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Neuromuscular disease

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https://www.readbyqxmd.com/read/29049016/skeletal-muscle-ultrasonography-in-nutrition-and-functional-outcome-assessment-of-critically-ill-children-experience-and-insights-from-pediatric-disease-and-adult-critical-care-studies-formula-see-text
#1
Chengsi Ong, Jan Hau Lee, Melvin K S Leow, Zudin A Puthucheary
Evidence suggests that critically ill children develop muscle wasting, which could affect outcomes. Muscle ultrasound has been used to track muscle wasting and association with outcomes in critically ill adults but not children. This review aims to summarize methodological considerations of muscle ultrasound, structural findings, and possibilities for its application in the assessment of nutrition and functional outcomes in critically ill children. Medline, Embase, and CINAHL databases were searched up until April 2016...
September 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29048368/the-structure-of-human-neuromuscular-junctions-some-unanswered-molecular-questions
#2
REVIEW
Clarke R Slater
The commands that control animal movement are transmitted from motor neurons to their target muscle cells at the neuromuscular junctions (NMJs). The NMJs contain many protein species whose role in transmission depends not only on their inherent properties, but also on how they are distributed within the complex structure of the motor nerve terminal and the postsynaptic muscle membrane. These molecules mediate evoked chemical transmitter release from the nerve and the action of that transmitter on the muscle...
October 19, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29046769/factors-that-affect-the-onset-of-action-of-non-depolarizing-neuromuscular-blocking-agents
#3
REVIEW
Yong Byum Kim, Tae-Yun Sung, Hong Seuk Yang
Neuromuscular blockade plays an important role in the safe management of patient airways, surgical field improvement, and respiratory care. Rapid-sequence induction of anesthesia is indispensable to emergency surgery and obstetric anesthesia, and its purpose is to obtain a stable airway, adequate depth of anesthesia, and appropriate respiration within a short period of time without causing irritation or damage to the patient. There has been a continued search for new neuromuscular blocking drugs (NMBDs) with a rapid onset of action...
October 2017: Korean Journal of Anesthesiology
https://www.readbyqxmd.com/read/29046475/evidence-for-mast-cells-contributing-to-neuromuscular-pathology-in-an-inherited-model-of-als
#4
Emiliano Trias, Sofía Ibarburu, Romina Barreto-Núñez, Valentina Varela, Ivan C Moura, Patrice Dubreuil, Olivier Hermine, Joseph S Beckman, Luis Barbeito
Evidence indicates that neuroinflammation contributes to motor neuron degeneration in amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease leading to progressive muscular paralysis. However, it remains elusive whether inflammatory cells can interact with degenerating distal motor axons, influencing the progressive denervation of neuromuscular junctions (NMJs). By analyzing the muscle extensor digitorum longus (EDL) following paralysis onset in the SOD1G93A rat model, we have observed a massive infiltration and degranulation of mast cells, starting after paralysis onset and correlating with progressive NMJ denervation...
October 19, 2017: JCI Insight
https://www.readbyqxmd.com/read/29043641/measuring-dynamic-behavior-of-trinucleotide-repeat-tracts-in-vivo-in-saccharomyces-cerevisiae
#5
Gregory M Williams, Jennifer A Surtees
Trinucleotide repeat (TNR) tracts are inherently unstable during DNA replication, leading to repeat expansions and/or contractions. Expanded tracts are the cause of over 40 neurodegenerative and neuromuscular diseases. In this chapter, we focus on the (CNG)n repeat sequences that, when expanded, lead to Huntington's disease (HD), myotonic dystrophy type 1 (DM1), and a number of other neurodegenerative diseases. We describe a series of in vivo assays, using the model system Saccharomyces cerevisiae, to determine and characterize the dynamic behavior of TNR tracts that are in the early stages of expansion, i...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29040265/analysis-of-peripheral-b-cells-and-autoantibodies-against-the-anti-nicotinic-acetylcholine-receptor-derived-from-patients-with-myasthenia-gravis-using-single-cell-manipulation-tools
#6
Tomohiro Makino, Ryuichi Nakamura, Maki Terakawa, Satoshi Muneoka, Kazuhiro Nagahira, Yuriko Nagane, Jyoji Yamate, Masakatsu Motomura, Kimiaki Utsugisawa
The majority of patients with myasthenia gravis (MG), an organ-specific autoimmune disease, harbor autoantibodies that attack the nicotinic acetylcholine receptor (nAChR-Abs) at the neuromuscular junction of skeletal muscles, resulting in muscle weakness. Single cell manipulation technologies coupled with genetic engineering are very powerful tools to examine T cell and B cell repertoires and the dynamics of adaptive immunity. These tools have been utilized to develop mAbs in parallel with hybridomas, phage display technologies and B-cell immortalization...
