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Neuromuscular disease

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https://www.readbyqxmd.com/read/29666207/split-hand-muscle-echo-intensity-index-as-a-reliable-imaging-marker-for-differential-diagnosis-of-amyotrophic-lateral-sclerosis
#1
Hung Youl Seok, Jinseok Park, Yoo Hwan Kim, Ki-Wook Oh, Seung Hyun Kim, Byung-Jo Kim
OBJECTIVE: The objective of this study was to investigate the usefulness of muscle ultrasound in evaluating dissociated small hand muscle atrophy, termed 'split hand', and its feasibility in the diagnosis of amyotrophic lateral sclerosis (ALS). METHODS: Forty-four patients with ALS, 18 normal subjects and 9 patients with other neuromuscular disorders were included in this study. The hand muscles were divided into three regions, the median-innervated lateral hand muscle group (ML), the ulnar-innervated lateral hand muscle (UL) and the ulnar-innervated medial hand muscle (UM), and the muscle echo intensity (EI) and compound muscle action potential (CMAP) were measured...
April 17, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29666144/sporadic-amyotrophic-lateral-sclerosis-sals-skeletal-muscle-response-to-cerebrospinal-fluid-from-sals-patients-in-a-rat-model
#2
Shruthi Shanmukha, Gayathri Narayanappa, Atchayaram Nalini, Phalguni Anand Alladi, Trichur R Raju
Skeletal muscle atrophy is the most prominent feature of amyotrophic lateral sclerosis (ALS), an adult-onset neurodegenerative disease of motor neurons. However, the contribution of skeletal muscle to disease progression remains elusive. Our previous studies have shown that intrathecal injection of cerebrospinal fluid from sporadic ALS patients (ALS-CSF) induces several degenerative changes in motor neurons and glia of neonatal rats. Here, we describe various pathologic events in the rat extensor digitorum longus muscle following intrathecal injection of ALS-CSF...
April 16, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29663410/the-negotiated-equilibrium-model-of-spinal-cord-function
#3
Jonathan R Wolpaw
The belief that the spinal cord is hardwired is no longer tenable. Like the rest of the CNS, the spinal cord changes during growth and aging, when new motor behaviours are acquired, and in response to trauma and disease. This paper describes a new model of spinal cord function that reconciles its recently appreciated plasticity with its long recognized reliability as the final common pathway for behaviour. According to this model, the substrate of each motor behaviour comprises brain and spinal plasticity: the plasticity in the brain induces and maintains the plasticity in the spinal cord...
April 16, 2018: Journal of Physiology
https://www.readbyqxmd.com/read/29663403/smoke-induced-neuromuscular-junction-degeneration-precedes-the-fibre-type-shift-and-atrophy-in-copd
#4
Sophia Kapchinsky, Madhusudanarao Vuda, Kayla Miguez, Daren Elkrief, Angela R de Souza, Carolyn J Baglole, Sudhakar Aare, Norah J MacMillan, Jacinthe Baril, Paul Rozakis, Vita Sonjak, Charlotte Pion, Mylène Aubertin-Leheudre, Jose A Morais, R Thomas Jagoe, Jean Bourbeau, Tanja Taivassalo, Russell T Hepple
A neurological basis for the fast fibre shift and atrophy seen in limb muscle of patients with chronic obstructive pulmonary disease (COPD) has not been considered previously. The objective of our study was: (i) to determine if denervation contributes to fast fibre shift and muscle atrophy in COPD; and (ii) to assess using a preclinical smoking mouse model whether chronic tobacco smoke (TS) exposure could initiate denervation by causing neuromuscular junction (NMJ) degeneration. Vastus lateralis muscle biopsies were obtained from severe COPD patients (n = 10 with low fat free mass index [FFMI], 65 y; n = 15 normal FFMI, 65 y) and healthy age- and activity-matched non-smoker control subjects (CON; n = 11, 67 y), to evaluate morphological and transcriptional markers of denervation...
April 16, 2018: Journal of Physiology
https://www.readbyqxmd.com/read/29662923/effects-of-electrical-stimulation-on-skeletal-muscle-of-old-sedentary-people
#5
Simone Mosole, Sandra Zampieri, Sandra Furlan, Ugo Carraro, Stefan Löefler, Helmut Kern, Pompeo Volpe, Alessandra Nori
Physical activity plays an important role in preventing muscle atrophy and chronic diseases in adults and in the elderly. Calcium (Ca2+ ) cycling and activation of specific molecular pathways are essential in contraction-induced muscle adaptation. This study attains human muscle sections and total homogenates prepared from biopsies obtained before (control) and after 9 weeks of training by electrical stimulation (ES) on a group of volunteers. The aim of the study was to investigate about the molecular mechanisms that support functional muscle improvement by ES...
