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Sample records for las alteraciones neuromusculares

  1. [Neuromuscular choristoma].

    PubMed

    Vajtai, I; Varga, Z; Hackel, J

    1999-08-01

    Neuromuscular choristomas are malformative pseudotumoral masses composed of striated muscle and peripheral nerves. This rare condition almost exclusively affects large nerve trunks of infants and young children, and may cause neurologic deficits of variable severity. We report a case of neuromuscular choristoma identified in the lumbosacral lipoma of a 4-month-old boy. The lesion was characterized by an organoid association of myogenic and neurogenic elements reminiscent of neuromuscular units. Myosin immunophenotyping revealed disordered groups of type-I and type-II extrafusal myofibers. There was no immunoreactivity for smooth muscle specific alpha actin. Most participating axons were of the large myelinated type. Pathogenetic theories of neuromuscular choristoma involve aberrant migration of developing peripheral nerves, dysgenesis of muscle spindles, as well as mesenchymal differentiation of the primitive neuroectoderm. Neuromuscular choristomas arising in connection with congenital lipomas may derive from a dysembryogenic process of stem cells of the caudal neural tube.

  2. Neuromuscular Scoliosis

    MedlinePlus

    ... degree of neuromuscular involvement. Diagnosis Incidence of Scoliosis Cerebral palsy (2 limbs involved) 25% Myelodysplasia (lower lumbar) 60% Spinal muscle atrophy 67% Friedreich ataxia 80% Cerebral palsy (4 limbs involved) 80% Duchenne muscular dystrophy 90% ...

  3. Neuromuscular scoliosis.

    PubMed

    Allam, Anand M; Schwabe, Aloysia L

    2013-11-01

    The purpose of this focused review is to provide an overview of neuromuscular scoliosis from the perspective of the rehabilitation physician. Scoliosis is a common consequence of neuromuscular diseases, including central nervous system disorders such as cerebral palsy and spinal cord injury; motor neuron disorders, for example, spinal muscular atrophy; muscle fiber disorders, for example, Duchenne muscular dystrophy; multifactorial disorders, for example, spina bifida; and many other neuropathic and myopathic conditions. Unlike adolescent idiopathic scoliosis, which is the most common form of spinal deformity, neuromuscular scoliosis is more severe and more progressive, and is associated with more morbidity. Factors that contribute to this spinal deformity include asymmetric paraplegia, imbalance of mechanical forces, intraspinal and congenital anomalies of the spine, altered sensory feedback, and abnormal posture via central pathways. Spinal deformity combined with limitations due to an underlying neuromuscular condition lead to significant physiologic impairments that affect limb movement, cardiopulmonary function, gait, standing, sitting, balance, trunk stability, bimanual activities, activities of daily living, and pain, as well as concerns with self-image and social interactions. Evaluation and management of this population requires understanding of disease progression, pulmonary status, functional limitations, indications for conservative and surgical interventions, and social considerations.

  4. Neuromuscular block.

    PubMed

    Bowman, W C

    2006-01-01

    Descriptions of the South American arrow poisons known as curares were reported by explorers in the 16th century, and their site of action in producing neuromuscular block was determined by Claude Bernard in the mid-19th century. Tubocurarine, the most important curare alkaloid, played a large part in experiments to determine the role of acetylcholine in neuromuscular transmission, but it was not until after 1943 that neuromuscular blocking drugs became established as muscle relaxants for use during surgical anaesthesia. Tubocurarine causes a number of unwanted effects, and there have been many attempts to replace it. The available drugs fall into two main categories: the depolarising blocking drugs and the nondepolarising blocking drugs. The former act by complex mixed actions and are now obsolete with the exception of suxamethonium, the rapid onset and brief duration of action of which remain useful for intubation at the start of surgical anaesthesia. The nondepolarising blocking drugs are reversible acetylcholine receptor antagonists. The main ones are the atracurium group, which possess a built-in self-destruct mechanism that makes them specially useful in kidney or liver failure, and the vecuronium group, which are specially free from unwanted side effects. Of this latter group, the compound rocuronium is of special interest because its rapid onset of action allows it to be used for intubation, and there is promise that its duration of action may be rapidly terminated by a novel antagonist, a particular cyclodextrin, that chelates the drug, thereby removing it from the acetylcholine receptors.

  5. Residual Neuromuscular Blockade.

    PubMed

    Plummer-Roberts, Anna L; Trost, Christina; Collins, Shawn; Hewer, Ian

    2016-02-01

    This article provides an update on residual neuromuscular blockade for nurse anesthetists. The neuromuscular junction, pharmacology for producing and reversing neuromuscular blockade, monitoring sites and methods, and patient implications relating to incomplete reversal of neuromuscular blockade are reviewed. Overall recommendations include using multiple settings when employing a peripheral nerve stimulator for monitoring return of neuromuscular function and administering pharmacologic reversal when the train-of-four ratio is below 0.9.

  6. TEACHING NEUROMUSCULAR RELAXATION.

    ERIC Educational Resources Information Center

    NORRIS, JEANNE E.; STEINHAUS, ARTHUR H.

    THIS STUDY ATTEMPTED TO FIND OUT WHETHER (1) THE METHODS FOR ATTAINING NEUROMUSCULAR RELAXATION THAT HAVE PROVED FRUITFUL IN THE ONE-TO-ONE RELATIONSHIP OF THE CLINIC CAN BE SUCCESSFULLY ADAPTED TO THE TEACHER-CLASS RELATIONSHIP OF THE CLASSROOM AND GYMNASIUM, AND (2) NEUROMUSCULAR RELAXATION CAN BE TAUGHT SUCCESSFULLY BY AN APPROPRIATELY TRAINED…

  7. Neuromuscular disorders in pregnancy.

    PubMed

    Guidon, Amanda C; Massey, E Wayne

    2012-08-01

    Preexisting and coincident neuromuscular disorders in pregnancy are challenging for clinicians because of the heterogeneity of disease and the limited data in the literature. Many questions arise regarding the effect of disease on the pregnancy, delivery, and newborn in addition to the effect of pregnancy on the course of disease. Each disorder has particular considerations and possible complications. An interdisciplinary team of physicians is essential. This article discusses the most recent literature on neuromuscular disorders in pregnancy including acquired root, plexus, and peripheral nerve lesions; acquired and inherited neuropathies and myopathies; disorders of the neuromuscular junction; and motor neuron diseases.

  8. Autoimmune Neuromuscular Disorders

    PubMed Central

    Kraker, Jessica; Živković, Saša A

    2011-01-01

    Autoimmune neuromuscular disorders affecting peripheral nerves, neuromuscular junction or muscle have a wide clinical spectrum with diverse pathogenetic mechanisms. Peripheral nervous system may be targeted in the context of complex immune reactions involving different cytokines, antigen-presenting cells, B cells and different types of T cells. Various immunomodulating and cytotoxic treatments block proliferation or activation of immune cells by different mechanisms attempting to control the response of the immune system and limit target organ injury. Most treatment protocols for autoimmune neuromuscular disorders are based on the use of corticosteroids, intravenous immunoglobulins and plasmapheresis, with cytotoxic agents mostly used as steroid-sparing medications. More recently, development of specific monoclonal antibodies targeting individual cell types allowed a different approach targeting specific immune pathways, but these new treatments are also associated with various adverse effects and their long-term efficacy is still unknown. PMID:22379454

  9. Biotherapies of neuromuscular disorders.

    PubMed

    Briand, J-F; Roy, M-O; Mourlane, F; André, C; Loux, N; Rougeau, C; Toursel, T; Braun, S

    2014-12-01

    This review focuses on the most recent data on biotherapeutic approaches, using DNA, RNA, recombinant proteins, or cells as therapeutic tools or targets for the treatment of neuromuscular diseases. Many of these novel technologies have now reached the clinical stage and have or are about to move to the market. Others, like genome editing are still in an early stage but hold great promise.

  10. Neuromuscular disorders in otolaryngology.

    PubMed

    Govett, G S; Amedee, R G

    1991-03-01

    Neuromuscular disorders pose an interesting diagnostic and therapeutic dilemma to clinicians. The initial manifestations of these disorders on physical examination are frequently subtle but may eventually progress to disabling, complex findings. Management options are varied and include pharmacologic, surgical, and other supportive modalities.

  11. Temperature and neuromuscular function.

    PubMed

    Racinais, S; Oksa, J

    2010-10-01

    This review focuses on the effects of different environmental temperatures on the neuromuscular system. During short duration exercise, performance improves from 2% to 5% with a 1 °C increase in muscle temperature. However, if central temperature increases (i.e., hyperthermia), this positive relation ceases and performance becomes impaired. Performance impairments in both cold and hot environment are related to a modification in neural drive due to protective adaptations, central and peripheral failures. This review highlights, to some extent, the different effects of hot and cold environments on the supraspinal, spinal and peripheral components of the neural drive involved in the up- and down-regulation of neuromuscular function and shows that temperature also affects the neural drive transmission to the muscle and the excitation-contraction coupling.

  12. Optimality in neuromuscular systems.

    PubMed

    Theodorou, Evangelos; Valero-Cuevas, Francisco J

    2010-01-01

    We provide an overview of optimal control methods to nonlinear neuromuscular systems and discuss their limitations. Moreover we extend current optimal control methods to their application to neuromuscular models with realistically numerous musculotendons; as most prior work is limited to torque-driven systems. Recent work on computational motor control has explored the used of control theory and estimation as a conceptual tool to understand the underlying computational principles of neuromuscular systems. After all, successful biological systems regularly meet conditions for stability, robustness and performance for multiple classes of complex tasks. Among a variety of proposed control theory frameworks to explain this, stochastic optimal control has become a dominant framework to the point of being a standard computational technique to reproduce kinematic trajectories of reaching movements (see [12]) In particular, we demonstrate the application of optimal control to a neuromuscular model of the index finger with all seven musculotendons producing a tapping task. Our simulations include 1) a muscle model that includes force- length and force-velocity characteristics; 2) an anatomically plausible biomechanical model of the index finger that includes a tendinous network for the extensor mechanism and 3) a contact model that is based on a nonlinear spring-damper attached at the end effector of the index finger. We demonstrate that it is feasible to apply optimal control to systems with realistically large state vectors and conclude that, while optimal control is an adequate formalism to create computational models of neuro-musculoskeletal systems, there remain important challenges and limitations that need to be considered and overcome such as contact transitions, curse of dimensionality, and constraints on states and controls.

  13. [Characteristics of neuromuscular scoliosis].

    PubMed

    Putzier, M; Groß, C; Zahn, R K; Pumberger, M; Strube, P

    2016-06-01

    Usually, neuromuscular scolioses become clinically symptomatic relatively early and are rapidly progressive even after the end of growth. Without sufficient treatment they lead to a severe reduction of quality of life, to a loss of the ability of walking, standing or sitting as well as to an impairment of the cardiopulmonary system resulting in an increased mortality. Therefore, an intensive interdisciplinary treatment by physio- and ergotherapists, internists, pediatricians, orthotists, and orthopedists is indispensable. In contrast to idiopathic scoliosis the treatment of patients with neuromuscular scoliosis with orthosis is controversially discussed, whereas physiotherapy is established and essential to prevent contractures and to maintain the residual sensorimotor function.Frequently, the surgical treatment of the scoliosis is indicated. It should be noted that only long-segment posterior correction and fusion of the whole deformity leads to a significant improvement of the quality of life as well as to a prevention of a progression of the scoliosis and the development of junctional problems. The surgical intervention is usually performed before the end of growth. A prolonged delay of surgical intervention does not result in an increased height but only in a deformity progression and is therefore not justifiable. In early onset neuromuscular scolioses guided-growth implants are used to guarantee the adequat development. Because of the high complication rates, further optimization of these implant systems with regard to efficiency and safety have to be addressed in future research.

  14. Computational Models for Neuromuscular Function

    PubMed Central

    Valero-Cuevas, Francisco J.; Hoffmann, Heiko; Kurse, Manish U.; Kutch, Jason J.; Theodorou, Evangelos A.

    2011-01-01

    Computational models of the neuromuscular system hold the potential to allow us to reach a deeper understanding of neuromuscular function and clinical rehabilitation by complementing experimentation. By serving as a means to distill and explore specific hypotheses, computational models emerge from prior experimental data and motivate future experimental work. Here we review computational tools used to understand neuromuscular function including musculoskeletal modeling, machine learning, control theory, and statistical model analysis. We conclude that these tools, when used in combination, have the potential to further our understanding of neuromuscular function by serving as a rigorous means to test scientific hypotheses in ways that complement and leverage experimental data. PMID:21687779

  15. Scoliosis in neuromuscular disorders.

    PubMed

    Pecak, F; Trontelj, J V; Dimitrijevic, M R

    1980-01-01

    Hundred-seventy patients with the 4 commonest degenerative neuro-muscular disorders (limbgirdle and Duchenne muscular dystrphy, Kugelberg-Welander's spinal muscular atrophy and peroneal muscular atrophy) were screened for scoliosis, which was found in 56%. Of 76 patients in the early stages of their respective disorders (stages 1-6 of Gardner-Medwin and Walton), scoliosis was found in 72%. The incidence of scoliosis was not related to the duration or degree of clinical weakness. Morphologically, scoliosis in these disorders was not found to differ from idiopathic scoliosis. Neither side nor site of the scoliosis were related to the distribution of muscle weakness as determined by manual testing.

  16. [Neuromuscular blockade monitoring. Part 1].

    PubMed

    Ariño-Irujo, J J; Calbet-Mañueco, A; De la Calle-Elguezabal, P A; Velasco-Barrio, J M; López-Timoneda, F; Ortiz-Gómez, J R; Fabregat-López, J; Palacio-Abizanda, F J; Fornet-Ruiz, I; Pérez-Cajaraville, J

    2010-03-01

    Many recent studies have underlined the importance of quantitative neuromuscular monitoring and the high incidence of residual block in clinical practice in spite of the use of nondepolarizing neuromuscular blockers of intermediate duration. Neuromuscular monitoring facilitates the tailoring of the muscular paralysis and appropriate patient recovery at the end of surgery. Monitoring also controls or prevents residual block and serves to guide the use of reversing agents. This review describes the physiology of neuromuscular junctions as well as the principles and patterns of nerve stimulation and clinical monitoring. In addition to drawing on their own experience, the authors have reviewed the literature available through evidence-based indexes and other databases up to December 2008. Most references found were case series and reviews. Quantitative monitoring is an evidence-based practice that should be applied in all situations in which a neuromuscular block is established.

  17. Update on neuromuscular pharmacology.

    PubMed

    Naguib, Mohamed; Brull, Sorin J

    2009-08-01

    We review the efficacy and safety of gantacurium and AV002, two novel, investigational fumarate-based nondepolarizing neuromuscular blockers, as well as sugammadex and cysteine, two novel reversal drugs that have no acetylcholinesterase inhibition properties. Gantacurium (with a pharmacodynamic profile similar to that of succinylcholine) and AV002 (with an intermediate duration of action) have shown efficacy in animals and, for gantacurium, in humans. Animal data have shown that exogenous administration of the amino acid cysteine accelerates the natural chemical degradation of both gantacurium and AV002 via the cysteine adduction pathway. Another reversal drug, sugammadex (a modified gamma-cyclodextrin and the first selective relaxant binding agent), forms very tight complexes in a 1: 1 ratio with steroidal neuromuscular blocking agents. In a multicenter phase-2 randomized controlled study in the European Union, the efficacy and safety of gantacurium were evaluated, but results have not yet been published. Sugammadex is currently available in the European Union, but the United States Food and Drug Administration has had concerns about its safety (hypersensitivity and allergic reactions) and has asked for additional safety data. It is hoped that the widespread use of sugammadex in the European Union will provide additional information.

  18. Neuromuscular toxicity of therapy.

    PubMed

    Mollman, J E

    1992-06-01

    The peripheral nervous system is frequently impaired in patients who have cancer. This impairment often results from toxicity of treatment but may also be due to direct invasion by tumor or may be part of a paraneoplastic syndrome. This review summarizes the recent literature regarding peripheral neuropathies and myopathies that are seen in patients with cancer. Highlights include the neuromuscular toxicity of some of the newer chemotherapeutic agents and immune mediators such as taxol and interleukin-2; a discussion of some of the agents being investigated for chemoprotection and rescue; an assessment of the evidence supporting the concept of motor neuron disease as a paraneoplastic disorder; and an interesting case report of megakaryoblastic leukemia invading peripheral nerves. Also summarized are some nice reviews and prospective studies of the toxicity of more conventional treatments.

  19. A surgeon's assessment of inadequate neuromuscular antagonism in a case of prolonged neuromuscular blockade.

    PubMed

    Lamberg, James J; Answine, Joseph F

    2013-04-01

    Evaluation of the degree of neuromuscular blockade by the surgeon using clinical criteria alone is unreliable. We report a case of prolonged neuromuscular blockade lasting 5.5 h, where an additional intra-operative dose of neuromuscular relaxant was given at the request of the surgical team. Possible causes of prolonged neuromuscular antagonism are discussed, as is the importance of neuromuscular assessment prior to the administration of additional neuromuscular blocking agents when receiving a surgeon request for additional neuromuscularblockade.

  20. Neuromuscular retraining for facial paralysis.

    PubMed

    Diels, H J; Combs, D

    1997-10-01

    Neuromuscular retraining is an effective method for rehabilitating facial musculature in patients with facial paralysis. This nonsurgical therapy has demonstrated improved functional outcomes and is an important adjunct to surgical treatment for restoring facial movement. Treatment begins with an intensive clinical evaluation and incorporates appropriate sensory feedback techniques into a patient-specific, comprehensive, home therapy program. This article discusses appropriate patients, timelines for referral, and basic treatment practices of facial neuromuscular retraining for restoring function and expression to the highest level possible.

  1. Neuromuscular disease and hypoventilation.

    PubMed

    Sivak, E D; Shefner, J M; Sexton, J

    1999-11-01

    Alveolar hypoventilation associated with neuromuscular disease can occur in acute and chronic forms. In the acute form, progressive weakness of respiratory muscles leads to rapid reduction in vital capacity followed by respiratory failure with hypoxemia and hypercarbia. Symptoms are those of acute respiratory failure, including dyspnea, tachypnea, and tachycardia. In the chronic form, impairment of the respiratory muscles affects mechanical properties of the lungs and chest wall, decreases the ability to clear secretions, and eventually may alter the function of the central respiratory centers. Symptoms include orthopnea, fatigue, disturbed sleep, and hypersomnolence. Treatment and outcome of the disease's chronic form are dependent on the underlying clinical cause of the alveolar hypoventilation. For chronic but stable diseases such as old polio, quadriplegia, or kyposcoliosis, mechanical support of minute ventilation can reverse symptoms. For chronic and progressive disease such as muscular dystrophy and amyotrophic lateral sclerosis, mechanical support of minute ventilation provides only symptomatic relief and is usually associated with deterioration to the point of complete ventilator dependency for survival. For the chronic progressive forms of alveolar hypoventilation, there is currently a need for quality randomized controlled clinical trials to define physiologic indicators and appropriate timing for mechanical support of minute ventilation.

  2. Neuromuscular disease classification system

    NASA Astrophysics Data System (ADS)

    Sáez, Aurora; Acha, Begoña; Montero-Sánchez, Adoración; Rivas, Eloy; Escudero, Luis M.; Serrano, Carmen

    2013-06-01

    Diagnosis of neuromuscular diseases is based on subjective visual assessment of biopsies from patients by the pathologist specialist. A system for objective analysis and classification of muscular dystrophies and neurogenic atrophies through muscle biopsy images of fluorescence microscopy is presented. The procedure starts with an accurate segmentation of the muscle fibers using mathematical morphology and a watershed transform. A feature extraction step is carried out in two parts: 24 features that pathologists take into account to diagnose the diseases and 58 structural features that the human eye cannot see, based on the assumption that the biopsy is considered as a graph, where the nodes are represented by each fiber, and two nodes are connected if two fibers are adjacent. A feature selection using sequential forward selection and sequential backward selection methods, a classification using a Fuzzy ARTMAP neural network, and a study of grading the severity are performed on these two sets of features. A database consisting of 91 images was used: 71 images for the training step and 20 as the test. A classification error of 0% was obtained. It is concluded that the addition of features undetectable by the human visual inspection improves the categorization of atrophic patterns.

  3. Telocytes in neuromuscular spindles

    PubMed Central

    Díaz-Flores, Lucio; Gutiérrez, Ricardo; Sáez, Francisco J; Díaz-Flores, Lucio; Madrid, Juan F

    2013-01-01

    A new cell type named telocyte (TC) has recently been identified in various stromal tissues, including skeletal muscle interstitium. The aim of this study was to investigate by means of light (conventional and immunohistochemical procedures) and electron microscopy the presence of TCs in adult human neuromuscular spindles (NMSs) and lay the foundations for future research on their behaviour during human foetal development and in skeletal muscle pathology. A large number of TCs were observed in NMSs and were characterized ultrastructurally by very long, initially thin, moniliform prolongations (telopodes – Tps), in which thin segments (podomeres) alternated with dilations (podoms). TCs formed the innermost and (partially) the outermost layers of the external NMS capsule and the entire NMS internal capsule. In the latter, the Tps were organized in a dense network, which surrounded intrafusal striated muscle cells, nerve fibres and vessels, suggesting a passive and active role in controlling NMS activity, including their participation in cell-to-cell signalling. Immunohistochemically, TCs expressed vimentin, CD34 and occasionally c-kit/CD117. In human foetus (22–23 weeks of gestational age), TCs and perineural cells formed a sheath, serving as an interconnection guide for the intrafusal structures. In pathological conditions, the number of CD34-positive TCs increased in residual NMSs between infiltrative musculoaponeurotic fibromatosis and varied in NMSs surrounded by lymphocytic infiltrate in inflammatory myopathy. We conclude that TCs are numerous in NMSs (where striated muscle cells, nerves and vessels converge), which provide an ideal microanatomic structure for TC study. PMID:23621814

  4. Analisis de Alteraciones EN la Imagen Debidas a Descolimacion de un Telescopio

    NASA Astrophysics Data System (ADS)

    Cobos, F. J.; Galan, M. J.

    1987-05-01

    Podemos considerar, en términos generales, que los espejos de un telescopio tienen una calidad óptica intrínseca, entendiendo por ésta la que se ha obtenido como resultado, fundamentalmente, de la destreza del personal del Taller Optico, que considerará terminadas las superficies ópticas cuando éstas satisfagan los requisitos de diseño y las pruebas de evaluación pertinentes. Debemos esperar que, una vez instalados los espejos en el telescopio, no se altere esta calidad de la óptica por un funcionamiento inadecuado de partes mecánicas del mismo. En los últimos años, en la medida que los problemas de infraestructuratura de nuestros Observatorios se han ido resolviendo, se ha hecho más patente la necesidad de llevar a la instrumentación existente al máximo de su potencial y parte esencial de ésta la conforman los mismos te lescopios. Mejorar la calidad óptica de las imágenes obtenidas con ellos ha hecho que sea prioritario el realizar una investigación más sistemática de sus características. Este trabajo ha tenido como objetivo primordial el usar un programa de diseño óptico, en el caso particular del telescopio UNAM212, con el fin de calcular y obtener gráficamente los diagramas de manchas de imagenes en foco y extrafocales, tanto con la óptica perfectamente alineada como descolimándola (mediante pequenos giros y descentramientos de los espejos). De esta manera, se hizo una evaluación de los efectos que estas alteraciones simuladas producirían en las imágenes focales y extra focales para así poder compararlas con las que realmente se han observado. Asimismo, se ha buscado información bibliográfica, en particular sobre los efectos de giros y descentramientos en las imágenes extrafocales, en lo que se ref iere a la falta de concentricidad de los círculos que forman la "dona" y a la distribución de intensidad luminosa en la misma. De ésta, l futuro un proceso que, haciendo uso de los detectores bidimensionales, nos permita Ilevar a

  5. Telocytes in neuromuscular spindles.

    PubMed

    Díaz-Flores, Lucio; Gutiérrez, Ricardo; Sáez, Francisco J; Díaz-Flores, Lucio; Madrid, Juan F

    2013-04-01

    A new cell type named telocyte (TC) has recently been identified in various stromal tissues, including skeletal muscle interstitium. The aim of this study was to investigate by means of light (conventional and immunohistochemical procedures) and electron microscopy the presence of TCs in adult human neuromuscular spindles (NMSs) and lay the foundations for future research on their behaviour during human foetal development and in skeletal muscle pathology. A large number of TCs were observed in NMSs and were characterized ultrastructurally by very long, initially thin, moniliform prolongations (telopodes - Tps), in which thin segments (podomeres) alternated with dilations (podoms). TCs formed the innermost and (partially) the outermost layers of the external NMS capsule and the entire NMS internal capsule. In the latter, the Tps were organized in a dense network, which surrounded intrafusal striated muscle cells, nerve fibres and vessels, suggesting a passive and active role in controlling NMS activity, including their participation in cell-to-cell signalling. Immunohistochemically, TCs expressed vimentin, CD34 and occasionally c-kit/CD117. In human foetus (22-23 weeks of gestational age), TCs and perineural cells formed a sheath, serving as an interconnection guide for the intrafusal structures. In pathological conditions, the number of CD34-positive TCs increased in residual NMSs between infiltrative musculoaponeurotic fibromatosis and varied in NMSs surrounded by lymphocytic infiltrate in inflammatory myopathy. We conclude that TCs are numerous in NMSs (where striated muscle cells, nerves and vessels converge), which provide an ideal microanatomic structure for TC study. © 2013 The Authors. Published by Foundation for Cellular and Molecular Medicine/Blackwell Publishing Ltd.

  6. American Association of Neuromuscular & Electrodiagnostic Medicine

    MedlinePlus

    Muscle & Nerve AANEM Foundation ABEM Login American Association of Neuromuscular & Electrodiagnostic Medicine Improving the Lives of Patients with Neuromuscular Diseases About Vision, Mission & Values Board & Committees Committee Index Board Nominations AANEM ...

  7. Neuromuscular control: introduction and overview.

    PubMed

    van Leeuwen, J L

    1999-05-29

    This paper introduces some basic concepts of the interdisciplinary field of neuromuscular control, without the intention to be complete. The complexity and multifaceted nature of neuromuscular control systems is briefly addressed. Principles of stability and planning of motion trajectories are discussed. Closed-loop and open-loop control are considered, together with the inherent stability properties of muscles and the geometrical design of animal bodies. Various modelling approaches, as used by several authors in the Philosophical Transactions of the Royal Society of London, Series B, May 1999 issue, such as inverse and forward dynamics are outlined. An introductory overview is presented of the other contributions in that issue.

  8. Neuromuscular control: introduction and overview.

    PubMed Central

    van Leeuwen, J L

    1999-01-01

    This paper introduces some basic concepts of the interdisciplinary field of neuromuscular control, without the intention to be complete. The complexity and multifaceted nature of neuromuscular control systems is briefly addressed. Principles of stability and planning of motion trajectories are discussed. Closed-loop and open-loop control are considered, together with the inherent stability properties of muscles and the geometrical design of animal bodies. Various modelling approaches, as used by several authors in the Philosophical Transactions of the Royal Society of London, Series B, May 1999 issue, such as inverse and forward dynamics are outlined. An introductory overview is presented of the other contributions in that issue. PMID:10382220

  9. Neuromuscular ultrasound of cranial nerves.

    PubMed

    Tawfik, Eman A; Walker, Francis O; Cartwright, Michael S

    2015-04-01

    Ultrasound of cranial nerves is a novel subdomain of neuromuscular ultrasound (NMUS) which may provide additional value in the assessment of cranial nerves in different neuromuscular disorders. Whilst NMUS of peripheral nerves has been studied, NMUS of cranial nerves is considered in its initial stage of research, thus, there is a need to summarize the research results achieved to date. Detailed scanning protocols, which assist in mastery of the techniques, are briefly mentioned in the few reference textbooks available in the field. This review article focuses on ultrasound scanning techniques of the 4 accessible cranial nerves: optic, facial, vagus and spinal accessory nerves. The relevant literatures and potential future applications are discussed.

  10. DYNAMIC NEUROMUSCULAR STABILIZATION & SPORTS REHABILITATION

    PubMed Central

    Kobesova, Alena; Kolar, Pavel

    2013-01-01

    Dynamic neuromuscular (core) stability is necessary for optimal athletic performance and is not achieved purely by adequate strength of abdominals, spinal extensors, gluteals or any other musculature; rather, core stabilization is accomplished through precise coordination of these muscles and intra‐abdominal pressure regulation by the central nervous system. Understanding developmental kinesiology provides a framework to appreciate the regional interdependence and the inter‐linking of the skeleton, joints, musculature during movement and the importance of training both the dynamic and stabilizing function of muscles in the kinetic chain. The Dynamic Neuromuscular Stabilization (DNS) approach provides functional tools to assess and activate the intrinsic spinal stabilizers in order to optimize the movement system for both pre‐habilitation and rehabilitation of athletic injuries and performance. Level of Evidence: 5 PMID:23439921

  11. Neuromuscular Ultrasound of Cranial Nerves

    PubMed Central

    Tawfik, Eman A.; Cartwright, Michael S.

    2015-01-01

    Ultrasound of cranial nerves is a novel subdomain of neuromuscular ultrasound (NMUS) which may provide additional value in the assessment of cranial nerves in different neuromuscular disorders. Whilst NMUS of peripheral nerves has been studied, NMUS of cranial nerves is considered in its initial stage of research, thus, there is a need to summarize the research results achieved to date. Detailed scanning protocols, which assist in mastery of the techniques, are briefly mentioned in the few reference textbooks available in the field. This review article focuses on ultrasound scanning techniques of the 4 accessible cranial nerves: optic, facial, vagus and spinal accessory nerves. The relevant literatures and potential future applications are discussed. PMID:25851889

  12. [Respiratory treatments in neuromuscular disease].

    PubMed

    Martínez Carrasco, C; Cols Roig, M; Salcedo Posadas, A; Sardon Prado, O; Asensio de la Cruz, O; Torrent Vernetta, A

    2014-10-01

    In a previous article, a review was presented of the respiratory pathophysiology of the patient with neuromuscular disease, as well as their clinical evaluation and the major complications causing pulmonary deterioration. This article presents the respiratory treatments required to preserve lung function in neuromuscular disease as long as possible, as well as in special situations (respiratory infections, spinal curvature surgery, etc.). Special emphasis is made on the use of non-invasive ventilation, which is changing the natural history of many of these diseases. The increase in survival and life expectancy of these children means that they can continue their clinical care in adult units. The transition from pediatric care must be an active, timely and progressive process. It may be slightly stressful for the patient before the adaptation to this new environment, with multidisciplinary care always being maintained. Copyright © 2013 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  13. Neuromuscular blockade in the elderly.

    PubMed

    Stankiewicz-Rudnicki, Michał

    2016-01-01

    The aim of the presented review is to highlight the clinical problem of postoperative residual curarization (PORC) following general anaesthesia in the elderly. Possible complications of PORC are described along with age-induced changes in pharmacokinetics of long and intermediate-acting neuromuscular blocking agents. This is intended to facilitate the selection and to promote appropriate intraoperative use of muscle relaxants in patients over the age of 65 years.

  14. Use of neuromuscular blockers in Brazil.

    PubMed

    Locks, Giovani de Figueiredo; Cavalcanti, Ismar Lima; Duarte, Nadia Maria Conceição; da Cunha, Rafael Martins; de Almeida, Maria Cristina Simões

    2015-01-01

    The objective of this study was to evaluate how Brazilian anesthesiologists are using neuromuscular blockers, focusing on how they establish the diagnosis of postoperative residual curarization and the incidence of complications associated with the use of neuromuscular blockers. A questionnaire was sent to anesthesiologists inviting them to participate in the study. The online data collection remained open from March 2012 to June 2013. During the study period, 1296 responses were collected. Rocuronium, atracurium, and cisatracurium were the main neuromuscular blockers used in cases of elective surgery. Succinylcholine and rocuronium were the main neuromuscular blockers used in cases of emergency surgery. Less than 15% of anesthesiologists reported the frequent use of neuromuscular function monitors. Only 18% of those involved in the study reported that all workplaces have such a monitor. Most respondents reported using only the clinical criteria to assess whether the patient is recovered from the muscle relaxant. Most respondents also reported always using some form of neuromuscular blockade reversal. The major complications attributed to neuromuscular blockers were residual curarization and prolonged blockade. Eighteen anesthesiologists reported death attributed to neuromuscular blockers. Residual or prolonged blockade is possibly recorded as a result of the high rate of using clinical criteria to diagnose whether the patient has recovered or not from motor block and, as a corollary, the poor use of neuromuscular transmission monitors in daily practice.

  15. Neuromuscular Ultrasound for Evaluation of the Diaphragm

    PubMed Central

    Sarwal, Aarti; Walker, Francis O.; Cartwright, Michael S.

    2012-01-01

    Neuromuscular clinicians are often asked to evaluate the diaphragm for diagnostic and prognostic purposes. Traditionally, this evaluation is accomplished through history, physical exam, fluoroscopic sniff test, nerve conduction studies, and electromyography (EMG). Nerve conduction studies and EMG in this setting are challenging, uncomfortable, and can cause serious complications such as pneumothorax. Neuromuscular ultrasound has emerged as a non-invasive technique that can be used in the structural and functional assessment of the diaphragm. This article reviews different techniques for assessing the diaphragm using neuromuscular ultrasound and the application of these techniques to enhance diagnosis and prognosis by neuromuscular clinicians. PMID:23382111

  16. Neuromuscular Control and Coordination during Cycling

    ERIC Educational Resources Information Center

    Li, Li

    2004-01-01

    The neuromuscular control aspect of cycling has been investigated through the effects of modifying posture and cadence. These studies show that changing posture has a more profound influence on neuromuscular coordination than does changing slope. Most of the changes with standing posture occur late in the downstroke: increased ankle and knee joint…

  17. Neuromuscular Control and Coordination during Cycling

    ERIC Educational Resources Information Center

    Li, Li

    2004-01-01

    The neuromuscular control aspect of cycling has been investigated through the effects of modifying posture and cadence. These studies show that changing posture has a more profound influence on neuromuscular coordination than does changing slope. Most of the changes with standing posture occur late in the downstroke: increased ankle and knee joint…

  18. Residual neuromuscular blockade in critical care.

    PubMed

    Wilson, Jason; Collins, Angela S; Rowan, Brea O

    2012-06-01

    Neuromuscular blockade is a pharmacological adjunct for anesthesia and for surgical interventions. Neuromuscular blockers can facilitate ease of instrumentation and reduce complications associated with intubation. An undesirable sequela of these agents is residual neuromuscular blockade. Residual neuromuscular blockade is linked to aspiration, diminished response to hypoxia, and obstruction of the upper airway that may occur soon after extubation. If an operation is particularly complex or requires a long anesthesia time, residual neuromuscular blockade can contribute to longer stays in the intensive care unit and more hours of mechanical ventilation. Given the risks of this medication class, it is essential to have an understanding of the mechanism of action of, assessment of, and factors affecting blockade and to be able to identify factors that affect pharmacokinetics.

  19. Mitochondrial dysfunction in neuromuscular disorders.

    PubMed

    Katsetos, Christos D; Koutzaki, Sirma; Melvin, Joseph J

    2013-09-01

    This review deciphers aspects of mitochondrial (mt) dysfunction among nosologically, pathologically, and genetically diverse diseases of the skeletal muscle, lower motor neuron, and peripheral nerve, which fall outside the traditional realm of mt cytopathies. Special emphasis is given to well-characterized mt abnormalities in collagen VI myopathies (Ullrich congenital muscular dystrophy and Bethlem myopathy), megaconial congenital muscular dystrophy, limb-girdle muscular dystrophy type 2 (calpainopathy), centronuclear myopathies, core myopathies, inflammatory myopathies, spinal muscular atrophy, Charcot-Marie-Tooth neuropathy type 2, and drug-induced peripheral neuropathies. Among inflammatory myopathies, mt abnormalities are more prominent in inclusion body myositis and a subset of polymyositis with mt pathology, both of which are refractory to corticosteroid treatment. Awareness is raised about instances of phenotypic mimicry between cases harboring primary mtDNA depletion, in the context of mtDNA depletion syndrome, and established neuromuscular disorders such as spinal muscular atrophy. A substantial body of experimental work, derived from animal models, attests to a major role of mitochondria (mt) in the early process of muscle degeneration. Common mechanisms of mt-related cell injury include dysregulation of the mt permeability transition pore opening and defective autophagy. The therapeutic use of mt permeability transition pore modifiers holds promise in various neuromuscular disorders, including muscular dystrophies.

  20. Neuromuscular Highlights-AAN 2005.

    PubMed

    Cheema, Zahid; Saperstein, David; Jackson, Carolyn; Newman, Daniel

    2006-06-01

    Summary of Neuromuscular Presentations at the 57 Annual AAN 2005 meeting in Miami Florida on topics of Facioscapulohumeral muscular dystrophy (FSHD), Duchenne muscular dystrophy (DMD), Diabetic Neuropathy, Charco Marie Tooth disease (CMT), Comparison of injected steroids versus Surgery for carpal tunnel syndrome, Rituximab in Anti-MAG associated polyneuropathy, Cannabis based medicine (CBM) in the treatment of neuropathic pain, utility of skin biopsy with intraepidermal nerve fiber density (IENFD) in sensory complaints, comparing sympathetic skin responses (SSRs) and skin biopsy in diagnosing small fiber sensory neuropathy, Chronic inflammatory demyelinating polyneuropathy (CIDP) clinical and electrophysiologic predictors, affect of limb warming in mild ulnar nerve conduction study (NCS) abnormalities, Tamoxifen affect in ALS, open label study of 3,4 DAP, Pyridostigmine and Ephedrine in fast channel syndrome, Mexilitine as an antimyotonia treatment in myotonic dystrophy (DM1), frontal lobe impairment evaluation in DM1 and DM2 patients and phenotype-genotype correlation in patients with dysferlinopathy.

  1. Novel drug development for neuromuscular blockade.

    PubMed

    Prabhakar, Amit; Kaye, Alan D; Wyche, Melville Q; Salinas, Orlando J; Mancuso, Kenneth; Urman, Richard D

    2016-01-01

    Pharmacological advances in anesthesia in recent decades have resulted in safer practice and better outcomes. These advances include improvement in anesthesia drugs with regard to efficacy and safety profiles. Although neuromuscular blockers were first introduced over a half century ago, few new neuromuscular blockers and reversal agents have come to market and even fewer have remained as common clinically employed medications. In recent years, newer agents have been studied and are presented in this review. With regard to nondepolarizer neuromuscular blocker agents, the enantiomers Gantacurium and CW002, which are olefinic isoquinolinium diester fumarates, have shown potential for clinical application. Advantages include ultra rapid reversal of neuromuscular blockade via cysteine adduction and minimal systemic hemodynamic effects with administration.

  2. School Intervention for the Neuromuscularly Handicapped Child.

    ERIC Educational Resources Information Center

    Hall, Colin D.; Porter, Patricia

    1983-01-01

    Difficulties encountered in school by 35 neuromuscularly handicapped children, (5 to 18 years old) were assessed, and methods of alleviating problems were developed. (SEW) Journal Availability: The C. V. Mosby Company, 11830 Westline Industrial Drive, St. Louis, MO 63141.

  3. Sugammadex: A revolutionary drug in neuromuscular pharmacology

    PubMed Central

    Nag, Kusha; Singh, Dewan Roshan; Shetti, Akshaya N.; Kumar, Hemanth; Sivashanmugam, T.; Parthasarathy, S.

    2013-01-01

    Sugammadex (ORG 25969) is a unique neuromuscular reversal drug; a novel cyclodextrin, the first in a new class of selective relaxant binding agents, which reverse neuromuscular blockade (NMB) with the aminosteroid non-depolarizing muscle relaxants rocuronium and vecuronium. Sugammadex can reverse moderate or deep NMB. The clinical use of sugammadex promises to eliminate many of the shortcomings in current anesthetic practice with regard to antagonism of rocuronium and other aminosteroid muscle relaxants. PMID:25885973

  4. [Translation and validation of the Individualised Neuromuscular Quality of Life scale for the Spanish population: quality of life assessment for persons with neuromuscular diseases].