2017: PloS One
https://www.readbyqxmd.com/read/29037990/roles-for-the-vcp-co-factors-npl4-and-ufd1-in-neuronal-function-in-drosophila-melanogaster
#7
Dwayne J Byrne, Mark J Harmon, Jeremy C Simpson, Craig Blackstone, Niamh C O'Sullivan
The VCP-Ufd1-Npl4 complex regulates proteasomal processing within cells by delivering ubiquitinated proteins to the proteasome for degradation. Mutations in VCP are associated with two neurodegenerative diseases, amyotrophic lateral sclerosis (ALS) and inclusion body myopathy with Paget's disease of the bone and frontotemporal dementia (IBMPFD), and extensive study has revealed crucial functions of VCP within neurons. By contrast, little is known about the functions of Npl4 or Ufd1 in vivo. Using neuronal-specific knockdown of Npl4 or Ufd1 in Drosophila melanogaster, we infer that Npl4 contributes to microtubule organization within developing motor neurons...
September 21, 2017: Journal of Genetics and Genomics, Yi Chuan Xue Bao
https://www.readbyqxmd.com/read/29036181/juvenile-myasthenia-gravis-in-norway-hla-drb1-04-04-is-positively-associated-with-prepubertal-onset
#8
T H Popperud, M K Viken, E Kerty, B A Lie
BACKGROUND: Juvenile myasthenia gravis (MG) is a rare autoantibody mediated autoimmune disorder targeting the neuromuscular endplate. The clinical hallmark is muscle weakness and fatigability. Disease aetiology is complex, including both genetic and environmental factors. The involvement of genes in the human leukocyte antigen (HLA) is well established in adult MG. However, HLA associations in European juvenile MG have not been studied. This case-control study aimed to investigate and characterize genetic risk factors in prepubertal and postpubertal onset juvenile MG...
2017: PloS One
https://www.readbyqxmd.com/read/29033729/use-of-rocuronium-and-sugammadex-under-neuromuscular-transmission-monitoring-in-a-patient-with-multiple-sclerosis
#9
Chryssoula Staikou, Martina Rekatsina
Multiple sclerosis (MS) is a potentially disabling disease characterized by demyelinating lesions in the central nervous system. One of the anesthetic challenges encountered in surgical patients with MS is the management of neuromuscular blockade (NMB) and its reversal. We report a case of a 31-year-old female patient suffering from MS, who underwent gynecological surgery under general anesthesia with sevoflurane, fentanyl, and rocuronium which was successfully reversed with sugammadex. Neuromuscular transmission (NMT) monitoring was used to guide the intraoperative doses of rocuronium and also the reversal of NMB by the use of sugammadex to ensure a safe tracheal extubation...
October 2017: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/29032483/regulation-of-cardiac-voltage-gated-sodium-channel-by-kinases-roles-of-protein-kinases-a-and-c
#10
Ademuyiwa S Aromolaran, Mohamed Chahine, Mohamed Boutjdir
In the heart, voltage-gated sodium (Nav) channel (Nav1.5) is defined by its pore-forming α-subunit and its auxiliary β-subunits, both of which are important for its critical contribution to the initiation and maintenance of the cardiac action potential (AP) that underlie normal heart rhythm. The physiological relevance of Nav1.5 is further marked by the fact that inherited or congenital mutations in Nav1.5 channel gene SCN5A lead to altered functional expression (including expression, trafficking, and current density), and are generally manifested in the form of distinct cardiac arrhythmic events, epilepsy, neuropathic pain, migraine, and neuromuscular disorders...
October 15, 2017: Handbook of Experimental Pharmacology
https://www.readbyqxmd.com/read/29030231/als-associated-mutation-sod1-g93a-leads-to-abnormal-mitochondrial-dynamics-in-osteocytes
#11
Huan Wang, Jianxun Yi, Xuejun Li, Yajuan Xiao, Kamal Dhakal, Jingsong Zhou
While the death of motor neuron is a pathological hallmark of amyotrophic lateral sclerosis (ALS), defects in other cell types or organs may also actively contribute to ALS disease progression. ALS patients experience progressive skeletal muscle wasting that may not only exacerbate neuronal degeneration, but likely has a significant impact on bone function. In our previous published study, we have discovered severe bone loss in an ALS mouse model with overexpression of ALS-associated mutation SOD1(G93A) (G93A)...
