation remodels mitochondrial metabolism in insects, suggesting that aerobic energy production during cold exposure could be a target of selection. Here, we test the hypothesis that cold adaptation improves the ability to maintain rates of aerobic energy production during cold exposure by using 31 P NMR on live flies...
May 3, 2018: Integrative Zoology
https://www.readbyqxmd.com/read/29720219/gne-myopathy-from-clinics-and-genetics-to-pathology-and-research-strategies
#5
REVIEW
Oksana Pogoryelova, José Andrés González Coraspe, Nikoletta Nikolenko, Hanns Lochmüller, Andreas Roos
GNE myopathy is an ultra-rare autosomal recessive disease, which starts as a distal muscle weakness and ultimately leads to a wheelchair bound state. Molecular research and animal modelling significantly moved forward understanding of GNE myopathy mechanisms and suggested therapeutic interventions to alleviate the symptoms. Multiple therapeutic attempts are being made to supplement sialic acid depleted in GNE myopathy muscle cells. Translational research field provided valuable knowledge through natural history studies, patient registries and clinical trial, which significantly contributed to bringing forward an era of GNE myopathy treatment...
May 2, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29718870/ctla-4-methylation-regulates-the-pathogenesis-of-myasthenia-gravis-and-the-expression-of-related-cytokines
#6
Ti-Kun Fang, Cheng-Jun Yan, Juan Du
BACKGROUND: Myasthenia gravis (MG) is a progressive autoimmune disease that occurs as a result of the failure of neuromuscular transmission and is characterized by muscle weakness. There has been evidence on the correlations between the genetic predisposition of cytotoxic T lymphocyte and the antigen-4 (CTLA-4) and MG. Thus, the present study was conducted to study is designed to examine the effects of CTLA-4 methylation on the pathogenesis of MG and the expressions of related cytokines...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29712789/macrophage-depletion-ameliorates-peripheral-neuropathy-in-aging-mice
#7
Xidi Yuan, Dennis Klein, Susanne Kerscher, Brian L West, Joachim Weis, Istvan Katona, Rudolf Martini
Aging is known as a major risk factor for the structure and function of the nervous system. There is urgent need to overcome such deleterious effects of age-related neurodegeneration. Here we show that peripheral nerves of 24-month-old aging C57Bl6 mice of either sex show similar pathological alterations as nerves from aging human individuals while 12 months old adult mice lack such alterations. Specifically, nerve fibers showed de- and remyelination and axonal lesion. Moreover, in the aging mice, neuromuscular junctions showed features typical for dying-back neuropathies, as revealed by a decline of pre-synaptic markers, associated with α-bungarotoxin-positive postsynapses...
April 30, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29695970/sport-specific-assessment-of-the-effectiveness-of-neuromuscular-training-in-young-athletes
#8
REVIEW
Erika Zemková, Dušan Hamar
Neuromuscular training in young athletes improves performance and decreases the risk of injuries during sports activities. These effects are primarily ascribed to the enhancement of muscle strength and power but also balance, speed and agility. However, most studies have failed to demonstrate significant improvement in these abilities. This is probably due to the fact that traditional tests do not reflect training methods (e.g., plyometric training vs. isometric or isokinetic strength testing, dynamic balance training vs...
2018: Frontiers in Physiology
https://www.readbyqxmd.com/read/29689734/evaluation-of-children-with-sma-type-1-under-treatment-with-nusinersen-within-the-expanded-access-program-in-germany
#9
Astrid Pechmann, Thorsten Langer, David Schorling, Sabine Stein, Sibylle Vogt, Ulrike Schara, Heike Kölbel, Oliver Schwartz, Andreas Hahn, Kerstin Giese, Jessika Johannsen, Jonas Denecke, Claudia Weiß, Manuela Theophil, Janbernd Kirschner
BACKGROUND: Spinal muscular atrophy (SMA) is a neuromuscular disorder characterized by muscle weakness and muscle atrophy. Nusinersen acts as a splicing modifier and has recently been approved for intrathecal treatment of SMA. OBJECTIVE: Prior to approval, nusinersen was provided to patients with SMA type 1 in Germany within an Expanded Access Program (EAP). In contrast to previous clinical trials, children of different age groups and different stages of the disease were treated with nusinersen...