    PubMed

    Fagoaga, J; Girabent-Farres, M; Bagur-Calafat, C

    2017-03-01

    Introduccion. La escala Individualized Neuromuscular Quality of Life (INQoL) es un cuestionario que valora la calidad de vida relacionada con la salud de personas adultas con enfermedades neuromusculares. Objetivo. Validar y analizar la fiabilidad de la version española de la INQoL, como instrumento de medicion de la calidad de vida relacionada con la salud en individuos con enfermedades neuromusculares. Pacientes y metodos. Se realiza una traduccion-retrotraduccion de la INQoL en la poblacion española y, posteriormente, para el analisis de fiabilidad se llevan a cabo dos mediciones, test-retest, a 50 pacientes de 19 a 67 años. De este modo se evalua la concordancia intraobservador y se evalua la consistencia interna de la escala. Resultados. El estudio de la fiabilidad del indice de concordancia intraobservador tiene un valor de excelente en siete de las diez subdimensiones y en la puntuacion total de la calidad de vida; de buena, en dos; y de moderada, en una. El analisis del alfa de Cronbach para las subdimensiones de la INQoL tiene un valor de excelente (> 0,818) en siete de ellas, asi como en la puntuacion total de la calidad de vida relacionada con la salud (0,928), un valor de buena consistencia interna en tres de las subdimensiones y de moderada en una. Conclusiones. La version española de la INQoL es un instrumento valido y fiable como herramienta de medicion de la calidad de vida en individuos adultos con enfermedades neuromusculares.

  5. Sugammadex: A Review of Neuromuscular Blockade Reversal.

    PubMed

    Keating, Gillian M

    2016-07-01

    Sugammadex (Bridion(®)) is a modified γ-cyclodextrin that reverses the effect of the steroidal nondepolarizing neuromuscular blocking agents rocuronium and vecuronium. Intravenous sugammadex resulted in rapid, predictable recovery from moderate and deep neuromuscular blockade in patients undergoing surgery who received rocuronium or vecuronium. Recovery from moderate neuromuscular blockade was significantly faster with sugammadex 2 mg/kg than with neostigmine, and recovery from deep neuromuscular blockade was significantly faster with sugammadex 4 mg/kg than with neostigmine or spontaneous recovery. In addition, recovery from neuromuscular blockade was significantly faster when sugammadex 16 mg/kg was administered 3 min after rocuronium than when patients spontaneously recovered from succinylcholine. Sugammadex also demonstrated efficacy in various special patient populations, including patients with pulmonary disease, cardiac disease, hepatic dysfunction or myasthenia gravis and morbidly obese patients. Intravenous sugammadex was generally well tolerated. In conclusion, sugammadex is an important option for the rapid reversal of rocuronium- or vecuronium-induced neuromuscular blockade.

  6. Neuromuscular blockade in the elderly patient.

    PubMed

    Lee, Luis A; Athanassoglou, Vassilis; Pandit, Jaideep J

    2016-01-01

    Neuromuscular blockade is a desirable or even essential component of general anesthesia for major surgical operations. As the population continues to age, and more operations are conducted in the elderly, due consideration must be given to neuromuscular blockade in these patients to avoid possible complications. This review considers the pharmacokinetics and pharmacodynamics of neuromuscular blockade that may be altered in the elderly. Compartment distribution, metabolism, and excretion of drugs may vary due to age-related changes in physiology, altering the duration of action with a need for reduced dosage (eg, aminosteroids). Other drugs (atracurium, cisatracurium) have more reliable duration of action and should perhaps be considered for use in the elderly. The range of interpatient variability that neuromuscular blocking drugs may exhibit is then considered and drugs with a narrower range, such as cisatracurium, may produce more predictable, and inherently safer, outcomes. Ultimately, appropriate neuromuscular monitoring should be used to guide the administration of muscle relaxants so that the risk of residual neuromuscular blockade postoperatively can be minimized. The reliability of various monitoring is considered. This paper concludes with a review of the various reversal agents, namely, anticholinesterase drugs and sugammadex, and the alterations in dosing of these that should be considered for the elderly patient.

  7. Clinical pharmacology of neuromuscular blocking agents.

    PubMed

    Fisher, D M

    1999-06-01

    The clinical pharmacology of neuromuscular blocking agents is described. During neuromuscular blockade, succinylcholine attaches to receptors in the motor end plate and depolarizes the neuromuscular junction, making the end plate refractory to acetylcholine. The nondepolarizing relaxants have a structure similar to that of succinylcholine and bind to the same receptors. Instead of depolarizing the junction, they block acetylcholine from binding to the receptor and cause channel blockade. As the concentration of nondepolarizing relaxant increases relative to acetylcholine, neuromuscular transmission is compromised. This relationship is used clinically to facilitate recovery from nondepolarizing agents. Succinylcholine is popular because its onset is faster than that of the nondepolarizing relaxants and metabolism by pseudocholinesterase clears it quickly. It is commonly given as an i.v. bolus to facilitate tracheal intubation. The onset of these agents varies widely and is dose dependent. Large doses are usually given to hasten the onset of paralysis; subsequent doses are adjusted according to response. The nondepolarizing agents interact with inhaled anesthetics, magnesium, and many antimicrobials. Drugs like neostigmine, edrophonium, and pyridostigmine antagonize neuromuscular blockade; an anticholinergic drug is typically administered to counteract the cardiovascular effects. The most serious adverse effects of succinylcholine are malignant hyperthermia syndrome, masseter muscle rigidity, and bradycardia. Some nondepolarizing relaxants (atracurium, mivacurium, and pancuronium) are associated with histamine release, occasionally causing serious hypotension and tachycardia. Neuromuscular blocking agents are essential to anesthesia. Older compounds produce greater toxicity than newer compounds, and several of these older compounds therefore are no longer in clinical use.

  8. Eccentric Exercise to Enhance Neuromuscular Control.

    PubMed

    Lepley, Lindsey K; Lepley, Adam S; Onate, James A; Grooms, Dustin R

    Neuromuscular alterations are a major causal factor of primary and secondary injuries. Though injury prevention programs have experienced some success, rates of injuries have not declined, and after injury, individuals often return to activity with functionality below clinical recommendations. Considering alternative therapies to the conventional concentric exercise approach, such as one that can target neuromuscular injury risk and postinjury alterations, may provide for more effective injury prevention and rehabilitation protocols. Peer-reviewed sources available on the Web of Science and MEDLINE databases from 2000 through 2016 were gathered using searches associated with the keywords eccentric exercise, injury prevention, and neuromuscular control. Eccentric exercise will reduce injury risk by targeting specific neural and morphologic alterations that precipitate neuromuscular dysfunction. Clinical review. Level 4. Neuromuscular control is influenced by alterations in muscle morphology and neural activity. Eccentric exercise beneficially modifies several underlying factors of muscle morphology (fiber typing, cross-sectional area, working range, and pennation angle), and emerging evidence indicates that eccentric exercise is also beneficial to peripheral and central neural activity (alpha motorneuron recruitment/firing, sarcolemma activity, corticospinal excitability, and brain activation). There is mounting evidence that eccentric exercise is not only a therapeutic intervention influencing muscle morphology but also targets unique alterations in neuromuscular control, influencing injury risk.

  9. Neuromuscular Adaptations to Reduced Use

    NASA Technical Reports Server (NTRS)

    Ploutz-Snyder, Lori

    2009-01-01

    This viewgraph presentation reviews the studies done to reduce neuromuscular strength loss during unilateral lower limb suspension (ULLS). Since there are animals that undergo fairly long periods of muscular disuse without any or minimal muscular atrophy, there is an answer to that might be applicable to human in situations that require no muscular use to diminish the effects of muscular atrophy. Three sets of ULLS studies were reviewed indicated that muscle strength decreased more than the muscle mass. The study reviewed exercise countermeasures to combat the atrophy, including: ischemia maintained during Compound muscle action potential (CMAP), ischemia and low load exercise, Japanese kaatsu, and the potential for rehabilitation or situations where heavy loading is undesirable. Two forms of countermeasures to unloading have been successful, (1) high-load resistance training has maintained muscle mass and strength, and low load resistance training with blood flow restriction (LL(sub BFR)). The LL(sub BFR) has been shown to increase muscle mass and strength. There has been significant interest in Tourniquet training. An increase in Growth Hormone(GH) has been noted for LL(sub BFR) exercise. An experimental study with 16 subjects 8 of whom performed ULLS, and 8 of whom performed ULLS and LL(sub BFR) exercise three times per week during the ULLS. Charts show the results of the two groups, showing that performing LL(sub BFR) exercise during 30 days of ULLS can maintain muscle size and strength and even improve muscular endurance.

  10. [Use of neuromuscular blockers in Brazil].

    PubMed

    Locks, Giovani de Figueiredo; Cavalcanti, Ismar Lima; Duarte, Nadia Maria Conceição; Cunha, Rafael Martins da; Almeida, Maria Cristina Simões de

    2015-01-01

    The objective of this study was to evaluate how Brazilian anesthesiologists are using neuromuscular blockers (NMB), focusing on how they establish the diagnosis of postoperative residual curarization and the incidence of complications associated with the use of NMB. A questionnaire was sent to anesthesiologists inviting them to participate in the study. The online data collection remained open from March 2012 to June 2013. During the study period, 1296 responses were collected. Rocuronium, atracurium, and cisatracurium were the main neuromuscular blockers used in cases of elective surgery. Succinylcholine and rocuronium were the main NMB used in cases of emergency surgery. Less than 15% of anesthesiologists reported the frequent use of neuromuscular function monitors. Only 18% of those involved in the study reported that all workplaces have such a monitor. Most respondents reported using only the clinical criteria to assess whether the patient is recovered from the muscle relaxant. Most respondents also reported always using some form of neuromuscular blockade reversal. The major complications attributed to NMB were residual curarization and prolonged blockade. Eighteen anesthesiologists reported death attributed to NMB. Residual or prolonged blockade is possibly recorded as a result of the high rate of using clinical criteria to diagnose whether the patient has recovered or not from motor block and, as a corollary, the poor use of neuromuscular transmission monitors in daily practice.

  11. Flightless Flies: Drosophila models of neuromuscular disease

    PubMed Central

    Lloyd, Thomas E.; Taylor, J. Paul

    2010-01-01

    The fruit fly, Drosophila melanogaster, has a long and rich history as an important model organism for biologists. In particular, study of the fruit fly has been essential to much of our fundamental understanding of the development and function of the nervous system. In recent years, studies using fruit flies have provided important insights into the pathogenesis of neurodegenerative and neuromuscular diseases. Fly models of spinal muscular atrophy, spinobulbar muscular atrophy, myotonic dystrophy, dystrophinopathies and other inherited neuromuscular diseases recapitulate many of the key pathologic features of the human disease. The ability to perform genetic screens holds promise for uncovering the molecular mechanisms of disease, and indeed, for identifying novel therapeutic targets. This review will summarize recent progress in developing fly models of neuromuscular diseases and will emphasize the contribution that Drosophila has made to our understanding of these diseases. PMID:20329357

  12. [Prolonged neuromuscular block after mivacurium injection].

    PubMed

    Viggiano, M; Soler, C; Dumont, J C; Pellissier, D; François, G

    1995-01-01

    Mivacurium, a new short acting non depolarizing neuromuscular blocker, is metabolized, as suxamethonium, by plasma cholinesterase. Therefore its duration of action is increased in patients with reduced plasma cholinesterase activity. We report a case of prolonged neuromuscular block after an i.v. bolus of mivacurium (0.20 mg.kg-1) in a 69 year-old ASA II woman with an unrecognized cholinesterase deficiency undergoing a lumbar sympathectomy for arteriopathy of the lower limbs. The duration of the block was 6 h and plasma cholinesterase concentrations were very low (540 and 610 UI.L-1), as well as the dibucaine number (16%), which suggests an homozygous enzymatic deficiency. Mechanical ventilation and sedation were continued until spontaneous return of full neuromuscular function.

  13. Aprotinin in pediatric neuromuscular scoliosis surgery.

    PubMed

    Kasimian, Stepan; Skaggs, David L; Sankar, Wudbhav N; Farlo, Joseph; Goodarzi, Mashallah; Tolo, Vernon T

    2008-12-01

    Reduction of blood transfusions in patients with neuromuscular scoliosis can decrease potential complications such as immune suppression, infection, hemolytic reaction and viral transmission. Aprotinin (Trasylol), Bayer), an antifibrinolytic, has proven to be effective in reducing blood loss in cardiac and liver surgery, but little data exists in patients undergoing spinal fusion for neuromuscular scoliosis. The purpose of this study was to evaluate the safety and efficacy of aprotinin in pediatric neuromuscular scoliosis patients undergoing spinal fusion. The medical records of all patients undergoing initial spinal fusions for neuromuscular scoliosis between January 1999 and March 2003 were reviewed to determine demographic data, perioperative data, wound drainage and number of transfusion required. Cases were compared to a matched group of historical controls. We had 14 patients in the aprotinin group and 17 in the control group. Total blood loss in the aprotinin group was significantly lower compared to the control group (715 vs. 2,110 ml; P = 0.007). Significantly less blood loss occurred in the aprotinin group when blood loss per kilogram was evaluated as well (23 vs. 60 ml/kg, respectively; P = 0.002). Intra-operative packed red blood cell (PRBC) transfusions were also significantly lower in the aprotinin group (1.25 vs. 3.16 units; P = 0.001). No clinical evidence of anaphylaxis, deep vein thrombosis (DVT) or renal failure was observed in the aprotinin group. After considering the price of drug therapy, operating room time, and the cost of blood products, the use of aprotinin saved an average of $8,577 per patient. In our series, the use of aprotinin resulted in decreased blood loss and a decreased rate of transfusions in children with neuromuscular scoliosis undergoing extensive spinal fusion. At out institution, the use of aprotinin is safe and cost effective for patients with neuromuscular scoliosis.

  14. Neuromuscular disorders in the intensive care unit.

    PubMed

    Marinelli, William A; Leatherman, James W

    2002-10-01

    Neuromuscular disorders encountered in the ICU can be categorized as muscular diseases that lead to ICU admission and those that are acquired in the ICU. This article discusses three neuromuscular disorders can lead to ICU admission and have a putative immune-mediated pathogenesis: the Guillian-Barré syndrome, myasthenia gravis, and dermatomyositis/polymyositis. It also reviews critical care polyneuropathy and ICU acquired myopathy, two disorders that, alone or in combination, are responsible for nearly all cases of severe ICU acquired muscle weakness.

  15. 21 CFR 882.5860 - Implanted neuromuscular stimulator.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... neuromuscular stimulator. (a) Identification. An implanted neuromuscular stimulator is a device that provides electrical stimulation to a patient's peroneal or femoral nerve to cause muscles in the leg to contract, thus... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Implanted neuromuscular stimulator....

  16. 21 CFR 882.5860 - Implanted neuromuscular stimulator.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 21 Food and Drugs 8 2014-04-01 2014-04-01 false Implanted neuromuscular stimulator. 882.5860... (CONTINUED) MEDICAL DEVICES NEUROLOGICAL DEVICES Neurological Therapeutic Devices § 882.5860 Implanted neuromuscular stimulator. (a) Identification. An implanted neuromuscular stimulator is a device that provides...

  17. 21 CFR 882.5860 - Implanted neuromuscular stimulator.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 21 Food and Drugs 8 2013-04-01 2013-04-01 false Implanted neuromuscular stimulator. 882.5860... (CONTINUED) MEDICAL DEVICES NEUROLOGICAL DEVICES Neurological Therapeutic Devices § 882.5860 Implanted neuromuscular stimulator. (a) Identification. An implanted neuromuscular stimulator is a device that provides...

  18. 21 CFR 882.5860 - Implanted neuromuscular stimulator.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Implanted neuromuscular stimulator. 882.5860... (CONTINUED) MEDICAL DEVICES NEUROLOGICAL DEVICES Neurological Therapeutic Devices § 882.5860 Implanted neuromuscular stimulator. (a) Identification. An implanted neuromuscular stimulator is a device that provides...

  19. Neuromuscular Disorders and Sleep in Critically Ill Patients

    PubMed Central

    Irfan, Muna; Selim, Bernardo; Rabinstein, Alejandro A.

    2016-01-01

    Synopsis Sleep-disordered breathing (SDB) is a frequent presenting manifestation of neuromuscular disorders and can lead to significant morbidity and mortality. If not promptly recognized and addressed early in the clinical course, SDB can lead to clinical deterioration with respiratory failure. In this article, we review the pathophysiologic basis of SDB in neuromuscular disorders, clinical features encountered in specific neuromuscular diseases, and diagnostic and management strategies for SDB in neuromuscular patients in the critical care setting. Non-invasive positive pressure ventilation (NIPV) has been a crucial advance in critical care management, improving sleep quality and often preventing or delaying mechanical ventilation and improving survival in neuromuscular patients. PMID:26118919

  20. Neuromuscular disorders and sleep in critically ill patients.

    PubMed

    Irfan, Muna; Selim, Bernardo; Rabinstein, Alejandro A; St Louis, Erik K

    2015-07-01

    Sleep-disordered breathing (SDB) is a frequent presenting manifestation of neuromuscular disorders and can lead to significant morbidity and mortality. If not recognized and addressed early in the clinical course, SDB can lead to clinical deterioration with respiratory failure. The pathophysiologic basis of SDB in neuromuscular disorders, clinical features encountered in specific neuromuscular diseases, and diagnostic and management strategies for SDB in neuromuscular patients in the critical care setting are reviewed. Noninvasive positive pressure ventilation has been a crucial advance in critical care management, improving sleep quality and often preventing or delaying mechanical ventilation and improving survival in neuromuscular patients.

  1. [The use of neuromuscular monitoring in Germany].

    PubMed

    Fuchs-Buder, T; Hofmockel, R; Geldner, G; Diefenbach, C; Ulm, K; Blobner, M

    2003-06-01

    As there are no reliable epidemiological data for the use of muscle relaxants in Germany,we conducted a mailing study. The aim of the study was to compare the use of muscle relaxants between German anaesthesia departments. In the present part of the presentation we focused on neuromuscular monitoring (NMM) and management of residual paralysis. A total number of 2,996 questionnaires were sent to all registered anaesthesiological facilities in Germany and the return was 68.6% (2054 questionnaires). In 574 of the returned questionnaires (28%) the regular use of NMM was confirmed. Intraoperative monitoring of neuromuscular block and assessment of neuromuscular recovery were the most frequent applications of NMM, i.e. 25% and 18% of returned questionnaires, respectively. Clinical signs, however, are still the most popular way to estimate the degree of neuromuscular blockade. Moreover, routine reversal at the end of surgery with a neostigmin/atropine mixture was not practiced in 75% of the anaesthesia departments. This survey revealed that NMM is still very rarely used in daily clinical practice. Especially the seldom use of NMM to assess residual paralysis has to be improved.

  2. Dynamic Flexibility and Proprioceptive Neuromuscular Facilitation.

    ERIC Educational Resources Information Center

    Hardy, Lew; Jones, David

    1986-01-01

    Two experiments are described which investigated whether results obtained in studies of static flexibility tranfer to dynamic flexibility. In both experiments, subjects were assigned to a group receiving proprioceptive neuromuscular facilitation training, ballistic stretching technique training or a control group. Results are presented and…

  3. Hypoventilation: neuromuscular and chest wall disorders.

    PubMed

    Goldstein, R S

    1992-09-01

    Patients with neuromuscular and chest wall disorders are vulnerable at night when alterations in ventilatory mechanics and control associated with their disease are imposed on the changes in mechanics and control associated with sleep. The physiologic and clinical consequences of these events may be reversed by nocturnal mechanical ventilatory support.

  4. Dynamic Flexibility and Proprioceptive Neuromuscular Facilitation.

    ERIC Educational Resources Information Center

    Hardy, Lew; Jones, David

    1986-01-01

    Two experiments are described which investigated whether results obtained in studies of static flexibility tranfer to dynamic flexibility. In both experiments, subjects were assigned to a group receiving proprioceptive neuromuscular facilitation training, ballistic stretching technique training or a control group. Results are presented and…

  5. Subcutaneous immunoglobulin in treating inflammatory neuromuscular disorders

    PubMed Central

    Yoon, Min-Suk; Gold, Ralf

    2015-01-01

    Objective: Intravenous immunoglobulin administration has long been used in the treatment of autoimmune neuromuscular disorders. Immunoglobulins may be administered by intramuscular, intravenous or subcutaneous routes. Methods: This is a report on the long-term clinical follow up of six patients with inflammatory neuromuscular disorders, that is, three chronic inflammatory demyelinating polyneuropathy (CIDP), one multifocal motor neuropathy (MMN), one inclusion body myositis (IBM) and one myasthenia gravis (MG), treated with subcutaneous immunoglobulins for a mean of 3.25 years. Results: One MMN and two CIDP patients received a weekly dose of subcutaneous immunoglobulins equivalent to intravenous immunoglobulin. One CIDP patient received a 50% dose reduction, the IBM patient received a 30% reduction and the MG patient a 20% reduction. The lower dose chosen in the majority of patients was based not only on clinical effects, but also on studies of primary immunodeficiency syndromes. One patient with CIDP showed clinical fluctuation, which was successfully treated with an adaptation of the dose of subcutaneous immunoglobulins, while the remaining patients with neuromuscular disorders had a stable clinical course for 2 years. No serious side effects were observed. Conclusions: Our results suggest that subcutaneous immunoglobulins can be an attractive alternative therapy in autoimmune neuromuscular disorders. PMID:26136842

  6. Selective relaxant binding agents for reversal of neuromuscular blockade.

    PubMed

    Bom, Anton; Epemolu, Ola; Hope, Frank; Rutherford, Samantha; Thomson, Karen

    2007-06-01

    Traditionally, reversal of neuromuscular blockade during anaesthesia was achieved by increasing the acetylcholine concentration in the neuromuscular junction using acetylcholinesterase inhibitors. However, this is ineffective against profound blockade. Furthermore, the increase in acetylcholine level is not limited to the neuromuscular junction, resulting in unwanted side effects requiring co-treatment with muscarinic antagonists. Selective relaxant binding agents offer a new approach for the reversal of neuromuscular blockade: encapsulation of the neuromuscular blocking agent, resulting in inactivation. As part of this new approach, cyclodextrin molecules have been designed that selectively encapsulate steroidal neuromuscular blocking agents. Both animal and human experiments have demonstrated that fast, effective and complete recovery from both normal and profound neuromuscular blockade is now possible. Furthermore, these cyclodextrin derivatives do not have the unwanted side effects of acetylcholinesterase inhibitors.

  7. Exploring the effect of laryngeal neuromuscular electrical stimulation on voice.

    PubMed

    Gorham-Rowan, M; Morris, R

    2016-11-01

    This study was conducted to explore the potential use of neuromuscular electrical stimulation as an adjunctive treatment for muscle tension dysphonia. Voice data and ratings of fatigue and soreness were obtained for two experiments. Experiment one examined the vocal effects of neuromuscular electrical stimulation applied to the neck for 15 minutes. Experiment two examined the recovery effect of laryngeal neuromuscular electrical stimulation following a vocal loading task among normophonic women. No significant differences in vocal function following 15 minutes of laryngeal neuromuscular electrical stimulation were found. Six of 11 participants receiving laryngeal neuromuscular electrical stimulation exhibited improved recovery following the vocal loading task. A short session of laryngeal neuromuscular electrical stimulation may be beneficial in reducing muscle fatigue for some individuals. Further investigation is warranted to determine the applicability of laryngeal neuromuscular electrical stimulation in voice therapy.

  8. Neuromuscular blockers--a means of palliation?

    PubMed

    Hawryluck, L

    2002-06-01

    As we die, our respiratory pattern is altered and we seem to gasp and struggle for each breath. Such gasping is commonly seen as a clear sign of dyspnoea and suffering by families and loved ones, however, it is unclear whether it is perceived at all by the dying person. Narcotics and sedatives do not seem to affect these gasping respirations. In this issue of the Journal of Medical Ethics, we are asked to consider whether the last gasp of a dying patient could be or, perhaps, even should be avoided by administering neuromuscular blockers to palliate dying patients. For many reasons, such as our current failure to alleviate pain and distress, stories of inadequate analgesia and sedation in critically ill paralysed patients and the inability to know the intent-whether to palliate or to euthanise-it would seem that administering neuromuscular blockers should not be ethically permissible.

  9. Gastrointestinal neuromuscular pathology in chronic constipation

    PubMed Central

    Knowles, Charles H.; Farrugia, Gianrico

    2014-01-01

    Some patients with chronic constipation may undergo colectomy yielding tissue appropriate to diagnosis of underlying neuromuscular pathology. The analysis of such tissue has, over the past 40 years, fuelled research that has explored the presence of neuropathy, myopathy and more recently changes in interstitial cells of Cajal (ICC). In this chapter, the data from these studies have been critically reviewed in the context of the significant methodological and interpretative issues that beset the field of gastrointestinal neuromuscular pathology. On this basis, reductions in ICC appear to a consistent finding but one whose role as a primary cause of slow transit constipation requires further evaluation. Findings indicative of significant neuropathy or myopathy are variable and in many studies subject to considerable methodological bias. Methods with practical diagnostic utility in the individual patient have rarely been employed and require further validation in respect of normative data. PMID:21382578

  10. Neuromuscular electrical stimulation for skeletal muscle function.

    PubMed

    Doucet, Barbara M; Lam, Amy; Griffin, Lisa

    2012-06-01

    Lack of neural innervation due to neurological damage renders muscle unable to produce force. Use of electrical stimulation is a medium in which investigators have tried to find a way to restore movement and the ability to perform activities of daily living. Different methods of applying electrical current to modify neuromuscular activity are electrical stimulation (ES), neuromuscular electrical stimulation (NMES), transcutaneous electrical nerve stimulation (TENS), and functional electrical stimulation (FES). This review covers the aspects of electrical stimulation used for rehabilitation and functional purposes. Discussed are the various parameters of electrical stimulation, including frequency, pulse width/duration, duty cycle, intensity/amplitude, ramp time, pulse pattern, program duration, program frequency, and muscle group activated, and how they affect fatigue in the stimulated muscle.

  11. Neuromuscular Electrical Stimulation for Skeletal Muscle Function

    PubMed Central

    Doucet, Barbara M.; Lam, Amy; Griffin, Lisa

    2012-01-01

    Lack of neural innervation due to neurological damage renders muscle unable to produce force. Use of electrical stimulation is a medium in which investigators have tried to find a way to restore movement and the ability to perform activities of daily living. Different methods of applying electrical current to modify neuromuscular activity are electrical stimulation (ES), neuromuscular electrical stimulation (NMES), transcutaneous electrical nerve stimulation (TENS), and functional electrical stimulation (FES). This review covers the aspects of electrical stimulation used for rehabilitation and functional purposes. Discussed are the various parameters of electrical stimulation, including frequency, pulse width/duration, duty cycle, intensity/amplitude, ramp time, pulse pattern, program duration, program frequency, and muscle group activated, and how they affect fatigue in the stimulated muscle. PMID:22737049

  12. Diurnal hypercapnia in patients with neuromuscular disease.

    PubMed

    Panitch, Howard B

    2010-03-01

    Subjects with progressive neuromuscular diseases undergo a typical sequence of respiratory compromise, leading from normal unassisted gas exchange to nocturnal hypoventilation with normal daytime gas exchange, and eventually to respiratory failure requiring continuous ventilatory support. Several different abnormalities in respiratory pump function have been described to explain the development of respiratory failure in subjects with neuromuscular weakness. Early in the progression of respiratory failure, the use of nocturnal assisted ventilation can reverse both night- and day-time hypercapnia. Eventually, however, diurnal hypercapnia will persist despite correction of nocturnal hypoventilation. The likely beneficial effects of mechanical ventilatory support include resting fatigue-prone respiratory muscles and resetting of the central chemoreceptors to PaCO(2). Recent experience shows that select patients who require daytime ventilation can be supported with non-invasive ventilation continuously to correct gas exchange abnormalities while avoiding detrimental aspects of tracheostomy placement. Copyright 2009 Elsevier Ltd. All rights reserved.

  13. Neuromuscular control of prey capture in frogs.

    PubMed Central

    Nishikawa, K C

    1999-01-01

    While retaining a feeding apparatus that is surprisingly conservative morphologically, frogs as a group exhibit great variability in the biomechanics of tongue protraction during prey capture, which in turn is related to differences in neuromuscular control. In this paper, I address the following three questions. (1) How do frog tongues differ biomechanically? (2) What anatomical and physiological differences are responsible? (3) How is biomechanics related to mechanisms of neuromuscular control? Frog species use three non-exclusive mechanisms to protract their tongues during feeding: (i) mechanical pulling, in which the tongue shortens as its muscles contract during protraction; (ii) inertial elongation, in which the tongue lengthens under inertial and muscular loading; and (iii) hydrostatic elongation, in which the tongue lengthens under constraints imposed by the constant volume of a muscular hydrostat. Major differences among these functional types include (i) the amount and orientation of collagen fibres associated with the tongue muscles and the mechanical properties that this connective tissue confers to the tongue as a whole; and (ii) the transfer of intertia from the opening jaws to the tongue, which probably involves a catch mechanism that increases the acceleration achieved during mouth opening. The mechanisms of tongue protraction differ in the types of neural mechanisms that are used to control tongue movements, particularly in the relative importance of feed-forward versus feedback control, in requirements for precise interjoint coordination, in the size and number of motor units, and in the afferent pathways that are involved in coordinating tongue and jaw movements. Evolution of biomechanics and neuromuscular control of frog tongues provides an example in which neuromuscular control is finely tuned to the biomechanical constraints and opportunities provided by differences in morphological design among species. PMID:10382226

  14. Neuromuscular Disorders - Multiple Languages: MedlinePlus

    MedlinePlus

    ... List of All Topics All Neuromuscular Disorders - Multiple Languages To use the sharing features on this page, please enable JavaScript. French (français) Japanese (日本語) Korean (한국어) Russian (Русский) Somali (af Soomaali) Spanish (español) Ukrainian (Українська) ...

  15. Neuro-muscular junction block stimulator simulator.

    PubMed

    Sprick, Cyle

    2006-03-01

    Improved technology and higher fidelity are making medical simulations increasingly popular. A simulated peripheral nerve stimulator and thumb actuator has been developed for use with the SimMan Universal Patient Simulator. This device incorporates a handheld control box, a McKibben pneumatic muscle and articulated thumb, and a remote software interface for the simulation facilitator. The system simulates the action of a peripheral nerve stimulator on the ulnar nerve, and the effects of neuromuscular junction blocking agents on the thumb motion.

  16. Neuromuscular Diseases Associated with HIV-1 Infection

    PubMed Central

    Robinson-Papp, Jessica; Simpson, David M.

    2010-01-01

    Neuromuscular disorders are common in HIV, occurring at all stages of disease and affecting all parts of the peripheral nervous system. These disorders have diverse etiologies including HIV itself, immune suppression and dysregulation, co-morbid illnesses and infections, and side effects of medications. In this article, we review the following HIV-associated conditions: distal symmetric polyneuropathy, inflammatory demyelinating polyneuropathy, mononeuropathy, mononeuropathy multiplex, autonomic neuropathy, progressive polyradiculopathy due to cytomegalovirus, herpes zoster, myopathy and other rarer disorders. PMID:19771594

  17. Scoliosis and the impact in neuromuscular disease.

    PubMed

    Mayer, Oscar Henry

    2015-01-01

    Scoliosis can alter respiratory mechanics by changing the orientation of the muscles and joints of the respiratory system and in severe forms can put a patient at risk of severe respiratory morbidity or respiratory failure. However, perhaps the most important factor in determining the pulmonary morbidity in scoliosis is the balance between the "load" or altered respiratory mechanics and the "pump" or the respiratory muscle strength. Therefore, scoliosis in patients with neuromuscular disease will both lead to increased "load" and a weakened "pump", an exceptionally unfortunate combination. While progressive neuromuscular disease by its nature does not respond favorably to attempts to improve respiratory muscle strength, the natural approach of early proactive management of the "load" and in the case of scoliosis a variety of different strategies have been tried with variable short term and long term results. Figuring this out requires both an understanding of the underlying pathophysiology of a particular neuromuscular condition and the available options for and timing of surgical intervention. Copyright © 2014 Elsevier Ltd. All rights reserved.

  18. Neuromuscular adaptation to actual and simulated weightlessness

    NASA Technical Reports Server (NTRS)

    Edgerton, V. R.; Roy, R. R.

    1994-01-01

    The chronic "unloading" of the neuromuscular system during spaceflight has detrimental functional and morphological effects. Changes in the metabolic and mechanical properties of the musculature can be attributed largely to the loss of muscle protein and the alteration in the relative proportion of the proteins in skeletal muscle, particularly in the muscles that have an antigravity function under normal loading conditions. These adaptations could result in decrements in the performance of routine or specialized motor tasks, both of which may be critical for survival in an altered gravitational field, i.e., during spaceflight and during return to 1 G. For example, the loss in extensor muscle mass requires a higher percentage of recruitment of the motor pools for any specific motor task. Thus, a faster rate of fatigue will occur in the activated muscles. These consequences emphasize the importance of developing techniques for minimizing muscle loss during spaceflight, at least in preparation for the return to 1 G after spaceflight. New insights into the complexity and the interactive elements that contribute to the neuromuscular adaptations to space have been gained from studies of the role of exercise and/or growth factors as countermeasures of atrophy. The present chapter illustrates the inevitable interactive effects of neural and muscular systems in adapting to space. It also describes the considerable progress that has been made toward the goal of minimizing the functional impact of the stimuli that induce the neuromuscular adaptations to space.

  19. THE ANALYSIS OF NEUROMUSCULAR MECHANISMS IN CHITON.

    PubMed

    Crozier, W J

    1920-07-20

    1. The degree of curvature of the body and of the girdle of a Chiton is determined by the activity of antagonistic muscle groups. At a certain, early stage in the strychninization of a Chiton the reciprocal inhibition involved in the natural use of these muscle groups is reversed, such that extensor muscles, rather than, as normally, flexor muscles, contract as the result of stimulation. This condition involves a reversal, under strychnine, of the normally positive stereotropism of the foot, and of the usual response of the mollusk to an increased illumination of its ventral surface. Strychnine reversal of this character is not a matter of the relative strength of the opposed muscle groups, for the flexor muscles are the more powerful and are the ones always shortened in tetanic contraction. 2. Nicotine, in contrast to strychnine, primarily induces contraction of flexor muscles. Its effects, moreover, are in a degree selective, being notably exerted on "cerebral" nervous structures. Curare is devoid of characteristic action on the neuromuscular responses of Chiton. 3. The chemical organization of the neuromuscular organs of Chiton, as far as revealed by these tests, corresponds to a more simple condition than is inferred for gastropods. In particular, the behavior with respect to curare resembles more that of the neuromuscular apparatus of flatworms.

  20. Neuromuscular adaptation to actual and simulated weightlessness

    NASA Technical Reports Server (NTRS)

    Edgerton, V. R.; Roy, R. R.

    1994-01-01

    The chronic "unloading" of the neuromuscular system during spaceflight has detrimental functional and morphological effects. Changes in the metabolic and mechanical properties of the musculature can be attributed largely to the loss of muscle protein and the alteration in the relative proportion of the proteins in skeletal muscle, particularly in the muscles that have an antigravity function under normal loading conditions. These adaptations could result in decrements in the performance of routine or specialized motor tasks, both of which may be critical for survival in an altered gravitational field, i.e., during spaceflight and during return to 1 G. For example, the loss in extensor muscle mass requires a higher percentage of recruitment of the motor pools for any specific motor task. Thus, a faster rate of fatigue will occur in the activated muscles. These consequences emphasize the importance of developing techniques for minimizing muscle loss during spaceflight, at least in preparation for the return to 1 G after spaceflight. New insights into the complexity and the interactive elements that contribute to the neuromuscular adaptations to space have been gained from studies of the role of exercise and/or growth factors as countermeasures of atrophy. The present chapter illustrates the inevitable interactive effects of neural and muscular systems in adapting to space. It also describes the considerable progress that has been made toward the goal of minimizing the functional impact of the stimuli that induce the neuromuscular adaptations to space.

  1. Residual neuromuscular blockade and postoperative critical respiratory events: literature review.

    PubMed

    Kiekkas, Panagiotis; Bakalis, Nick; Stefanopoulos, Nikolaos; Konstantinou, Evangelos; Aretha, Diamanto

    2014-11-01

    To investigate and synthesise published literature on the associations between residual neuromuscular blockade and critical respiratory events of postoperative adult patients in the postanaesthesia care unit. Residual neuromuscular blockade continues to be common among patients transferred to the postanaesthesia care unit after general anaesthesia, while negative effects of residual neuromuscular blockade on respiratory function have been demonstrated in laboratory volunteers. Literature review. Using key terms, a search was conducted in Cumulative Index for Nursing and Allied Health Literature, PubMed, Web of Science, Cochrane Database and EMBASE (January 1990-May 2013) for clinical trials or observational studies on the associations between residual neuromuscular blockade and critical respiratory events, published in English-language journals. Nine articles met the inclusion criteria. Residual neuromuscular blockade definition threshold differed between studies. Among critical respiratory events, only hypoxaemia was investigated in all included studies. Residual neuromuscular blockade was significantly associated with increased incidence of hypoxaemia during postanaesthesia care unit stay in most studies, while associations with the rest of the critical respiratory events were inconclusive. Although limited, existing research has provided evidence that patients with residual neuromuscular blockade are at high risk of early postoperative hypoxaemia. Further studies are needed to investigate independent associations between residual neuromuscular blockade and critical respiratory events, along with causality of these associations. The clinical importance of residual neuromuscular blockade for groups at high risk of critical respiratory events should also be investigated. Healthcare professionals have to be aware of the increased risk of hypoxaemia in patients with residual neuromuscular blockade. Efforts to decrease residual neuromuscular blockade incidence

  2. FACTORS MODIFYING THE ACTION OF NEUROMUSCULAR BLOCKING AGENTS.

    DTIC Science & Technology

    neuromuscular blocking properties of another intravenous anaesthetic the eugenol derivative G.34075 and a recently synthesised relaxant diallyl-nortoxiferine were also investigated with this preparation. (Author)

  3. Neuromuscular Manifestations of West Nile Virus Infection

    PubMed Central

    Leis, A. Arturo; Stokic, Dobrivoje S.

    2012-01-01

    The most common neuromuscular manifestation of West Nile virus (WNV) infection is a poliomyelitis syndrome with asymmetric paralysis variably involving one (monoparesis) to four limbs (quadriparesis), with or without brainstem involvement and respiratory failure. This syndrome of acute flaccid paralysis may occur without overt fever or meningoencephalitis. Although involvement of anterior horn cells in the spinal cord and motor neurons in the brainstem are the major sites of pathology responsible for neuromuscular signs, inflammation also may involve skeletal or cardiac muscle (myositis, myocarditis), motor axons (polyradiculitis), and peripheral nerves [Guillain–Barré syndrome (GBS), brachial plexopathy]. In addition, involvement of spinal sympathetic neurons and ganglia provides an explanation for autonomic instability seen in some patients. Many patients also experience prolonged subjective generalized weakness and disabling fatigue. Despite recent evidence that WNV may persist long-term in the central nervous system or periphery in animals, the evidence in humans is controversial. WNV persistence would be of great concern in immunosuppressed patients or in those with prolonged or recurrent symptoms. Support for the contention that WNV can lead to autoimmune disease arises from reports of patients presenting with various neuromuscular diseases that presumably involve autoimmune mechanisms (GBS, other demyelinating neuropathies, myasthenia gravis, brachial plexopathies, stiff-person syndrome, and delayed or recurrent symptoms). Although there is no specific treatment or vaccine currently approved in humans, and the standard remains supportive care, drugs that can alter the cascade of immunobiochemical events leading to neuronal death may be potentially useful (high-dose corticosteroids, interferon preparations, and intravenous immune globulin containing WNV-specific antibodies). Human experience with these agents seems promising based on anecdotal reports

  4. Neuromuscular Functions on Experimental Acute Methanol Intoxication

    PubMed Central

    Moral, Ali Reşat; Çankayalı, İlkin; Sergin, Demet; Boyacılar, Özden

    2015-01-01

    Objective The incidence of accidental or suicidal ingestion of methyl alcohol is high and methyl alcohol intoxication has high mortality. Methyl alcohol intoxication causes severe neurological sequelae and appears to be a significant problem. Methyl alcohol causes acute metabolic acidosis, optic neuropathy leading to permanent blindness, respiratory failure, circulatory failure and death. It is metabolised in the liver, and its metabolite formic acid has direct toxic effects, causing oxidative stress, mitochondrial damage and increased lipid peroxidation associated with the mechanism of neurotoxicity. Methanol is known to cause acute toxicity of the central nervous system; however, the effects on peripheral neuromuscular transmission are unknown. In our study, we aimed to investigate the electrophysiological effects of experimentally induced acute methanol intoxication on neuromuscular transmission in the early period (first 24 h). Methods After approval by the Animal Experiment Ethics Committee of Ege University, the study was carried out on 10 Wistar rats, each weighing about 200 g. During electrophysiological recordings and orogastric tube insertion, the rats were anaesthetised using intra-peritoneal (IP) injection of ketamine 100 mg kg−1 and IP injection of xylazine 10 mg kg−1. The rats were given 3 g kg−1 methyl alcohol by the orogastric tube. Electrophysiological measurements from the gastrocnemius muscle were compared with baseline. Results Latency measurements before and 24 h after methanol injection were 0.81±0.11 ms and 0.76±0.12 ms, respectively. CMAP amplitude measurements before and 24 h after methanol injection were 9.85±0.98 mV and 9.99±0.40 mV, respectively. CMAP duration measurements before and 24 h after methanol injection were 9.86±0.03 ms and 9.86±0.045 ms, respectively. Conclusion It was concluded that experimental methanol intoxication in the acute phase (first 24 h) did not affect neuromuscular function. PMID:27366524

  5. Perisurgical management of patients with neuromuscular disorders.

    PubMed

    Bertorini, Tulio E

    2004-05-01

    Patients with neuromuscular disorders who undergo surgical procedures are particularly predisposed to complications during the perioperative period. Such complications may arise from respiratory failure, arrhythmias,or infections, and particularly MH. It is recommended that these patients be monitored for respiratory and cardiovascular complications and receive proper respiratory toilet, physio-therapy, and incentive respirometry. Proper electrolyte balance is mandatory. They should be monitored in the ICU when necessary. Excessive sedation of these patients, and drugs that could aggravate weakness or cause MH, should be avoided. Those at risk of MH should not receive drugs that may precipitate an attack.