October 10, 2017: Bone
https://www.readbyqxmd.com/read/29021758/acute-neuromuscular-adaptations-in-the-postural-control-of-patients-with-parkinson-s-disease-after-perturbed-walking
#12
Cristian F Pasluosta, Simon Steib, Sarah Klamroth, Heiko Gaßner, Julia Goßler, Julius Hannink, Vinzenz von Tscharner, Klaus Pfeifer, Juergen Winkler, Jochen Klucken, Bjoern M Eskofier
Patients suffering from Parkinson's disease (PD) present motor impairments reflected in the dynamics of the center of pressure (CoP) adjustments during quiet standing. One method to study the dynamics of CoP adjustments is the entropic half-life (EnHL), which measures the short-term correlations of a time series at different time scales. Changes in the EnHL of CoP time series suggest neuromuscular adaptations in the control of posture. In this study, we sought to investigate the immediate changes in the EnHL of CoP adjustments of patients with PD during one session of perturbed (experimental group) and unperturbed treadmill walking (control group)...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/29019017/leptospirosis-associated-catastrophic-respiratory-failure-supported-by-extracorporeal-membrane-oxygenation
#13
Tamara Cantwell, Andrés Ferre, Nicolette Van Sint Jan, Rodrigo Blamey, Jorge Dreyse, Cristian Baeza, Rodrigo Diaz, Tomás Regueira
A previously healthy, 39-year-old obese farmer, arrived hypotensive and tachycardic, with fever, myalgia, headache, abdominal pain, diarrhea, and progressive dyspnea. Ten days before symptoms onset, he was in direct contact with mice and working in a contaminated drain. Patient laboratory showed acute kidney injury and thrombocytopenia. Chest X-ray exhibited bilateral diffuse interstitial infiltrates. First-line empirical antibiotics were started and influenza discarded. Patient evolved with severe respiratory failure, associated with hemoptysis, and rapidly severe hemodynamic compromise...
October 10, 2017: Journal of Artificial Organs: the Official Journal of the Japanese Society for Artificial Organs
https://www.readbyqxmd.com/read/29018589/neurological-complications-in-a-polynesian-traveler-with-dengue
#14
Maegan Lm Doi, Sydney Y Tatsuno, Gurdev Singh, Eric M Tatsuno, Marjorie M Mau
In recent times, there has been an increased focus on mosquito-borne Flaviviruses, in particular dengue and Zika. With the reappearance of dengue in Hawai'i and the mainland United States (US), clinicians should be aware of both the common presentations of dengue, as well as other less common complications associated with the disease. Dengue can result in neurologic disorders such as encephalopathy, encephalitis, immune-mediated syndromes, neuromuscular dysfunction, and neuro-ophthalmologic disorders. We present an interesting case of dengue that initially presented with classic symptoms (arthropathy, biphasic fever, and rash) and subsequently developed into a neurologic movement disorder with muscle tightening and twitching of the face, chest, and extremities...
October 2017: Hawai'i Journal of Medicine & Public Health: a Journal of Asia Pacific Medicine & Public Health
https://www.readbyqxmd.com/read/29016857/biallelic-mutation-of-unc50-encoding-a-protein-involved-in-achr-trafficking-is-responsible-for-arthrogryposis
#15
Emanuela Abiusi, Manuela D'Alessandro, Klaus Dieterich, Loic Quevarec, Sandrina Turczynski, Aurore-Cecile Valfort, Paulette Mezin, Pierre Simon Jouk, Marta Gut, Ivo Gut, Jean Louis Bessereau, Judith Melki
Arthrogryposis multiplex congenita (AMC) is a developmental condition characterized by multiple joint contractures resulting from reduced or absent fetal movements. Homozygosity mapping of disease loci combined with whole exome sequencing in a consanguineous family presenting with lethal AMC allowed the identification of a homozygous frameshift deletion in UNC50 gene (c.750_751del:p.Cys251Phefs*4) in the index case. To assess the effect of the mutation, an equivalent mutation in the Caenorhabditis elegans orthologous gene was created using CRISPR/Cas9...