April 16, 2018: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/29687368/fluorescent-determination-of-succinylcholine-chloride-by-naphthalimide-stilbazolium-dye-%C3%A2-cp5a
#10
Qianxia Pan, Zihui He, Dongdong Xia, Xiaomin Gu, Haibo Wang, Jie Sun
Succinylcholine Chloride (SCC), a short-acting neuromuscular relaxant, is non-fluorescent in aqueous solutions. This property makes it impossible to be determined by direct fluorescent method. Naphthalimide dye (NA) exhibits very strong fluorescence emissions in aqueous solution, after complexing with carboxylatopillar[5]arene (CP5A) in aqueous solutions, the fluorescent quenched intensity of complex was observed. On the contraty, stilbazolium dye (SA) exhibits weak fluorescence emissions in aqueous solution, after being included by CP5A, a fluorescence enhancement was observed...
April 23, 2018: Journal of Fluorescence
https://www.readbyqxmd.com/read/29685414/a-novel-capn3-mutation-in-late-onset-limb-girdle-muscular-dystrophy-with-early-respiratory-insufficiency
#11
Jennifer M Martinez-Thompson, Steven A Moore, Teerin Liewluck
We describe a 70 year-old independently ambulatory man with a 10-year history of progressive axial and limb-girdle weakness, hyperCKemia, and a 5-year history of dyspnea requiring nocturnal ventilatory support due to a known c.1309C>T (p.Arg437Cys) variant and a novel in-frame deletion of exons 17-19 in the calpain-3 encoding gene (CAPN3). Pulmonary function tests revealed neuromuscular respiratory weakness. Biceps femoris biopsy showed chronic myopathic changes, numerous lobulated fibers, and reduced calpain-3 immunoreactivity...
April 20, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29683988/case-report-ocular-myasthenia-gravis-associated-with-in-vitro-fertilization-procedures
#12
Yung Ju Yoo, Sang Beom Han, Hee Kyung Yang, Jeong-Min Hwang
SIGNIFICANCE: Ocular myasthenia gravis is a localized form of myasthenia gravis, which is a postsynaptic disorder of the neuromuscular junction that causes fluctuating weakness of extraocular muscles resulting from autoimmune mechanisms. In women with myasthenia, changes in sex hormone levels and administration of corticosteroids can trigger or worsen symptoms of myasthenia gravis. PURPOSE: To describe a case of seronegative ocular myasthenia gravis whose first symptom appeared a day after in vitro fertilization procedure...
April 20, 2018: Optometry and Vision Science: Official Publication of the American Academy of Optometry
https://www.readbyqxmd.com/read/29681644/paraneoplastic-lambert-eaton-syndrome-in-a-patient-with-disseminated-metastatic-cancer
#13
Gregorio Arellano-Aguilar, Erik Santiago Núñez-Mojica, José Luis Gutiérrez-Velazco, Luis Gerardo Domínguez-Carrillo
Background: Neurological paraneoplastic syndromes are rare, occur in 0.01% of all cancer patients; like part of them, the Lambert-Eaton syndrome is an autoimmune presynaptic disorder of neuromuscular transmission characterized by muscle weakness and neurovegetative dysfunction, and often associated with small cell lung cancer. Case report: A 72 years old female with a family history of lung cancer and leukemia, with 7 months of dry cough and 3 months with waist and pelvic muscle weakness, oropharyngeal dysphagia, dry mouth, chronic constipation and weight loss of 10 kg...