  6. [Functional characteristics and distribution of neuromuscular spindles].

    PubMed

    Pintea, V; Ganţă, C

    1987-01-01

    The mm. lumbricales and the mm. capsulares of the shoulder and hip joints of the horse are rich in neuromuscular spindles (NMS). The mm. interossei of the pig, but not of the horse, have NMS too. In the m. orbicularis of the eye, m.hyo-epiglotticus and the m. palatinus of the horse NMS are absent. In the vestigial muscles, as m. extensor digitorum brevis and m. of Thiernesse, NMS are absent too. A role of modulator for the proximal muscles of the limbs by the distal muscles is hypothesized, as well as the absence of NMS in striated muscles not involved in locomotion.

  7. Neuromuscular Development and Regulation of Myosin Expression

    NASA Technical Reports Server (NTRS)

    Bodine, Sue

    1997-01-01

    The proposed experiments were designed to determine whether the absence of gravity during embryogenesis influences the postnatal development of the neuromuscular system. Further, we examined the effects of reduced gravity on hindlimb muscles of the pregnant rats. Microgravity may have short and long-term effects on the development of muscle fiber type differentiation and force producing capabilities. Microgravity will reduce muscle fiber size and cause a shift in myosin heavy chain expression from slow to fast in hindlimb muscles of the adult pregnant rats.

  8. [Transition experience of patients with neuromuscular disease].

    PubMed

    Greif, Valeria; Ugo, Florencia; de Castro Pérez, M Fernanda; Mozzoni, Julieta; Aguerre, Verónica; Saldías, Milagros; Monges, M Soledad

    2017-02-01

    Neuromuscular diseases are mostly genetic disorders, with chronic and progressive course. Affected people are at high risk of developing physical and emotional disabilities. In the last decades, the advance in technology and science has increased chronic pediatric patients survival rate, thus requiring an ongoing assistance in adult hospitals, making the transition a necessity and a challenge. This article reports the clinical practice designed between Hospital Garrahan and Hospital Ramos Mejía for the transition of 27 adolescents during 2015, setting achievements, findings and challenges resulting from this experience. Sociedad Argentina de Pediatría.

  9. Recent achievements in restorative neurology: Progressive neuromuscular diseases

    SciTech Connect

    Dimitrijevic, M.R.; Kakulas, B.A.; Vrbova, G.

    1986-01-01

    This book contains 27 chapters. Some of the chapter titles are: Computed Tomography of Muscles in Neuromuscular Disease; Mapping the Genes for Muscular Dystrophy; Trophic Factors and Motor Neuron Development; Size of Motor Units and Firing Rate in Muscular Dystrophy; Restorative Possibilities in Relation to the Pathology of Progressive Neuromuscular Disease; and An Approach to the Pathogenesis of some Congenital Myopathies.

  10. Neuromuscular Characteristics of Endurance--And Power-Trained Athletes

    ERIC Educational Resources Information Center

    Koceja, David M.; Davison, Edwin; Robertson, Christopher T.

    2004-01-01

    In response to chronic physical training, the human neuromuscular system undergoes significant and specific adaptations. More importantly, these influences are the result of the type and quantity of physical activity. One of the simplest neuromuscular mechanisms is the spinal stretch reflex. The reflex system was previously viewed as inflexible,…

  11. Neuromuscular Blockade and Reversal Agents: A Primer for Postanesthesia Nurses.

    ERIC Educational Resources Information Center

    Pesci, Barbara R.

    1986-01-01

    Presents a comprehensive review of neuromuscular blocking agents, reversal agents used in anesthesia, and factors affecting reversal. It is aimed at nurses who provide care to patients recovering from anesthesia. It discusses the neuromuscular transmission system, depolarizing muscle relaxants, nondepolarizing blocking agents, and criteria for…

  12. Neuromuscular Characteristics of Endurance--And Power-Trained Athletes

    ERIC Educational Resources Information Center

    Koceja, David M.; Davison, Edwin; Robertson, Christopher T.

    2004-01-01

    In response to chronic physical training, the human neuromuscular system undergoes significant and specific adaptations. More importantly, these influences are the result of the type and quantity of physical activity. One of the simplest neuromuscular mechanisms is the spinal stretch reflex. The reflex system was previously viewed as inflexible,…

  13. 21 CFR 882.5810 - External functional neuromuscular stimulator.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false External functional neuromuscular stimulator. 882.5810 Section 882.5810 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN... External functional neuromuscular stimulator. (a) Identification. An external functional...

  14. 21 CFR 882.5810 - External functional neuromuscular stimulator.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 21 Food and Drugs 8 2011-04-01 2011-04-01 false External functional neuromuscular stimulator. 882.5810 Section 882.5810 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN... External functional neuromuscular stimulator. (a) Identification. An external functional...

  15. Neuromuscular Blockade and Reversal Agents: A Primer for Postanesthesia Nurses.

    ERIC Educational Resources Information Center

    Pesci, Barbara R.

    1986-01-01

    Presents a comprehensive review of neuromuscular blocking agents, reversal agents used in anesthesia, and factors affecting reversal. It is aimed at nurses who provide care to patients recovering from anesthesia. It discusses the neuromuscular transmission system, depolarizing muscle relaxants, nondepolarizing blocking agents, and criteria for…

  16. Motoneuron and sensory neuron plasticity to varying neuromuscular activity levels

    NASA Technical Reports Server (NTRS)

    Ishihara, Akihiko; Roy, Roland R.; Ohira, Yoshinobu; Edgerton, V. Reggie

    2002-01-01

    The size and phenotypic properties of the neural and muscular elements of the neuromuscular unit are matched under normal conditions. When subjected to chronic decreases or increases in neuromuscular activity, however, the adaptations in these properties are much more limited in the neural compared with the muscular elements.

  17. Prevalencia de alteraciones en la interfase vitreoretiniana detectadas mediante tomografía de coherencia óptica de dominio espectral.

    PubMed

    Jacob, Julie; Stalmans, Peter

    2017-07-11

    Objetivo: El objetivo principal del presente estudio consistió en determinar la prevalencia de los cambios en la interfase vitreomacular (IVM) mediante tomografía de coherencia óptica (OCT) en la población general. En segundo lugar, se describieron otros cambios de la OCT. Métodos: Las anomalías en la IVM se diagnosticaron mediante OCT y se distribuyeron de acuerdo con la clasificación del grupo International Vitreomacular Traction Study (estudio internacional de tracción vitreomacular, IVTS, por sus siglas en inglés) y se dividieron en 3 grados según John et al. [Retina 2014;34:442-446]. Resultados: La prevalencia calculada de anomalías vitreomaculares en la población belga de ≥50 años fue del 1,17% [intervalo de confianza (IC 0,38-3,62)] en el caso de tracción vitreomacular (TVM) focal de grado 1; del 0,39% (IC 0,05-2,76) en el caso de TVM focal de grado 2; del 8,17% (IC 5,33-12,53) en el caso de adhesión vitreomacular focal; y del 17,9% (IC 13,41-23,9) en el caso de adhesión vitreomacular difusa. Conclusiones: Se presentó la prevalencia de anomalías vitreomaculares en un estudio de cohortes belga. Estos resultados concuerdan en gran medida con los datos presentados previamente sobre la prevalencia de la TVM. Un conocimiento correcto sobre la epidemiología de las alteraciones en la IVM y un diagnóstico temprano permitirán una intervención satisfactoria. © 2017 S. Karger AG, Basel.

  18. Novel neuromuscular blocking drugs and antagonists.

    PubMed

    Heerdt, Paul M; Sunaga, Hiroshi; Savarese, John J

    2015-08-01

    This review summarizes recent progress in the development of new muscle relaxants that are inactivated by cysteine, and considers the evolving paradigm of selective relaxant binding or degrading agents that can reverse neuromuscular blockade at any time. The benzylisoquinoline compound gantacurium is a nondepolarizing muscle relaxant with an ultrashort duration largely determined by the rapid rate at which endogenous L-cysteine binds to, and permanently inactivates, the molecule. Although the clinical development of gantacurium has been hampered by modest histamine release, preclinical studies demonstrating that the drug can be rapidly reversed by injecting L-cysteine led to the development of CW002, an intermediate duration molecule that can also be reversed at any time by L-cysteine injection. Clinical trials with CW002 are now underway. The ability to reverse complete paralysis with cysteine dovetails with the established selective aminosteroid binding agent sugammadex, and the recently described universal relaxant binding agent calabadion. Taken together, the concept of rapid reversal at any time raises the question of whether an ultrashort nondepolarizing drug is needed if safe and cost-effective relaxant binding agents are available. The gantacurium derivative CW002 is an intermediate duration, nondepolarizing, cysteine-inactivated, neuromuscular blocking drug currently in clinical trials. Like sugammadex reversal of rocuronium, CW002 can be reversed at any time by cysteine injection.

  19. Rabies virus binding at neuromuscular junctions.

    PubMed

    Burrage, T G; Tignor, G H; Smith, A L

    1985-04-01

    Morphological, immunocytochemical, biochemical, and immunological techniques have been used to describe rabies virus binding to a sub-cellular unit and molecular complex at the neuromuscular junction (NMJ). Early after infection in vivo, virus antigen and virus particles were found by immunofluorescence, electron microscopy and immunoelectron microscopy in regions of high density acetylcholine receptors (AChR) at NMJs. One monoclonal antibody (alpha-Mab) to the alpha subunit of the AChR blocked attachment of radio-labeled rabies virus to cultured muscle cells bearing high density patches of AChR. A sub-cellular structure, resembling an array of AChR monomers, bound both rabies virus antigens and alpha-Mab. By immunoblotting with electrophoretically transferred motor endplate proteins, rabies virus proteins and alpha-Mab bound to two proteins of 43 000 and 110 000 daltons. A rabies virus glycoprotein antibody detected virus antigen bound to the 110 000 dalton protein. An auto-immune (anti-idiotypic) response followed immunization of mice with rabies virus glycoprotein antigen; the antibody was directed to the 110 000 dalton protein. This auto-antibody altered the kinetics of neutralization by rabies virus antibody and induced the formation of rabies virus antibody after inoculation of mice. These results define, at the neuromuscular junction, a rabies virus receptor which may be part of the acetylcholine receptor complex.

  20. Neuromuscular disruption with ultrashort electrical pulses

    NASA Astrophysics Data System (ADS)

    Pakhomov, Andrei; Kolb, Juergen F.; Joshi, Ravindra P.; Schoenbach, Karl H.; Dayton, Thomas; Comeaux, James; Ashmore, John; Beason, Charles

    2006-05-01

    Experimental studies on single cells have shown that application of pulsed voltages, with submicrosecond pulse duration and an electric field on the order of 10 kV/cm, causes sudden alterations in the intracellular free calcium concentration, followed by immobilization of the cell. In order to examine electrical stimulation and incapacitation with such ultrashort pulses, experiments on anesthetized rats have been performed. The effect of single, 450 nanosecond monopolar pulses have been compared with that of single pulses with multi-microsecond duration (TASER pulses). Two conditions were explored: 1. the ability to elicit a muscle twitch, and, 2. the ability to suppress voluntary movement by using nanosecond pulses. The second condition is relevant for neuromuscular incapacitation. The preliminary results indicate that for stimulation microsecond pulses are advantageous over nanosecond pulses, whereas for incapacitation, the opposite seems to apply. The stimulation effects seem to scale with electrical charge, whereas the disruption effects don't follow a simple scaling law. The increase in intensity (time of incapacitation) for a given pulse duration, is increasing with electrical energy, but is more efficient for nanosecond than for microsecond pulses. This indicates different cellular mechanisms for incapacitation, most likely subcellular processes, which have been shown to become increasingly important when the pulse duration is shortened into the nanosecond range. If further studies can confirm these initial results, consequences of reduced pulse duration are a reduction in weight and volume of the pulse delivery system, and likely, because of the lower required energy for neuromuscular incapacitation, reduced safety risks.

  1. Neuromuscular Dysfunction in Experimental Sepsis and Glutamine

    PubMed Central

    Çankayalı, İlkin; Boyacılar, Özden; Demirağ, Kubilay; Uyar, Mehmet; Moral, Ali Reşat

    2016-01-01

    Background: Electrophysiological studies show that critical illness polyneuromyopathy appears in the early stage of sepsis before the manifestation of clinical findings. The metabolic response observed during sepsis causes glutamine to become a relative essential amino acid. Aims: We aimed to assess the changes in neuromuscular transmission in the early stage of sepsis after glutamine supplementation. Study Design: Animal experimentation. Methods: Twenty male Sprague-Dawley rats were randomized into two groups. Rats in both groups were given normal feeding for one week. In the study group, 1 g/kg/day glutamine was added to normal feeding by feeding tube for one week. Cecal ligation and perforation (CLP) surgery was performed at the end of one week. Before and 24 hours after CLP, compound muscle action potentials were recorded from the gastrocnemius muscle. Results: Latency measurements before and 24 hours after CLP were 0.68±0.05 ms and 0.80±0.09 ms in the control group and 0.69±0.07 ms and 0.73±0.07 ms in the study group (p<0.05). Conclusion: Since enteral glutamine prevented compound muscle action potentials (CMAP) latency prolongation in the early phase of sepsis, it was concluded that enteral glutamine replacement might be promising in the prevention of neuromuscular dysfunction in sepsis; however, further studies are required. PMID:27308070

  2. Reversal of neuromuscular block in companion animals.

    PubMed

    Jones, Ronald S; Auer, Ulrike; Mosing, Martina

    2015-09-01

    To review the evidence regarding the reversal of neuromuscular block (NMB) in companion animals with emphasis on the development and use of newer agents. Data sources include scientific reviews and original research publications in both human and veterinary literature using Pubmed and Scopus as search data bases. Unpublished and locally published data on reversal of NMB are presented. Residual NMB has been shown to increase morbidity and mortality in humans and needs to be avoided. It can be detected only by adequate neuromuscular monitoring. The proper use of reversal agents avoids residual NMB and recurarization should not occur. Anticholinesterase inhibitors, such as edrophonium and neostigmine have been used to reverse NMB when the need for this has been established. Reversal is influenced by several factors and a number of undesirable side- effects of these drugs have been reported. Sugammadex, a γ-cyclodextrin, which was designed specifically to encapsulate rocuronium, is more rapid in its actions, has fewer side effects and can reverse profound NMB induced by aminosteroidal muscle relaxants. © 2015 Association of Veterinary Anaesthetists and the American College of Veterinary Anesthesia and Analgesia.

  3. THE ROLE OF THE NEUROMUSCULAR MEDICINE SPECIALIST AND PHYSIATRY IN THE MANAGEMENT OF NEUROMUSCULAR DISEASE

    PubMed Central

    McDonald, Craig M.; Fowler, William M.

    2012-01-01

    Synopsis The neuromuscular medicine, and physiatry specialists are key health care providers who work cooperatively with a multidisciplinary team to provide coordinated care for persons with Neuromuscular diseases (NMDs). The director or coordinator of the team must be aware of the potential issues specific to NMDs and be able to access the interventions that are the foundations for proper care in NMD. These include health maintenance and proper monitoring of disease progression and complications to provide anticipatory, preventive care and optimum management. Ultimate goals include maximizing health and functional capacities, performing medical monitoring and surveillance to inhibit and prevent complications, and promoting access and full integration into the community in order to optimize quality of life. PMID:22938874

  4. Novel pharmacological approaches for the antagonism of neuromuscular blockade.

    PubMed

    Pic, Lisa C

    2005-02-01

    Gamma cyclodextrin and purified plasma cholinesterase are 2 novel pharmacological agents being investigated as to their suitability for antagonism of neuromuscular blockade. Both of these agents are devoid of cholinergic stimulation and the accompanying side effects because their action is independent of acetylcholinesterase inhibition. Gamma cyclodextrin antagonizes the steroidal neuromuscular blocker rocuronium via the chemical encapsulation of the molecule forming a "host-guest" complex through van der Waals and hydrophobic interactions in the plasma. Encapsulation decreases plasma drug concentrations, shifting the neuromuscular blocking drug molecules from the neuromuscular junction back to the plasma compartment resulting in a rapid recovery of the neuromuscular function. Org 25969, a modified gamma cyclodextrin, will antagonize profound neuromuscular block induced by rocuronium in approximately 2 minutes. A commercial preparation of purified human plasma cholinesterase has been shown to be effective in reversing succinylcholine or mivacurium-induced block. Administration of exogenous plasma cholinesterase also has been shown to be effective in antagonizing mivacurium-induced neuromuscular block, cocaine toxicity, and organophosphate poisoning.

  5. Surgical advances in the treatment of neuromuscular scoliosis

    PubMed Central

    Canavese, Federico; Rousset, Marie; Le Gledic, Benoit; Samba, Antoine; Dimeglio, Alain

    2014-01-01

    Neuromuscular disorders are a group of diseases affecting the neuro-musculo-skeletal system. Children with neuromuscular disorders frequently develop progressive spinal deformities with cardio-respiratory compromise in the most severe cases. The incidence of neuromuscular scoliosis is variable, inversely correlated with ambulatory abilities and with a reported risk ranging from 80% to 100% in non-ambulatory patients. As surgical and peri-operative techniques have improved, more severely affected children with complex neuromuscular deformities and considerable co-morbidities are now believed to be candidates for extensive surgery for spinal deformity. This article aimed to provide a comprehensive review of how neuromuscular spinal deformities can affect normal spine balance and how these deformities can be treated with segmental instrumentation and sub-laminar devices. Older concepts have been integrated with newer scientific data to provide the reader with a basis for better understanding of how treatment of neuromuscular scoliosis has evolved over the past few decades. Recent advances, as well as challenges that remain to be overcome, in the surgical treatment of neuromuscular curves with sub-laminar devices and in the management of post-operative infections are outlined. PMID:24829875

  6. Applied pharmacology of common neuromuscular blocking agents in critical care.

    PubMed

    Prielipp, R C; Coursin, D B

    1994-02-01

    While all neuromuscular blocking agents (NMBAs) effectively interrupt neuromuscular transmission, it must be emphasized that these drugs are completely devoid of analgesic, sedative, or amnestic properties. The increasing use of NMBAs in the ICU requires familiarity with their basic pharmacologic properties, as well as an appreciation of potential problems associated with chronic (> 24 hrs) neuromuscular blockade. Although NMBAs possess an impressive safety record, the majority of recommendations for neuromuscular blocker use in the ICU are extrapolated from short-term perioperative studies in healthy patients. NMBAs are structurally related to acetylcholine and their main site of action is the postjunctional nicotinic acetylcholine receptor, although prejunctional interaction may be an important component of total activity. These drugs act to either sustain a depolarization at the postjunctional membrane (succinylcholine), or they inhibit neuromuscular transmission by a competitive (non-depolarizing) blocking mechanism. Adverse hemodynamic consequences may result from concurrent stimulation of muscarinic receptors, autonomic ganglia, histamine, or catecholamine release associated with some agents. The metabolism and excretion of NMBAs may be altered in ICU patients with end-organ dysfunction, concurrent medications, electrolyte, acid-base, and nutritional abnormalities, along with underlying nervous system and muscle pathology. Prolonged weakness after discontinuation of NMBAs is increasingly recognized after these agents are used for extended periods. This phenomenon may be related to alterations in the pharmacokinetics and pharmacodynamics, along with altered physiology of the neuromuscular junction, nervous system, or muscle, or other undefined toxic effects. A sound knowledge of the basic physiology of the neuromuscular junction, neuromuscular blocker pharmacology, and standard techniques to assess the degree of neuromuscular blockade provides the rationale

  7. [Prolonged phase II neuromuscular blockade following succinylcholine administration].

    PubMed

    Jurkolow, G; Fuchs-Buder, T; Lemoine, A; Raft, J; Rocq, N; Meistelman, C

    2014-03-01

    Patients who are given a single dose of succinylcholine normally undergo a short-acting depolarizing phase I neuromuscular block but rarely a phase II block. Prolonged neuromuscular blockade occurs after a single dose of succinylcholine in case of genetically determined abnormal plasma butyrylcholinesterase activity. It is mandatory to use monitoring to detect this side effect. We report a case of a patient with abnormal plasma butyrylcholinesterase activity undergoing a six-hour prolonged neuromuscular phase II block, after a single dose of succinylcholine.

  8. Neuromuscular ultrasound findings in polyneuropathy secondary to disulfiram.

    PubMed

    Stone, Sarah L; Cartwright, Michael S; Panea, Oana R; Vann, Ryan C; Magruder, John L; Walker, Francis O

    2014-12-01

    Disulfiram toxicity can cause multiple neurologic problems, including a reversible distal sensorimotor axonal polyneuropathy. Although electrodiagnosis and biopsy results have been described in the diagnosis and management of patients with disulfiram associated polyneuropathy, neuromuscular ultrasound findings have not been reported. The authors present a case of electrodiagnostically confirmed axonal polyneuropathy with relative sural sparing secondary to disulfiram and describe the neuromuscular ultrasound findings in this individual. Ultrasound demonstrated distal enlargement with slight side-to-side asymmetry and normal proximal cross-sectional area in the lower extremity nerves. Neuromuscular ultrasound is another diagnostic modality that may be used to assist in the diagnosis of patients with polyneuropathy secondary to disulfiram.

  9. Neuromuscular problems in foot and ankle: evaluation and workup.

    PubMed

    Hunt, Kenneth J; Ryu, Jessica H

    2014-03-01

    It is essential to determine the functional goals of the patient during the workup and treatment planning stages of neuromuscular disorders involving the foot and ankle. Accurate diagnosis, and informed discussion of treatment options, must be in the context of the patient's disease, cognition, comorbidities, functional attributes, and family environment. A thorough history and physical examination aid in appropriate diagnostic workup and optimal orthopedic management of each patient. In this article, general considerations in the workup of suspected neuromuscular disorders and issues pertinent to specific congenital and acquired neuromuscular disorders affecting foot and ankle function are reviewed.

  10. Hand orthotic device influence on fine neuromuscular control.

    PubMed

    Simard, T G; Ladd, H W

    1976-06-01

    With the aid of electromyography, voluntary control of fine neuromuscular activity of deltoid and extensor digitorum communis muscles was studied in patients with motor dysfunction of the upper limb. A sequential training procedure was carried out both while the patient was wearing and not wearing a hand orthosis, during a static posture and dynamic hand movements. There was no difference between the levels of neuromuscular control achieved while wearing and not wearing the orthotic device, but the quality of the neuromuscular control was superior in the proximal limb muscle to that in the more distal muscle.

  11. Load-dependent regulation of neuromuscular system

    NASA Technical Reports Server (NTRS)

    Ohira, Yoshinobu; Kawano, Fuminori; Stevens, James L.; Wang, Xiao D.; Ishihara, Akihiko

    2004-01-01

    Roles of gravitational loading, sarcomere length, and/or tension development on the electromyogram (EMG) of soleus and afferent neurogram recorded at the L5 segmental level of spinal cord were investigated during parabolic flight of a jet airplane or hindlimb suspension in conscious rats. Both EMG and neurogram levels were increased when the gravity levels were elevated from 1-G to 2-G during the parabolic flight. They were decreased when the hindlimbs were unloaded by exposure to actual microgravity or by suspension. These phenomena were related to passive shortening of muscle fibers and/or sarcomeres. Unloading-related decrease in sarcomere length was greater at the central rather than the proximal and distal regions of fibers. These activities and tension development were not detected when the mean sarcomere length was less than 2.03 micrometers. It is suggested that load-dependent regulation of neuromuscular system is related to the tension development which is influenced by sarcomere length.

  12. Load-dependent regulation of neuromuscular system

    NASA Technical Reports Server (NTRS)

    Ohira, Yoshinobu; Kawano, Fuminori; Stevens, James L.; Wang, Xiao D.; Ishihara, Akihiko

    2004-01-01

    Roles of gravitational loading, sarcomere length, and/or tension development on the electromyogram (EMG) of soleus and afferent neurogram recorded at the L5 segmental level of spinal cord were investigated during parabolic flight of a jet airplane or hindlimb suspension in conscious rats. Both EMG and neurogram levels were increased when the gravity levels were elevated from 1-G to 2-G during the parabolic flight. They were decreased when the hindlimbs were unloaded by exposure to actual microgravity or by suspension. These phenomena were related to passive shortening of muscle fibers and/or sarcomeres. Unloading-related decrease in sarcomere length was greater at the central rather than the proximal and distal regions of fibers. These activities and tension development were not detected when the mean sarcomere length was less than 2.03 micrometers. It is suggested that load-dependent regulation of neuromuscular system is related to the tension development which is influenced by sarcomere length.

  13. [Neuromuscular electric stimulation therapy in otorhinolaryngology].

    PubMed

    Miller, S; Kühn, D; Jungheim, M; Schwemmle, C; Ptok, M

    2014-02-01

    Animal experiments have shown that after specific nerve traumatization, neuromuscular electrostimulation (NMES) can promote nerve regeneration and reduce synkinesia without negatively interfering with normal regeneration processes. NMES is used routinely in physical rehabilitation medicine. This systematic literature search in the Cochrane Central Register of Controlled Trials, the Cochrane Database of Systematic Reviews, the DAHTA database, the Health Technology Assessment Database and MEDLINE or PubMed considered studies on the use of NMES in otorhinolaryngology that have been published in German or English. The search identified 180 studies. These were evaluated and relevant studies were included in the further evaluation. In the fields of otorhinolaryngology and phoniatry/paediatric audiology, clinical studies investigating the effects of NMES on facial and laryngeal paresis, as well as dysphonia and dysphagia have been carried out. The evidence collected to date is encouraging; particularly for the treatment of certain forms of dysphagia and laryngeal paresis.

  14. Muscle Biopsy Evaluation in Neuromuscular Disorders

    PubMed Central

    Joyce, Nanette C.; Oskarsson, Björn; Jin, Lee-Way

    2014-01-01

    Synopsis Muscle biopsy is a commonly ordered diagnostic procedure, used by clinicians who evaluate patients with weakness suspected to be caused by muscle disease. This article reviews the indications for a muscle biopsy, and then serves as a step-by-step guide reviewing the processes of muscle selection through to interpreting the biopsy report. The goal of this article is to aid the clinician in preparing for a muscle biopsy procedure so that they may avoid common pitfalls and obtain optimal results from this minimally invasive procedure. We review the basic anatomical structure of normal muscle to provide a foundation for understanding common patterns of pathologic change observed in muscle disease, and then present both common and disease-specific histopathologic findings, focused for illustrative purposes on a select group of neuromuscular diseases. PMID:22938878

  15. On the neuromuscular paralysis produced by procaine

    PubMed Central

    Hirst, G. D. S.; Wood, D. R.

    1971-01-01

    1. The effects of procaine on neuromuscular transmission in the rat phrenic nerve diaphragm preparation have been examined using intracellular recording techniques. 2. The paralysis produced by procaine resembled that produced by tubocurarine and it is concluded that this effect of procaine results from a change in post-junctional sensitivity to transmitter; at low rates of stimulation procaine did not depress the transmitter release. 3. During high frequency stimulation of the phrenic nerve a distinct form of prejunctional failure was observed. It is suggested that this depression results from a combination of local anaesthesia and anoxia and that it would explain reports of a reduction in the amount of transmitter released when assayed in a conventional manner. PMID:4396131

  16. [The hip joint in neuromuscular disorders].

    PubMed

    Strobl, W M

    2009-07-01

    Physiologic motor and biomechanical parameters are prerequisites for normal hip development and hip function. Disorders of muscle activity and lack of weight bearing due to neuromuscular diseases may cause clinical symptoms such as an unstable hip or reduced range of motion. Disability and handicap because of pain, hip dislocation, osteoarthritis, gait disorders, or problems in seating and positioning are dependent on the severity of the disease, the time of occurrence, and the means of prevention and treatment. Preservation of pain-free and stable hip joints should be gained by balancing muscular forces and by preventing progressive dislocation. Most important is the exact indication of therapeutic options such as movement and standing therapy as well as drugs and surgery.

  17. Cardiorespiratory and neuromuscular responses to motocross riding.

    PubMed

    Konttinen, Tomi; Kyröläinen, Heikki; Häkkinen, Keijo

    2008-01-01

    The aim of the present study was to examine physiological and neuromuscular responses during motocross riding at individual maximal speed together with the riding-induced changes in maximal isometric force production. Seven A-level (group A) and 5 hobby-class (group H) motocross-riders performed a 30-minute riding test on a motocross track and maximal muscle strength and oxygen uptake (VO2max) tests in a laboratory. During the riding the mean (+/-SD) VO2 reduced in group A from 86 +/- 10% to 69 +/- 6% of the maximum (P < 0.001), whereas in group H the corresponding reduction was from 94 +/- 25% to 82 +/- 20% (P < 0.05). This relative VO2 during the riding correlated with riding speed (r = 0.70, P < 0.01). Heart rate (HR) was maintained at the level of 97 +/- 7% of its maximum in group A and at 98 +/- 3% in group H. Mean muscle activation of the lower body during riding varied between 24% and 38% of its maximum in group A and between 40% and 45% in group H. In conclusion, motocross is a sport that causes great physical stress and demands on both skill and physical capacity of the rider. Physical stress occurs as the result of handling of the bike when receiving continuous impacts in the situation requiring both aerobic and anaerobic metabolism. Our data suggest that both maximal capacity and strain during the ride should be measured to analyze the true physiological and neuromuscular demands of motocross ride. For the practice, this study strongly suggests to train not only aerobic and anaerobic capacity but also to use strength and power training for successful motocross riding.

  18. Explosive neuromuscular performance of males versus females.

    PubMed

    Hannah, Ricci; Minshull, Claire; Buckthorpe, Matthew W; Folland, Jonathan P

    2012-05-01

    The purpose of the study was to investigate sex-related differences in explosive muscular force production, as measured by electromechanical delay (EMD) and rate of force development (RFD), and to examine the physiological mechanisms responsible for any differences. The neuromuscular performance of untrained males (n = 20) and females (n = 20) was assessed during a series of isometric knee extension contractions; explosive and maximal voluntary efforts, as well as supramaximal evoked twitches and octets (eight pulses at 300 Hz). Evoked and voluntary EMD were determined from twitch and explosive contractions. The RFD was recorded over consecutive 50 ms time windows from force onset during evoked and explosive contractions, and normalized to maximal strength. Neuromuscular activity during explosive voluntary contractions was measured with EMG of the superficial knee extensors normalized to maximal M-wave. Muscle size (thickness) and muscle-tendon unit (MTU) stiffness were assessed using ultrasonic images of the vastus lateralis at rest and during ramped contractions. Males and females had similar evoked and voluntary EMD. Males were 33% stronger (P < 0.001) and their absolute RFD was 26-56% greater (all time points P < 0.05) compared with females. Muscle size (P < 0.001) and absolute MTU stiffness were also greater for males (P < 0.05). However, normalized RFD was similar for both sexes during the first 150 ms of the explosive voluntary contractions (P > 0.05). This was consistent with the similar normalized twitch and octet RFD, MTU stiffness and agonist EMG (all P > 0.05). When differences in maximal strength were accounted for, the evoked capacity of the knee extensors for explosive force production and the ability to utilize that capacity during explosive voluntary contractions was similar for males and females.

  19. Cotrel-Dubousset instrumentation in neuromuscular scoliosis.

    PubMed

    Piazzolla, Andrea; Solarino, G; De Giorgi, S; Mori, C M; Moretti, L; De Giorgi, G

    2011-05-01

    The study design is retrospective. The aim is to describe our experience about the treatment of patients with neuromuscular scoliosis (NMS) using Cotrel-Dubousset instrumentation. Neuromuscular scoliosis are difficult deformities to treat. A careful assessment and an understanding of the primary disease and its prognosis are essential for planning treatment which is aimed at maximizing function. These patients may have pelvic obliquity, dislocation of the hip, limited balance or ability to sit, back pain, and, in some cases, a serious decrease in pulmonary function. Spinal deformity is difficult to control with a brace, and it may progress even after skeletal maturity has been reached. Surgery is the main stay of treatment for selected patients. The goals of surgery are to correct the deformity producing a balanced spine with a level pelvis and a solid spinal fusion to prevent or delay secondary respiratory complications. The instrumented spinal fusion (ISF) with second-generation instrumentation (e.g., Luque-Galveston and unit rod constructs), are until 1990s considered the gold standard surgical technique for neuromuscular scoliosis (NMS). Still in 2008 Tsirikos et al. said that "the Unit rod instrumentation is a common standard technique and the primary instrumentation system for the treatment of pediatric patients with cerebral palsy and neuromuscular scoliosis because it is simple to use, it is considerably less expensive than most other systems, and can achieve good deformity correction with a low loss of correction, as well as a low prevalence of associated complications and a low reoperation rate." In spite of the Cotrel-Dubousset (CD) surgical technique, used since the beginning of the mid 1980s, being already considered the highest level achieved in correction of scoliosis by a posterior approach, Teli et al., in 2006, said that reports are lacking on the results of third-generation instrumentation for the treatment of NMS. Patients with neuromuscular

  20. The role of proprioception and neuromuscular stability in carpal instabilities.

    PubMed

    Hagert, E; Lluch, A; Rein, S

    2016-01-01

    Carpal stability has traditionally been defined as dependent on the articular congruity of joint surfaces, the static stability maintained by intact ligaments, and the dynamic stability caused by muscle contractions resulting in a compression of joint surfaces. In the past decade, a fourth factor in carpal stability has been proposed, involving the neuromuscular and proprioceptive control of joints. The proprioception of the wrist originates from afferent signals elicited by sensory end organs (mechanoreceptors) in ligaments and joint capsules that elicit spinal reflexes for immediate joint stability, as well as higher order neuromuscular influx to the cerebellum and sensorimotor cortices for planning and executing joint control. The aim of this review is to provide an understanding of the role of proprioception and neuromuscular control in carpal instabilities by delineating the sensory innervation and the neuromuscular control of the carpus, as well as descriptions of clinical applications of proprioception in carpal instabilities.

  1. Genetics of Pediatric-Onset Motor Neuron and Neuromuscular Diseases

    ClinicalTrials.gov

    2015-08-24

    Spinal Muscular Atrophy; Charcot-Marie-Tooth Disease; Muscular Dystrophy; Spinal Muscular Atrophy With Respiratory Distress 1; Amyotrophic Lateral Sclerosis; Motor Neuron Disease; Neuromuscular Disease; Peroneal Muscular Atrophy; Fragile X Syndrome

  2. Teaching Visually Impaired Adults with a Neuromuscular Disorder.

    ERIC Educational Resources Information Center

    Williams, Susan

    1983-01-01

    The effects of four neuromuscular disorders (stroke, Parkinson's disease, Huntington's disease, and Lou Gehrig's disease) on concommitant visual impairments are considered. Rehabilitation approaches and equipment that help clients cope with the condition are described. (CL)

  3. Teaching Visually Impaired Adults with a Neuromuscular Disorder.

    ERIC Educational Resources Information Center

    Williams, Susan

    1983-01-01

    The effects of four neuromuscular disorders (stroke, Parkinson's disease, Huntington's disease, and Lou Gehrig's disease) on concommitant visual impairments are considered. Rehabilitation approaches and equipment that help clients cope with the condition are described. (CL)

  4. The potential of disease management for neuromuscular hereditary disorders.

    PubMed

    Chouinard, Maud-Christine; Gagnon, Cynthia; Laberge, Luc; Tremblay, Carmen; Côté, Charlotte; Leclerc, Nadine; Mathieu, Jean

    2009-01-01

    Neuromuscular hereditary disorders require long-term multidisciplinary rehabilitation management. Although the need for coordinated healthcare management has long been recognized, most neuromuscular disorders are still lacking clinical guidelines about their long-term management and structured evaluation plan with associated services. One of the most prevalent adult-onset neuromuscular disorders, myotonic dystrophy type 1, generally presents several comorbidities and a variable clinical picture, making management a constant challenge. This article presents a healthcare follow-up plan and proposes a nursing case management within a disease management program as an innovative and promising approach. This disease management program and model consists of eight components including population identification processes, evidence-based practice guidelines, collaborative practice, patient self-management education, and process outcomes evaluation (Disease Management Association of America, 2004). It is believed to have the potential to significantly improve healthcare management for neuromuscular hereditary disorders and will prove useful to nurses delivering and organizing services for this population.

  5. Neuromuscular blockade: what was, is and will be.

    PubMed

    Schepens, Tom; Cammu, Guy

    2014-01-01

    Non-depolarizing neuromuscular blocking agents (NMBAs) produce neuromuscular blockade by competing with acetylcholine at the neuromuscular junction, whereas depolarizing NMBAs open receptor channels in a manner similar to that of acetylcholine. Problems with NMBAs include malignant hyperthermia caused by succinylcholine, anaphylaxis with the highest incidence for succinylcholine and rocuronium, and residual neuromuscular blockade. To reverse these blocks, anticholinesterases can act indirectly by increasing the amount of acetylcholine in the neuromuscular junction; sugammadex is the only selective relaxant binding agent (SRBA) in clinical use. At all levels of blockade, recovery after sugammadex is faster than after neostigmine. Sugammadex potentially also has some other advantages over neostigmine that are related to neostigmine's increase in the amount of acetylcholine and the necessity of co-administering anticholinergics. However, hypersensitivity reactions, including anaphylaxis, have occurred in some patients and healthy volunteers after sugammadex and remain an issue for the FDA. In the near future, we may see the emergence of new SRBAs and of easier-to-use technologies that can routinely monitor neuromuscular transmissions in daily practice. The nature of the effect of sugammadex on freeing nicotinic acetylcholine receptors located outside the neuromuscular junction from NMBAs is unknown. Moreover, it is uncertain whether the full removal of the competing antagonists (by SRBAs) at the neuromuscular junction impacts the efficiency of acetylcholine transmission. In a recent pilot study in healthy volunteers, we demonstrated increased electromyographic diaphragm activity after sugammadex, compared to neostigmine. Further research is needed to elucidate the role of NMBAs and their reversal agents in the central control of breathing, respiratory muscle activity, and respiratory outcomes.

  6. Control of neuromuscular blockade in the presence of sensor faults.

    PubMed

    Lemos, João M; Magalhães, Hugo; Mendonça, Teresa; Dionísio, Rui

    2005-11-01

    The problem of embedding sensor fault tolerance in feedback control of neuromuscular blockade is considered. For tackling interruptions of feedback measurements, a structure based upon Bayesian inference as well as a predictive filter is proposed. This algorithm is general and can be applied to different situations. Here, it is incorporated in an adaptive automatic system for feedback control of neuromuscular blockade using continuous infusion of muscle relaxants. A significant contribution consists in the experimental clinical testing of the algorithm in patients undergoing surgery.