January 2018: Gerontology & Geriatric Medicine
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
#6
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/29660267/recovery-process-and-determinants-of-adverse-event-occurrence-in-bronchoscopic-procedures-performed-under-general-anesthesia
#7
Derya Özden Omaygenç, Nermin Ünal, Saadet İpek Edipoğlu, Tuğçe Barca Şeker, Mehmet Akif Özgül, Demet Turan, Cengiz Özdemir, İbrahim Oğuz Karaca, Erdoğan Çetinkaya
OBJECTIVE: Regarding the fact that rigid bronchoscopy is generally performed under general anesthesia and this patient subgroup is remarkably morbid, encountering procedure and/or anesthesia related complications are highly likely. Here, we aimed to assess factors influencing recovery and detect possible determinants of adverse event occurence during these operations performed in a tertiary referral centre. METHODS: 81 consecutive ASA I-IV patients were recruited for this investigation...
April 16, 2018: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/29658931/optical-cross-sectional-muscle-area-determination-of-drosophila-melanogaster-adult-indirect-flight-muscles
#8
Estela Selma-Soriano, Rubén Artero, Beatriz Llamusi
Muscle mass wasting, known as muscle atrophy, is a common phenotype in Drosophila models of neuromuscular diseases. We have used the indirect flight muscles (IFMs) of flies, specifically the dorso-longitudinal muscles (DLM), as the experimental subject to measure the atrophic phenotype brought about by different genetic causes. In this protocol, we describe how to embed fly thorax muscles for semi thin sectioning, how to obtain a good contrast between muscle and the surrounding tissue, and how to process optical microscope images for semiautomatic acquisition of quantifiable data and analysis...
March 31, 2018: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29657216/colistin-induced-myasthenic-syndrome-in-a-patient-with-end-stage-renal-disease
#9
Pratim Sengupta, Sumanta Biswas
With resurgence of multidrug resistance (MDR) bacteria and no new novel broad-spectrum antibiotic in research pipeline, usage of older generation antibiotics, once discarded due to their toxicity profile are becoming popular again. Often these drugs are the only option left in managing MDR bacteria-related sepsis. Colistin is one of such antibiotic which is often used in recent times after decades of its avoidance due to its diverse toxicity profile. In this case report, we present a rare myasthenic syndrome like neuromuscular complication developed in a patient after receiving colistin for treatment of MDR Klebsiella-related urosepsis...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29656576/repurposing-carbamazepine-for-the-treatment-of-amyotrophic-lateral-sclerosis-in-sod1-g93a-mouse-model
#10
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/29655528/a-mobile-app-for-patients-with-pompe-disease-and-its-possible-clinical-applications
#11
Giulia Ricci, Sigrid Baldanzi, Fabrizio Seidita, Chiara Proietti, Francesca Carlini, Silvia Peviani, Giovanni Antonini, Andrea Vianello, Gabriele Siciliano
In recent years, the potential of smart technology to provide innovative solutions for disease management has raised high expectations for patients' and healthcare professionals' community. We developed a mobile app, called AIGkit, specifically designed for adult patients with Pompe disease, with the aim to help them manage the burden of illness-related factors, and also to provide clinicians with continuous tracking of each patient in real-time and ambient conditions of everyday life. We present the AIGkit as an innovative approach exploiting cutting-edge technology to improve quality of care and research into neuromuscular disorders...