2018: Cirugia y Cirujanos
https://www.readbyqxmd.com/read/29666602/myasthenia-gravis-induced-by-ipilimumab-in-a-patient-with-metastatic-melanoma
#14
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/29662906/quantitative-relationships-between-pulmonary-function-and-residual-neuromuscular-blockade
#15
ShuYing Fu, WenDong Lin, XiNing Zhao, ShengJin Ge, ZhangGang Xue
Background: Neuromuscular blockade is a risk factor for postoperative respiratory weakness during the immediate postoperative period. The quantitative relationships between postoperative pulmonary-function impairment and residual neuromuscular blockade are unknown. Methods: 113 patients who underwent elective laparoscopic cholecystectomy were enrolled in this study. They all had a pulmonary-function test (PFT) during the preoperative evaluation. Predictive values based on demographic data were also recorded...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29655456/lambert-eaton-myasthenic-syndrome
#16
REVIEW
Vita G Kesner, Shin J Oh, Mazen M Dimachkie, Richard J Barohn
Lambert-Eaton myasthenic syndrome is a paraneoplastic or primary autoimmune neuromuscular junction disorder characterized by proximal weakness, autonomic dysfunction and ariflexia. The characteristic symptoms are thought to be caused by antibodies generated against the P/Q-type voltage-gated calcium channels present on presynaptic nerve terminals and by diminished release of acetylcholine. More than half of Lambert-Eaton myasthenic syndrome cases are associated with small cell lung carcinoma. Diagnosis is confirmed by serologic testing and electrophysiologic studies...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655455/congenital-myasthenic-syndromes
#17
REVIEW
Perry B Shieh, Shin J Oh
The congenital myasthenic syndromes (CMS) are a group of rare genetic conditions characterized by abnormal neuromuscular transmission. Typically, these conditions have been the result of a dysfunctional protein that is present in the presynaptic terminal, the synaptic cleft, or the postsynaptic terminal. Many of these syndromes present within the first few years of life with fluctuating and fatiguable weakness in a distribution similar to myasthenia gravis, although a limb-girdle distribution and late onset are also seen in certain specific types of CMS...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655448/generalized-myasthenia-gravis-classification-clinical-presentation-natural-history-and-epidemiology
#18
REVIEW
Michael K Hehir, Nicholas J Silvestri
Myasthenia gravis (MG) is a rare disease, but the most common disorder of the neuromuscular junction. It is the prototypic autoimmune disease most commonly caused by antibodies to the acetylcholine receptor (AChR) leading to characteristic fatigable weakness of the ocular, bulbar, respiratory, axial, and limb muscles. The majority of patients with MG first present with ocular symptoms. Most patients with MG will experience at least 1 exacerbation of symptoms throughout the course of their illness. This article will cover the epidemiology, clinical presentation, classification, and natural history of MG...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29652962/-myasthenia-gravis-and-its-association-with-lymphoproliferative-disorders-a-case-series
#19
Gabriel Cea, Andrés Gallardo V, María Elena Cabrera C
Myasthenia gravis (MG) is a rare autoimmune disease of the neuromuscular junction. It is characterized by variable weakness and excessive fatigability of skeletal muscles. In the last few years, numerous reports have been published showing the association between autoimmune diseases, such as systemic erythematous lupus or rheumatoid arthritis, with lymphoid neoplasias. The association between MG and lymphoid neoplasia seems to be less frequent. To analyze this association we reviewed the MG patients in the Department of Neurology, Hospital Salvador of Santiago, Chile...
December 2017: Revista Médica de Chile
https://www.readbyqxmd.com/read/29652169/contribution-of-neuromuscular-factors-to-quadriceps-asymmetry-after-anterior-cruciate-ligament-reconstruction
#20
Alexa K Johnson, Riann M Palmieri-Smith, Lindsey K Lepley
CONTEXT:   To quantify quadriceps weakness after anterior cruciate ligament reconstruction (ACLR), researchers have often analyzed only peak torque. However, analyzing other characteristics of the waveform, such as the rate of torque development (RTD), time to peak torque (TTP), and central activation ratio (CAR), can lend insight into the underlying neuromuscular factors that regulate torque development. OBJECTIVE:   To determine if interlimb neuromuscular asymmetry was present in patients with ACLR at the time of clearance to return to activity...
April 13, 2018: Journal of Athletic Training
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