  7. Study on Neuromuscular Blockade Action of Verapamil in Albino Rats

    PubMed Central

    Nagaral, Jayashree; GH, Shashikala; K, Jagadeesh; Kumar K, Sharath; GS, Jayanth; PK, Chennaveerappa; Patil, Rajani

    2013-01-01

    Background: Calcium Channel Blockers (CCBs) are now widely employed in the treatment of cardiovascular diseases and peri operative hypertension. It has been reported that calcium channel blockers inhibit neuromuscular transmission. They have been shown to increase the neuromuscular blockade produced by neuromuscular blocking agents in in-vitro muscle nerve preparations. The present study is undertaken to demonstrate the effect of calcium channel blocker, verapamil on neuromuscular transmission in albino rats. Objectives: To study the neuromuscular blockade action of verapamil in albino rats. Methods: Twenty four albino rats of either sex weigh 150-250gms are selected and are randomly divided into 4 equal groups. The experimental rats are divided into four groups of 6 rats each and they are given the following treatment. Group 1(Control) - Normal saline (1ml/ kg), Group 2 (Standard) - Pancuronium (0.04 mg/kg) Group 3-Verapamil (2.5mg/kg), Group 4-given Verapamil (10mg/kg). The time of onset of hind limb paralysis and total duration of recovery are noted using inclined screen method. Results: Analysis of the results of group 3 that was received 2.5mg/kg of Verapamil, there was no onset of paralysis, in group 4 that received injection Verapamil 10mg/kg, showed neuromuscular blockade activity. The mean onset of hind limb paralysis was delayed compared to standard group and the mean duration of hind limb paralysis was shorter than standard group. It was statistically significant (P≤ 0.05). Interpretation and conclusion: It is generally held that external calcium is not necessary for the contraction of mammalian skeletal muscle, the demonstration of inward calcium currents that can be abolished by CCBs in these muscles prompted to re-examine the effect of Verapamil on the neuromuscular transmission. The present study allows us to determine the neuromuscular blockade activity of Verapamil. PMID:24086855

  8. Effects of Pivoting Neuromuscular Training on Pivoting Control and Proprioception

    PubMed Central

    Lee, Song Joo; Ren, Yupeng; Chang, Alison H.; Geiger, François; Zhang, Li-Qun

    2014-01-01

    Purpose Pivoting neuromuscular control and proprioceptive acuity may play an important role in ACL injuries. The goal of this study was to investigate whether pivoting neuromuscular training on an offaxis elliptical trainer (POINT) could improve pivoting neuromuscular control, proprioceptive acuity, and functional performance. Methods Among 41 subjects, 21 subjects participated in 18 sessions of POINT (3 sessions/week for 6 weeks), and 20 subjects served as controls who did their regular workout. Both groups received pre-, mid-, and post-intervention evaluations. Propensity score analysis with multivariable regression adjustment was used to investigate the effect of training on pivoting neuromuscular control (pivoting instability, leg pivoting stiffness, maximum internal and external pivoting angles), proprioceptive acuity, and functional performance in both groups. Results Compared to the control group, the training group significantly improved pivoting neuromuscular control as reduced pivoting instability, reduced maximum internal and external pivoting angles, increased leg pivoting stiffness, and decreased entropy of time to peak EMG in the gluteus maximus and lateral gastrocnemius under pivoting perturbations. Furthermore, the training group enhanced weight-bearing proprioceptive acuity and improved the single leg hop distance. Conclusion Improvement of pivoting neuromuscular control in functional weight-bearing activities and task performances following POINT may help develop lower limb injury prevention and rehabilitation methods to reduce ACL and other musculoskeletal injuries associated with pivoting sports. PMID:24389517

  9. Effects of pivoting neuromuscular training on pivoting control and proprioception.

    PubMed

    Lee, Song Joo; Ren, Yupeng; Chang, Alison H; Geiger, François; Zhang, Li-Qun

    2014-07-01

    Pivoting neuromuscular control and proprioceptive acuity may play an important role in anterior cruciate ligament injuries. The goal of this study was to investigate whether pivoting off-axis intensity adjustable neuromuscular control training (POINT) could improve pivoting neuromuscular control, proprioceptive acuity, and functional performance. Among 41 subjects, 21 subjects participated in 18 sessions of POINT (three sessions per week for 6 wk), and 20 subjects served as controls who did their regular workout. Both groups received pre-, mid-, and postintervention evaluations. Propensity score analysis with multivariable regression adjustment was used to investigate the effect of training on pivoting neuromuscular control (pivoting instability, leg pivoting stiffness, maximum internal, and external pivoting angles), proprioceptive acuity, and functional performance in both groups. Compared with the control group, the training group significantly improved pivoting neuromuscular control as reduced pivoting instability, reduced maximum internal and external pivoting angles, increased leg pivoting stiffness, and decreased entropy of time to peak EMG in the gluteus maximus and lateral gastrocnemius under pivoting perturbations. Furthermore, the training group enhanced weight-bearing proprioceptive acuity and improved the single leg hop distance. Improvement of pivoting neuromuscular control in functional weight-bearing activities and task performances after POINT may help develop lower limb injury prevention and rehabilitation methods to reduce anterior cruciate ligament and other musculoskeletal injuries associated with pivoting sports.

  10. Neuromuscular disease. DOK7 gene therapy benefits mouse models of diseases characterized by defects in the neuromuscular junction.

    PubMed

    Arimura, Sumimasa; Okada, Takashi; Tezuka, Tohru; Chiyo, Tomoko; Kasahara, Yuko; Yoshimura, Toshiro; Motomura, Masakatsu; Yoshida, Nobuaki; Beeson, David; Takeda, Shin'ichi; Yamanashi, Yuji

    2014-09-19

    The neuromuscular junction (NMJ) is the synapse between a motor neuron and skeletal muscle. Defects in NMJ transmission cause muscle weakness, termed myasthenia. The muscle protein Dok-7 is essential for activation of the receptor kinase MuSK, which governs NMJ formation, and DOK7 mutations underlie familial limb-girdle myasthenia (DOK7 myasthenia), a neuromuscular disease characterized by small NMJs. Here, we show in a mouse model of DOK7 myasthenia that therapeutic administration of an adeno-associated virus (AAV) vector encoding the human DOK7 gene resulted in an enlargement of NMJs and substantial increases in muscle strength and life span. When applied to model mice of another neuromuscular disorder, autosomal dominant Emery-Dreifuss muscular dystrophy, DOK7 gene therapy likewise resulted in enlargement of NMJs as well as positive effects on motor activity and life span. These results suggest that therapies aimed at enlarging the NMJ may be useful for a range of neuromuscular disorders.

  11. Neuromuscular Fatigue During 200 M Breaststroke

    PubMed Central

    Conceição, Ana; Silva, António J.; Barbosa, Tiago; Karsai, István; Louro, Hugo

    2014-01-01

    The aims of this study were: i) to analyze activation patterns of four upper limb muscles (duration of the active and non-active phase) in each lap of 200m breaststroke, ii) quantify neuromuscular fatigue, with kinematics and physiologic assessment. Surface electromyogram was collected for the biceps brachii, deltoid anterior, pectoralis major and triceps brachii of nine male swimmers performing a maximal 200m breaststroke trial. Swimming speed, SL, SR, SI decreased from the 1st to the 3rd lap. SR increased on the 4th lap (35.91 ± 2.99 stroke·min-1). Peak blood lactate was 13.02 ± 1.72 mmol·l-1 three minutes after the maximal trial. The EMG average rectified value (ARV) increased at the end of the race for all selected muscles, but the deltoid anterior and pectoralis major in the 1st lap and for biceps brachii, deltoid anterior and triceps brachii in the 4th lap. The mean frequency of the power spectral density (MNF) decreased at the 4th lap for all muscles. These findings suggest the occurrence of fatigue at the beginning of the 2nd lap in the 200m breaststroke trial, characterized by changes in kinematic parameters and selective changes in upper limb muscle action. There was a trend towards a non-linear fatigue state. Key Points Fatigue in the upper limbs occurs in different way as it described by 100m swimming events. Neuromuscular fatigue was estimated by analyzing the physiological changes (high blood lactate concentrations), biomechanical changes in the swimming stroke characteristics (decreased in swimming velocity), and by the changes in the EMG amplitude and frequency parameters at the end of the swimming bout. The amplitude signal of EMG provided by the ARV demonstrated an increase at the end with the respect to the beginning for all muscles under study, excepted for the muscle deltoid anterior. The mean frequency (MNF) in our study decrease at the end of the swimming in the 4th lap relative to the 1st lap for all muscles under observation, along the

  12. Animal models for genetic neuromuscular diseases.

    PubMed

    Vainzof, Mariz; Ayub-Guerrieri, Danielle; Onofre, Paula C G; Martins, Poliana C M; Lopes, Vanessa F; Zilberztajn, Dinorah; Maia, Lucas S; Sell, Karen; Yamamoto, Lydia U

    2008-03-01

    The neuromuscular disorders are a heterogeneous group of genetic diseases, caused by mutations in genes coding sarcolemmal, sarcomeric, and citosolic muscle proteins. Deficiencies or loss of function of these proteins leads to variable degree of progressive loss of motor ability. Several animal models, manifesting phenotypes observed in neuromuscular diseases, have been identified in nature or generated in laboratory. These models generally present physiological alterations observed in human patients and can be used as important tools for genetic, clinic, and histopathological studies. The mdx mouse is the most widely used animal model for Duchenne muscular dystrophy (DMD). Although it is a good genetic and biochemical model, presenting total deficiency of the protein dystrophin in the muscle, this mouse is not useful for clinical trials because of its very mild phenotype. The canine golden retriever MD model represents a more clinically similar model of DMD due to its larger size and significant muscle weakness. Autosomal recessive limb-girdle MD forms models include the SJL/J mice, which develop a spontaneous myopathy resulting from a mutation in the Dysferlin gene, being a model for LGMD2B. For the human sarcoglycanopahties (SG), the BIO14.6 hamster is the spontaneous animal model for delta-SG deficiency, whereas some canine models with deficiency of SG proteins have also been identified. More recently, using the homologous recombination technique in embryonic stem cell, several mouse models have been developed with null mutations in each one of the four SG genes. All sarcoglycan-null animals display a progressive muscular dystrophy of variable severity and share the property of a significant secondary reduction in the expression of the other members of the sarcoglycan subcomplex and other components of the Dystrophin-glycoprotein complex. Mouse models for congenital MD include the dy/dy (dystrophia-muscularis) mouse and the allelic mutant dy(2J)/dy(2J) mouse

  13. Sevoflurane enhances neuromuscular blockade by increasing the sensitivity of skeletal muscle to neuromuscular blockers.

    PubMed

    Ye, Ling; Zuo, Yunxia; Zhang, Peng; Yang, Pingliang

    2015-01-01

    The aim of this study was to investigate the effects of sevoflurane on skeletal muscle contractility. In the first part, twenty-two American Society of Anesthesiology (ASA I-II) female adult patients undergoing elective hysterectomy surgery inhaled sevoflurane 1.0, 1.5 and 2.0 minimum alveolar concentrations (MAC) in succession. Neuromuscular function was assessed at each dose. In the second part, forty-four ASA I-II female adult patients were randomized into four groups: group 1 (propofol + atracurium, sevoflurane 0 MAC), and groups 2 to 4 (atracurium + sevoflurane 1.0, 1.5 and 2.0 MAC, respectively). In group 1, patients were anesthetized by propofol. Then 0.01 mg/kg atracurium was injected into the tested arm intravenously after the arterial blood flow was blocked using a tourniquet. For the other 3 groups, patients inhaled 1.0 MAC, 1.5 MAC, or 2.0 MAC of sevoflurane. Then 0.01 mg/kg atracurium was injected. Neuromuscular function was recorded for the 4 groups. Neuromuscular function was assessed by acceleromyography measurement of evoked responses to train-of four (TOF) stimuli (2 Hz for 2 s applied every 12 s) at the adductor pollicis using a TOF-Guard(TM) neuromuscular transmission monitor. Amplitudes of first response (T1) in each TOF sequence and the ratios of fourth TOF response (T4) to the first were similar at 1.0 MAC, 1.5 MAC, and 2.0 MAC sevoflurane. Compared to baseline, there was no significant change in the TOF value after inhaling 1.0 MAC, 1.5 MAC, or 2.0 MAC sevoflurane. Compared to group 1, there was no significant difference in atracurium onset time (time to reach TOF ratio = 0.25) in group 2 ( 5.6 ± 1.8 min vs. 6.5 ± 1.7 min, P>0.05), or degree of adductor pollicis block (subject number with TOF ratio = 0, 5 vs. 2 subjects, p = 0.3). However, inhaling 1.5 or 2.0 MAC sevoflurane decreased atracurium onset time (4.6 ± 1.5 min and 4.0 ± 1.3 min vs. 6.5 ± 1.7 min, P<0.01 and P<0.001, respectively), and enhanced the block degree (9 and 10 vs. 2

  14. Sevoflurane enhances neuromuscular blockade by increasing the sensitivity of skeletal muscle to neuromuscular blockers

    PubMed Central

    Ye, Ling; Zuo, Yunxia; Zhang, Peng; Yang, Pingliang

    2015-01-01

    The aim of this study was to investigate the effects of sevoflurane on skeletal muscle contractility. In the first part, twenty-two American Society of Anesthesiology (ASA I-II) female adult patients undergoing elective hysterectomy surgery inhaled sevoflurane 1.0, 1.5 and 2.0 minimum alveolar concentrations (MAC) in succession. Neuromuscular function was assessed at each dose. In the second part, forty-four ASA I-II female adult patients were randomized into four groups: group 1 (propofol + atracurium, sevoflurane 0 MAC), and groups 2 to 4 (atracurium + sevoflurane 1.0, 1.5 and 2.0 MAC, respectively). In group 1, patients were anesthetized by propofol. Then 0.01 mg/kg atracurium was injected into the tested arm intravenously after the arterial blood flow was blocked using a tourniquet. For the other 3 groups, patients inhaled 1.0 MAC, 1.5 MAC, or 2.0 MAC of sevoflurane. Then 0.01 mg/kg atracurium was injected. Neuromuscular function was recorded for the 4 groups. Neuromuscular function was assessed by acceleromyography measurement of evoked responses to train-of four (TOF) stimuli (2 Hz for 2 s applied every 12 s) at the adductor pollicis using a TOF-GuardTM neuromuscular transmission monitor. Amplitudes of first response (T1) in each TOF sequence and the ratios of fourth TOF response (T4) to the first were similar at 1.0 MAC, 1.5 MAC, and 2.0 MAC sevoflurane. Compared to baseline, there was no significant change in the TOF value after inhaling 1.0 MAC, 1.5 MAC, or 2.0 MAC sevoflurane. Compared to group 1, there was no significant difference in atracurium onset time (time to reach TOF ratio = 0.25) in group 2 ( 5.6 ± 1.8 min vs. 6.5 ± 1.7 min, P>0.05), or degree of adductor pollicis block (subject number with TOF ratio = 0, 5 vs. 2 subjects, p = 0.3). However, inhaling 1.5 or 2.0 MAC sevoflurane decreased atracurium onset time (4.6 ± 1.5 min and 4.0 ± 1.3 min vs. 6.5 ± 1.7 min, P<0.01 and P<0.001, respectively), and enhanced the block degree (9 and 10 vs. 2

  15. Electrophysiology of autonomic neuromuscular transmission involving ATP.

    PubMed

    Sneddon, P

    2000-07-03

    Electrophysiological investigations of autonomic neuromuscular transmission have provided great insights into the role of ATP as a neurotransmitter. Burnstock and Holman made the first recordings of excitatory junction potentials (e.j.p.s) produced by sympathetic nerves innervating the smooth muscle of the guinea-pig vas deferens. This led to the identification of ATP as the mediator of e.j.p.s in this tissue, where ATP acts as a cotransmitter with noradrenaline. The e.j.p.s are mediated solely by ATP acting on P2X(1) receptors leading to action potentials and a rapid phasic contraction, whilst noradrenaline mediates a slower, tonic contraction which is not dependent on membrane depolarisation. Subsequent electrophysiological studies of the autonomic innervation of smooth muscles of the urogenital, gastrointestinal and cardiovascular systems have revealed a similar pattern of response, where ATP mediates a fast electrical and mechanical response, whilst another transmitter such as noradrenaline, acetylcholine, nitric oxide or a peptide mediates a slower response. The modulation of junction potentials by a variety of pre-junctional receptors and the mechanism of inactivation of ATP as a neurotransmitter will also be described.

  16. Assessment of Motor Units in Neuromuscular Disease.

    PubMed

    Henderson, Robert D; McCombe, Pamela A

    2017-01-01

    The motor unit comprises the anterior horn cell, its axon, and the muscle fibers that it innervates. Although the true number of motor units is unknown, the number of motor units appears to vary greatly between different muscles and between different individuals. Assessment of the number and function of motor units is needed in diseases of the anterior horn cell and other motor nerve disorders. Amyotrophic lateral sclerosis is the most important disease of anterior horn cells. The need for an effective biomarker for assessing disease progression and for use in clinical trials in amyotrophic lateral sclerosis has stimulated the study of methods to measure the number of motor units. Since 1970 a number of different methods, including the incremental, F-wave, multipoint, and statistical methods, have been developed but none has achieved widespread applicability. Two methods (MUNIX and the multipoint incremental method) are in current use across multiple centres and are discussed in detail in this review, together with other recently published methods. Imaging with magnetic resonance and ultrasound is increasingly being applied to this area. Motor unit number estimates have also been applied to other neuromuscular diseases such as spinal muscular atrophy, compression neuropathies, and prior poliomyelitis. The need for an objective measure for the assessment of motor units remains tantalizingly close but unfulfilled in 2016.

  17. Stem cell route to neuromuscular therapies.

    PubMed

    Partridge, Terence A

    2003-02-01

    As applied to skeletal muscle, stem cell therapy is a reincarnation of myoblast transfer therapy that has resulted from recent advances in the cell biology of skeletal muscle. Both strategies envisage the reconstruction of damaged muscle from its precursors, but stem cell therapy employs precursors that are earlier in the developmental hierarchy. It is founded on demonstrations of apparently multipotential cells in a wide variety of tissues that can assume, among others, a myogenic phenotype. The main demonstrated advantage of such cells is that they are capable of colonizing many tissues, including skeletal and cardiac muscle via the blood vascular system, thereby providing the potential for a body-wide distribution of myogenic progenitors. From a practical viewpoint, the chief disadvantage is that such colonization has been many orders of magnitude too inefficient to be useful. Proposals for overcoming this drawback are the subject of much speculation but, so far, relatively little experimentation. This review attempts to give some perspective to the status of the stem cell as a therapeutic instrument for neuromuscular disease and to identify issues that need to be addressed for application of this technology.

  18. Scapholunate Instability: Proprioception and Neuromuscular Control

    PubMed Central

    Salva-Coll, Guillem; Garcia-Elias, Marc; Hagert, Elisabet

    2013-01-01

    From a kinetic point of view, the wrist is considered stable when it is capable of resisting load without suffering injury. Several prerequisites are necessary for the wrist to be kinetically stable: bone morphology, normal articulating surfaces, ligaments, the sensorimotor system, the muscles crossing the wrist, and all nerves connecting to ligaments and muscles. Failure of any one of these factors may result in carpal instability. The terms “scapholunate (SL) dissociation” and “SL instability” refer to one of the most frequent types of wrist instability, resulting from rupture or attenuation of the SL supporting ligaments. From a radiologic point of view, SL instability may be dynamic or static. Unlike static instabilities, which tend to be painful and dysfunctional, a good proportion of dynamic SL instabilities remain asymptomatic and stable for prolonged periods of time. Such a lack of symptoms suggests that a ligament rupture, in itself, is not enough for a joint to become unstable. Certainly, the process of achieving stability is multifactorial and involves normal joint surfaces, ligaments, muscles, and a complex network of neural connections linking all these elements. In this article, we will review the neuromuscular stabilization of the SL joint and the proprioceptive mechanisms that contribute to the dynamic carpal stabilization. PMID:24436806

  19. [Neuromuscular biopsy and diagnosis of vasculitis].

    PubMed

    Vital, Anne; Vital, Claude

    2006-09-01

    One characteristic histological lesion on biopsy specimens is mandatory to establish the diagnosis of vasculitis. Combined nerve and muscle biopsies, by the same cutaneous incision, improve significantly the percentage of positive results. Nerve fragments should be taken in every patient presenting sensory manifestations. Such vasculitic lesions are present in medium-sized arterioles and/or small vessels, and correspond mainly to 4 necrotizing vasculitis: panarteritis nodosa (PAN), microscopic polyangiitis (MPA), Churg and Strauss syndrome and Wegener granulomatosis. Microvasculitis should be added to these classical entities, because it corresponds to small vessel wall infiltration by inflammatory cells, as observed in PAN and MPA, but without any necrosis. Microvasculitis has to be differentiated from the inflammatory cell infiltrates surrounding small vessels. However, such perivascular inflammatory cell infiltrates enable the diagnosis of probable vasculitis when associated with clusters of neo-vessels, hemosiderin deposits, or a focal damage of nerve fibers. Grossly, one third of vasculitis diagnosis is confirmed on muscle fragments, a second third on nerve fragments, and the last third on both nerve and muscle fragments. Moreover, in the search for vasculitis, an unpredicted diagnosis of lymphoma or amyloidosis is occasionally established on the neuro-muscular biopsy.

  20. Neuromuscular dentistry: Occlusal diseases and posture

    PubMed Central

    Khan, Mohd Toseef; Verma, Sanjeev Kumar; Maheshwari, Sandhya; Zahid, Syed Naved; Chaudhary, Prabhat K.

    2013-01-01

    Neuromuscular dentistry has been a controversial topic in the field of dentistry and still remains debatable. The issue of good occlusion and sound health has been repeatedly discussed. Sometimes we get complains of sensitive teeth and sometimes of tired facial muscles on getting up in the morning. Owing to the intimate relation of masticatory apparatus with the cranium and cervico-scapular muscular system, the disorders in any system, draw attention from concerned clinicians involved in management, to develop an integrated treatment protocol for the suffering patients. There may be patients reporting to the dental clinics after an occlusal restoration or extraction, having pain in or around the temporomandibular joint, headache or neck pain. Although their esthetic demands must not be undermined during the course of treatment plan, whenever dental treatment of any sort is planned, occlusion/bite should be given prime importance. Very few dentist are able to diagnose the occlusal disease and of those who diagnose many people resort to aggressive treatment modalities. This paper aims to report the signs of occlusal disease, and discuss their association with TMDs and posture. PMID:25737904

  1. Silent synapses in neuromuscular junction development.

    PubMed

    Tomàs, Josep; Santafé, Manel M; Lanuza, Maria A; García, Neus; Besalduch, Nuria; Tomàs, Marta

    2011-01-01

    In the last few years, evidence has been found to suggest that some synaptic contacts become silent but can be functionally recruited before they completely retract during postnatal synapse elimination in muscle. The physiological mechanism of developmental synapse elimination may be better understood by studying this synapse recruitment. This Mini-Review collects previously published data and new results to propose a molecular mechanism for axonal disconnection. The mechanism is based on protein kinase C (PKC)-dependent inhibition of acetylcholine (ACh) release. PKC activity may be stimulated by a methoctramine-sensitive M2-type muscarinic receptor and by calcium inflow though P/Q- and L-type voltage-dependent calcium channels. In addition, tropomyosin-related tyrosine kinase B (trkB) receptor-mediated brain-derived neurotrophic factor (BDNF) activity may oppose the PKC-mediated ACh release depression. Thus, a balance between trkB and muscarinic pathways may contribute to the final functional suppression of some neuromuscular synapses during development. © 2010 Wiley-Liss, Inc.

  2. Myasthenia gravis and the neuromuscular junction.

    PubMed

    Querol, Luis; Illa, Isabel

    2013-10-01

    Myasthenic disorders are a well characterized group of diseases of the neuromuscular junction. Their pathogenesis is diverse, including genetic and autoimmune mechanisms. We review recent findings on risk factors, pathogenesis and treatment of autoimmune myasthenia gravis. Better knowledge of congenital myasthenia has led to the development of efficient diagnostic algorithms that have therapeutic implications. New epidemiological and genetic risk factors have been identified and are considered to play a role in the development of myasthenia gravis. The study of the role of innate immunity in myasthenia gravis has identified relevant pathways to explain myasthenia gravis causes. The description of the pathogenic role of IgG4 anti-MuSK antibodies has revealed heterogeneous immune mechanisms that should lead to more specific therapies. Rituximab seems to be particularly effective in MuSK myasthenia gravis, and eculizumab arises as an option in refractory AChR myasthenia gravis. Therapeutic algorithms need to be tailored to each myasthenia subtype. Increasing knowledge about the environmental and genetic risk factors and basic immunopathogenesis of myasthenia gravis, including the role of innate immunity, regulatory T cell impairment and autoantibody heterogeneity, is providing a rationale for treatment with new biological agents. Current immunotherapies in myasthenia gravis undoubtedly provide benefits, but also cause side-effects. Controlled trials are, therefore, needed to confirm initial results from pilot studies.

  3. [Treatment of oropharyngeal dysphagia with neuromuscular electrostimulaiton].

    PubMed

    Terré, Rosa; Martinell, Montse; González, Beatriz; Ejarque, Judith; Mearin, Fermín

    2013-02-16

    Oropharyngeal dysphagia is highly prevalent in stroke patients, with a high mortality and morbidity. Neuromuscular electrostimulation (NMES) is a new and potentially useful therapy. We assessed the therapeutic effectiveness and safety of NMES in the treatment of oropharyngeal dysphagia in patients with stroke. Prospective study of stroke patients, with tracheal aspiration revealed by videofluoroscopy (VDF), who underwent conventional therapy of oropharyngeal dysphagia as well as NMES. We did a follow-up at the end of the treatment and 3 months later. Patients underwent 18 courses of treatment (15-20) with a mean stimulation intensity of 12.45mA (range: 6.6-16.7mA). Before starting therapy, 8 patients were fed exclusively by means of a gastrostomy tube; after the treatment, only one patient needed it. No patient had complications. VDF parameters before treatment: oral transition time, swallowing reflex shooting (SRS) and pharyngeal transition time were longer and the hyoid elevation was reduced. After the treatment, the number of patients with tracheal aspiration decreased to 6, but only in 6 it persisted for the three analyzed viscosities (liquid, nectar and pudding), with an overall improvement of the VDF parameters. The only predictive factor of therapeutic effectiveness was the delay in the SRS. NMES is an effective and safe treatment for stroke patients with oropharyngeal dysphagia. Copyright © 2012 Elsevier España, S.L. All rights reserved.

  4. Pulmonary Rehabilitation in Patients with Neuromuscular Disease

    PubMed Central

    2006-01-01

    In neuromuscular disease (NMD) patients with progressive muscle weakness, respiratory muscles are also affected and hypercapnia can increase gradually as the disease progresses. The fundamental respiratory problems NMD patients experience are decreased alveolar ventilation and coughing ability. For these reasons, it is necessary to precisely evaluate pulmonary function to provide the proper inspiratory and expiratory muscle aids in order to maintain adequate respiratory function. As inspiratory muscle weakening progresses, NMD patients experience hypoventilation. At this point, respiratory support by mechanical ventilator should be initiated to relieve respiratory distress symptoms. Patients with adequate bulbar muscle strength and cognitive function who use a non-invasive ventilation aid, via a mouthpiece or a nasal mask, may have their hypercapnia and associated symptoms resolved. For a proper cough assist, it is necessary to provide additional insufflation to patients with inspiratory muscle weakness before using abdominal thrust. Another effective method for managing airway secretions is a device that performs mechanical insufflation-exsufflation. In conclusion, application of non-invasive respiratory aids, taking into consideration characterization of respiratory pathophysiology, have made it possible to maintain a better quality of life in addition to prolonging the life span of patients with NMD. PMID:16807978

  5. Vaccination recommendations for patients with neuromuscular disease.

    PubMed

    Esposito, Susanna; Bruno, Claudio; Berardinelli, Angela; Filosto, Massimiliano; Mongini, Tiziana; Morandi, Lucia; Musumeci, Olimpia; Pegoraro, Elena; Siciliano, Gabriele; Tonin, Paola; Marrosu, Gianni; Minetti, Carlo; Servida, Maura; Fiorillo, Chiara; Conforti, Giorgio; Scapolan, Silvia; Ansaldi, Filippo; Vianello, Andrea; Castaldi, Silvana; Principi, Nicola; Toscano, Antonio; Moggio, Maurizio

    2014-10-14

    Neuromuscular diseases (NMDs) encompass a broad spectrum of conditions. Because infections may be relevant to the final prognosis of most NMDs, vaccination appears to be the simplest and most effective solution for protecting NMD patients from vaccine-preventable infections. However, very few studies have evaluated the immunogenicity, safety, tolerability, and efficacy of different vaccines in NMD patients; therefore, detailed vaccination recommendations for NMD patients are not available. Here, we present vaccination recommendations from a group of Italian Scientific Societies for optimal disease prevention in NMD patients that maintain high safety levels. We found that NMD patients can be classified into two groups according to immune function: patients with normal immunity and patients who are immunocompromised, including those who intermittently or continuously take immunosuppressive therapy. Patients with normal immunity and do not take immunosuppressive therapy can be vaccinated as healthy subjects. In contrast, immunocompromised patients, including those who take immunosuppressive therapy, should receive all inactivated vaccines as well as influenza and pneumococcal vaccines; these patients should not be administered live attenuated vaccines. In all cases, the efficacy and long-term persistence of immunity from vaccination in NMD patients can be lower than in normal subjects. Household contacts of immunocompromised NMD patients should also be vaccinated appropriately. Copyright © 2014 Elsevier Ltd. All rights reserved.

  6. Neuromuscular dentistry: Occlusal diseases and posture.

    PubMed

    Khan, Mohd Toseef; Verma, Sanjeev Kumar; Maheshwari, Sandhya; Zahid, Syed Naved; Chaudhary, Prabhat K

    2013-01-01

    Neuromuscular dentistry has been a controversial topic in the field of dentistry and still remains debatable. The issue of good occlusion and sound health has been repeatedly discussed. Sometimes we get complains of sensitive teeth and sometimes of tired facial muscles on getting up in the morning. Owing to the intimate relation of masticatory apparatus with the cranium and cervico-scapular muscular system, the disorders in any system, draw attention from concerned clinicians involved in management, to develop an integrated treatment protocol for the suffering patients. There may be patients reporting to the dental clinics after an occlusal restoration or extraction, having pain in or around the temporomandibular joint, headache or neck pain. Although their esthetic demands must not be undermined during the course of treatment plan, whenever dental treatment of any sort is planned, occlusion/bite should be given prime importance. Very few dentist are able to diagnose the occlusal disease and of those who diagnose many people resort to aggressive treatment modalities. This paper aims to report the signs of occlusal disease, and discuss their association with TMDs and posture.

  7. Pulmonary rehabilitation in patients with neuromuscular disease.

    PubMed

    Kang, Seong-Woong

    2006-06-30

    In neuromuscular disease (NMD) patients with progressive muscle weakness, respiratory muscles are also affected and hypercapnia can increase gradually as the disease progresses. The fundamental respiratory problems NMD patients experience are decreased alveolar ventilation and coughing ability. For these reasons, it is necessary to precisely evaluate pulmonary function to provide the proper inspiratory and expiratory muscle aids in order to maintain adequate respiratory function. As inspiratory muscle weakening progresses, NMD patients experience hypoventilation. At this point, respiratory support by mechanical ventilator should be initiated to relieve respiratory distress symptoms. Patients with adequate bulbar muscle strength and cognitive function who use a non-invasive ventilation aid, via a mouthpiece or a nasal mask, may have their hypercapnia and associated symptoms resolved. For a proper cough assist, it is necessary to provide additional insufflation to patients with inspiratory muscle weakness before using abdominal thrust. Another effective method for managing airway secretions is a device that performs mechanical insufflation-exsufflation. In conclusion, application of non-invasive respiratory aids, taking into consideration characterization of respiratory pathophysiology, have made it possible to maintain a better quality of life in addition to prolonging the life span of patients with NMD.

  8. Lumbopelvic flexibility modulates neuromuscular responses during trunk flexion-extension.

    PubMed

    Sánchez-Zuriaga, Daniel; Artacho-Pérez, Carla; Biviá-Roig, Gemma

    2016-06-01

    Various stimuli such as the flexibility of lumbopelvic structures influence the neuromuscular responses of the trunk musculature, leading to different load sharing strategies and reflex muscle responses from the afferents of lumbopelvic mechanoreceptors. This link between flexibility and neuromuscular response has been poorly studied. The aim of this study was to investigate the relationship between lumbopelvic flexibility and neuromuscular responses of the erector spinae, hamstring and abdominal muscles during trunk flexion-extension. Lumbopelvic movement patterns were measured in 29 healthy women, who were separated into two groups according to their flexibility during trunk flexion-extension. The electromyographic responses of erector spinae, rectus abdominis and biceps femoris were also recorded. Subjects with greater lumbar flexibility had significantly less pelvic flexibility and vice versa. Subjects with greater pelvic flexibility had a higher rate of relaxation and lower levels of hamstring activation during maximal trunk flexion. The neuromuscular response patterns of the hamstrings seem partially modulated by pelvic flexibility. Not so with the lumbar erector spinae and lumbar flexibility, despite the assertions of some previous studies. The results of this study improve our knowledge of the relationships between trunk joint flexibility and neuromuscular responses, a relationship which may play a role in low back pain. Copyright © 2016 Elsevier Ltd. All rights reserved.

  9. Effects of melatonin on gallbladder neuromuscular function in acute cholecystitis.

    PubMed

    Gomez-Pinilla, Pedro J; Camello, Pedro J; Pozo, María J

    2007-10-01

    Gallbladder stasis is associated to experimental acute cholecystitis. Impaired contractility could be, at least in part, the result of inflammation-induced alterations in the neuromuscular function. This study was designed to determine the changes in gallbladder neurotransmission evoked by acute inflammation and to evaluate the protective and therapeutic effects of melatonin. Experimental acute cholecystitis was induced in guinea pigs by common bile duct ligation for 2 days, and then the neuromuscular function was evaluated using electrical field stimulation (EFS; 5-40 Hz). In a group of animals with the bile duct ligated for 2 days, a deligation of the duct was performed, and after 2 days, the neuromuscular function was studied. The EFS-evoked isometric gallbladder contraction was significantly lower in cholecystitic tissue. In addition, inflammation changed the pharmacological profile of these contractions that were insensitive to tetrodotoxin but sensitive to atropine and omega-conotoxin, indicating that acute cholecystitis affects action potential propagation in the intrinsic nerves. Nitric oxide (NO)-mediated neurotransmission was reduced by inflammation, which also increased the reactivity of sensitive fibers. Melatonin treatment prevented qualitative changes in gallbladder neurotransmission, but it did not improve EFS-induced contractility. The hormone recovered gallbladder neuromuscular function once the biliary obstruction was resolved, even when the treatment was started after the onset of gallbladder inflammation. These findings show for the first time the therapeutic potential of melatonin in the recovery of gallbladder neuromuscular function during acute cholecystitis.

  10. Drosophila Nesprin-1 controls glutamate receptor density at neuromuscular junctions.

    PubMed

    Morel, Véronique; Lepicard, Simon; Rey, Alexandre N; Parmentier, Marie-Laure; Schaeffer, Laurent

    2014-09-01

    Nesprin-1 is a core component of a protein complex connecting nuclei to cytoskeleton termed LINC (linker of nucleoskeleton and cytoskeleton). Nesprin-1 is anchored to the nuclear envelope by its C-terminal KASH domain, the disruption of which has been associated with neuronal and neuromuscular pathologies, including autosomal recessive cerebellar ataxia and Emery-Dreifuss muscular dystrophy. Here, we describe a new and unexpected role of Drosophila Nesprin-1, Msp-300, in neuromuscular junction. We show that larvae carrying a deletion of Msp-300 KASH domain (Msp-300 (∆KASH) ) present a locomotion defect suggestive of a myasthenia, and demonstrate the importance of muscle Msp-300 for this phenotype, using tissue-specific RNAi knock-down. We show that Msp-300 (∆KASH) mutants display abnormal neurotransmission at the larval neuromuscular junction, as well as an imbalance in postsynaptic glutamate receptor composition with a decreased percentage of GluRIIA-containing receptors. We could rescue Msp-300 (∆KASH) locomotion phenotypes by GluRIIA overexpression, suggesting that the locomotion impairment associated with the KASH domain deletion is due to a reduction in junctional GluRIIA. In summary, we found that Msp-300 controls GluRIIA density at the neuromuscular junction. Our results suggest that Drosophila is a valuable model for further deciphering how Nesprin-1 and LINC disruption may lead to neuronal and neuromuscular pathologies.

  11. N-glycosylation requirements in neuromuscular synaptogenesis.

    PubMed

    Parkinson, William; Dear, Mary Lynn; Rushton, Emma; Broadie, Kendal

    2013-12-01

    Neural development requires N-glycosylation regulation of intercellular signaling, but the requirements in synaptogenesis have not been well tested. All complex and hybrid N-glycosylation requires MGAT1 (UDP-GlcNAc:α-3-D-mannoside-β1,2-N-acetylglucosaminyl-transferase I) function, and Mgat1 nulls are the most compromised N-glycosylation condition that survive long enough to permit synaptogenesis studies. At the Drosophila neuromuscular junction (NMJ), Mgat1 mutants display selective loss of lectin-defined carbohydrates in the extracellular synaptomatrix, and an accompanying accumulation of the secreted endogenous Mind the gap (MTG) lectin, a key synaptogenesis regulator. Null Mgat1 mutants exhibit strongly overelaborated synaptic structural development, consistent with inhibitory roles for complex/hybrid N-glycans in morphological synaptogenesis, and strengthened functional synapse differentiation, consistent with synaptogenic MTG functions. Synapse molecular composition is surprisingly selectively altered, with decreases in presynaptic active zone Bruchpilot (BRP) and postsynaptic Glutamate receptor subtype B (GLURIIB), but no detectable change in a wide range of other synaptic components. Synaptogenesis is driven by bidirectional trans-synaptic signals that traverse the glycan-rich synaptomatrix, and Mgat1 mutation disrupts both anterograde and retrograde signals, consistent with MTG regulation of trans-synaptic signaling. Downstream of intercellular signaling, pre- and postsynaptic scaffolds are recruited to drive synaptogenesis, and Mgat1 mutants exhibit loss of both classic Discs large 1 (DLG1) and newly defined Lethal (2) giant larvae [L(2)GL] scaffolds. We conclude that MGAT1-dependent N-glycosylation shapes the synaptomatrix carbohydrate environment and endogenous lectin localization within this domain, to modulate retention of trans-synaptic signaling ligands driving synaptic scaffold recruitment during synaptogenesis.

  12. Homeostatic plasticity at the Drosophila neuromuscular junction.

    PubMed

    Frank, C Andrew

    2014-03-01

    In biology, homeostasis refers to how cells maintain appropriate levels of activity. This concept underlies a balancing act in the nervous system. Synapses require flexibility (i.e. plasticity) to adjust to environmental challenges. Yet there must also exist regulatory mechanisms that constrain activity within appropriate physiological ranges. An abundance of evidence suggests that homeostatic regulation is critical in this regard. In recent years, important progress has been made toward identifying molecules and signaling processes required for homeostatic forms of neuroplasticity. The Drosophila melanogaster third instar larval neuromuscular junction (NMJ) has been an important experimental system in this effort. Drosophila neuroscientists combine genetics, pharmacology, electrophysiology, imaging, and a variety of molecular techniques to understand how homeostatic signaling mechanisms take shape at the synapse. At the NMJ, homeostatic signaling mechanisms couple retrograde (muscle-to-nerve) signaling with changes in presynaptic calcium influx, changes in the dynamics of the readily releasable vesicle pool, and ultimately, changes in presynaptic neurotransmitter release. Roles in these processes have been demonstrated for several molecules and signaling systems discussed here. This review focuses primarily on electrophysiological studies or data. In particular, attention is devoted to understanding what happens when NMJ function is challenged (usually through glutamate receptor inhibition) and the resulting homeostatic responses. A significant area of study not covered in this review, for the sake of simplicity, is the homeostatic control of synapse growth, which naturally, could also impinge upon synapse function in myriad ways. This article is part of the Special Issue entitled 'Homeostatic Synaptic Plasticity'. Copyright © 2013 Elsevier Ltd. All rights reserved.