October 15, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28992936/-short-term-exercise-based-cardiac-rehabilitation-induced-changes-in-cardiorespiratory-mechanical-and-neuromuscular-responses-to-progressive-exercise-testing
#16
P-M Leprêtre, M Ghannem, S Delanaud, N Jaunet, L Gaillard, A Barnabé, T Porcher, T Weissland
Previous studies showed that changes in peak of oxygen uptake value (VO2peak) with training were poorly related to changes in Maximal Tolerated Power output (MTP) among patients with cardiovascular disease. This result could be due to a difference between cardiopulmonary adaptation to training and the skeletal muscle conditioning. OBJECTIVE: The aim of the study was to compare the responses to exercise training of electromyographic activities of vastus lateralis (rms-EMG) and respiratory parameters...
October 6, 2017: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/28988152/acute-effects-of-static-stretching-on-the-shear-elastic-moduli-of-the-medial-and-lateral-gastrocnemius-muscles-in-young-and-elderly-women
#17
Masatoshi Nakamura, Tome Ikezoe, Satoru Nishishita, Jun Umehara, Misaka Kimura, Noriaki Ichihashi
PURPOSE: Generally, static stretching (SS) is the recommended intervention for a decline in the range of motion among elderly adults. However, no study has investigated the acute effects of SS on the shear elastic modulus in elderly people. The aims of the present study were to investigate the acute effects of SS on the shear elastic moduli of the medial and lateral gastrocnemius muscles and to examine the differences in these acute effects between young and elderly women. METHODS: This study included 15 healthy young women (age: 23...
September 25, 2017: Musculoskeletal Science & Practice
https://www.readbyqxmd.com/read/28987470/potential-therapeutic-impact-of-omega-3-long-chain-polyunsaturated-fatty-acids-on-inflammation-markers-in-duchenne-muscular-dystrophy-a-double-blind-controlled-randomized-trial
#18
Maricela Rodríguez-Cruz, Oriana Del Rocío Cruz-Guzmán, Tomás Almeida-Becerril, Alan Donovan Solís-Serna, Salvador Atilano-Miguel, Juan Raúl Sánchez-González, Lourdes Barbosa-Cortés, Eugenia Dolores Ruíz-Cruz, Juan Carlos Huicochea, Alan Cárdenas-Conejo, Rosa Elena Escobar-Cedillo, Carlos Alberto Yam-Ontiveros, Edgar F Ricárdez-Marcial
BACKGROUND & AIMS: Duchenne Muscular Dystrophy (DMD) is the most frequent dystrophy in childhood generated by a deficiency in dystrophin. DMD is a neuromuscular disease and its clinical course comprises chronic inflammation and gradual muscle weakness. Supplementation of omega-3 long chain-Polyunsaturated Fatty Acids (ω-3 long chain-PUFA) reduces inflammatory markers in various disorders. The goal of this research was to analyze the influence of ω-3 long chain-PUFA intake on gene expression and blood inflammatory markers in boys with DMD...
September 23, 2017: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28987188/diseases-of-the-skeletal-muscle
#19
Edoardo Malfatti, Norma Beatriz Romero
After the advances created by the use of cryostat sections and histochemistry 60 years ago, muscle histopathology is now living a real renaissance. In the field of genetic neuromuscular disorders, muscle biopsy analysis is fundamental to address questions about pathogenicity and protein expression when new genes are discovered through next-generation sequencing approaches. Moreover, the identification of the same gene mutated in previously considered distinct histopathologic entities imposes a constant reassessment of morphologic boundaries in several groups of disorders...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28986620/-imaging-of-primary-muscular-diseases-what-do-neurologists-expect-from-radiologists
#20
REVIEW
M P Wattjes, A Fischmann, D Fischer
Imaging, in particular magnetic resonance imaging (MRI), has in recent years increasingly become a crucial tool for the diagnostics of inherited and acquired muscular diseases. The aim of imaging in neuromuscular disorders goes beyond the detection and quantification of degenerative muscular changes, such as fatty degeneration and includes recognition of very early signs of muscular pathologies presenting as muscular edema. Therefore, imaging is a valuable diagnostic method to support the clinical diagnosis and to narrow down the differential diagnoses, leading to specific additional diagnostic tests in order to establish the correct diagnosis...
October 6, 2017: Der Radiologe
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