March 12, 2018: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29655448/generalized-myasthenia-gravis-classification-clinical-presentation-natural-history-and-epidemiology
#12
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/29655446/practical-anatomy-of-the-neuromuscular-junction-in-health-and-disease
#13
REVIEW
Hiroshi Nishimune, Kazuhiro Shigemoto
Neuromuscular junctions (NMJs) form between nerve terminals of spinal cord motor neurons and skeletal muscles, and perisynaptic Schwann cells and kranocytes cap NMJs. One muscle fiber has one NMJ, which is innervated by one motor nerve terminal. NMJs are excitatory synapses that use P/Q-type voltage-gated calcium channels to release the neurotransmitter acetylcholine. Acetylcholine receptors accumulate at the postsynaptic specialization called the end plate on the muscle fiber membrane, the sarcolemma. Proteins essential for the organization of end plates include agrin secreted from nerve terminals, Lrp4 and MuSK receptors for agrin, and Dok-7 and rapsyn cytosolic proteins in the muscle...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29652962/-myasthenia-gravis-and-its-association-with-lymphoproliferative-disorders-a-case-series
#14
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/29651734/palivizumab-use-in-infants-with-down-syndrome-report-from-the-german-synagis%C3%A2-registry-2009-2016
#15
Arne Simon, Susanne Gehrmann, Gudrun Wagenpfeil, Stefan Wagenpfeil
Infants with Down syndrome (DS) face an increased risk of respiratory tract infections. Recent studies describe DS as independent risk factor for a complicated clinical course in infants with respiratory syncytial virus (RSV) infection. The prospective observational German Synagis™ Registry comprises data from 249 children below 25 months of age with DS and palivizumab prophylaxis 2009-2016 (1191 administrations; mean 4.8 per patient and season). The median gestational age and the birth weight in patients without and with DS were 31 versus 37 weeks (P < 0...
April 12, 2018: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29651162/quantitative-methods-to-monitor-rna-biomarkers-in-myotonic-dystrophy
#16
Marzena Wojciechowska, Krzysztof Sobczak, Piotr Kozlowski, Saam Sedehizadeh, Agnieszka Wojtkowiak-Szlachcic, Karol Czubak, Robert Markus, Anna Lusakowska, Anna Kaminska, J David Brook
Myotonic dystrophy type 1 (DM1) and type 2 (DM2) are human neuromuscular disorders associated with mutations of simple repetitive sequences in affected genes. The abnormal expansion of CTG repeats in the 3'-UTR of the DMPK gene elicits DM1, whereas elongated CCTG repeats in intron 1 of ZNF9/CNBP triggers DM2. Pathogenesis of both disorders is manifested by nuclear retention of expanded repeat-containing RNAs and aberrant alternative splicing. The precise determination of absolute numbers of mutant RNA molecules is important for a better understanding of disease complexity and for accurate evaluation of the efficacy of therapeutic drugs...
April 12, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29649746/degradation-of-alpha-synuclein-by-dendritic-cell-factor-1-delays-neurodegeneration-and-extends-lifespan-in-drosophila
#17
Shiqing Zhang, Ruili Feng, Yanhui Li, Linhua Gan, Fangfang Zhou, Shiquan Meng, Qian Li, Tieqiao Wen
Parkinson's disease (PD) is a common neurodegenerative disease associated with the progressive loss of dopaminergic neurons in the substantia nigra. Proteinaceous depositions of alpha-synuclein (α-syn) and its mutations, A30P and A53T, are one important characteristic of PD. However, little is known about their aggregation and degradation mechanisms. Dendritic cell factor 1 (DCF1) is a membrane protein that plays important roles in nerve development in mouse. In this study, we aimed to show that DCF1 overexpression in a PD Drosophila model significantly ameliorates impaired locomotor behavior in third instar larvae and normalizes neuromuscular junction growth...
March 13, 2018: Neurobiology of Aging
https://www.readbyqxmd.com/read/29649521/modelling-motor-neuron-disease-in-fruit-flies-lessons-from-spinal-muscular-atrophy
#18
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
#19
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/29645070/-isolated-girdle-weakness-expansion-of-the-phenotypic-spectrum-of-the-merrf-8344a-g-mutation-of-mitochondrial-dna
#20
A Erdocia-Goni, A Alonso-Jimenez, C Ramon-Carbajo, E Garcia-Arumi, P Casquero, E Gallardo, J Diaz-Manera
INTRODUCTION: The differential diagnosis of diseases that are accompanied by adult-onset girdle weakness is broad and includes motor neurone, neuromuscular junction or muscular diseases. The 8344A>G mutation of the MTTK gene of mitochondrial DNA usually presents with involvement of multiple organs associated (or not) with girdle weakness. To date no cases of isolated girdle weakness have been reported as the presenting symptom of this mutation. CASE REPORT: A 57-year-old male, with a four-year history of isolated clinical signs of progressive girdle weakness...
April 16, 2018: Revista de Neurologia
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