  13. Neuromuscular transmission in the athymic nude mouse.

    PubMed

    Schofield, G G; Marshall, I G

    1980-10-01

    No major differences were observed in the mechanical properties of diaphragm, extensor digitorum longus and soleus muscles from athymic nude and control mice. Denervated soleus muscles from nudes and controls showed no significant differences in their sensitivities to the cholinoceptor agonists acetylcholine and carbachol, either in the absence or presence of the anticholinesterase, physostigmine, suggesting that postjunctional receptor function is essentially normal. Phrenic nerve-diaphragm preparations from nudes were less sensitive to the twitch-augmenting effects of neostigmine. No difference in the time course of endplate potentials (epps) between nudes and controls was seen either in the absence or presence of neostigmine. Hence the observed differences in twitch augmentation are unlikely to be due to differences in acetylcholinesterase activity in the two muscles. In normal mice miniature endplate potential (mepp) amplitude decreased and mepp frequency increased with age. These changes were associated with an increase in muscle fibre diameter and a concomitant decrease in membrane resistance. Such changes did not occur in nude mice; thus mepp amplitude remained, high as in young normal muscle. It is suggested that the thymus may play a role in muscle development and that the effects on neuromuscular transmission are secondary to changes in development. In cut diaphragm muscles transmitter reversal potentials in nudes and controls were not different. Although there was no difference in the amplitude of the first epp of a train, or in the immediately releasable acetylcholine store, the quantal content of the first epp, the probability of transmitter release, the total nerve terminal acetylcholine store and the transmitter mobilization rate were all reduced. It is considered probable that all the measurable differences in transmitter release can be explained in terms of the nude muscle fibre diameter being small and being associated with a small nerve terminal

  14. Pain in Youths With Neuromuscular Disease

    PubMed Central

    Engel, Joyce M.; Kartin, Deborah; Carter, Gregory T.; Jensen, Mark P.; Jaffe, Kenneth M.

    2011-01-01

    To examine the prevalence and characteristics of pain in children with neuromuscular disease (NMD), 42 youths with NMD underwent a comprehensive evaluation including a detailed intake interview and structured questionnaire that included demographic and functional data. Youths who reported chronic pain were further queried about pain characteristics, locations, and intensity using an 11-point numerical rating scale and a modified Brief Pain Inventory (BPI). The sample consisted of 24 males (57%) and 18 females (43%), ages ranging from 9 to 20 years (M = 14.8, SD = 2.96). Participants included 14 (37%) with Duchenne-muscular dystrophy, 6 (14%) with myotonic dystrophy, 2 (5%) with Becker dystrophy, 2 (5%) with limb-girdle dystrophy, 2 (5%) with congenital muscular dystrophy, 1 (2%) facioscapulohumeral, and 15 (36%) were classified as “other NMD.” Twenty-one (50%) were ambulatory; 26 (62%) used power wheelchairs/scooters, 9 (2%) used manual wheelchairs, 3 (.07%) used crutches/canes, and 1 (2%) used a walker. A total of 23 (55%) of the youths reported having chronic pain. Current pain intensity was 1.30(range=0–6), mean pain intensity over the past week was 2.39 (range = 0–7), mean pain duration was 8.75 hours (SD=12.84). Pain in the legs was most commonly reported and 83% reported using pain medications. This study indicates that chronic pain is a significant problem in youths with NMD. These data strongly support making comprehensive pain assessment and management an integral part of the standard of care for youths with NMD. PMID:19820205

  15. Single Fiber Electromyographic Jitter to Detect Acute Changes in Neuromuscular Function in Young and Adult Rats

    EPA Science Inventory

    Introduction: Exposure to irreversible cholinesterase (ChE)-inhibiting compounds, such as organophosphates may produce neuromuscular dysfunction. However, less is known about changes in neuromuscular transmission after treatment with reversible ChE-inhibitors. These studies adapt...

  16. Single Fiber Electromyographic Jitter to Detect Acute Changes in Neuromuscular Function in Young and Adult Rats

    EPA Science Inventory

    Introduction: Exposure to irreversible cholinesterase (ChE)-inhibiting compounds, such as organophosphates may produce neuromuscular dysfunction. However, less is known about changes in neuromuscular transmission after treatment with reversible ChE-inhibitors. These studies adapt...

  17. Perceived pain and temporomandibular disorders in neuromuscular diseases.

    PubMed

    Fischer, Michael J; Riedlinger, Kathrin; Schoser, Benedikt; Bernateck, Michael

    2009-10-01

    Little is known about pain associated with temporomandibular disorders (TMD) in neuromuscular diseases. Inpatients (N = 134) with neuromuscular disorder diagnoses were given questionnaires to estimate pain localization and intensity. Research Diagnostic Criteria for Temporomandibular Disorders and the Temporomandibular Index (TMI) were utilized to assess TMD. Pain was reported by 116 patients (86%). Legs (52%) and arms (33%) were the most common locations for pain localization, but the highest Pearson correlations (TMI vs. perceived pain) appeared for pain located in the trunk and arms (0.861, P < 0.01). No correlation between TMI and diagnosis group existed except for "acquired myopathy" and "miscellaneous neuromuscular diseases." These results suggest that the degree of TMD does not correlate with pain according to disease, although common mechanisms might be responsible for pain development in specific body regions connected with TMD. Most important, higher levels of TMD are associated with higher levels of perceived pain.

  18. Neuromuscular control and rehabilitation of the unstable ankle.

    PubMed

    Hung, You-Jou

    2015-06-18

    Lateral ankle sprain is a common orthopedic injury with a very high recurrence rate in athletes. After decades of research, it is still unclear what contributes to the high recurrence rate of ankle sprain, and what is the most effective intervention to reduce the incident of initial and recurrent injuries. In addition, clinicians often implement balance training as part of the rehabilitation protocol in hopes of enhancing the neuromuscular control and proprioception of the ankle joint. However, there is no consensus on whether the neuromuscular control and proprioception are compromised in unstable ankles. To reduce the prevalence of ankle sprains, the effectiveness of engaging balance training to enhance the neuromuscular control and proprioception of the ankle joint is also questionable.

  19. Neuromuscular control and rehabilitation of the unstable ankle

    PubMed Central

    Hung, You-jou

    2015-01-01

    Lateral ankle sprain is a common orthopedic injury with a very high recurrence rate in athletes. After decades of research, it is still unclear what contributes to the high recurrence rate of ankle sprain, and what is the most effective intervention to reduce the incident of initial and recurrent injuries. In addition, clinicians often implement balance training as part of the rehabilitation protocol in hopes of enhancing the neuromuscular control and proprioception of the ankle joint. However, there is no consensus on whether the neuromuscular control and proprioception are compromised in unstable ankles. To reduce the prevalence of ankle sprains, the effectiveness of engaging balance training to enhance the neuromuscular control and proprioception of the ankle joint is also questionable. PMID:26085985

  20. Gastrointestinal neuromuscular apparatus: An underestimated target of gut microbiota

    PubMed Central

    Guarino, Michele Pier Luca; Cicala, Michele; Putignani, Lorenza; Severi, Carola

    2016-01-01

    Over the last few years, the importance of the resident intestinal microbiota in the pathogenesis of several gastro-intestinal diseases has been largely investigated. Growing evidence suggest that microbiota can influence gastro-intestinal motility. The current working hypothesis is that dysbiosis-driven mucosal alterations induce the production of several inflammatory/immune mediators which affect gut neuro-muscular functions. Besides these indirect mucosal-mediated effects, the present review highlights that recent evidence suggests that microbiota can directly affect enteric nerves and smooth muscle cells functions through its metabolic products or bacterial molecular components translocated from the intestinal lumen. Toll-like receptors, the bacterial recognition receptors, are expressed both on enteric nerves and smooth muscle and are emerging as potential mediators between microbiota and the enteric neuromuscular apparatus. Furthermore, the ongoing studies on probiotics support the hypothesis that the neuromuscular apparatus may represent a target of intervention, thus opening new physiopathological and therapeutic scenarios. PMID:28018095

  1. Neuromuscular problems in the burn patient: cause and prevention.

    PubMed

    Helm, P A; Pandian, G; Heck, E

    1985-07-01

    Neuromuscular complications of the burn patient that occur during hospitalization frequently are overlooked. Eighty-eight patients with signs of weakness or complaints of easy fatigability were examined clinically and electrodiagnostically. The most frequently diagnosed neuromuscular abnormality in this study was generalized peripheral neuropathy. Other specific neuromuscular problems, in order of frequency of occurrence, were found to involve the deltoid muscle, peroneal nerve, ulnar nerve, median nerve, and brachial plexus. These lesions are found to be commonly due to (1) poor positioning, both in bed and in the operating room, and (2) heavy bulky dressings over superficial nerves. Thus, it is concluded that many of these injuries offer a significant potential for prevention through attention to physiologic positioning and meticulous patient care.

  2. Confocal Microendoscopy of Neuromuscular Synapses in Living Mice.

    PubMed

    Blanco, Gonzalo; Ribchester, Richard R

    2012-03-01

    Here we describe a step-by-step method for vital imaging of neuromuscular junctions (NMJ) and axons using fiber-optic confocal microendoscopy (CME). A commercially available system, the Cellvizio Lab, can be applied to transgenic mouse lines expressing yellow fluorescent protein in all or pseudorandom sub-subsets of motor neurons. Microscopic imaging in vivo is achieved by means of a flexible optical fiber probe that excites and collects the emitted light from fluorescently labeled structures. The hand-held probe is introduced through small skin incisions to visualize nerves and neuromuscular junctions from superficial muscles. Interpolation software then reconstructs the images in real time. The images are of sufficient quality to permit screening of axonal and neuromuscular synaptic integrity and other aspects of their phenotype in live animals. Curr. Protoc. Mouse Biol. 2:1-8 © 2012 by John Wiley & Sons, Inc. Copyright © 2012 John Wiley & Sons, Inc.

  3. Hexosamine Biosynthetic Pathway Mutations Cause Neuromuscular Transmission Defect

    PubMed Central

    Senderek, Jan; Müller, Juliane S.; Dusl, Marina; Strom, Tim M.; Guergueltcheva, Velina; Diepolder, Irmgard; Laval, Steven H.; Maxwell, Susan; Cossins, Judy; Krause, Sabine; Muelas, Nuria; Vilchez, Juan J.; Colomer, Jaume; Mallebrera, Cecilia Jimenez; Nascimento, Andres; Nafissi, Shahriar; Kariminejad, Ariana; Nilipour, Yalda; Bozorgmehr, Bita; Najmabadi, Hossein; Rodolico, Carmelo; Sieb, Jörn P.; Steinlein, Ortrud K.; Schlotter, Beate; Schoser, Benedikt; Kirschner, Janbernd; Herrmann, Ralf; Voit, Thomas; Oldfors, Anders; Lindbergh, Christopher; Urtizberea, Andoni; von der Hagen, Maja; Hübner, Angela; Palace, Jacqueline; Bushby, Kate; Straub, Volker; Beeson, David; Abicht, Angela; Lochmüller, Hanns

    2011-01-01

    Neuromuscular junctions (NMJs) are synapses that transmit impulses from motor neurons to skeletal muscle fibers leading to muscle contraction. Study of hereditary disorders of neuromuscular transmission, termed congenital myasthenic syndromes (CMS), has helped elucidate fundamental processes influencing development and function of the nerve-muscle synapse. Using genetic linkage, we find 18 different biallelic mutations in the gene encoding glutamine-fructose-6-phosphate transaminase 1 (GFPT1) in 13 unrelated families with an autosomal recessive CMS. Consistent with these data, downregulation of the GFPT1 ortholog gfpt1 in zebrafish embryos altered muscle fiber morphology and impaired neuromuscular junction development. GFPT1 is the key enzyme of the hexosamine pathway yielding the amino sugar UDP-N-acetylglucosamine, an essential substrate for protein glycosylation. Our findings provide further impetus to study the glycobiology of NMJ and synapses in general. PMID:21310273

  4. Toxoplasmic encephalitis during mycophenolate mofetil immunotherapy of neuromuscular disease

    PubMed Central

    Chahin, Nizar

    2015-01-01

    Objective: To show that immunotherapy with medications such mycophenolate mofetil (MMF) can cause serious complications in patients with neuromuscular disorders. Methods: Two patients with neuromuscular disorders on immunotherapy with long-term MMF who developed toxoplasmic encephalitis (TE) were included in this case series. Results: One patient with myasthenia gravis and one patient with inflammatory myopathy on immunotherapy with long-term MMF developed severe TE. Diagnosis was based on clinical presentation, MRI brain imaging characteristics, and CSF PCR positivity for Toxoplasma gondii. Both patients were treated with pyrimethamine, sulfadiazine, and leucovorin for 2 months without clinical improvement, and both died. Conclusions: Immunotherapy with medications such as MMF can cause devastating TE in non-HIV patients with neuromuscular disorders. Early consideration and recognition of this complication is important to possibly prevent unfavorable outcomes. The utility of screening and prophylaxis against toxoplasmosis in individuals with neuroimmunologic disorders and other autoimmune disorders who receive immunosuppressive therapy requires future study. PMID:25635260

  5. Neuromuscular blockade with vecuronium in paediatric patients with burn injury.

    PubMed Central

    Mills, A K; Martyn, J A

    1989-01-01

    1. The effect of body surface area (BSA) burn injury on neuromuscular pharmacodynamics of vecuronium was evaluated. 2. The neuromuscular responses of 15 burned children were compared with those in five controls. 3. The effective dose for 50% suppression of twitch tension (ED50) was 34 micrograms kg-1 for patients with less than 40% BSA burn, 55 micrograms kg-1 for 40-60% BSA burn, and 65 micrograms kg-1 for patients with greater than 60% BSA burn. These values were significantly higher than the value for control patients, which was 18 micrograms kg-1. 4. Burn injury induces a resistance to the neuromuscular effects of vecuronium, the magnitude of which is related to burn size. PMID:2570602

  6. Real-world experience with neuromuscular blockade reversal.

    PubMed

    Groudine, Scott B; Minkowitz, Harold S; Valentine, Danny L

    2017-11-01

    Neuromuscular blocking agents are used in many surgical procedures and have enabled new surgical advances. The expanded landscape of neuromuscular blockade (NMB) reversal drugs allows for fast and complete NMB reversal and the reduction of postoperative complications from residual block. In the United States, neostigmine/glycopyrrolate and sugammadex are the primary agents for pharmacologic antagonism of neuromuscular blocking agents. Whereas neostigmine and an anticholinergic have been available for decades, sugammadex has only recently become available. We present real-world cases in a variety of surgical procedures and clinical settings in which the use of NMB reversal agents played a significant role in the patients’ clinical outcome. Online access: http://courses.elseviercme.com/nmb/711.

  7. Frequency of Use and Cost of Selected Anesthetic Induction and Neuromuscular Blocking Agents

    DTIC Science & Technology

    2011-07-21

    identify the most frequently used agents for induction and neuromuscular blockade for intubation, and to identify variables which affected these...p. 196). Therefore, the purpose of this study is (a) to identify the most frequently used agents for induction and neuromuscular blockade for...discussions of which agent to use for neuromuscular blockade during tracheal intubation. "Succinylcholine is the only depolarizing neuromuscular blocking

  8. Neuromuscular block after intra-arterially injected acetylcholine

    PubMed Central

    Pinelli, P.; Tonali, P.; Gambi, D.

    1973-01-01

    It has been suggested that the effect of ACTH in myasthenia gravis may be ascribed to an action involving neuromuscular transmission which favours repolarization processes, with a tendency towards hyperpolarization of the membranes of muscle fibres and motor nerve endings. A similar mechanism has been postulated for the action of ACTH in epilepsy (Klein, 1970). A direct or indirect action on nerve membrane would interfere with depolarization. There is evidence of raised concentration of intracellular potassium and increased outflow of sodium ions which would cause hyperpolarization of the membrane. This paper studies the effect of ACTH on the late block of neuromuscular transmission caused by acetylcholine (ACTH). Images PMID:4350704

  9. Augmentative and alternative communication for people with progressive neuromuscular disease.

    PubMed

    Ball, Laura J; Fager, Susan; Fried-Oken, Melanie

    2012-08-01

    Individuals with progressive neuromuscular disease often experience complex communication needs and consequently find that interaction using their natural speech may not sufficiently meet their daily needs. Increasingly, assistive technology advances provide accommodations for and/or access to communication. Assistive technology related to communication is referred to as augmentative and alternative communication (AAC). The nature of communication challenges in progressive neuromuscular diseases can be as varied as the AAC options currently available. AAC systems continue to be designed and implemented to provide targeted assistance based on an individual's changing needs.

  10. [Organization of therapeutic aid to patients with hereditary neuromuscular diseases].

    PubMed

    Kalinin, V A; Temin, P A; Arkhipov, B A; Zavadenko, N N

    1989-01-01

    The paper summarizes experience gained for many years by the All-Union Research Methodological Center for Study of Hereditary Neuromuscular Diseases. The specialists of the Center render counselling and therapeutic assistance to patients afflicted with neuromuscular diseases. The counselling and diagnostic services are characterized by the fact that it is based on the activity of a large hospital intended for the treatment of various diseases. The problems which are being solved by the out- and inpatient services of the Center are considered in detail. The advantages of the setting up of the common in- and outpatient complex on the basis of the hospital intended for the treatment of various diseases are described.

  11. Prevention and management of limb contractures in neuromuscular diseases.

    PubMed

    Skalsky, Andrew J; McDonald, Craig M

    2012-08-01

    Limb contractures are a common impairment in neuromuscular diseases. They contribute to increased disability from decreased motor performance, mobility limitations, reduced functional range of motion, loss of function for activities of daily living, and increased pain. The pathogenesis of contractures is multifactorial. Myopathic conditions are associated with more severe limb contractures compared with neuropathic disorders. Although the evidence supporting the efficacy of multiple interventions to improve range of motion in neuromuscular diseases in a sustained manner is lacking, there are generally accepted principles with regard to splinting, bracing, stretching, and surgery that help minimize the impact or disability from contractures.

  12. Electrophysiological and Ultrastructural Characterization of Neuromuscular Junctions in Diaphragm Muscle of Acetylcholinesterase Knockout Mice

    DTIC Science & Technology

    2008-04-01

    Electrophysiological and Ultrastructural Characterization of Neuromuscular Junctions in 5a. CONTRACT NUMBER Diaphragm Muscle of Acetylcholinesterase Knockout Mice...AChE +/+) and acetylcholinesterase knockout (AChE -/-) mice to determine the compensatory mechanism manifested by the neuromuscular junction to...had smaller nerve terminals and diminished pre- and postsynaptic surface contacts relative to neuromuscular junctions of AChE +/+ mice. The

  13. Las Vegas

    NASA Technical Reports Server (NTRS)

    2001-01-01

    This image of Las Vegas, NV was acquired on August, 2000 and covers an area 42 km (25 miles) wide and 30 km (18 miles) long. The image displays three bands of the reflected visible and infrared wavelength region, with a spatial resolution of 15 m. McCarran International Airport to the south and Nellis Air Force Base to the NE are the two major airports visible. Golf courses appear as bright red areas of worms. The first settlement in Las Vegas (which is Spanish for The Meadows) was recorded back in the early 1850s when the Mormon church, headed by Brigham Young, sent a mission of 30 men to construct a fort and teach agriculture to the Indians. Las Vegas became a city in 1905 when the railroad announced this city was to be a major division point. Prior to legalized gambling in 1931, Las Vegas was developing as an agricultural area. Las Vegas' fame as a resort area became prominent after World War II. The image is located at 36.1 degrees north latitude and 115.1 degrees west longitude.

    The U.S. science team is located at NASA's Jet Propulsion Laboratory, Pasadena, Calif. The Terra mission is part of NASA's Science Mission Directorate.

  14. Las Vegas

    NASA Image and Video Library

    2001-10-22

    This image of Las Vegas, NV was acquired on August, 2000 and covers an area 42 km (25 miles) wide and 30 km (18 miles) long. The image displays three bands of the reflected visible and infrared wavelength region, with a spatial resolution of 15 m. McCarran International Airport to the south and Nellis Air Force Base to the NE are the two major airports visible. Golf courses appear as bright red areas of worms. The first settlement in Las Vegas (which is Spanish for The Meadows) was recorded back in the early 1850s when the Mormon church, headed by Brigham Young, sent a mission of 30 men to construct a fort and teach agriculture to the Indians. Las Vegas became a city in 1905 when the railroad announced this city was to be a major division point. Prior to legalized gambling in 1931, Las Vegas was developing as an agricultural area. Las Vegas' fame as a resort area became prominent after World War II. The image is located at 36.1 degrees north latitude and 115.1 degrees west longitude. http://photojournal.jpl.nasa.gov/catalog/PIA11096

  15. Drug Development and Challenges for Neuromuscular Clinical Trials.

    PubMed

    El Mouelhi, Mohamed

    2016-03-01

    Drug development process faces many challenges, including those encountered in clinical trials for neuromuscular diseases. Drug development is a lengthy and highly costly process. Out of 10 compounds entering first study in man (phase 1), only one compound reaches the market after an average of 14 years with a cost of $2.7 billion. Nevertheless, according to the Centers for Medicare and Medicaid services, prescription drugs constituted only 9 % of each health care dollar spent in USA in 2013. Examples of challenges encountered in neuromuscular clinical trials include lack of validated patient-reported outcome tools, blinding issues, and the use of placebo in addition to lack of health authority guidance for orphan diseases. Patient enrollment challenge is the leading cause of missed clinical trial deadlines observed in about 80 % of clinical trials, resulting in delayed availability of potentially life-saving therapies. Another specific challenge introduced by recent technology is the use of social media and risk of bias. Sharing personal experiences while in the study could easily introduce bias among patients that would interfere with accurate interpretation of collected data. To minimize this risk, recent neuromuscular studies incorporate as an inclusion criterion the patient's agreement not to share any of study experiences through social media with other patients during the study conduct. Consideration of these challenges will allow timely response to the high unmet medical needs for many neuromuscular diseases.

  16. Neuromuscular activation patterns during treadmill walking after space flight

    NASA Technical Reports Server (NTRS)

    Layne, C. S.; McDonald, P. V.; Bloomberg, J. J.

    1997-01-01

    Astronauts adopt a variety of neuromuscular control strategies during space flight that are appropriate for locomoting in that unique environment, but are less than optimal upon return to Earth. We report here the first systematic investigation of potential adaptations in neuromuscular activity patterns associated with postflight locomotion. Astronaut-subjects were tasked with walking on a treadmill at 6.4 km/h while fixating a visual target 30 cm away from their eyes after space flights of 8-15 days. Surface electromyography was collected from selected lower limb muscles and normalized with regard to mean amplitude and temporal relation to heel strike. In general, high correlations (more than 0.80) were found between preflight and postflight activation waveforms for each muscle and each subject: however relative activation amplitude around heel strike and toe off was changed as a result of flight. The level of muscle cocontraction and activation variability, and the relationship between the phasic characteristics of the ankle musculature in preparation for toe off also were altered by space flight. Subjects also reported oscillopsia during treadmill walking after flight. These findings indicate that, after space flight, the sensory-motor system can generate neuromuscular-activation strategies that permit treadmill walking, but subtle changes in lower-limb neuromuscular activation are present that may contribute to increased lower limb kinematic variability and oscillopsia also present during postflight walking.

  17. The sagital balance in idiopatic and neuromuscular scoliosis.

    PubMed

    Borges, Paulo Alvim; Ocampos, Guilherme Pereira; Mancuso Filho, José Antonio; Letaif, Olavo Biraghi; Marcon, Raphael Martus; Cristante, Alexandre Fogaça

    2014-01-01

    To describe and compare the distribution of spinopelvic parameters (SPP) in a Brazilian population with idiopathic scoliosis (IS) and neuromuscular scoliosis (NMS), and evaluate the association between pelvic incidence (PI) and lumbar lordosis (LL). Medical records investigation was performed. Sagital balance angles were measured in patients with neuromuscular and idiopathic scoliosis. IS SAMPLE MEANS (IN DEGREES): PI 55.55; Sacral Slope (SS) 45.35; Pelvic Tilt (PT) 10.19; Lumbar Lordosis (LL) 43.48; and Thoracic Kyphosis (TK) 32.10. In NMS: PI 53.77; SS 42.31; PT 11.46; LL 49.46; and TK 45.69. No statistically significant differences in PEP distribution were found between the two types of scoliosis (p=0,057). The association between pelvic incidence and lumbar lordosis is low among idiopathic scoliosis (R=0,074) and neuromuscular scoliosis (R=0,274). PEP measurements in a Brazilian population of idiopathic scoliosis and neuromuscular scoliosis patients are similar to those in the international literature and do not differ statistically between them. The association between LL and PI could not be assessed in this study. Level of Evidence IV, Case Series.

  18. Neuromuscular training for rehabilitation of sports injuries: a systematic review.

    PubMed

    Zech, Astrid; Hübscher, Markus; Vogt, Lutz; Banzer, Winfried; Hänsel, Frank; Pfeifer, Klaus

    2009-10-01

    Although proprioceptive and neuromuscular exercises are considered to be part and parcel of rehabilitation programs after sport injuries, there is an uncertainty regarding the effectiveness of corresponding training interventions. The objective of this review was to evaluate the effectiveness of proprioceptive and neuromuscular training (PT/NT) for the treatment of ankle, knee, and shoulder joint injuries. Two independent reviewers performed a literature search in various databases and reference lists of articles. Data of included trials were then extracted, and methodological quality was assessed by using predetermined forms. Fifteen trials met the inclusion criteria. PT/NT was effective at increasing functionality as well as at decreasing the incidence of recurrent injuries and "giving way" episodes after ankle sprains and in conservative treatment of anterior cruciate ligament injuries. However, conflicting results or no efficacy of training were reported for static postural control, joint position sense, neuromuscular control, joint laxity, and lower extremity strength. No study that examined PT/NT after shoulder injuries was found. From this review, it can be concluded that proprioceptive and neuromuscular interventions after ankle and knee joint injuries can be effective for the prevention of recurrent injuries and the improvement of joint functionality.

  19. Neuromuscular alterations during walking in persons with moderate knee osteoarthritis.

    PubMed

    Hubley-Kozey, C L; Deluzio, K J; Landry, S C; McNutt, J S; Stanish, W D

    2006-08-01

    This paper compared the neuromuscular responses during walking between those with early-stage knee osteoarthritis (OA) to asymptomatic controls. The rationale for studying those with mild to moderate knee OA was to determine the alterations in response to dynamic loading that might be expected before severe pain, joint space narrowing and joint surface changes occur. We used pattern recognition techniques to explore both amplitude and shape changes of the surface electromyograms recorded from seven muscles crossing the knee joint of 40 subjects with knee OA and 38 asymptomatic controls during a walking task. The principal patterns for each muscle grouping explained over 83% of the variance in the waveforms. This result supported the notion that the main neuromuscular patterns were similar between asymptomatic controls and those with OA, reflecting the specific roles of the major muscles during walking. ANOVA revealed significant (p<0.05) differences in the principal pattern scores reflecting both amplitude and shape alterations in the OA group and among muscles. These differences captured subtle changes in the neuromuscular responses of the subjects with OA throughout different phases of the gait cycle and most likely reflected changes in the mechanical environment (joint loading, instability) and pain. The subjects with OA attempted to increase activity of the lateral sites and reduce activity in the medial sites, having minimal but prolonged activity during late stance. Therefore, alterations in neuromuscular responses were found even in this high functioning group with moderate knee OA.

  20. Neuromuscular activation patterns during treadmill walking after space flight

    NASA Technical Reports Server (NTRS)

    Layne, C. S.; McDonald, P. V.; Bloomberg, J. J.

    1997-01-01

    Astronauts adopt a variety of neuromuscular control strategies during space flight that are appropriate for locomoting in that unique environment, but are less than optimal upon return to Earth. We report here the first systematic investigation of potential adaptations in neuromuscular activity patterns associated with postflight locomotion. Astronaut-subjects were tasked with walking on a treadmill at 6.4 km/h while fixating a visual target 30 cm away from their eyes after space flights of 8-15 days. Surface electromyography was collected from selected lower limb muscles and normalized with regard to mean amplitude and temporal relation to heel strike. In general, high correlations (more than 0.80) were found between preflight and postflight activation waveforms for each muscle and each subject: however relative activation amplitude around heel strike and toe off was changed as a result of flight. The level of muscle cocontraction and activation variability, and the relationship between the phasic characteristics of the ankle musculature in preparation for toe off also were altered by space flight. Subjects also reported oscillopsia during treadmill walking after flight. These findings indicate that, after space flight, the sensory-motor system can generate neuromuscular-activation strategies that permit treadmill walking, but subtle changes in lower-limb neuromuscular activation are present that may contribute to increased lower limb kinematic variability and oscillopsia also present during postflight walking.

  1. Neuromuscular Electrical Stimulation for Motor Restoration in Hemiplegia

    PubMed Central

    Knutson, Jayme S.; Fu, Michael J.; Sheffler, Lynne R.; Chae, John

    2015-01-01

    Synopsis This article reviews the most common therapeutic and neuroprosthetic applications of neuromuscular electrical stimulation (NMES) for upper and lower extremity stroke rehabilitation. Fundamental NMES principles and purposes in stroke rehabilitation are explained. NMES modalities used for upper and lower limb rehabilitation are described and efficacy studies are summarized. The evidence for peripheral and central mechanisms of action is also summarized. PMID:26522909

  2. Assessment of Neuromuscular Function Using Percutaneous Electrical Nerve Stimulation.

    PubMed

    Rozand, Vianney; Grosprêtre, Sidney; Stapley, Paul J; Lepers, Romuald

    2015-09-13

    Percutaneous electrical nerve stimulation is a non-invasive method commonly used to evaluate neuromuscular function from brain to muscle (supra-spinal, spinal and peripheral levels). The present protocol describes how this method can be used to stimulate the posterior tibial nerve that activates plantar flexor muscles. Percutaneous electrical nerve stimulation consists of inducing an electrical stimulus to a motor nerve to evoke a muscular response. Direct (M-wave) and/or indirect (H-reflex) electrophysiological responses can be recorded at rest using surface electromyography. Mechanical (twitch torque) responses can be quantified with a force/torque ergometer. M-wave and twitch torque reflect neuromuscular transmission and excitation-contraction coupling, whereas H-reflex provides an index of spinal excitability. EMG activity and mechanical (superimposed twitch) responses can also be recorded during maximal voluntary contractions to evaluate voluntary activation level. Percutaneous nerve stimulation provides an assessment of neuromuscular function in humans, and is highly beneficial especially for studies evaluating neuromuscular plasticity following acute (fatigue) or chronic (training/detraining) exercise.

  3. Biochemistry of Neuromuscular Diseases: A Course for Undergraduate Students

    ERIC Educational Resources Information Center

    Ohlendieck, Kay

    2002-01-01

    This article outlines an undergraduate course focusing on supramolecular membrane protein complexes involved in the molecular pathogenesis of neuromuscular disorders. The emphasis of this course is to introduce students to the key elements involved in the ion regulation and membrane stabilization during muscle contraction and the role of these…

  4. Biochemistry of Neuromuscular Diseases: A Course for Undergraduate Students

    ERIC Educational Resources Information Center

    Ohlendieck, Kay

    2002-01-01

    This article outlines an undergraduate course focusing on supramolecular membrane protein complexes involved in the molecular pathogenesis of neuromuscular disorders. The emphasis of this course is to introduce students to the key elements involved in the ion regulation and membrane stabilization during muscle contraction and the role of these…

  5. Neuromuscular and metabolic characteristics of elite basketball referees.

    PubMed

    Bonganha, V; Cavaglieri, C R; Daniel, J F; Mercadante, L A; Montagner, P C; Borin, J P

    2013-06-01

    The dynamics of Basketball refereeing has changed and more recently the championships started to use the participation of three referees, but there is still a lack of information about the physical characteristics and performance of the referees. The aim of this study was to characterize the neuromuscular and metabolic performances and body composition of Brazilian elite basketball referees, with a level of national and international refereeing. Thirty-seven referees participated in the study (international level N.=17 and national level N.=20). We evaluated anthropometric and body composition variables, among them: height, body mass, body mass index (BMI) and body fat (%); metabolic parameters: lactate at rest and post-exercise; and neuromuscular performance: speed, explosive lower limbs strength, flexibility and aerobic capacity. The main results showed a statistically significant difference in age, in which the international referees were older than the national level (41.94±6.71; 37.30±7.23; P=0.036). There were not significant differences between the levels for neuromuscular and metabolic parameters and body composition. Comparing the results with athletes, the referees showed lower neuromuscular and metabolic parameters and the body composition showed higher body fat than basketball players. These data may represent physiological parameters to be considered in the prescription of physical training during the preparation and competition period.

  6. 21 CFR 882.5860 - Implanted neuromuscular stimulator.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 21 Food and Drugs 8 2012-04-01 2012-04-01 false Implanted neuromuscular stimulator. 882.5860 Section 882.5860 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES NEUROLOGICAL DEVICES Neurological Therapeutic Devices § 882.5860...

  7. 21 CFR 882.5810 - External functional neuromuscular stimulator.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 21 Food and Drugs 8 2013-04-01 2013-04-01 false External functional neuromuscular stimulator. 882.5810 Section 882.5810 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES NEUROLOGICAL DEVICES Neurological Therapeutic Devices §...

  8. 21 CFR 882.5810 - External functional neuromuscular stimulator.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 21 Food and Drugs 8 2014-04-01 2014-04-01 false External functional neuromuscular stimulator. 882.5810 Section 882.5810 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES NEUROLOGICAL DEVICES Neurological Therapeutic Devices §...

  9. 21 CFR 882.5810 - External functional neuromuscular stimulator.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 21 Food and Drugs 8 2012-04-01 2012-04-01 false External functional neuromuscular stimulator. 882.5810 Section 882.5810 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES NEUROLOGICAL DEVICES Neurological Therapeutic Devices §...

  10. Facial rehabilitation: a neuromuscular reeducation, patient-centered approach.

    PubMed

    Vanswearingen, Jessie

    2008-05-01

    Individuals with facial paralysis and distorted facial expressions and movements secondary to a facial neuromotor disorder experience substantial physical, psychological, and social disability. Previously, facial rehabilitation has not been widely available or considered to be of much benefit. An emerging rehabilitation science of neuromuscular reeducation and evidence for the efficacy of facial neuromuscular reeducation, a process of facilitating the return of intended facial movement patterns and eliminating unwanted patterns of facial movement and expression, may provide patients with disorders of facial paralysis or facial movement control opportunity for the recovery of facial movement and function. We provide a brief overview of the scientific rationale for facial neuromuscular reeducation in the structure and function of the facial neuromotor system, the neuropsychology of facial expression, and relations among expressions, movement, and emotion. The primary purpose is to describe principles of neuromuscular reeducation, assessment and outcome measures, approach to treatment, the process, including surface-electromyographic biofeedback as an adjunct to reeducation, and the goal of enhancing the recovery of facial expression and function in a patient-centered approach to facial rehabilitation.

  11. Disorders of neuromuscular transmission due to natural environmental toxins.

    PubMed

    Senanayake, N; Román, G C

    1992-01-01

    A variety of natural toxins of animal, plant, and bacterial origin are capable of causing disorders of neuromuscular transmission. Animal toxins include venomous snakes and arthropods, venoms of certain marine creatures, skin secretions of dart-poison frogs, and poisonous fish, shellfish, and crabs. There are plant poisons such as curare, and bacterial poisons such as botulinum toxin. These act at single or multiple sites of the neuromuscular apparatus interfering with voltage-gated ion channels, acetylcholine release, depolarization of the postsynaptic membrane, or generation and spread of the muscle action potential. The specific actions of these toxins are being widely exploited in the study of neuromuscular physiology and pathology. Some toxins have proved to be valuable pharmaceutical agents. Poisoning by natural neurotoxins is an important public health hazard in many parts of the world, particularly in the tropics. Poisoning may occur by a bite or a sting of a venomous animal, or by the ingestion of poisonous fish, shellfish or other marine delicacies. Contaminated food is a vehicle for poisons such as botulinum toxin. Clinically, a cardinal feature in the symptomatology is muscle paralysis with a distribution characteristic of myasthenia gravis, affecting muscles innervated by cranial nerves, neck flexors, proximal limb muscles, and respiratory muscles. Respiratory paralysis may end fatally. This paper reviews from the clinical and pathophysiologic viewpoints, naturally occurring environmental neurotoxins acting at the neuromuscular junction.

  12. Neuromuscular Electrical Stimulation for Motor Restoration in Hemiplegia.

    PubMed

    Knutson, Jayme S; Fu, Michael J; Sheffler, Lynne R; Chae, John

    2015-11-01

    This article reviews the most common therapeutic and neuroprosthetic applications of neuromuscular electrical stimulation (NMES) for upper and lower extremity stroke rehabilitation. Fundamental NMES principles and purposes in stroke rehabilitation are explained. NMES modalities used for upper and lower limb rehabilitation are described, and efficacy studies are summarized. The evidence for peripheral and central mechanisms of action is also summarized.

  13. The sagital balance in idiopatic and neuromuscular scoliosis

    PubMed Central

    Borges, Paulo Alvim; Ocampos, Guilherme Pereira; Mancuso, José Antonio; Letaif, Olavo Biraghi; Marcon, Raphael Martus; Cristante, Alexandre Fogaça

    2014-01-01

    OBJECTIVES: To describe and compare the distribution of spinopelvic parameters (SPP) in a Brazilian population with idiopathic scoliosis (IS) and neuromuscular scoliosis (NMS), and evaluate the association between pelvic incidence (PI) and lumbar lordosis (LL). METHOD: Medical records investigation was performed. Sagital balance angles were measured in patients with neuromuscular and idiopathic scoliosis. RESULTS: IS sample means (in degrees): PI 55.55; Sacral Slope (SS) 45.35; Pelvic Tilt (PT) 10.19; Lumbar Lordosis (LL) 43.48; and Thoracic Kyphosis (TK) 32.10. In NMS: PI 53.77; SS 42.31; PT 11.46; LL 49.46; and TK 45.69. No statistically significant differences in PEP distribution were found between the two types of scoliosis (p=0,057). The association between pelvic incidence and lumbar lordosis is low among idiopathic scoliosis (R=0,074) and neuromuscular scoliosis (R=0,274). CONCLUSION: PEP measurements in a Brazilian population of idiopathic scoliosis and neuromuscular scoliosis patients are similar to those in the international literature and do not differ statistically between them. The association between LL and PI could not be assessed in this study. Level of Evidence IV, Case Series. PMID:25246845

  14. Pivoting neuromuscular control and proprioception in females and males.

    PubMed

    Lee, Song Joo; Ren, Yupeng; Kang, Sang Hoon; Geiger, François; Zhang, Li-Qun

    2015-04-01

    Noncontact ACL injuries occur most commonly in pivoting sports and are much more frequent in females than in males. However, information on sex differences in proprioceptive acuity under weight-bearing and leg neuromuscular control in pivoting is scarce. The objective of this study was to investigate sex differences in pivoting neuromuscular control during strenuous stepping tasks and proprioceptive acuity under weight-bearing. 21 male and 22 female subjects were recruited to evaluate pivoting proprioceptive acuity under weight-bearing, and pivoting neuromuscular control (in terms of leg pivoting instability, stiffness, maximum internal and external pivoting angles, and entropy of time-to-peak EMG in lower limb muscles) during strenuous stepping tasks performed on a novel offaxis elliptical trainer. Compared to males, females had significantly lower proprioceptive acuity under weight-bearing in both internal and external pivoting directions, higher pivoting instability, larger maximum internal pivoting angle, lower leg pivoting stiffness, and higher entropy of time-to-peak EMG in the gastrocnemius muscles during strenuous stepping tasks with internal and external pivoting perturbations. Results of this study may help us better understand factors contributing to ACL injuries in females and males, develop training strategies to improve pivoting neuromuscular control and proprioceptive acuity, and potentially reduce ACL and lower-limb musculoskeletal injuries.

  15. Adverse effects of neuromuscular blockers and their antagonists.

    PubMed

    Naguib, M; Magboul, M M

    1998-06-01

    Among all the drugs used for general anesthesia, neuromuscular blockers appear to play a prominent role in the incidence of severe adverse reactions. It now seems likely that most serious adverse drug reactions occurring during anesthesia are immunological in type. The frequency of life-threatening anaphylactic or anaphylactoid reactions occurring during anesthesia has been estimated to be between 1 in 1000 and 1 in 25,000 anesthetic procedures, with the neuromuscular blockers being involved in 80% of cases. The mortality from such serious reactions is reported to be in the range of 3.4 to 6%. The highly immunogenic drug, suxamethonium chloride (succinylcholine), was found to be the most hazardous agent. Drug-specific immunoglobulin E antibodies to suxamethonium chloride and other neuromuscular blockers have been demonstrated. This sensitivity to neuromuscular blockers seems to be a long-lasting phenomenon. During anesthesia, the clinical features of an allergic reaction are often masked. Tachycardia and circulatory collapse may be the only signs of an allergic reaction, and they are easily misdiagnosed. Bronchospasm is reported to be present in about 40% of cases. Successful management of these patients includes stabilisation during the acute reaction and avoidance of future reactions. The latter is based on the identification of the causative drug and potentially cross-reacting compounds. The use of suxamethonium chloride is associated with many other adverse effects, such as fasciculations, myalgia, potassium release, changes in the heart rate, increases in intragastric and intraocular pressures, and malignant hyperthermia. Because of the dangers of hyperkalemic cardiac arrest suxamethonium chloride administration in children with unrecognised muscular dystrophy, there have now been moves to limit the use of this drug in children. Although neuromuscular blockers are designed to specifically block nicotinic cholinergic receptors at the neuromuscular junction

  16. Stunted PFC activity during neuromuscular control under stress with obesity.

    PubMed

    Mehta, Ranjana K

    2016-02-01

    Obesity is an established risk factor for impaired cognition, which is primarily regulated by the prefrontal cortex (PFC). However, very little is known about the neural pathways that underlie obesity-related declines in neuromuscular control, particularly under stress. The purpose of this study was to determine the role of the PFC on neuromuscular control during handgrip exertions under stress with obesity. Twenty non-obese and obese young adults performed submaximal handgrip exertions in the absence and presence of a concurrent stressful task. Primary dependent measures included oxygenated hemoglobin (HbO2: a measure of PFC activity) and force fluctuations (an indicator of neuromuscular control). Higher HbO2 levels in the PFC were observed in the non-obese compared to the obese group (P = 0.009). In addition, higher HbO2 levels were observed in the stress compared to the control condition in the non-obese group; however, this trend was reversed in the obese group (P = 0.043). In general, force fluctuations increased by 26% in the stress when compared to the control condition (P = 0.001) and obesity was associated with 39% greater force fluctuation (P = 0.024). Finally, while not significant, obesity-related decrements in force fluctuations were magnified under stress (P = 0.063). The current study provides the first evidence that neuromuscular decrements with obesity were associated with impaired PFC activity and this relationship was augmented in stress conditions. These findings are important because they provide new information on obesity-specific changes in brain function associated with neuromuscular control since the knowledge previously focused largely on obesity-specific changes in peripheral muscle capacity.

  17. Neuromuscular blocking effect of fluoxetine and its interaction with rocuronium.

    PubMed

    Patel, J C; Barvaliya, M J; Patel, T K; Tripathi, C B

    2013-04-01

    As selective serotonin reuptake inhibitors have an inhibitory effect on nicotinic acetylcholine receptors, they may affect the neuromuscular transmission and interact with neuromuscular blockers. This study was designed to observe the effect of fluoxetine on neuromuscular transmission and its interaction with rocuronium using the rat phrenic nerve hemidiaphragm and rabbit head drop methods. Rat phrenic nerve hemidiaphragms were mounted and stimulated using a train of four pulses (TOF). The effect of fluoxetine was studied on both indirectly and directly stimulated basal twitch responses by plotting cumulative dose response curves (DRCs). DRCs of rocuronium were obtained in the absence, and presence of 5 μm and 20 μm fluoxetine to study its interaction. ED5 , ED50 and ED95 values of rocuronium DRCs in absence and presence of fluoxetine were calculated. Fluoxetine significantly inhibited twitch responses in both indirect and directly stimulated preparations. Fluoxetine (20 μm) caused an increase in the potency of rocuronium such that the ED50 and ED95 values of rocuronium DRCs were significantly decreased. Partially inhibited twitch responses by fluoxetine (100 μm) were not reversed by neostigmine (3.3 μm) or 3,4 diaminopyridine (0.25 μm). Rabbits were given fluoxetine 0.25 mg kg(-1) and 1 mg kg(-1) orally for 15 days, and on 15th day, rocuronium infusion was given, and time for head drop was recorded. The time of head drop was significantly reduced in fluoxetine pretreated as compared to control group. Fluoxetine blocks the neuromuscular transmission and increases the potency of rocuronium-induced neuromuscular block.

  18. Neuromuscular activity of Micrurus laticollaris (Squamata: Elapidae) venom in vitro.

    PubMed

    Carbajal-Saucedo, Alejandro; Floriano, Rafael Stuani; Dal Belo, Cháriston André; Olvera-Rodríguez, Alejandro; Alagón, Alejandro; Rodrigues-Simioni, Léa

    2014-01-17

    In this work, we have examined the neuromuscular activity of Micrurus laticollaris (Mexican coral snake) venom (MLV) in vertebrate isolated nerve-muscle preparations. In chick biventer cervicis preparations, the MLV induced an irreversible concentration- and time-dependent (1-30 µg/mL) neuromuscular blockade, with 50% blockade occurring between 8 and 30 min. Muscle contractures evoked by exogenous acetylcholine were completely abolished by MLV, whereas those of KCl were also significantly altered (86% ± 11%, 53% ± 11%, 89% ± 5% and 89% ± 7% for one, three, 10 and 30 µg of venom/mL, respectively; n = 4; p < 0.05). In mouse phrenic nerve-diaphragm preparations, MLV (1-10 µg/mL) promoted a slight increase in the amplitude of twitch-tension (3 µg/mL), followed by neuromuscular blockade (n = 4); the highest concentration caused complete inhibition of the twitches (time for 50% blockade = 26 ± 3 min), without exhibiting a previous neuromuscular facilitation. The venom (3 µg/mL) induced a biphasic modulation in the frequency of miniature end-plate potentials (MEPPs)/min, causing a significant increase after 15 min, followed by a decrease after 60 min (from 17 ± 1.4 (basal) to 28 ± 2.5 (t15) and 12 ± 2 (t60)). The membrane resting potential of mouse diaphragm preparations pre-exposed or not to d-tubocurarine (5 µg/mL) was also significantly less negative with MLV (10 µg/mL). Together, these results indicate that M. laticollaris venom induces neuromuscular blockade by a combination of pre- and post-synaptic activities.

  19. Endomicroscopy and electromyography of neuromuscular junctions in situ

    PubMed Central

    Brown, Rosalind; Dissanayake, Kosala N; Skehel, Paul A; Ribchester, Richard R

    2014-01-01

    Objective Electromyography (EMG) is used routinely to diagnose neuromuscular dysfunction in a wide range of peripheral neuropathies, myopathies, and neuromuscular degenerative diseases including motor neuron diseases such as amyotrophic lateral sclerosis (ALS). Definitive neurological diagnosis may also be indicated by the analysis of pathological neuromuscular innervation in motor-point biopsies. Our objective in this study was to preempt motor-point biopsy by combining live imaging with electrophysiological analysis of slow degeneration of neuromuscular junctions (NMJs) in vivo. Methods We combined conventional needle electromyography with fiber-optic confocal endomicroscopy (CEM), using an integrated hand-held, 1.5-mm-diameter probe. We utilized as a test bed, various axotomized muscles in the hind limbs of anaesthetized, double-homozygous thy1.2YFP16: WldS mice, which coexpress the Wallerian-degeneration Slow (WldS) protein and yellow fluorescent protein (YFP) in motor neurons. We also tested exogenous vital stains, including Alexa488-α-bungarotoxin; the styryl pyridinium dye 4-Di-2-Asp; and a GFP conjugate of botulinum toxin Type A heavy chain (GFP-HcBoNT/A). Results We show that an integrated EMG/CEM probe is effective in longitudinal evaluation of functional and morphological changes that take place over a 7-day period during axotomy-induced, slow neuromuscular synaptic degeneration. EMG amplitude declined in parallel with overt degeneration of motor nerve terminals. EMG/CEM was safe and effective when nerve terminals and motor endplates were selectively stained with vital dyes. Interpretation Our findings constitute proof-of-concept, based on live imaging in an animal model, that combining EMG/CEM may be useful as a minimally invasive precursor or alternative to motor-point biopsy in neurological diagnosis and for monitoring local administration of potential therapeutics. PMID:25540801

  20. Neuromuscular Activity of Micrurus laticollaris (Squamata: Elapidae) Venom in Vitro

    PubMed Central

    Carbajal-Saucedo, Alejandro; Floriano, Rafael Stuani; Dal Belo, Cháriston André; Olvera-Rodríguez, Alejandro; Alagón, Alejandro; Rodrigues-Simioni, Léa

    2014-01-01

    In this work, we have examined the neuromuscular activity of Micrurus laticollaris (Mexican coral snake) venom (MLV) in vertebrate isolated nerve-muscle preparations. In chick biventer cervicis preparations, the MLV induced an irreversible concentration- and time-dependent (1–30 µg/mL) neuromuscular blockade, with 50% blockade occurring between 8 and 30 min. Muscle contractures evoked by exogenous acetylcholine were completely abolished by MLV, whereas those of KCl were also significantly altered (86% ± 11%, 53% ± 11%, 89% ± 5% and 89% ± 7% for one, three, 10 and 30 µg of venom/mL, respectively; n = 4; p < 0.05). In mouse phrenic nerve-diaphragm preparations, MLV (1–10 µg/mL) promoted a slight increase in the amplitude of twitch-tension (3 µg/mL), followed by neuromuscular blockade (n = 4); the highest concentration caused complete inhibition of the twitches (time for 50% blockade = 26 ± 3 min), without exhibiting a previous neuromuscular facilitation. The venom (3 µg/mL) induced a biphasic modulation in the frequency of miniature end-plate potentials (MEPPs)/min, causing a significant increase after 15 min, followed by a decrease after 60 min (from 17 ± 1.4 (basal) to 28 ± 2.5 (t15) and 12 ± 2 (t60)). The membrane resting potential of mouse diaphragm preparations pre-exposed or not to d-tubocurarine (5 µg/mL) was also significantly less negative with MLV (10 µg/mL). Together, these results indicate that M. laticollaris venom induces neuromuscular blockade by a combination of pre- and post-synaptic activities. PMID:24445448

  1. Corticosteroids and neuromuscular blockers in development of critical illness neuromuscular abnormalities: A historical review.

    PubMed

    Wilcox, Susan R

    2017-02-01

    Weakness is common in critically ill patients, associated with prolonged mechanical ventilation and increased mortality. Corticosteroids and neuromuscular blockade (NMB) administration have been implicated as etiologies of acquired weakness in the intensive care unit. Medical literature since the 1970s is replete with case reports and small case series of patients with weakness after receiving high-dose corticosteroids, prolonged NMB, or both. Several risk factors for weakness appear in the early literature, including large doses of steroids, the dose and duration of NMB, hyperglycemia, and the duration of mechanical ventilation. With improved quality of data, however, the association between weakness and steroids or NMB wanes. This may reflect changes in clinical practice, such as a reduction in steroid dosing, use of cisatracurium besylate instead of aminosteroid NMBs, improved glycemic control, or trends in minimizing mechanical ventilatory support. Thus, based on the most recent and high-quality literature, neither corticosteroids in commonly used doses nor NMB is associated with increased duration of mechanical ventilation, the greatest morbidity of weakness. Minimizing ventilator support as soon as the patient's condition allows may be associated with a reduction in weakness-related morbidity.

  2. Neonatal presentation of lethal neuromuscular glycogen storage disease type IV.

    PubMed

    Escobar, L F; Wagner, S; Tucker, M; Wareham, J

    2012-10-01

    A total of 11 types of glycogen storage disorders have been recognized with variable clinical presentations. Type IV, also known as Andersen disease, represents a rare subtype that can induce severe clinical findings early in life. We report on a patient with early fetal onset of symptoms with severe neuromuscular findings at birth. The pregnancy was further complicated by polyhydramnios and depressed fetal movement. At birth severe hypotonia was noticed requiring active resuscitation and then mechanical ventilation. His lack of expected course for hypoxic ischemic encephalopathy prompted genetic testing, including a muscle biopsy, which confirmed the diagnosis of glycogen storage disease IV (GSD IV). Mutation analysis of the glycogen branching enzyme 1 gene demonstrated a previously unrecognized mutation. We review recent information on early presentation of GSD IV with particular interest in the presentation of the neonatal lethal neuromuscular form of this rare disorder.

  3. Sustained Neuromuscular Blockade after Vecuronium Use in a Premature Infant

    PubMed Central

    Sahni, Mitali; Richardson, C. Joan; Jain, Sunil K.

    2015-01-01

    Background Prolonged use of neuromuscular blocking agents (NMBAs) is very common in critically ill children both in pediatric and neonatal intensive care units. There are no guidelines available for use of NMBAs in children or neonates in the US, and the data for their safety in this age group is limited. Case Description Our case describes prolonged neuromuscular blockade following concurrent use of a NMBA along with aminoglycosides and steroids in the setting of renal failure in a premature infant. Conclusion Prolonged use of NMBAs in preterm infants should be avoided if possible or should be restricted to the shortest possible duration and the smallest possible physiologically effective dose. Concurrent use of NMBAs with aminoglycoside and steroids should be avoided, especially in the setting of renal failure. PMID:26495168

  4. One clinic's experience with carbon fiber orthoses in neuromuscular disease.

    PubMed

    Mnatsakanian, Ani; Kissel, John T; Terry, Philip; King, Wendy M

    2017-02-01

    The purpose of this study was to summarize our experience with off-the-shelf anterior shell carbon fiber ankle-foot orthoses (CFAFOs) prescribed to adult neuromuscular patients in an outpatient clinic. We studied ambulatory patients who were seen in Muscular Dystrophy Association or amyotrophic lateral sclerosis clinics between 2011 and 2014 and prescribed anterior shell CFAFOs. Charts were reviewed with attention to diagnosis, satisfaction with use, and reasons for acceptance or rejection. We included individuals who were currently using AFOs and those being prescribed AFOs for the first time. We were especially interested in reasons for acceptance or rejection of the orthosis. Two hundred eighty-three charts were reviewed. Of these, 109 of 123 (89%) patients were satisfied or extremely satisfied with the anterior shell CFAFOs, including 38 who had previously used other styles. Anterior shell CFAFOs should be considered for most neuromuscular patients with distal leg weakness. Muscle Nerve 55: 202-205, 2017. © 2016 Wiley Periodicals, Inc.

  5. The ageing neuromuscular system and sarcopenia: a mitochondrial perspective

    PubMed Central

    Picard, Martin; Turnbull, Doug M.

    2016-01-01

    Abstract Skeletal muscles undergo structural and functional decline with ageing, culminating in sarcopenia. The underlying neuromuscular mechanisms have been the subject of intense investigation, revealing mitochondrial abnormalities as potential culprits within both nerve and muscle cells. Implicated mechanisms involve impaired mitochondrial dynamics, reduced organelle biogenesis and quality control via mitophagy, accumulation of mitochondrial DNA (mtDNA) damage and respiratory chain defect, metabolic disturbance, pro‐apoptotic signalling, and oxidative stress. This article provides an overview of the cellular mechanisms whereby mitochondria may promote maladaptive changes within motor neurons, the neuromuscular junction (NMJ) and muscle fibres. Lifelong physical activity, which promotes mitochondrial health across tissues, is emerging as an effective countermeasure for sarcopenia. PMID:26921061

  6. Responses of neuromuscular systems under gravity or microgravity environment.

    PubMed

    Ishihara, Akihiko; Kawano, Fuminori; Wang, Xiao Dong; Ohira, Yoshinobu

    2004-11-01

    Hindlimb suspension of rats induces induces fiber atrophy and type shift of muscle fibers. In contrast, there is no change in the cell size or oxidative enzyme activity of spinal motoneurons innervating muscle fibers. Growth-related increases in the cell size of muscle fibers and their spinal motoneurons are inhibited by hindlimb suspension. Exposure to microgravity induces atrophy of fibers (especially slow-twitch fibers) and shift of fibers from slow- to fast-twitch type in skeletal muscles (especially slow, anti-gravity muscles). In addition, a decrease in the oxidative enzyme activity of spinal motoneurons innervating slow-twitch fibers and of sensory neurons in the dorsal root ganglion is observed following exposure to microgravity. It is concluded that neuromuscular activities are important for maintaining metabolism and function of neuromuscular systems at an early postnatal development and that gravity effects both efferent and afferent neural pathways.

  7. Ethical considerations in paediatric neurology: neuromuscular disease and epilepsy.

    PubMed

    Bodensteiner, John B; Ng, Yu-Tze

    2011-09-01

    The pace of developing technology with respect to many diagnostic tests, as well as available treatments including artificial ventilation, may have progressed at a faster rate than our ethical, humane ability to decide on the optimal choices for our patients. In fact, who should make these choices; physicians or patients and families? Certain ethical aspects of neuromuscular disorders and epilepsy are reviewed. For neuromuscular disease, the example of Duchenne muscular dystrophy (DMD) with regards to genetic testing, relatively early wheelchair placement and individualised invasive ventilation is discussed. In epilepsy, performing neurosurgery in severely impaired children is probably appropriate in some cases if desired by the family. Financial and human costs restrict therapies and testing for epilepsy as well as other neurological and medical diseases. Whether it is ethical to consider costs in medical treatment or not, it is certainly a reality.

  8. [Propanidid as an antagonist: competitive inhibitor of neuromuscular plaque acetylcholinesterase].

    PubMed

    Pesando, O T; Piazza, L; Berruti, G; Sciandra, G

    1980-06-01

    Propanidid is a competitive inhibitor not only of serum cholinesterase, but of neuromuscular plaque and brain acetylcholinesterase also. The I50 of plaque acetylcholinesterase occurs at much higher concentrations of anaesthetic than for serum enzyme, this may depend on the environmental conditions under which propanidide has to act, or on acetylcholinesterase's high degree of specificity by comparison with pseudocholinesterase. Succinylcholine also inhibits serum cholinesterase but not plaque enzyme. With respect to the serum enzyme, the inhibition curve of the two drugs combined is similar to that of propanidide alone. The prolongation of apnoea observed during propanidide-induced anaesthesia, when succinylcholine is administered, does not appear to be due to diminution of serum cholinesterase activity but rather to interference of the two other drugs with neuromuscular transmission.

  9. [Neuromuscular disease: respiratory clinical assessment and follow-up].

    PubMed

    Martínez Carrasco, C; Villa Asensi, J R; Luna Paredes, M C; Osona Rodríguez de Torres, F B; Peña Zarza, J A; Larramona Carrera, H; Costa Colomer, J

    2014-10-01

    Patients with neuromuscular disease are an important group at risk of frequently suffering acute or chronic respiratory failure, which is their main cause of death. They require follow-up by a pediatric respiratory medicine specialist from birth or diagnosis in order to confirm the diagnosis and treat any respiratory complications within a multidisciplinary context. The ventilatory support and the cough assistance have improved the quality of life and long-term survival for many of these patients. In this paper, the authors review the pathophysiology, respiratory function evaluation, sleep disorders, and the most frequent respiratory complications in neuromuscular diseases. The various treatments used, from a respiratory medicine point of view, will be analyzed in a next paper. Copyright © 2013 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  10. Neuromuscular blockers in surgery and intensive care, Part 1.

    PubMed

    McManus, M C

    2001-12-01

    The historical development, pharmacology, pharmacodynamics, pharmacokinetics, clinical applications, pharmacologic basis for selection, adverse effects, and cost of neuromuscular blockers (NMBs) are discussed. The first NMB to be used was tubocurarine. During neurotransmission, acetylcholine is synthesized, stored in vesicles at the neuromuscular junction, released into the synapse, and bound to nicotinic receptors in the muscle end plate. For muscle contraction to occur, the impulse generated in a neuron's cell body must create an action potential that is chemically transmitted across the synapse. The postsynaptic nicotinic receptor at the neuromuscular junction is the major site of action of depolarizing and nondepolarizing NMBs. All NMBs have the potential for cross-reactivity at other nicotinic and muscarinic sites. Drug interactions most commonly occur between NMBs and inhalation anesthetics, certain antimicrobials, calcium-channel blockers, and anticholinesterases. When selecting an NMB, an agent's onset and duration of action must be considered. NMBs can be used on a short-term or long-term basis. Apart from cost, the choice of an NMB is made on the basis of its adverse-reaction profile, pharmacokinetics, and indications for use. Monitoring tools, their use, the rationale for their use, and the interpretation of the results they provide are unique. The patterns of peripheral nerve stimulation vary and elicit different characteristics of nondepolarizing neuromuscular blockade. The effectiveness of reversal agents is proportional to the degree of blockade. The mechanism of action of anticholinesterases involves inhibition of acetylcholinesterase. The expensive NMBs should be conserved for use in surgery, while the cheaper, long-acting [corrected] agents should be used in the intensive care unit. An understanding of the pharmacology, pharmacodynamics, and pharmacokinetics of NMBs will help health care providers gain expertise in the selection and use of these

  11. Neuromuscular blockers in surgery and intensive care, Part 2.

    PubMed

    McManus, M C

    2001-12-15

    The historical development, pharmacology, pharmacodynamics, pharmacokinetics, clinical applications, pharmacologic basis for selection, adverse effects, and cost of neuromuscular blockers (NMBs) are discussed. The first NMB to be used was tubocurarine. During neurotransmission, acetylcholine is synthesized, stored in vesicles at the neuromuscular junction, released into the synapse, and bound to nicotinic receptors in the muscle end plate. For muscle contraction to occur, the impulse generated in a neuron's cell body must create an action potential that is chemically transmitted across the synapse. The postsynaptic nicotinic receptor at the neuromuscular junction is the major site of action of depolarizing and nondepolarizing NMBs. All NMBs have the potential for cross-reactivity at other nicotinic and muscarinic sites. Drug interactions most commonly occur between NMBs and inhalation anesthetics, certain antimicrobials, calcium-channel blockers, and anticholinesterases. When selecting an NMB, an agent's onset and duration of action must be considered. NMBs can be used on a short-term or long-term basis. Apart from cost, the choice of an NMB is made on the basis of its adverse-reaction profile, pharmacokinetics, and indications for use. Monitoring tools, their use, the rationale for their use, and the interpretation of the results they provide are unique. The patterns of peripheral nerve stimulation vary and elicit different characteristics of nondepolarizing neuromuscular blockade. The effectiveness of reversal agents is proportional to the degree of blockade. The mechanism of action of anticholinesterases involves inhibition of acetylcholinesterase. The expensive NMBs should be conserved for use in surgery, while the cheaper, long-acting [corrected] agents should be used in the intensive care unit. An understanding of the pharmacology, pharmacodynamics, and pharmacokinetics of NMBs will help health care providers gain expertise in the selection and use of these

  12. Pruritus associated with onabotulinumtoxinA treatment of neuromuscular pain.

    PubMed

    Ho, Derek; Jagdeo, Jared

    2015-02-01

    OnabotulinumtoxinA is one of the most widely used agents for cosmetic and medical treatment. Studies have shown that onabotulinumtoxinA is safe and effective with minimal adverse events, and is often well tolerated by patients. We present a patient who developed neuropathic pruritus five days after treatment with onabotulinumtoxinA for neuromuscular pain. This case highlights the treatment of pruritus associated with onabotulinumtoxinA and the therapeutic method to resolve the patient's pruritus.

  13. Report on Adaptive Force, A Specific Neuromuscular Function

    PubMed Central

    Hoff, Marko; Heinke, Nancy; Bittmann, Frank

    2015-01-01

    In real life motions, as well as in sports, the adaptation of the neuromuscular systems to externally applied forces plays an important role. The term Adaptive Force (AF) shall characterize the ability of the nerve-muscle-system to adapt to impacting external forces during isometric and eccentric muscle action. The focus in this paper is on the concept of this neuromuscular action, which is not yet described in this way. A measuring system was constructed and evaluated for this specific neuromuscular function, but only the main information of the evaluation of the measuring system and the preliminary reference values are mentioned here, while an article with detailed description will be published separately. This paper concentrates on the three following points: 1) What is the peculiarity of this neuromuscular function, introduced as AF? 2) Is the measuring system able to capture its specific characteristics and which phases of measurement occur? 3) It seems reasonable to discuss if AF can be distinguished and classified among the known force concepts. The article describes the measuring system and how it is able to capture special features of real life motions like submaximal intensities and the subjects’ option to react adequately on external varying forces. Furthermore, within one measurement the system records three different force qualities: the isometric submaximal Adaptive Force (AFiso), the maximal isometric Adaptive Force (AFisomax) and the maximal eccentric Adaptive Force (AFeccmax). Each of these phases provide different and unique information on the nerve-muscle-system that are discussed in detail. Important, in terms of the Adaptive Force, seems to be the combination of conditional and coordinative abilities. This project was funded by the Federal Ministry of Economy and Technology (Project ZIM KF2262301FO9). PMID:26913155

  14. [Morphometric characteristics of neuromuscular spindles in hypertrophied skeletal muscle].

    PubMed

    Mytskan, B M; Mel'man, E P

    1986-11-01

    Skeletal muscle hypertrophy in young male rats was found to be accompanied by adaptive changes in neuromuscular spindles. The changes consisted in connective capsule thickening, increased diameter of NMS and intrafusal muscle fibers, expanded afferent and efferent nerve terminals, increased microcirculatory bed capacity. The quantitative and qualitative shifts observed in NMS structure are morphologically equivalent to the rise in their functional potential, which forms the basis for the functional changes in conditions of increasing skeletal muscle hypertrophy.

  15. Current status on electrodiagnostic standards and guidelines in neuromuscular disorders.

    PubMed

    Fuglsang-Frederiksen, Anders; Pugdahl, Kirsten

    2011-03-01

    The aim of this review is to present the status of electrodiagnostic standards and guidelines in neuromuscular disorders. Electrodiagnostic guidelines are developed on the background of medical technology assessment, wherefore a short presentation of medical technology assessment is given covering: (1) Evidence-based medicine, i.e. "to do the right thing", describing practice parameters and the STARD initiative which introduces evidence-based medicine in electrodiagnostic medicine, (2) Continuous quality improvement, i.e. "to do the thing right", describing variation among laboratories in methods and interpretation of tests, and the need for medical audit and implementation of electrodiagnostic guidelines, (3) Outcome studies, i.e. "is it worthwhile to do the right thing right?". In electrodiagnostic medicine there are very few outcome studies. Standards and guidelines described in the literature for different neuromuscular disorders are presented, often as figures or tables. These cover guidelines developed in detail for CIDP by expert consensus multicentre groups by AAN, INCAT, EFNS/PNS and for other inflammatory demyelinating neuropathies are described, as well as guidelines differentiating between demyelinating pathophysiology and axonal loss by motor and sensory nerve conduction studies. Furthermore, electrodiagnostic guidelines for ALS as detailed in the El Escorial, the modified El Escorial and the recent supplementary Awaji criteria are described and presented in a comprehensive table. Only few electrodiagnostic guidelines are published for nerve entrapment, cervical radiculopathy and neuromuscular transmission failure whereas none are known for myopathy. If no electrodiagnostic criteria for a given disorder exist, criteria for the electrodiagnostic examination are described if present. It is concluded that future research is needed in order to develop more electrodiagnostic guidelines in neuromuscular disorders by international expert consensus groups

  16. Fatty replacement of lower paraspinal muscles: normal and neuromuscular disorders

    SciTech Connect

    Hader, H.; Gadoth, N.; Heifetz, H.

    1983-11-01

    The physiologic replacement of the lower paraspinal muscles by fat was evaluated in 157 patients undergoing computed tomography for reasons unrelated to abnormalities of the locomotor system. Five patients with neuromuscular disorders were similarly evaluated. The changes were graded according to severity at three spinal levels: lower thoracic-upper lumbar, midlumbar, and lumbosacral. The results were analyzed in relation to age and gender. It was found that fatty replacement of paraspinal muscles is a normal age-progressive phenomenon most prominent in females. It progresses down the spine, being most advanced in the lumbosacral region. The severest changes in the five patients with neuromuscular disorders (three with poliomyelitis and two with progressive muscular dystrophy) consisted of complete muscle group replacement by fat. In postpoliomyelitis atrophy, the distribution was typically asymmetric and sometimes lacked clinical correlation. In muscular dystrophy, fatty replacement was symmetric, showing relative sparing of the psoas and multifidus muscles. In patients with neuromuscular diseases, computed tomography of muscles may be helpful in planning a better rehabilitation regimen.

  17. Neuromuscular electric stimulation in patellofemoral dysfunction: literature review

    PubMed Central

    dos Santos, Ricardo Lucas; Souza, Márcia Leal São Pedro; dos Santos, Fernanda Andrade

    2013-01-01

    Patellofemoral dysfunction is a fairly common deficiency among young individuals that primarily affects females and may be characterized by pain, swelling and retropatellar crepitation. The purpose of this review of literature from the period between 2005 and 2011 was to systematize knowledge in relation to the increase in quadriceps muscle strength and pain relief in patients with patellofemoral dysfunction, using neuromuscular electrical stimulation and resistance exercises. The inclusion criteria were intervention articles from the past six years, in English, Spanish and Portuguese, which used muscle strengthening and neuromuscular electrical stimulation for rehabilitation obtained through searches in the electronic databases Medline and Lilacs and in the Bireme library. The bibliographic search yielded 28 references, of which nine were excluded in accordance with the aims and inclusion criteria while 16 articles were selected for reading of the abstracts and subsequent analysis. Mediumfrequency Neuromuscular Electrical Stimulation (NMES) can be used in association with resistance exercises as an adjuvant in the treatment of patellofemoral dysfunction (PFD), both to achieve muscle rebalance and for pain relief. PMID:24453645

  18. Methods for Multiloop Identification of Visual and Neuromuscular Pilot Responses.

    PubMed

    Olivari, Mario; Nieuwenhuizen, Frank M; Venrooij, Joost; Bülthoff, Heinrich H; Pollini, Lorenzo

    2015-12-01

    In this paper, identification methods are proposed to estimate the neuromuscular and visual responses of a multiloop pilot model. A conventional and widely used technique for simultaneous identification of the neuromuscular and visual systems makes use of cross-spectral density estimates. This paper shows that this technique requires a specific noninterference hypothesis, often implicitly assumed, that may be difficult to meet during actual experimental designs. A mathematical justification of the necessity of the noninterference hypothesis is given. Furthermore, two methods are proposed that do not have the same limitations. The first method is based on autoregressive models with exogenous inputs, whereas the second one combines cross-spectral estimators with interpolation in the frequency domain. The two identification methods are validated by offline simulations and contrasted to the classic method. The results reveal that the classic method fails when the noninterference hypothesis is not fulfilled; on the contrary, the two proposed techniques give reliable estimates. Finally, the three identification methods are applied to experimental data from a closed-loop control task with pilots. The two proposed techniques give comparable estimates, different from those obtained by the classic method. The differences match those found with the simulations. Thus, the two identification methods provide a good alternative to the classic method and make it possible to simultaneously estimate human's neuromuscular and visual responses in cases where the classic method fails.

  19. Role of Radiologic Imaging in Genetic and Acquired Neuromuscular Disorders

    PubMed Central

    Zanato, Riccardo; Coran, Alessandro; Beltrame, Valeria; Stramare, Roberto

    2015-01-01

    Great technologic and clinical progress have been made in the last two decades in identifying genetic defects of several neuromuscular diseases, as Spinal Muscular Atrophy, genetic muscular dystrophies and other genetic myopathies. The diagnosis is usually challenging, due to great variability in genetic abnormalities and clinical phenotypes and the poor specificity of complementary analyses, i.e., serum creatine kinase (CK) and electrophysiology. Muscle biopsy represents the gold standard for the diagnosis of genetic neuromuscular diseases, but clinical imaging of muscle tissue is an important diagnostic tool to identify and quantifyies muscle damage. Radiologic imaging is, indeed, increasingly used as a diagnostic tool to describe patterns and the extent of muscle involvement, thanks to modern techniques that enable to definethe definition of degrees of muscle atrophy and changes in connective tissue. They usually grade the severity of the disease process with greater accuracy than clinical scores. Clinical imaging is more than complementary to perform muscle biopsy, especially as ultrasound scans are often mandatory to identify the muscle to be biopsied. We will here detail and provideWe will herein provide detailed examples of the radiologic methods that can be used in genetic and acquired neuromuscular disorders, stressing pros and cons. Key Words: Muscle Imaging, MRI, CT, genetic muscle disorders, myopathies, dystrophies PMID:26913153

  20. Defective Membrane Remodeling in Neuromuscular Diseases: Insights from Animal Models

    PubMed Central

    Muller, Jean; Laporte, Jocelyn

    2012-01-01

    Proteins involved in membrane remodeling play an essential role in a plethora of cell functions including endocytosis and intracellular transport. Defects in several of them lead to human diseases. Myotubularins, amphiphysins, and dynamins are all proteins implicated in membrane trafficking and/or remodeling. Mutations in myotubularin, amphiphysin 2 (BIN1), and dynamin 2 lead to different forms of centronuclear myopathy, while mutations in myotubularin-related proteins cause Charcot-Marie-Tooth neuropathies. In addition to centronuclear myopathy, dynamin 2 is also mutated in a dominant form of Charcot-Marie-Tooth neuropathy. While several proteins from these different families are implicated in similar diseases, mutations in close homologues or in the same protein in the case of dynamin 2 lead to diseases affecting different tissues. This suggests (1) a common molecular pathway underlying these different neuromuscular diseases, and (2) tissue-specific regulation of these proteins. This review discusses the pathophysiology of the related neuromuscular diseases on the basis of animal models developed for proteins of the myotubularin, amphiphysin, and dynamin families. A better understanding of the common mechanisms between these neuromuscular disorders will lead to more specific health care and therapeutic approaches. PMID:22496665

  1. Vibration or balance training on neuromuscular performance in osteopenic women.

    PubMed

    Stolzenberg, N; Belavý, D L; Rawer, R; Felsenberg, D

    2013-11-01

    Maintaining neuromuscular function in older age is an important topic for aging societies, especially for older women with low bone density who may be at risk of falls and bone fracture. This randomized controlled trial investigated the effect of resistive exercise with either whole-body vibration training (VIB) or coordination/balance training (BAL) on neuromuscular function (countermovement jump, multiple 1-leg hopping, sit-to-stand test). 68 postmenopausal women with osteopenia or osteoporosis were recruited for the study. 57 subjects completed the 9-month, twice weekly, intervention period. All subjects conducted 30 min of resistance exercise each training day. The VIB-group performed additional training on the Galileo vibration exercise device. The BAL-group performed balance training. An "intent-to-treat" analysis showed greater improvement in the VIB-group for peak countermovement power (p=0.004). The mean [95% confidence interval] effect size for this parameter was a  + 0.9[0.3 to 1.5] W/kg greater change in VIB than BAL after 9 months. In multiple 1-leg hopping, a significantly better performance in the VIB-group after the intervention period was seen on a "per-protocol" analysis only. Both groups improved in the sit-to-stand test. The current study provides evidence that short-duration whole-body vibration exercise can have a greater impact on some aspects of neuromuscular function in post-menopausal women with low bone density than proprioceptive training.

  2. Neuromuscular interactions around the knee in children, adults and elderly

    PubMed Central

    Kellis, Eleftherios; Mademli, Lida; Patikas, Dimitrios; Kofotolis, Nikolaos

    2014-01-01

    Although injury and neuromuscular activation patterns may be common for all individuals, there are certain factors which differentiate neuromuscular activity responses between children, adults and elderly. The purpose of this study is to review recent evidence on age differences in neural activation and muscle balances around the knee when performing single joint movements. Particularly, current evidence indicates that there are some interesting similarities in the neuromuscular mechanisms by which children or the elderly differ compared with adults. Both children and elderly display a lower absolute muscle strength capacity than adults which cannot fully be explained by differences in muscle mass. Quadriceps activation failure is a common symptom of all knee injuries, irrespective of age but it is likely that its effect is more evident in children or adults. While one might expect that antagonist co-activation would differ between age categories, it appears that this is not the case. Although hamstring: quadriceps ratio levels are altered after knee injury, it is not clear whether this is an age specific response. Finally, evidence suggests that both children and the elderly display less stiffness of the quadriceps muscle-tendon unit than adults which affects their knee joint function. PMID:25232523

  3. Development and plasticity of the Drosophila larval neuromuscular junction.

    PubMed

    Menon, Kaushiki P; Carrillo, Robert A; Zinn, Kai

    2013-01-01

    The Drosophila larval neuromuscular system is relatively simple, containing only 32 motor neurons in each abdominal hemisegment, and its neuromuscular junctions (NMJs) have been studied extensively. NMJ synapses exhibit developmental and functional plasticity while displaying stereotyped connectivity. Drosophila Type I NMJ synapses are glutamatergic, while the vertebrate NMJ uses acetylcholine as its primary neurotransmitter. The larval NMJ synapses use ionotropic glutamate receptors (GluRs) that are homologous to AMPA-type GluRs in the mammalian brain, and they have postsynaptic scaffolds that resemble those found in mammalian postsynaptic densities. These features make the Drosophila neuromuscular system an excellent genetic model for the study of excitatory synapses in the mammalian central nervous system. The first section of the review presents an overview of NMJ development. The second section describes genes that regulate NMJ development, including: (1) genes that positively and negatively regulate growth of the NMJ, (2) genes required for maintenance of NMJ bouton structure, (3) genes that modulate neuronal activity and alter NMJ growth, (4) genes involved in transsynaptic signaling at the NMJ. The third section describes genes that regulate acute plasticity, focusing on translational regulatory mechanisms. As this review is intended for a developmental biology audience, it does not cover NMJ electrophysiology in detail, and does not review genes for which mutations produce only electrophysiological but no structural phenotypes. Copyright © 2013 Wiley Periodicals, Inc.

  4. Adiposity, physical activity and neuromuscular performance in children.

    PubMed

    Haapala, Eero A; Väistö, Juuso; Lintu, Niina; Tompuri, Tuomo; Brage, Soren; Westgate, Kate; Ekelund, Ulf; Lampinen, Eeva-Kaarina; Sääkslahti, Arja; Lindi, Virpi; Lakka, Timo A

    2016-09-01

    We investigated the associations of body fat percentage (BF%), objectively assessed moderate-to-vigorous physical activity (MVPA) and different types of physical activity assessed by a questionnaire with neuromuscular performance. The participants were 404 children aged 6-8 years. BF% was assessed using dual-energy x-ray absorptiometry and physical activity by combined heart rate and movement sensing and a questionnaire. The results of 50-m shuttle run, 15-m sprint run, hand grip strength, standing long jump, sit-up, modified flamingo balance, box-and-block and sit-and-reach tests were used as measures of neuromuscular performance. Children who had a combination of higher BF% and lower levels of physical activity had the poorest performance in 50-m shuttle run, 15-m sprint run and standing long jump tests. Higher BF% was associated with slower 50-m shuttle run and 15-m sprint times, shorter distance jumped in standing long jump test, fewer sit-ups, more errors in balance test and less cubes moved in box-and-block test. Higher levels of physical activity and particularly MVPA assessed objectively by combined accelerometer and heart rate monitor were related to shorter 50-m shuttle run and 15-m sprint times. In conclusion, higher BF% and lower levels of physical activity and particularly the combination of these two factors were associated with worse neuromuscular performance.

  5. A biodynamic feedthrough model based on neuromuscular principles.

    PubMed

    Venrooij, Joost; Abbink, David A; Mulder, Mark; van Paassen, Marinus M; Mulder, Max; van der Helm, Frans C T; Bulthoff, Heinrich H

    2014-07-01

    A biodynamic feedthrough (BDFT) model is proposed that describes how vehicle accelerations feed through the human body, causing involuntary limb motions and so involuntary control inputs. BDFT dynamics strongly depend on limb dynamics, which can vary between persons (between-subject variability), but also within one person over time, e.g., due to the control task performed (within-subject variability). The proposed BDFT model is based on physical neuromuscular principles and is derived from an established admittance model-describing limb dynamics-which was extended to include control device dynamics and account for acceleration effects. The resulting BDFT model serves primarily the purpose of increasing the understanding of the relationship between neuromuscular admittance and biodynamic feedthrough. An added advantage of the proposed model is that its parameters can be estimated using a two-stage approach, making the parameter estimation more robust, as the procedure is largely based on the well documented procedure required for the admittance model. To estimate the parameter values of the BDFT model, data are used from an experiment in which both neuromuscular admittance and biodynamic feedthrough are measured. The quality of the BDFT model is evaluated in the frequency and time domain. Results provide strong evidence that the BDFT model and the proposed method of parameter estimation put forward in this paper allows for accurate BDFT modeling across different subjects (accounting for between-subject variability) and across control tasks (accounting for within-subject variability).

  6. Oxidative stress disrupts purinergic neuromuscular transmission in the inflamed colon

    PubMed Central

    Roberts, Jane A; Durnin, Leonie; Sharkey, Keith A; Mutafova-Yambolieva, Violeta N; Mawe, Gary M

    2013-01-01

    Colitis, induced by trinitrobenzene sulfonic acid (TNBS) in guinea pig, leads to decreased purinergic neuromuscular transmission resulting in a reduction in inhibitory junction potentials (IJPs) in colonic circular muscle. We explored possible mechanisms responsible for this inflammation-induced neurotransmitter plasticity. Previous studies have suggested that the deficit in inflamed tissue involves decreased ATP release. We therefore hypothesized that decreased purinergic transmission results from inflammation-induced free radical damage to mitochondria, leading to decreased purine synthesis and release. Stimulus-induced release of purines was measured using high-performance liquid chromatography, and quantities of all purines measured were significantly reduced in the inflamed colons as compared to controls. To test whether decreased mitochondrial function affects the IJP, colonic muscularis preparations were treated with the mitochondrial ATP synthase inhibitors oligomycin or dicyclohexylcarbodiimide, which resulted in a significant reduction of IJP amplitude. Induction of oxidative stress in vitro, by addition of H2O2 to the preparation, also significantly reduced IJP amplitude. Purinergic neuromuscular transmission was significantly restored in TNBS-inflamed guinea pigs, and in dextran sodium sulfate-inflamed mice, treated with a free radical scavenger. Furthermore, propulsive motility in the distal colons of guinea pigs with TNBS colitis was improved by in vivo treatment with the free radical scavenger. We conclude that oxidative stress contributes to the reduction in purinergic neuromuscular transmission measured in animal models of colitis, and that these changes can be prevented by treatment with a free radical scavenger, resulting in improved motility. PMID:23732648

  7. Muscle fiber hypotrophy with intact neuromuscular junctions. A study of a patient with congenital neuromuscular disease and ophthalmoplegia.

    PubMed

    Bender, A N; Bender, M B

    1977-03-01

    An infant born with severe but nonprogressive somatic and cranial muscle weakness including bilateral external ophthalmoplegia was studied with a motor-point muscle biopsy. There was a strinking generalized decrease in the size of muscle fibers (hypotrophy), most marked in the type I fibers. Many of the small fibers were immature, resembling myotubes. Neuromuscular junctions on severely hypotrophic fibers were normal with esterase staining and by ultrastructural criteria. Although these are unusual clinical and biopsy characteristics, this infant's condition bears a resemblance to two other congenital nonprogressive neuromuscular diseases:myotubular myopathy and congenital fiber type disproportion. In these conditions and in our patient, there is no primary degenerative process affecting nerve or muscle but, rather, an apparent lack of maturation of fetal muscle fibers, indicating a defective normal trophic interaction between nerve and muscle.

  8. Role of amino acid infusion in delayed recovery from neuromuscular blockers.

    PubMed

    Kalra, Seema; Wadhwa, Rachna

    2010-03-01

    This case report highlights the anaesthetic management of a patient who had residual muscle paralysis following neuromuscular blockade, which was attributed to hypothermia and corrected by administration of amino acid solution. The various causes of residual neuromuscular blockade should be considered when treating such a patient. Amino acid infusion has been found to hasten the recovery from neuromuscular block due to vecuronium bromide aggravated by hypothermia.

  9. Feasibility of neuromuscular electrical stimulation in critically ill patients.

    PubMed

    Segers, Johan; Hermans, Greet; Bruyninckx, Frans; Meyfroidt, Geert; Langer, Daniel; Gosselink, Rik

    2014-12-01

    Critically ill patients often develop intensive care unit-acquired weakness. Reduction in muscle mass and muscle strength occurs early after admission to the intensive care unit (ICU). Although early active muscle training could attenuate this intensive care unit-acquired weakness, in the early phase of critical illness, a large proportion of patients are unable to participate in any active mobilization. Neuromuscular electrical stimulation (NMES) could be an alternative strategy for muscle training. The aim of this study was to investigate the safety and feasibility of NMES in critically ill patients. This is an observational study. The setting is in the medical and surgical ICUs of a tertiary referral university hospital. Fifty patients with a prognosticated prolonged stay of at least 6 days were included on day 3 to 5 of their ICU stay. Patients with preexisting neuromuscular disorders and patients with musculoskeletal conditions limiting quadriceps contraction were excluded. Twenty-five minutes of simultaneous bilateral NMES of the quadriceps femoris muscle. This intervention was performed 5 days per week (Monday-Friday). Effective muscle stimulation was defined as a palpable and visible contraction (partial or full muscle bulk). The following parameters, potentially affecting contraction upon NMES, were assessed: functional status before admission to the ICU (Barthel index), type and severity of illness (Acute Physiology And Chronic Health Evaluation II score and sepsis), treatments possibly influencing the muscle contraction (corticosteroids, vasopressors, inotropes, aminoglycosides, and neuromuscular blocking agents), level of consciousness (Glasgow Coma Scale, score on 5 standardized questions evaluating awakening, and sedation agitation scale), characteristics of stimulation (intensity of the NMES, number of sessions per patient, and edema), and neuromuscular electrophysiologic characteristics. Changes in heart rate, blood pressure, oxygen saturation

  10. Neuromuscular control of trunk stability: clinical implications for sports injury prevention.

    PubMed

    Zazulak, Bohdanna; Cholewicki, Jacek; Reeves, N Peter

    2008-09-01

    Recent prospective evidence supports the hypothesis that impaired trunk control is a contributing factor to sports injuries of the spine as well as to segments of the kinetic chain. The current concepts regarding neuromuscular control of trunk stability are best described from a systems engineering perspective. In the analysis of current neuromuscular training protocols for sports injury prevention, these principles are applied to identify components that optimize neuromuscular control of trunk stability. Current perspectives of neuromuscular learning can be applied clinically to aid in the formulation of injury prevention strategies.

  11. Neuromuscular complexity during gait is not responsive to medication in persons with Parkinson's disease.

    PubMed

    Roemmich, Ryan T; Fregly, Benjamin J; Hass, Chris J

    2014-09-01

    The purpose of this study was to investigate the effects of dopaminergic therapy on neuromuscular complexity during gait and on the relationship between neuromuscular complexity and gait speed in persons with Parkinson's disease (PD). Nine persons with PD walked at self-selected speed for 5 min after having withdrawn from dopaminergic medication for at least 12 h and while optimally-medicated. Electromyographic recordings were taken from eight leg muscles bilaterally. Non-negative matrix factorization was applied to reduce the dimensionality of the electromyographic signals into motor modules. We assessed neuromuscular complexity by investigating the number, structure, and timing of the modules. We also investigated the influence of dopaminergic medication on the relationships between neuromuscular complexity and gait speed. Though gait speed increased significantly after medication intake, medication did not affect neuromuscular complexity. Neuromuscular complexity was significantly associated with gait speed only while the participants were medicated. Thus, the supraspinal structures that govern neuromuscular complexity during gait do not appear to be solely dopaminergically-influenced in PD. The lack of dopaminergic influence on neuromuscular complexity may explain why persons with PD exhibit gait slowness even while medicated, and an intervention that restores neuromuscular complexity may result in gait speed improvement in PD.

  12. A case series of re-establishment of neuromuscular block with rocuronium after sugammadex reversal.

    PubMed

    Iwasaki, Hajime; Sasakawa, Tomoki; Takahoko, Kenichi; Takagi, Shunichi; Nakatsuka, Hideki; Suzuki, Takahiro; Iwasaki, Hiroshi

    2016-06-01

    We report the use of rocuronium to re-establish neuromuscular block after reversal with sugammadex. The aim of this study was to investigate the relationship between the dose of rocuronium needed to re-establish neuromuscular block and the time interval between sugammadex administration and re-administration of rocuronium. Patients who required re-establishment of neuromuscular block within 12 h after the reversal of rocuronium-induced neuromuscular block with sugammadex were included. After inducing general anesthesia and placing the neuromuscular monitor, the protocol to re-establish neuromuscular block was as follows. An initial rocuronium dose of 0.6 mg/kg was followed by additional 0.3 mg/kg doses every 2 min until train-of-four responses were abolished. A total of 11 patients were enrolled in this study. Intervals between sugammadex and second rocuronium were 12-465 min. Total dose of rocuronium needed to re-establish neuromuscular block was 0.6-1.2 mg/kg. 0.6 mg/kg rocuronium re-established neuromuscular block in all patients who received initial sugammadex more than 3 h previously. However, when the interval between sugammadex and second rocuronium was less than 2 h, more than 0.6 mg/kg rocuronium was necessary to re-establish neuromuscular block.

  13. Effects on posture by different neuromuscular afferent stimulations and proprioceptive insoles: Rasterstereographic evaluation.

    PubMed

    Dankerl, Peter; Keller, Andrea Kerstin; Häberle, Lothar; Stumptner, Thomas; Pfaff, Gregor; Uder, Michael; Forst, Raimund

    2016-06-01

    Proprioceptive neuromuscular stimulating insoles are increasingly applied in treating functional complaints, chronic pain, foot disorders and so on. To evaluate rasterstereography as a tool in objectifying postural changes resulting from neuromuscular afferent stimulation and proprioceptive neuromuscular stimulating insoles and to compare the respective effects on posture. This is a prospective experimental study. A total of 27 healthy volunteers were consecutively exposed to six different varying intense neuromuscular afferent stimulating test conditions at three different times. One test condition featured proprioceptive neuromuscular stimulating insoles. In each test condition, a sequence of 12 rasterstereographic recordings of back shape was documented. Changes between six different test conditions and over time for 14 posture characterising parameters were investigated, for example, trunk inclination, pelvic torsion, lateral deviation of the spine's amplitude or sagittal spinal curve. Standard deviation of our rasterstereographic measurements (±2.67 mm) was better than in most comparable reference values. Different neuromuscular stimuli were found to provoke significant changes to various posture parameters, including trunk inclination, pelvic torsion and so on ( each p < 0.001, F-tests). Proprioceptive neuromuscular stimulating insoles induced significant changes for parameter lateral deviation of the spine's amplitude (p = 0.03). Neuromuscular afferent stimulation and proprioceptive neuromuscular stimulating insoles induce postural changes, which can be detected reliably by rasterstereography. We demonstrated that rasterstereography - a radiation-free imaging modality - enables visualisation and documentation of subtle postural changes induced by varying intense neuromuscular afferent stimulation and the application of proprioceptive neuromuscular stimulating insoles. © The International Society for Prosthetics and Orthotics 2014.

  14. Use of Single Fiber Electromyographic Jitter to Detect Acute Changes in Neuromuscular Function in Young and Adult Rats

    EPA Science Inventory

    INTRODUCTION: Exposure to irreversible cholinesterase (ChE)-inhibiting compounds, such as organophosphates may produce neuromuscular dysfunction. However, less is known about changes in neuromuscular transmission after treatment with reversible ChE-inhibitors. These studies adapt...

  15. Clinical use of creatine in neuromuscular and neurometabolic disorders.

    PubMed

    Tarnopolsky, Mark A

    2007-01-01

    Many of the neuromuscular (e.g., muscular dystrophy) and neurometabolic (e.g., mitochondrial cytopathies) disorders share similar final common pathways of cellular dysfunction that may be favorably influenced by creatine monohydrate (CrM) supplementation. Studies using the mdx model of Duchenne muscular dystrophy have found evidence of enhanced mitochondrial function, reduced intra-cellular calcium and improved performance with CrM supplementation. Clinical trials in patients with Duchenne and Becker's muscular dystrophy have shown improved function, fat-free mass, and some evidence of improved bone health with CrM supplementation. In contrast, the improvements in function in myotonic dystrophy and inherited neuropathies (e.g., Charcot-Marie-Tooth) have not been significant. Some studies in patients with mitochondrial cytopathies have shown improved muscle endurance and body composition, yet other studies did not find significant improvements in patients with mitochondrial cytopathy. Lower-dose CrM supplementation in patients with McArdle's disease (myophosphorylase deficiency) improved exercise capacity, yet higher doses actually showed some indication of worsened function. Based upon known cellular pathologies, there are potential benefits from CrM supplementation in patients with steroid myopathy, inflammatory myopathy, myoadenylate deaminase deficiency, and fatty acid oxidation defects. Larger randomized control trials (RCT) using homogeneous patient groups and objective and clinically relevant outcome variables are needed to determine whether creatine supplementation will be of therapeutic benefit to patients with neuromuscular or neurometabolic disorders. Given the relatively low prevalence of some of the neuromuscular and neurometabolic disorders, it will be necessary to use surrogate markers of potential clinical efficacy including markers of oxidative stress, cellular energy charge, and gene expression patterns.

  16. Neuromuscular responses to simulated brazilian jiu-jitsu fights.

    PubMed

    da Silva, Bruno Victor Corrêa; Ide, Bernardo Neme; de Moura Simim, Mário Antônio; Marocolo, Moacir; da Mota, Gustavo Ribeiro

    2014-12-09

    The aim of this study was to investigate the neuromuscular performance responses following successive Brazilian Jiu-Jitsu (BJJ) fights. Twenty-three BJJ athletes (age: 26.3 ± 6.3 years; body mass: 79.4 ± 9.7 kg; body height: 1.80 ± 0.1 m) undertook 3 simulated BJJ fights (10 min duration each separated by 15 min of rest). Neuromuscular performance was measured by the bench press throw (BPT) and vertical counter movement jump (VCMJ) tests, assessed before the 1st fight (Pre) and after the last one (Post). Blood lactate (LA) was measured at Pre, 1 min Post, and 15 min Post fights. Paired t-tests were employed in order to compare the BPT and VCMJ results. One-way ANOVA with Bonferroni post hoc tests were utilized to compare LA responses. The results revealed a significant (p < 0.05) increase in VCMJ performance (40.8 ± 5.5 cm Pre vs. 42.0 ± 5.8 cm Post), but no significant changes in the BPT (814 ± 167 W Pre vs. 835 ± 213 W Post) were observed. LA concentration increased significantly (p < 0.05) at Post, both in the 1st min (10.4 ± 2.7 mmol L-1) and the 15th min (6.4 ± 2.5 mmol L-1) of recovery. We concluded that successive simulated BJJ fights demanded considerable anaerobic contribution of ATP supply, reinforcing the high-intensity intermittent nature of the sport. Nevertheless, no negative impact on acute neuromuscular performance (power) was observed.

  17. Case report: Neuromuscular block induced by rocuronium following sugammadex administration.

    PubMed

    Askin, Tugba; Unver, Suheyla; Oguz, Deniz; Kutay, Kubra

    2017-02-01

    We present a case in which rocuronium was applied for muscle relaxation following the administration of sugammadex. An emergency surgery under general anesthesia was planned for a 43-year-old male patient due to an L1 vertebral corpus and right tibia-fibula shaft fracture. Anesthesia was induced with fentanyl, propofol and lidocaine. After applying only 30mg of the total induction dose of rocuronium, it was learned that the neurological examination should be controlled again from the surgeon because of the controversial of the neurological deficit. As a result, patient awakened from anesthesia. We administered 2mg/kg sugammadex and spontaneous breathing of patient returned immediately. The patient became conscious and orientated immediately afterwards. The neurological examination of the lower extremities was performed. The patient was anesthetized once again and 0.6mg/kg rocuronium was given in order to gain neoromuscular block approximately 10min after sugammadex administration. 2min later, the patient was smoothly intubated. Neuromuscular monitorization was not used because of emergency. We administered 2mg/kg sugammadex at the end of the procedure and the patient was extubated. The most suitable time for the re-establishment of rocuronium following sugammadex is currently unclear. This case showed that neuromuscular block can be effectively re-induced by rocuronium following the reversal of rocuronium-induced neuromuscular block with sugammadex. In this case, we consider that the ability to effectively reuse normal induction doses of rocuronium is an important clinical observation. Copyright © 2016 Elsevier Inc. All rights reserved.

  18. Neuromuscular adaptations to isoload versus isokinetic eccentric resistance training.

    PubMed

    Guilhem, Gaël; Cornu, Christophe; Maffiuletti, Nicola A; Guével, Arnaud

    2013-02-01

    The purpose of this study was to compare neuromuscular adaptations induced by work-matched isoload (IL) versus isokinetic (IK) eccentric resistance training. A total of 31 healthy subjects completed a 9-wk IL (n = 11) or IK (n = 10) training program for the knee extensors or did not train (control group; n = 10). The IL and IK programs consisted of 20 training sessions, which entailed three to five sets of eight repetitions in the respective modalities. The amount of work and the mean angular velocity were strictly matched between IL and IK conditions. Neuromuscular tests were performed before and after training and consisted of the assessment of quadriceps muscle strength, muscle architecture (vastus lateralis), EMG activity, and antagonist coactivation. IL, but not IK, eccentric resistance training enhanced eccentric strength at short muscle length (+20%), high-velocity eccentric strength (+15%), muscle thickness (+10%), and fascicle angle measured at rest (+11%; P < 0.05). Agonist EMG activity increased almost similarly for the two modalities, whereas antagonist coactivation was unaffected by training. IL proved to be more effective than IK training for improving quadriceps muscle strength and structure. It is conjectured that the rapid acceleration of the load in the early phase of IL eccentric movements (i.e., at short muscle lengths), which results in greater torque and angular velocities compared with IK actions, is the main determinant of strength and neuromuscular adaptations to eccentric training. These findings have important consequences for the optimization of IL and IK eccentric exercise for resistance training and rehabilitation purposes.

  19. Neuromuscular Changes and Damage after Isoload versus Isokinetic Eccentric Exercise.

    PubMed

    Doguet, Valentin; Nosaka, Kazunori; Plautard, Mathieu; Gross, Raphaël; Guilhem, GaËL; Guével, Arnaud; Jubeau, Marc

    2016-12-01

    This study compared the effects of isoload (IL) and isokinetic (IK) knee extensor eccentric exercises on changes in muscle damage and neuromuscular parameters to test the hypothesis that the changes would be different after IL and IK exercises. Twenty-two young men were paired based on their strength and placed in the IL (N = 11) or the IK (N = 11) group. The IL group performed 15 sets of 10 eccentric contractions with a 150% of predetermined one-repetition maximum load. The IK group performed 15 sets of several maximal eccentric contractions matched set by set for the total amount of work and mean angular velocity with the IL group. Muscle damage markers (voluntary isometric peak torque, muscle soreness, and creatine kinase activity) and neuromuscular variables (e.g., voluntary activation, H-reflex, M-wave, and evoked torque) were measured before, immediately after, and 24, 48, 72, and 96 h postexercise. Voluntary isometric peak torque decreased to the same extent (P = 0.94) in both groups immediately after (IL = -40.6% ± 13.8% vs IK = -42.4% ± 10.2%) to 96 h after the exercise (IL = -21.8% ± 28.5% vs IK = -26.7% ± 23.5%). Neither peak muscle soreness (IL = 48.1 ± 28.2 mm vs IK = 54.7 ± 28.9 mm, P = 0.57) nor creatine kinase activity (IL = 12,811 ± 22,654 U·L vs IK = 15,304 ± 24,739 U·L, P = 0.59) significantly differed between groups. H-reflex (IL = -23% vs IK = -35%) and M-wave (IL = -10% vs IK = -17%) significantly decreased immediately postexercise similarly between groups. The changes in muscle damage and neuromuscular function after the exercise are similar between IL and IK, suggesting that resistance modality has little effects on acute muscle responses.

  20. Neuromuscular activity of Bothrops fonsecai snake venom in vertebrate preparations

    PubMed Central

    Fernandes, Carla T; Giaretta, Vânia MA; Prudêncio, Luiz S; Toledo, Edvana O; da Silva, Igor RF; Collaço, Rita CO; Barbosa, Ana M; Hyslop, Stephen; Rodrigues-Simioni, Léa; Cogo, José C

    2014-01-01

    The neuromuscular activity of venom from Bothrops fonsecai, a lancehead endemic to southeastern Brazil, was investigated. Chick biventer cervicis (CBC) and mouse phrenic nerve-diaphragm (PND) preparations were used for myographic recordings and mouse diaphragm muscle was used for membrane resting potential (RP) and miniature end-plate potential (MEPP) recordings. Creatine kinase release and muscle damage were also assessed. In CBC, venom (40, 80 and 160μg/ml) produced concentration- and time-dependent neuromuscular blockade (50% blockade in 85±9 min and 73±8 min with 80 and 160μg/ml, respectively) and attenuated the contractures to 110μM ACh (78–100% inhibition) and 40mM KCl (45–90% inhibition). The venom-induced decrease in twitch-tension in curarized, directly-stimulated preparations was similar to that in indirectly stimulated preparations. Venom (100 and 200μg/ml) also caused blockade in PND preparations (50% blockade in 94±13 min and 49±8 min with 100 and 200μg/ml, respectively) but did not alter the RP or MEPP amplitude. In CBC, venom caused creatine kinase release and myonecrosis. The venom-induced decrease in twitch-tension and in the contractures to ACh and K+ were abolished by preincubating venom with commercial antivenom. These findings indicate that Bothrops fonsecai venom interferes with neuromuscular transmission essentially through postsynaptic muscle damage that affects responses to ACh and KCl. These actions are effectively prevented by commercial antivenom. PMID:25028603

  1. CHO Mouth Rinsing Improves Neuromuscular Performance During Isokinetic Fatiguing Exercise.

    PubMed

    Bazzucchi, Ilenia; Patrizio, Federica; Felici, Francesco; Nicolò, Andrea; Sacchetti, Massimo

    2016-12-14

    To determine whether repeated CHO mouth rinsing would improve neuromuscular performance during high intensity fatiguing contractions. 18 young men (26.1±5.0 yr; 22.9±1,9 BMI) performed 3 maximal voluntary isometric contractions (MVICPRE). Immediately after, they completed 10 s mouth rinse with either 6.4% maltodextrine solution [MAL] or 7.1% glucose solution [GLU] or water [W] or artificially sweetened solution [PLA] or a control trial with no rinse [CON] in a cross over protocol. 5 sets of 30 isokinetic fatiguing contractions at 180°s(-1) and a MVICPOST with their elbow flexors were performed after each mouth rinse. Mechanical and electromyographic signals (EMG) were recorded from the biceps brachii muscle and parameters of interest analyzed. 1) When rinsing the mouth with a solution containing CHO, independently of the sweetness, isokinetic performance was enhanced as shown by the greater total work achieved in comparison with CON; 2) the decay of torque and mean fiber conduction velocity (MFCV) recorded at the end of the fatiguing task was lower when rinsing the mouth with GLU compared to CON; 3) the torque recorded during the MVICPOST was greater with CHO with respect to CON and this was associated to a lower decay of MFCV. CHO mouth rinse counteracts the fatigue-induced decline in neuromuscular performance, supporting the notion that CHO rinse may activate positive afferent signals able to modify the motor output. Repeated mouth rinsing with sweet and non-sweet CHO-containing solutions can improve neuromuscular performance during an isokinetic intermittent fatiguing task.

  2. Neuromuscular pharmacodynamics of mivacurium in adults with major burns

    PubMed Central

    Han, T.-H.; Martyn, J. A. J.

    2011-01-01

    Background Mivacurium is metabolized by plasma pseudocholinesterase (PChE) enzyme, which is decreased in burns. We tested whether the decreased metabolism of mivacurium due to decreased PChE activity can overcome the pharmacodynamic resistance to non-depolarizing relaxants previously seen in major burns. Methods Thirty adults with 35 (13)% [mean (sd)] burn were studied at 5–91 post-burn days and 31 non-burns matched controls. Mivacurium 0.2 mg kg−1 was administered as a single bolus. Neuromuscular block was monitored with single-twitch response using TOF-Watch™. Onset time (drug administration to maximal twitch suppression) and spontaneous recovery were measured. Results Onset time was significantly prolonged in burns when compared with non-burns (115 vs 90 s; P<0.001). The PChE levels were lower in burns [1432 (916) vs 2866 (731) IU litre−1; P<0.001] and the neuromuscular recovery to 50% of baseline twitch height was prolonged in burns (41 vs 26 min; P<0.001). There was a significant correlation between PChE and time to 50% recovery for the whole group together (r=−0.6; P<0.001). The dibucaine numbers were not different. Conclusions The prolonged onset time suggests resistance to neuromuscular effects, whereas the prolonged recovery suggests increased sensitivity. This divergent response can be explained by qualitative and quantitative changes in acetylcholine receptor expression causing resistance and decreased PChE activity causing sensitivity. Despite using a relatively large dose of mivacurium (0.2 mg kg−1) in the presence of decreased PChE levels, this did not overcome the resistance resulting from up-regulated receptors. PMID:21354998

  3. Neuromuscular performance of explosive power athletes versus untrained individuals.

    PubMed

    Tillin, Neale A; Jimenez-Reyes, Pedro; Pain, Matthew T G; Folland, Jonathan P

    2010-04-01

    Electromechanical delay (EMD) and rate of force development (RFD) are determinants of explosive neuromuscular performance. We may expect a contrast in EMD and RFD between explosive power athletes, who have a demonstrable ability for explosive contractions, and untrained individuals. However, comparison and the neuromuscular mechanisms for any differences have not been studied. The neuromuscular performance of explosive power athletes (n = 9) and untrained controls (n = 10) was assessed during a series of twitch, tetanic, explosive, and maximum voluntary isometric knee extensions. Knee extension force and EMG of the superficial quadriceps were measured in three 50-ms time windows from their onset and were normalized to strength and maximal M-wave (Mmax), respectively. Involuntary and voluntary EMD were determined from twitch and explosive voluntary contractions, respectively, and were similar for both groups. The athletes were 28% stronger, and their absolute RFD in the first 50 ms was twofold that of controls. Athletes had greater normalized RFD (4.86 ± 1.46 vs 2.81 ± 1.20 MVC·s(-1)) and neural activation (mean quadriceps, 0.26 ± 0.07 vs 0.15 ± 0.06 Mmax) during the first 50 ms of explosive voluntary contractions. Surprisingly, the controls had a greater normalized RFD in the second 50 ms (6.68 ± 0.92 vs 7.93 ± 1.11 MVC·s-1) and a greater change in EMG preceding this period. However, there were no differences in the twitch response or normalized tetanic RFD between groups. The differences in voluntary normalized RFD between athletes and controls were explained by agonist muscle neural activation and not by the similar intrinsic contractile properties of the groups.

  4. Antivenom for Neuromuscular Paralysis Resulting From Snake Envenoming

    PubMed Central

    Silva, Anjana; Hodgson, Wayne C.; Isbister, Geoffrey K.

    2017-01-01

    Antivenom therapy is currently the standard practice for treating neuromuscular dysfunction in snake envenoming. We reviewed the clinical and experimental evidence-base for the efficacy and effectiveness of antivenom in snakebite neurotoxicity. The main site of snake neurotoxins is the neuromuscular junction, and the majority are either: (1) pre-synaptic neurotoxins irreversibly damaging the presynaptic terminal; or (2) post-synaptic neurotoxins that bind to the nicotinic acetylcholine receptor. Pre-clinical tests of antivenom efficacy for neurotoxicity include rodent lethality tests, which are problematic, and in vitro pharmacological tests such as nerve-muscle preparation studies, that appear to provide more clinically meaningful information. We searched MEDLINE (from 1946) and EMBASE (from 1947) until March 2017 for clinical studies. The search yielded no randomised placebo-controlled trials of antivenom for neuromuscular dysfunction. There were several randomised and non-randomised comparative trials that compared two or more doses of the same or different antivenom, and numerous cohort studies and case reports. The majority of studies available had deficiencies including poor case definition, poor study design, small sample size or no objective measures of paralysis. A number of studies demonstrated the efficacy of antivenom in human envenoming by clearing circulating venom. Studies of snakes with primarily pre-synaptic neurotoxins, such as kraits (Bungarus spp.) and taipans (Oxyuranus spp.) suggest that antivenom does not reverse established neurotoxicity, but early administration may be associated with decreased severity or prevent neurotoxicity. Small studies of snakes with mainly post-synaptic neurotoxins, including some cobra species (Naja spp.), provide preliminary evidence that neurotoxicity may be reversed with antivenom, but placebo controlled studies with objective outcome measures are required to confirm this. PMID:28422078

  5. Assessing neuromuscular mechanisms in human-exoskeleton interaction.

    PubMed

    Sylla, N; Bonnet, V; Venture, G; Armande, N; Fraisse, P

    2014-01-01

    In this study, we propose to evaluate a 7 DOF exoskeleton in terms of motion control. Using criteria from the human motor control literature, inverse optimization was performed to assess an industrial screwing movement. The results of our study show that the hybrid composition of the free arm movement was accurately determined. At contrary, when wearing the exoskeleton, which produces an arbitrary determined torque compensation, the motion is different from the naturally adopted one. This study is part of the evaluation and comprehension of the complex neuromuscular mechanism resulting in wearing an exoskeleton several hours per day for industrial tasks assistance.

  6. [Sugammadex, a novel drug for neuromuscular blockade reversal].

    PubMed

    Fernández Meré, L A; Alvarez-Blanco, M

    2010-02-01

    Significant progress in the management of aminosteroid nondepolarizing neuromuscular blockers will follow the introduction of sugammadex (Org 25969). Safety and rapid recovery of muscle force will improve and the adverse effects of acetylcholinesterase inhibitors will be avoided. Sugammadex is a modified gamma-cyclodextrin agent developed for the specific reversal of rocuronium and, to a lesser extent, vecuronium. This novel drug functions by means of encapsulation (chelation). Sugammadex was recently approved by the European Medicines Evaluation Agency and became available in Spain in 2009, leading to a series of changes related to patient safety and surgical conditions. We review the literature on sugammadex published to date.

  7. Functional neuromuscular stimulation in 4 patients with complete paraplegia.

    PubMed

    Hjeltnes, N; Lannem, A

    1990-05-01

    The effect of functional neuromuscular stimulation (FNS) on muscle strength and endurance was studied in 3 patients with long-standing complete injuries (T7-T12) and in 1 patient with a recent complete injury (T5). All 4 patients became strong enough to rise, stand and to walk a few steps within parallel bars. However, only 1 patient was able to walk without parallel bars (60 m). The energy demand of FNS-assisted walking was measured to be more than 60% of the maximal endurance capacity of this patient. This finding partly explains the low patient acceptance of FNS.

  8. Schwann Cells in Neuromuscular Junction Formation and Maintenance.

    PubMed

    Barik, Arnab; Li, Lei; Sathyamurthy, Anupama; Xiong, Wen-Cheng; Mei, Lin

    2016-09-21

    The neuromuscular junction (NMJ) is a tripartite synapse that is formed by motor nerve terminals, postjunctional muscle membranes, and terminal Schwann cells (TSCs) that cover the nerve-muscle contact. NMJ formation requires intimate communications among the three different components. Unlike nerve-muscle interaction, which has been well characterized, less is known about the role of SCs in NMJ formation and maintenance. We show that SCs in mice lead nerve terminals to prepatterned AChRs. Ablating SCs at E8.5 (i.e., prior nerve arrival at the clusters) had little effect on aneural AChR clusters at E13.5, suggesting that SCs may not be necessary for aneural clusters. SC ablation at E12.5, a time when phrenic nerves approach muscle fibers, resulted in smaller and fewer nerve-induced AChR clusters; however, SC ablation at E15.5 reduced AChR cluster size but had no effect on cluster density, suggesting that SCs are involved in AChR cluster maturation. Miniature endplate potential amplitude, but not frequency, was reduced when SCs were ablated at E15.5, suggesting that postsynaptic alterations may occur ahead of presynaptic deficits. Finally, ablation of SCs at P30, after NMJ maturation, led to NMJ fragmentation and neuromuscular transmission deficits. Miniature endplate potential amplitude was reduced 3 d after SC ablation, but both amplitude and frequency were reduced 6 d after. Together, these results indicate that SCs are not only required for NMJ formation, but also necessary for its maintenance; and postsynaptic function and structure appeared to be more sensitive to SC ablation. Neuromuscular junctions (NMJs) are critical for survival and daily functioning. Defects in NMJ formation during development or maintenance in adulthood result in debilitating neuromuscular disorders. The role of Schwann cells (SCs) in NMJ formation and maintenance was not well understood. We genetically ablated SCs during development and after NMJ formation to investigate the consequences

  9. Tenascin is present in human muscle spindles and neuromuscular junctions.

    PubMed

    Pedrosa-Domellöf, F; Virtanen, I; Thornell, L E

    1995-10-06

    We used immunocytochemistry to investigate the presence of tenascin, an extracellular matrix glycoprotein with very restricted tissue distribution, in human skeletal muscle. Tenascin was found in a short segment of the muscle spindle fibres, in the equatorial region where the sensory endings are found, and in the outer layers of the spindle capsule. Tenascin was also found in the neuromuscular junctions of the extrafusal fibres. The close spatial relationship between tenascin and both sensory and motor nerve endings shown here suggests that this glycoprotein is of functional importance in adult nerve-muscle contacts in human skeletal muscle.

  10. Quantum dot ex vivo labeling of neuromuscular synapses.

    PubMed

    Orndorff, Rebecca L; Warnement, Michael R; Mason, John N; Blakely, Randy D; Rosenthal, Sandra J

    2008-03-01

    Nicotinic receptors (nAchRs) are responsible for fast excitatory signaling by the neurotransmitter acetylcholine (Ach). They are present on the postsynaptic membrane at neuromuscular junctions (NMJs) and also at brain synapses. Alpha-bungarotoxin (alpha-BTX), a high-affinity nAchR antagonist, inhibits Ach binding and neurotransmission. Here we demonstrate biotinylated alpha-BTX, bound to native mouse diaphragm nAchRs, can be quantified and visualized ex vivo using streptavidin-conjugated quantum dots. This approach provides a novel methodology for the direct assessment of the presence and mobility of neurotransmitter receptors in native tissue.

  11. To build a synapse: signaling pathways in neuromuscular junction assembly

    PubMed Central

    Wu, Haitao; Xiong, Wen C.; Mei, Lin

    2010-01-01

    Synapses, as fundamental units of the neural circuitry, enable complex behaviors. The neuromuscular junction (NMJ) is a synapse type that forms between motoneurons and skeletal muscle fibers and that exhibits a high degree of subcellular specialization. Aided by genetic techniques and suitable animal models, studies in the past decade have brought significant progress in identifying NMJ components and assembly mechanisms. This review highlights recent advances in the study of NMJ development, focusing on signaling pathways that are activated by diffusible cues, which shed light on synaptogenesis in the brain and contribute to a better understanding of muscular dystrophy. PMID:20215342

  12. Schwann Cells in Neuromuscular Junction Formation and Maintenance

    PubMed Central

    Barik, Arnab; Li, Lei; Sathyamurthy, Anupama; Xiong, Wen-Cheng

    2016-01-01

    The neuromuscular junction (NMJ) is a tripartite synapse that is formed by motor nerve terminals, postjunctional muscle membranes, and terminal Schwann cells (TSCs) that cover the nerve-muscle contact. NMJ formation requires intimate communications among the three different components. Unlike nerve-muscle interaction, which has been well characterized, less is known about the role of SCs in NMJ formation and maintenance. We show that SCs in mice lead nerve terminals to prepatterned AChRs. Ablating SCs at E8.5 (i.e., prior nerve arrival at the clusters) had little effect on aneural AChR clusters at E13.5, suggesting that SCs may not be necessary for aneural clusters. SC ablation at E12.5, a time when phrenic nerves approach muscle fibers, resulted in smaller and fewer nerve-induced AChR clusters; however, SC ablation at E15.5 reduced AChR cluster size but had no effect on cluster density, suggesting that SCs are involved in AChR cluster maturation. Miniature endplate potential amplitude, but not frequency, was reduced when SCs were ablated at E15.5, suggesting that postsynaptic alterations may occur ahead of presynaptic deficits. Finally, ablation of SCs at P30, after NMJ maturation, led to NMJ fragmentation and neuromuscular transmission deficits. Miniature endplate potential amplitude was reduced 3 d after SC ablation, but both amplitude and frequency were reduced 6 d after. Together, these results indicate that SCs are not only required for NMJ formation, but also necessary for its maintenance; and postsynaptic function and structure appeared to be more sensitive to SC ablation. SIGNIFICANCE STATEMENT Neuromuscular junctions (NMJs) are critical for survival and daily functioning. Defects in NMJ formation during development or maintenance in adulthood result in debilitating neuromuscular disorders. The role of Schwann cells (SCs) in NMJ formation and maintenance was not well understood. We genetically ablated SCs during development and after NMJ formation to

  13. The neuromuscular transform constrains the production of functional rhythmic behaviors.

    PubMed

    Brezina, V; Weiss, K R

    2000-01-01

    We continue our study of the properties and the functional role of the neuromuscular transform (NMT). The NMT is an input-output relation that formalizes the processes by which patterns of motor neuron firing are transformed to muscle contractions. Because the NMT acts as a dynamic, nonlinear, and modifiable filter, the transformation is complex. In the preceding paper we developed a framework for analysis of the NMT and identified with it principles by which the NMT transforms different firing patterns to contractions. The ultimate question is functional, however. In sending different firing patterns through the NMT, the nervous system is seeking to command different functional behaviors, with specific contraction requirements. To what extent do the contractions that emerge from the NMT actually satisfy those requirements? In this paper we extend our analysis to address this issue. We define representative behavioral tasks and corresponding measures of performance, for a single neuromuscular unit, for two antagonistic units, and, in a real illustration, for the accessory radula closer (ARC)-opener neuromuscular system of Aplysia. We focus on cyclical, rhythmic behaviors which reveal the underlying principles particularly clearly. We find that, although every pattern of motor neuron firing produces some state of muscle contraction, only a few patterns produce functional behavior, and even fewer produce efficient functional behavior. The functional requirements thus dictate certain patterns to the nervous system. But many desirable functional behaviors are not possible with any pattern. We examine, in particular, how rhythmic behaviors degrade and disintegrate as the nervous system attempts to speed up their cycle frequency. This happens because, with fixed properties, the NMT produces only a limited range of contraction shapes that are kinetically well matched to the firing pattern only on certain time scales. Thus the properties of the NMT constrain and restrict

  14. Critical illness associated neuromuscular disorders -- keep them in mind.

    PubMed

    Nemes, Réka; Molnár, Levente; Fülep, Zoltán; Fekete, Kálra; Berhés, Mariann; Fülesdi, Béla

    2014-11-30

    Neuromuscular disorders complicating sepsis and critical illness are not new and scarce phenomena yet they receive little attention in daily clinical practice. Critical illness polyneuropathy and myopathy affect nearly half of the patients with sepsis. The difficult weaning from the ventilator, the prolonged intensive care unit and hospital stay, the larger complication and mortality rate these disorders predispose to, put a large burden on the patient and the health care system. The aim of this review is to give an insight into the pathophysiological background, diagnostic possibilities and potential preventive and therapeutic measures in connection with these disorders to draw attention to their significance and underline the importance of preventive approach.

  15. Bone health and associated metabolic complications in neuromuscular diseases.

    PubMed

    Joyce, Nanette C; Hache, Lauren P; Clemens, Paula R

    2012-11-01

    This article reviews the recent literature regarding bone health as it relates to the patient living with neuromuscular disease (NMD). Studies defining the scope of bone-related disease in NMD are scant. The available evidence is discussed, focusing on abnormal calcium metabolism, increased fracture risk, and the prevalence of both scoliosis and hypovitaminosis D in Duchenne muscular dystrophy, amyotrophic lateral sclerosis, and spinal muscular atrophy. Future directions are discussed, including the urgent need for studies both to determine the nature and extent of poor bone health, and to evaluate the therapeutic effect of available osteoporosis treatments in patients with NMD.

  16. PREVENTION AND MANAGEMENT OF LIMB CONTRACTURES IN NEUROMUSCULAR DISEASES

    PubMed Central

    Skalsky, Andrew J.; McDonald, Craig M.

    2012-01-01

    Synopsis Limb contractures are a common impairment in neuromuscular diseases (NMD). They contribute to increased disability due to decreased motor performance, mobility limitations, reduced functional range of motion, loss of function for activities of daily living (ADL), and increased pain. The pathogenesis of contractures is multifactorial. Myopathic conditions are associated with more severe limb contractures in comparison to neuropathic disorders. Although the evidence supporting the efficacy of multiple interventions to improve ROM in NMD in a sustained manner is lacking, there are generally accepted principles with regard to splinting, bracing, stretching, and surgery that help minimize the impact or disability from the contractures. PMID:22938881

  17. Calcium channels, neuromuscular synaptic transmission and neurological diseases.

    PubMed

    Urbano, Francisco J; Pagani, Mario R; Uchitel, Osvaldo D

    2008-09-15

    Voltage-dependent calcium channels are essential in neuronal signaling and synaptic transmission, and their functional alterations underlie numerous human disorders whether monogenic (e.g., ataxia, migraine, etc.) or autoimmune. We review recent work on Ca(V)2.1 or P/Q channelopathies, mostly using neuromuscular junction preparations, and focus specially on the functional hierarchy among the calcium channels recruited to mediate neurotransmitter release when Ca(V)2.1 channels are mutated or depleted. In either case, synaptic transmission is greatly compromised; evidently, none of the reported functional replacements with other calcium channels compensates fully.

  18. Does quadriceps neuromuscular activation capability explain mobility function among older men and women?

    USDA-ARS?s Scientific Manuscript database

    Age-related impairment of neuromuscular activation has been shown to contribute to weakness in older adults. However, it is unclear to what extent impaired neuromuscular activation independently accounts for decline of mobility function. The hypothesis of this study is that capability to produce rap...

  19. Does neuromuscular activation capability explain mobility function among older men and women?

    USDA-ARS?s Scientific Manuscript database

    Age-related impairment of neuromuscular activation has been shown to contribute to weakness in older adults. However, it is unclear to what extent impaired neuromuscular activation independently accounts for decline of mobility function. The hypothesis of this study is that capability to produce rap...

  20. Neuromuscular determinants of maximum walking speed in well-functioning older adults

    USDA-ARS?s Scientific Manuscript database

    Maximum walking speed may offer an advantage over usual walking speed for clinical assessment of age-related declines in mobility function that are due to neuromuscular impairment. The objective of this study was to determine the extent to which maximum walking speed is affected by neuromuscular fun...

  1. Fatigue in neuromuscular disorders: focus on Guillain-Barré syndrome and Pompe disease.

    PubMed

    de Vries, J M; Hagemans, M L C; Bussmann, J B J; van der Ploeg, A T; van Doorn, P A

    2010-03-01

    Fatigue accounts for an important part of the burden experienced by patients with neuromuscular disorders. Substantial high prevalence rates of fatigue are reported in a wide range of neuromuscular disorders, such as Guillain-Barré syndrome and Pompe disease. Fatigue can be subdivided into experienced fatigue and physiological fatigue. Physiological fatigue in turn can be of central or peripheral origin. Peripheral fatigue is an important contributor to fatigue in neuromuscular disorders, but in reaction to neuromuscular disease fatigue of central origin can be an important protective mechanism to restrict further damage. In most cases, severity of fatigue seems to be related with disease severity, possibly with the exception of fatigue occurring in a monophasic disorder like Guillain-Barré syndrome. Treatment of fatigue in neuromuscular disease starts with symptomatic treatment of the underlying disease. When symptoms of fatigue persist, non-pharmacological interventions, such as exercise and cognitive behavioral therapy, can be initiated.

  2. Functional connectivity under optogenetic control allows modeling of human neuromuscular disease

    PubMed Central

    Steinbeck, Julius A.; Jaiswal, Manoj K.; Calder, Elizabeth L; Kishinevsky, Sarah; Weishaupt, Andreas; Toyka, Klaus V.; Goldstein, Peter A.; Studer, Lorenz

    2015-01-01

    Summary Capturing the full potential of human pluripotent stem cell (PSC)-derived neurons in disease modeling and regenerative medicine requires analysis in complex functional systems. Here we establish optogenetic control in human PSC-derived spinal motorneurons and show that co-culture of these cells with human myoblast-derived skeletal muscle builds a functional all-human neuromuscular junction that can be triggered to twitch upon light stimulation. To model neuromuscular disease we incubated these co-cultures with IgG from myasthenia gravis patients and active complement. Myasthenia gravis is an autoimmune disorder that selectively targets neuromuscular junctions. We saw a reversible reduction in the amplitude of muscle contractions, representing a surrogate marker for the characteristic loss of muscle strength seen in this disease. The ability to recapitulate key aspects of disease pathology and its symptomatic treatment suggests that this neuromuscular junction assay has significant potential for modeling of neuromuscular disease and regeneration. PMID:26549107

  3. The immediate effect of neuromuscular joint facilitation on the rotation of the tibia during walking

    PubMed Central

    Li, Desheng; Huang, Qiuchen; Huo, Ming; Hiiragi, Yukinobu; Maruyama, Hitoshi

    2017-01-01

    [Purpose] The aim of this study was to investigate the change in tibial rotation during walking among young adults after neuromuscular joint facilitation therapy. [Subjects and Methods] The subjects were twelve healthy young people (6 males, 6 females). A neuromuscular joint facilitation intervention and nonintervention were performed. The interventions were performed one after the other, separated by a 1-week interval. The order of the interventions was completely randomized. The rotation of the tibia during walking was evaluated before and after treatment. [Results] The neuromuscular joint facilitation group demonstrated increased lateral rotation of the tibia in the overall gait cycle and stance phase, and decreased medial rotation of the tibia in the overall gait cycle, stance phase, and swing phase after the neuromuscular joint facilitation intervention. In the control group, there were no significant differences. [Conclusion] These results suggest neuromuscular joint facilitation intervention has an immediate effect on the rotational function of the knee. PMID:28210049

  4. The Wnt and BMP Families of Signaling Morphogens at the Vertebrate Neuromuscular Junction

    PubMed Central

    Henríquez, Juan P.; Krull, Catherine E.; Osses, Nelson

    2011-01-01

    The neuromuscular junction has been extensively employed in order to identify crucial determinants of synaptogenesis. At the vertebrate neuromuscular synapse, extracellular matrix and signaling proteins play stimulatory and inhibitory roles on the assembly of functional synapses. Studies in invertebrate species have revealed crucial functions of early morphogens during the assembly and maturation of the neuromuscular junction. Here, we discuss growing evidence addressing the function of Wnt and Bone morphogenetic protein (BMP) signaling pathways at the vertebrate neuromuscular synapse. We focus on the emerging role of Wnt proteins as positive and negative regulators of postsynaptic differentiation. We also address the possible involvement of BMP pathways on motor neuron behavior for the assembly and/or regeneration of the neuromuscular junction. PMID:22272112

  5. The role of neuromuscular inhibition in hamstring strain injury recurrence.

    PubMed

    Fyfe, Jackson J; Opar, David A; Williams, Morgan D; Shield, Anthony J

    2013-06-01

    Hamstring strain injuries are amongst the most common and problematic injuries in a wide range of sports that involve high speed running. The comparatively high rate of hamstring injury recurrence is arguably the most concerning aspect of these injuries. A number of modifiable and nonmodifiable risk factors are proposed to predispose athletes to hamstring strains. Potentially, the persistence of risk factors and the development of maladaptations following injury may explain injury recurrence. Here, the role of neuromuscular inhibition following injury is discussed as a potential mechanism for several maladaptations associated with hamstring re-injury. These maladaptations include eccentric hamstring weakness, selective hamstring atrophy and shifts in the knee flexor torque-joint angle relationship. Current evidence indicates that athletes return to competition after hamstring injury having developed maladaptations that predispose them to further injury. When rehabilitating athletes to return to competition following hamstring strain injury, the role of neuromuscular inhibition in re-injury should be considered. Crown Copyright © 2013. Published by Elsevier Ltd. All rights reserved.

  6. Contralaterally Controlled Neuromuscular Electrical Stimulation for Recovery of Ankle Dorsiflexion

    PubMed Central

    Knutson, Jayme S.; Hansen, Kristine; Nagy, Jennifer; Bailey, Stephanie N.; Gunzler, Douglas D.; Sheffler, Lynne R.; Chae, John

    2013-01-01

    Objective Compare the effects of contralaterally controlled neuromuscular electrical stimulation (CCNMES) versus cyclic neuromuscular electrical stimulation (NMES) on lower extremity impairment, functional ambulation, and gait characteristics. Design Twenty-six stroke survivors with chronic (≥6mo) footdrop during ambulation were randomly assigned to six weeks of CCNMES or cyclic NMES. Both groups had ten sessions per week of self-administered home application of either CCNMES or cyclic NMES plus two sessions per week of gait training with a physical therapist. Primary outcomes included lower extremity Fugl-Meyer score, modified Emory Functional Ambulation Profile, and gait velocity. Assessments were made at pretreatment, posttreatment, and at 1 and 3 months posttreatment. Results There were no significant differences between groups in the outcome trajectories for any of the measures. With data from both groups pooled, there were significant but modest and sustained improvements in the Fugl-Meyer score and the modified Emory Functional Ambulation Profile, but not in gait velocity. Conclusions The results support the hypothesis that gait training combined with either CCNMES or cyclic NMES reduces lower extremity impairment and functional ambulation, but do not support the hypothesis that CCNMES is more effective than cyclic NMES in chronic patients. PMID:23867888

  7. [Use of neuromuscular blockers in the critical patient].

    PubMed

    Sandiumenge, A; Anglés, R; Martínez-Melgar, J L; Torrado, H

    2008-02-01

    The use of neuromuscular blockers (NMB) is a frequent practice in Intensive Care Units. However most of the experience with the use of these agents come from the operating room used to deal with patients with different characteristics from those admitted in the ICU. Recent advances on neuromonitoring and the commercialization of newer agents make necessary the update in the management of NMB in the ICU. The NMB agent should be chosen attending to its pharmacokinetics and the physiopathology of the critically ill patient. Those NMB with organ-independent metabolism as well as those with rapid onset of action are the preferred ones for the use in the critically ill patient substituting older depolarizing agents and those whose metabolism is dependent on the liver and/or kidney, organs frequently impaired in the critically ill patients. Neuromuscular blocking in the critically ill patient should be done according to protocols and monitor its effects in order to avoid complications related to its prolonged use.

  8. Measurement of acceleration: a new method of monitoring neuromuscular function.

    PubMed

    Viby-Mogensen, J; Jensen, E; Werner, M; Nielsen, H K

    1988-01-01

    A new method for monitoring neuromuscular function based on measurement of acceleration is presented. The rationale behind the method is Newton's second law, stating that the acceleration is directly proportional to the force. For measurement of acceleration, a piezo-electric ceramic wafer was used. When this piezo electrode was fixed to the thumb, an electrical signal proportional to the acceleration was produced whenever the thumb moved in response to nerve stimulation. The electrical signal was registered and analysed in a Myograph 2000 neuromuscular transmission monitor. In 35 patients anaesthetized with halothane, train-of-four ratios measured with the accelerometer (ACT-TOF) were compared with simultaneous mechanical train-of-four ratios (FDT-TOF). Control ACT-TOF ratios were significantly higher than control FDT-TOF ratios: 116 +/- 12 and 98 +/- 4 (mean +/- s.d.), respectively. In five patients not given any relaxant during the anaesthetic procedure (20-60 min), both responses were remarkably constant. In 30 patients given vecuronium, a close linear relationship was found during recovery between ACT-TOF and FDT-TOF ratios. It is concluded that the method fulfils the basic requirements for a simple and reliable clinical monitoring tool.

  9. Hipocrates: a robust system for the control of neuromuscular blockade.

    PubMed

    Mendonça, Teresa; Magalhães, Hugo; Lago, Pedro; Esteves, Simão

    2004-08-01

    Development of an automatic system (software package Hipocrates) for the control of neuromuscular blockade by continuous infusion of the non-depolarising types of muscle relaxant drugs presently used in anaesthesia, namely atracurium, cisatracurium, vecuronium and rocuronium. Hipocrates incorporates control strategies based upon classical, adaptive and robust control, as well as a wide range of noise reduction techniques and on-line adaptation to patient-specific characteristics. Therefore, the system provides strong robustness to inter- and intra-individual variability of the patients responses or unexpected circumstances and adaptation to the individual requirements. The control system is easy to set up and to use in a clinical environment. It consists of a portable PC computer, a Datex AS/3 NMT sensor and a B/Braun compact perfusion pump. In the simulation mode the software package incorporates sophisticated generation of pharmacokinetic/pharmacodynamic models driven by simulated drug administration regimes (bolus, continuous infusion and a combination of both). Hipocrates is an advanced standalone application for the control of neuromuscular blockade with a friendly graphic interface. It has been extensively validated, and it can be used on patients undergoing surgery as well as for simulation studies. Therefore Hipocrates also provides an excellent environment for education and training purposes.

  10. Inherited disorders of the neuromuscular junction: an update.

    PubMed

    Rodríguez Cruz, Pedro M; Palace, Jacqueline; Beeson, David

    2014-11-01

    Congenital myasthenic syndromes (CMSs) are a group of heterogeneous inherited disorders caused by mutations in genes affecting the function and structure of the neuromuscular junction. This review updates the reader on established and novel subtypes of congenital myasthenia, and the treatment strategies for these increasingly heterogeneous disorders. The discovery of mutations associated with the N-glycosylation pathway and in the family of serine peptidases has shown that causative genes encoding ubiquitously expressed molecules can produce defects at the human neuromuscular junction. By contrast, mutations in lipoprotein-like receptor 4 (LRP4), a long-time candidate gene for congenital myasthenia, and a novel phenotype of myasthenia with distal weakness and atrophy due to mutations in AGRN have now been described. In addition, a pathogenic splicing mutation in a nonfunctional exon of CHRNA1 has been reported emphasizing the importance of analysing nonfunctional exons in genetic analysis. The benefit of salbutamol and ephedrine alone or combined with pyridostigmine or 3,4-DAP is increasingly being reported for particular subtypes of CMS.

  11. Motor unit recruitment during neuromuscular electrical stimulation: a critical appraisal.

    PubMed

    Bickel, C Scott; Gregory, Chris M; Dean, Jesse C

    2011-10-01

    Neuromuscular electrical stimulation (NMES) is commonly used in clinical settings to activate skeletal muscle in an effort to mimic voluntary contractions and enhance the rehabilitation of human skeletal muscles. It is also used as a tool in research to assess muscle performance and/or neuromuscular activation levels. However, there are fundamental differences between voluntary- and artificial-activation of motor units that need to be appreciated before NMES protocol design can be most effective. The unique effects of NMES have been attributed to several mechanisms, most notably, a reversal of the voluntary recruitment pattern that is known to occur during voluntary muscle contractions. This review outlines the assertion that electrical stimulation recruits motor units in a nonselective, spatially fixed, and temporally synchronous pattern. Additionally, it synthesizes the evidence that supports the contention that this recruitment pattern contributes to increased muscle fatigue when compared with voluntary actions and provides some commentary on the parameters of electrical stimulation as well as emerging technologies being developed to facilitate NMES implementation. A greater understanding of how electrical stimulation recruits motor units, as well as the benefits and limitations of its use, is highly relevant when using this tool for testing and training in rehabilitation, exercise, and/or research.

  12. Neuromuscular and endocrine control of an avian courtship behavior.

    PubMed

    Schlinger, B A; Schultz, J D; Hertel, F

    2001-09-01

    In many species of birds, males perform complex visual and acoustic courtship displays to attract and stimulate females. Some of these displays involve considerable use of the wings and legs, suggesting that they may be controlled by sexually dimorphic spinal motoneurons and their target muscles. Sex steroid hormones are known to organize and activate many sexually dimorphic phenotypes, so these neuromuscular systems may also be steroid sensitive. To test these ideas, we have begun studies of wild golden-collared manakins (Manacus vitellinus) in Central America. Males of this species establish a courtship arena in the forest, where they perform an elaborate dance that includes use of their wings to generate loud snapping sounds. Here we describe male golden-collared manakin courtship behavior, including the various "wingsnaps." We also review our studies, and those of others, showing sexually dimorphic properties of manakin wings, the wing musculature, and sex steroid accumulation in the spinal cord. These data suggest that manakins are useful models for evaluating steroid control of complex peripheral neuromuscular systems.

  13. Influence of Fatigue in Neuromuscular Control of Spinal Stability

    PubMed Central

    Granata, Kevin P.; Slota, Greg P.; Wilson, Sara E.

    2006-01-01

    Lifting-induced fatigue may influence neuromuscular control of spinal stability. Stability is primarily controlled by muscle recruitment, active muscle stiffness, and reflex response. Fatigue has been observed to affect each of these neuromuscular parameters and may therefore affect spinal stability. A biomechanical model of spinal stability was implemented to evaluate the effects of fatigue on spinal stability. The model included a 6-degree-of-freedom representation of the spine controlled by 12 deformable muscles from which muscle recruitment was determined to simultaneously achieve equilibrium and stability. Fatigue-induced reduction in active muscle stiffness necessitated increased antagonistic cocontraction to maintain stability resulting in increased spinal compression with fatigue. Fatigueinduced reduction in force-generating capacity limited the feasible set of muscle recruitment patterns, thereby restricting the estimated stability of the spine. Electromyographic and trunk kinematics from 21 healthy participants were recorded during sudden-load trials in fatigued and unfatigued states. Empirical data supported the model predictions, demonstrating increased antagonistic cocontraction during fatigued exertions. Results suggest that biomechanical factors including spinal load and stability should be considered when performing ergonomic assessments of fatiguing lifting tasks. Potential applications of this research include a biomechanical tool for the design of administrative ergonomic controls in manual materials handling industries. PMID:15151156

  14. Neuromuscular performance characteristics of open-wheel and rally drivers.

    PubMed

    Backman, Jani; Häkkinen, Keijo; Ylinen, Jari; Häkkinen, Arja; Kyröläinen, Heikki

    2005-11-01

    The purpose of the present study was to investigate neuromuscular performance characteristics in open-wheel and rally drivers using the cross-sectional study design. The subjects (N = 28) consisted of experienced international-level open-wheel drivers (n = 9), experienced international-level rally drivers (n = 9) and a physically active nondriving male control group (n = 10). In 3 separate test sessions, speed, muscle strength, and endurance tests were performed. The rally drivers had higher (p < 0.05) grip, shoulder flexion, and ankle plantar flexion strength, as compared to the control group. The open-wheel drivers showed higher strengths (p < 0.05) than the controls in neck forces, grip, shoulder flexion, and leg extension. The rally drivers were stronger (p < 0.05) than the open-wheel drivers in grip, plantar flexion, and trunk extension forces, whereas the open-wheel drivers were stronger (p < 0.01) than the rally drivers in neck lateral flexions and extension forces. Thus, competitive long-term open-wheel and rally drivers differ specifically in neuromuscular performance. For practice, these findings suggest that rally drivers should concentrate on training hand, ankle, and trunk muscles, whereas open-wheel drivers should train neck muscles, especially, and all other muscle groups rather equally.

  15. Long-term neuromuscular training and ankle joint position sense.

    PubMed

    Kynsburg, A; Pánics, G; Halasi, T

    2010-06-01

    Preventive effect of proprioceptive training is proven by decreasing injury incidence, but its proprioceptive mechanism is not. Major hypothesis: the training has a positive long-term effect on ankle joint position sense in athletes of a high-risk sport (handball). Ten elite-level female handball-players represented the intervention group (training-group), 10 healthy athletes of other sports formed the control-group. Proprioceptive training was incorporated into the regular training regimen of the training-group. Ankle joint position sense function was measured with the "slope-box" test, first described by Robbins et al. Testing was performed one day before the intervention and 20 months later. Mean absolute estimate errors were processed for statistical analysis. Proprioceptive sensory function improved regarding all four directions with a high significance (p<0.0001; avg. mean estimate error improvement: 1.77 degrees). This was also highly significant (p< or =0.0002) in each single directions, with avg. mean estimate error improvement between 1.59 degrees (posterior) and 2.03 degrees (anterior). Mean absolute estimate errors at follow-up (2.24 degrees +/-0.88 degrees) were significantly lower than in uninjured controls (3.29 degrees +/-1.15 degrees) (p<0.0001). Long-term neuromuscular training has improved ankle joint position sense function in the investigated athletes. This joint position sense improvement can be one of the explanations for injury rate reduction effect of neuromuscular training.

  16. The neuromuscular approach towards interdisciplinary cooperation in medicine.

    PubMed

    Yurchenko, Maksim; Hubálková, Hana; Klepáček, Ivo; Machoň, Vladimír; Mazánek, Jiří

    2014-02-01

    The contemporary approach of dentistry towards interdisciplinary cooperation is based on the neuromuscular concept. In recent years many authors have pointed out a correlation between orthopaedic and dental findings. Furthermore, there is an intimate biomechanical interrelationship of occlusion with cerebral fluid circulation, left and right equilibrium, gaze stabilisation and headache. The influence of a dental occlusion and temporomandibular joint (TMJ) status on general health has been widely analysed in the current scientific literature. The aim of this analytic study was to display the deep relationship between dentistry and other fields of medicine, and to show the necessity of wide cooperation between dentists and physicians. This study was based on a review of 41 sources, including specialised articles and books. The significance of different anatomical and physiological preconditions (occlusion, muscles and TMJ status) was considered and evaluated separately. However, as a result of modern concepts of general body health, extending cooperation between different fields of medicine is essential. The practical application of the principles of neuromuscular dentistry enables one to increase greatly the treatment efficiency of aches in muscles, headaches, postural dysfunctions as well as of many other diseases. However, the dentist's contribution to the development of an interdisciplinary approach is underestimated. Many theoretical aspects of the interdisciplinary relationship have not been sufficiently examined, hence the practical consequences remain unclear. Further research in the field is urgently needed. © 2013 FDI World Dental Federation.

  17. Fetal akinesia: review of the genetics of the neuromuscular causes.

    PubMed

    Ravenscroft, Gianina; Sollis, Elliot; Charles, Adrian K; North, Kathryn N; Baynam, Gareth; Laing, Nigel G

    2011-12-01

    Fetal akinesia refers to a broad spectrum of disorders in which the unifying feature is a reduction or lack of fetal movement. Fetal akinesias may be caused by defects at any point along the motor system pathway including the central and peripheral nervous system, the neuromuscular junction and the muscle, as well as by restrictive dermopathy or external restriction of the fetus in utero. The fetal akinesias are clinically and genetically heterogeneous, with causative mutations identified to date in a large number of genes encoding disparate parts of the motor system. However, for most patients, the molecular cause remains unidentified. One reason for this is because the tools are only now becoming available to efficiently and affordably identify mutations in a large panel of disease genes. Next-generation sequencing offers the promise, if sufficient cohorts of patients can be assembled, to identify the majority of the remaining genes on a research basis and facilitate efficient clinical molecular diagnosis. The benefits of identifying the causative mutation(s) for each individual patient or family include accurate genetic counselling and the options of prenatal diagnosis or preimplantation genetic diagnosis. In this review, we summarise known single-gene disorders affecting the spinal cord, peripheral nerves, neuromuscular junction or skeletal muscles that result in fetal akinesia. This audit of these known molecular and pathophysiological mechanisms involved in fetal akinesia provides a basis for improved molecular diagnosis and completing disease gene discovery.

  18. Musculoskeletal and neuromuscular interventions: a physical approach to cystic fibrosis.

    PubMed Central

    Massery, Mary

    2005-01-01

    Children with CF are living longer than ever before, and thus issues pertaining to quality of life rather than just longevity of life need to be addressed by the entire healthcare team. This article addressed the issues pertaining to the external support of the dysfunctional internal organs: the secondary musculoskeletal (postural) and neuromuscular control deficits that occur to the maturing child with CF. The research pointed towards starting PT interventions for these deficits during the pre-pubescent phase when postural deficits were just emerging, but a suggestion was also made to explore whether these deficits can be even more effectively monitored and treated at an earlier age. The dual relationship between the muscles used to meet the increased respiratory demands of CF and the normal postural demands of physical activities was described through a model based on a soda-pop can and pressure support. A pre-pubescent child with a typical progression of CF was presented as a case report to illustrate how a PT programme that was focused on postural deficits could be implemented and what type of outcomes might be possible. The child made significant changes within a relatively short time frame of 4 months, proposing that the musculoskeletal and neuromuscular systems may play a significant role in the medical and physical long-term outcomes of CF. For that reason, the physical as well as medical needs of the patient should be incorporated into a comprehensive multi-system approach to the disease across the lifespan. PMID:16025768

  19. Postoperative Residual Neuromuscular Paralysis at an Australian Tertiary Children's Hospital

    PubMed Central

    O'Dea, Brendan; Meyerkort, Luke; Hegarty, Mary; von Ungern-Sternberg, Britta S.

    2015-01-01

    Purpose. Residual neuromuscular blockade (RNMB) is known to be a significant but frequently overlooked complication after the use of neuromuscular blocking agents (NMBA). Aim of this prospective audit was to investigate the incidence and severity of RNMB at our Australian tertiary pediatric center. Methods. All children receiving NMBA during anesthesia were included over a 5-week period at the end of 2011 (Mondays to Fridays; 8 a.m.–6 p.m.). At the end of surgery, directly prior to tracheal extubation, the train-of-four (TOF) ratio was assessed quantitatively. Data related to patient postoperative outcome was collected in the postoperative acute care unit. Results. Data of 64 patients were analyzed. Neostigmine was given in 34 cases and sugammadex in 1 patient. The incidence of RNMB was 28.1% overall (without reversal: 19.4%; after neostigmine: 37.5%; n.s.). Severe RNMB (TOF ratio < 0.7) was found in 6.5% after both no reversal and neostigmine, respectively. Complications in the postoperative acute care unit were infrequent, with no differences between reversal and no reversal groups. Conclusions. In this audit, RNMB was frequently observed, particularly in cases where patients were reversed with neostigmine. These findings underline the well-known problems associated with the use of NMBA that are not fully reversed. PMID:26064105

  20. Neuromuscular rate of force development deficit in Parkinson disease.

    PubMed

    Hammond, Kelley G; Pfeiffer, Ronald F; LeDoux, Mark S; Schilling, Brian K

    2017-06-01

    Bradykinesia and reduced neuromuscular force exist in Parkinson disease. The interpolated twitch technique has been used to evaluate central versus peripheral manifestations of neuromuscular strength in healthy, aging, and athletic populations, as well as moderate to advanced Parkinson disease, but this method has not been used in mild Parkinson disease. This study aimed to evaluate quadriceps femoris rate of force development and quantify potential central and peripheral activation deficits in individuals with Parkinson disease. Nine persons with mild Parkinson Disease (Hoehn & Yahr≤2, Unified Parkinson Disease Rating Scale total score=mean 19.1 (SD 5.0)) and eight age-matched controls were recruited in a cross-sectional investigation. Quadriceps femoris voluntary and stimulated maximal force and rate of force development were evaluated using the interpolated twitch technique. Thirteen participants satisfactorily completed the protocol. Individuals with early Parkinson disease (n=7) had significantly slower voluntary rate of force development (p=0.008; d=1.97) and rate of force development ratio (p=0.004; d=2.18) than controls (n=6). No significant differences were found between groups for all other variables. Persons with mild-to-moderate Parkinson disease display disparities in rate of force development, even without deficits in maximal force. The inability to produce force at a rate comparable to controls is likely a downstream effect of central dysfunction of the motor pathway in Parkinson disease. Copyright © 2017. Published by Elsevier Ltd.

  1. Effect of electrostimulation training-detraining on neuromuscular fatigue mechanisms.

    PubMed

    Jubeau, Marc; Zory, Raphaël; Gondin, Julien; Martin, Alain; Maffiuletti, Nicola A

    2007-08-31

    The aim of this study was to evaluate the effects of neuromuscular electrical stimulation (NMES) training and subsequent detraining on neuromuscular fatigue mechanisms. Ten young healthy men completed one NMES fatigue protocol before and after a NMES training program of 4 weeks and again after 4 weeks of detraining. Muscle fatigue (maximal voluntary torque loss), central fatigue (activation failure), and peripheral fatigue (transmission failure and contractile failure) of the plantar flexor muscles were assessed by using a series of electrically evoked and voluntary contractions with concomitant electromyographic and torque recordings. At baseline, maximal voluntary torque decreased significantly with fatigue (P<0.001), due to both activation and transmission failure. After detraining, maximal voluntary torque loss was significantly reduced (P<0.05). In the same way, the relative decrease in muscle activation after training and detraining was significantly lower compared to baseline values (P<0.05). Short-term NMES training-detraining of the plantar flexor muscles significantly reduced the muscle fatigue associated to one single NMES exercise session. This was mainly attributable to a reduction in activation failure, i.e., lower central fatigue, probably as a result of subject's accommodation to pain and discomfort during NMES.

  2. Sarcocystis fayeri in skeletal muscle of horses with neuromuscular disease.

    PubMed

    Aleman, Monica; Shapiro, Karen; Sisó, Silvia; Williams, Diane C; Rejmanek, Daniel; Aguilar, Beatriz; Conrad, Patricia A

    2016-01-01

    Recent reports of Sarcocystis fayeri-induced toxicity in people consuming horse meat warrant investigation on the prevalence and molecular characterization of Sarcocystis spp. infection in horses. Sarcocysts in skeletal muscle of horses have been commonly regarded as an incidental finding. In this study, we investigated the prevalence of sarcocysts in skeletal muscle of horses with neuromuscular disease. Our findings indicated that S. fayeri infection was common in young mature horses with neuromuscular disease and could be associated with myopathic and neurogenic processes. The number of infected muscles and number of sarcocysts per muscle were significantly higher in diseased than in control horses. S. fayeri was predominantly found in low oxidative highly glycolytic myofibers. This pathogen had a high glycolytic metabolism. Common clinical signs of disease included muscle atrophy, weakness with or without apparent muscle pain, gait deficits, and dysphagia in horses with involvement of the tongue and esophagus. Horses with myositis were lethargic, apparently painful, stiff, and reluctant to move. Similar to humans, sarcocystosis and cardiomyopathy can occur in horses. This study did not establish causality but supported a possible association (8.9% of cases) with disease. The assumption of Sarcocysts spp. being an incidental finding in every case might be inaccurate. Copyright © 2015 Elsevier B.V. All rights reserved.

  3. [Six-minute walk test in children with neuromuscular disease.

    PubMed

    Cruz-Anleu, Israel Didier; Baños-Mejía, Benjamín Omar; Galicia-Amor, Susana

    2013-01-01

    Background: neuromuscular diseases affect the motor unit. When they evolve, respiratory complications are common; the six-minute walk test plays an important role in the assessment of functional capacity. Methods: prospective, transversal, descriptive and observational study. We studied seven children with a variety of neuromuscular diseases and spontaneous ambulation. We tested their lung function, and administered a six-minute walk test and a test of respiratory muscle strength to these children. Results: the age was 9.8 ± 2.4 years. All patients were males. Forced vital capacity decreased in three patients (42.8 %), forced expiratory volume during the first second (2.04 ± 1.4 L) and peak expiratory flow (4.33 ± 3.3 L/s) were normal. The maximum strength of respiratory muscles was less than 60 % of predicted values. The distance covered in the six-minute walk test was lower when compared with healthy controls (29.9 %). Conclusions: the six-minute walk test can be a useful tool in early stages of this disease, since it is easy to perform and well tolerated by the patients.

  4. A survey of the management of neuromuscular blockade monitoring in Australia and New Zealand.

    PubMed

    Phillips, S; Stewart, P A; Bilgin, A B

    2013-05-01

    This survey of anaesthetists in Australia and New Zealand aimed to investigate their attitudes and practice relating to the management of neuromuscular blockade monitoring. All medical practitioner members (3188) of the Australian and New Zealand Societies of Anaesthetists were invited to complete an anonymous survey, which was available online for two months. A total of 678 survey questionnaires were completed (response rate 21%). Most respondents (71.4%) underestimated the incidence of residual neuromuscular blockade and 63.2% believed this to be a significant clinical problem. Objective monitoring of neuromuscular function was used routinely only by 17% of respondents, although 70% believed routine monitoring would reduce the incidence of residual neuromuscular blockade. Only 25% of respondents correctly indicated that quantitative train-of-four counts of greater than 90% were the accepted criteria for safe extubation, with 52% using clinical judgement only. Only 29% of respondents believed neuromuscular function monitors should be part of minimum monitoring standards; quantitative neuromuscular function monitors were not available in 42% of the hospitals in which the respondents practiced. Despite the low response rate, the large sample size and heterogeneity of respondents make the findings of this survey concerning. There is a need for more education, availability of appropriate monitoring equipment and evidence-based guidelines for management of neuromuscular blockade in Australia and New Zealand.

  5. Clinical Practice Guidelines for Sustained Neuromuscular Blockade in the Adult Critically Ill Patient.

    PubMed

    Murray, Michael J; DeBlock, Heidi; Erstad, Brian; Gray, Anthony; Jacobi, Judi; Jordan, Che; McGee, William; McManus, Claire; Meade, Maureen; Nix, Sean; Patterson, Andrew; Sands, M Karen; Pino, Richard; Tescher, Ann; Arbour, Richard; Rochwerg, Bram; Murray, Catherine Friederich; Mehta, Sangeeta

    2016-11-01

    To update the 2002 version of "Clinical practice guidelines for sustained neuromuscular blockade in the adult critically ill patient." A Task Force comprising 17 members of the Society of Critical Medicine with particular expertise in the use of neuromuscular-blocking agents; a Grading of Recommendations Assessment, Development, and Evaluation expert; and a medical writer met via teleconference and three face-to-face meetings and communicated via e-mail to examine the evidence and develop these practice guidelines. Annually, all members completed conflict of interest statements; no conflicts were identified. This activity was funded by the Society for Critical Care Medicine, and no industry support was provided. Using the Grading of Recommendations Assessment, Development, and Evaluation system, the Grading of Recommendations Assessment, Development, and Evaluation expert on the Task Force created profiles for the evidence related to six of the 21 questions and assigned quality-of-evidence scores to these and the additional 15 questions for which insufficient evidence was available to create a profile. Task Force members reviewed this material and all available evidence and provided recommendations, suggestions, or good practice statements for these 21 questions. The Task Force developed a single strong recommendation: we recommend scheduled eye care that includes lubricating drops or gel and eyelid closure for patients receiving continuous infusions of neuromuscular-blocking agents. The Task Force developed 10 weak recommendations. 1) We suggest that a neuromuscular-blocking agent be administered by continuous intravenous infusion early in the course of acute respiratory distress syndrome for patients with a PaO2/FIO2 less than 150. 2) We suggest against the routine administration of an neuromuscular-blocking agents to mechanically ventilated patients with status asthmaticus. 3) We suggest a trial of a neuromuscular-blocking agents in life-threatening situations

  6. Development and potential clinical impairment of ultra-short-acting neuromuscular blocking agents.

    PubMed

    Lien, C A

    2011-12-01

    Developing a non-depolarizing neuromuscular blocking agent that, like succinylcholine, has a rapid onset and a short duration of effect remains a goal of ongoing research. While rocuronium fills a portion of this need, the large doses required for rapid intubation render it a much longer-acting neuromuscular blocking agent. Postoperative residual neuromuscular block (NMB) is an increasingly recognized complication of non-depolarizing neuromuscular blocking agents. This occurs because of dosing choices for neuromuscular blocking agents and anticholinesterases as well as insensitivity of typically used monitors of depth of NMB. While antagonism of NMB is necessary with partial recovery, it is unnecessary with more complete recovery. Even when monitoring with an accelerograph, reversal of NMB is complicated. In addition to the pharmacodynamics of the individual neuromuscular blocking agents, factors such as timing of anticholinesterase administration, dose of anticholinesterase, concomitant medications, electrolyte abnormalities, and hepatic or renal disease can influence the degree of reversal. Sugammadex works differently than anticholinesterases and, when administered in appropriate doses, can reverse even profound block induced with vecuronium or rocuronium. Two new fumarate neuromuscular blocking agents have a rapid onset of effect and can be reversed at any time by administration of cysteine, which could significantly reduce the risk of postoperative residual NMB.

  7. Role of Amicar in surgery for neuromuscular scoliosis.

    PubMed

    Thompson, George H; Florentino-Pineda, Ivan; Poe-Kochert, Connie; Armstrong, Douglas G; Son-Hing, Jochen

    2008-11-15

    A retrospective case-control study. Evaluate the effectiveness of Amicar in decreasing perioperative blood loss in patients with neuromuscular scoliosis undergoing posterior spinal fusion (PSF) and segmental spinal instrumentation (SSI). Previously, a preliminary prospective; prospective randomized double-blind; same-day anterior and posterior spinal fusion; and fibrinogen studies have demonstrated Amicar to be effective in decreasing total perioperative blood loss and transfusion requirements in surgery for idiopathic scoliosis. Increased fibrinogen secretion is a possible explanation. We are now analyzing its effectiveness in neuromuscular scoliosis. Amicar was administered at 100 mg/kg over 15 minute not to exceed 5 g after anesthesia induction. Maintenance is 10 mg/kg/h until wound closure. There were 2 study groups: group 1 (n = 34), no Amicar and group 2 (n = 62) who received Amicar. The majority of patients in both groups had cerebral palsy. Total perioperative blood loss was determined from the estimated intraoperative blood loss and measured postoperative suction drainage. Total perioperative blood loss and transfusion requirements (cell saver and allogeneic) were compared using chi or Fisher exact test. There was statistically less estimated intraoperative blood loss, total perioperative blood loss, and transfusion requirements in group 2. Postoperative suction drainage was also less but did not reach statistical significance. In group 1, estimated intraoperative blood loss, measured postoperative suction drainage, and total perioperative blood loss were 2194 +/- 1626 mL, 903 +/- 547 mL, and 3055 +/- 1852 mL, whereas in group 2, it was 1125 +/- 715 mL, 695 +/- 489 mL, and 1805 +/- 940 mL. Transfusion requirements were 1548 +/- 962 mL in group 1 but only 660 +/- 589 mL in group 2 (P < 0.0001). Amicar was equally effective in all diagnoses. There were no complications related to the use of Amicar. Amicar was highly effective in decreasing perioperative

  8. Neuromuscular blockers in early acute respiratory distress syndrome.

    PubMed

    Papazian, Laurent; Forel, Jean-Marie; Gacouin, Arnaud; Penot-Ragon, Christine; Perrin, Gilles; Loundou, Anderson; Jaber, Samir; Arnal, Jean-Michel; Perez, Didier; Seghboyan, Jean-Marie; Constantin, Jean-Michel; Courant, Pierre; Lefrant, Jean-Yves; Guérin, Claude; Prat, Gwenaël; Morange, Sophie; Roch, Antoine

    2010-09-16

    In patients undergoing mechanical ventilation for the acute respiratory distress syndrome (ARDS), neuromuscular blocking agents may improve oxygenation and decrease ventilator-induced lung injury but may also cause muscle weakness. We evaluated clinical outcomes after 2 days of therapy with neuromuscular blocking agents in patients with early, severe ARDS. In this multicenter, double-blind trial, 340 patients presenting to the intensive care unit (ICU) with an onset of severe ARDS within the previous 48 hours were randomly assigned to receive, for 48 hours, either cisatracurium besylate (178 patients) or placebo (162 patients). Severe ARDS was defined as a ratio of the partial pressure of arterial oxygen (PaO2) to the fraction of inspired oxygen (FIO2) of less than 150, with a positive end-expiratory pressure of 5 cm or more of water and a tidal volume of 6 to 8 ml per kilogram of predicted body weight. The primary outcome was the proportion of patients who died either before hospital discharge or within 90 days after study enrollment (i.e., the 90-day in-hospital mortality rate), adjusted for predefined covariates and baseline differences between groups with the use of a Cox model. The hazard ratio for death at 90 days in the cisatracurium group, as compared with the placebo group, was 0.68 (95% confidence interval [CI], 0.48 to 0.98; P=0.04), after adjustment for both the baseline PaO2:FIO2 and plateau pressure and the Simplified Acute Physiology II score. The crude 90-day mortality was 31.6% (95% CI, 25.2 to 38.8) in the cisatracurium group and 40.7% (95% CI, 33.5 to 48.4) in the placebo group (P=0.08). Mortality at 28 days was 23.7% (95% CI, 18.1 to 30.5) with cisatracurium and 33.3% (95% CI, 26.5 to 40.9) with placebo (P=0.05). The rate of ICU-acquired paresis did not differ significantly between the two groups. In patients with severe ARDS, early administration of a neuromuscular blocking agent improved the adjusted 90-day survival and increased the time off

  9. Age‐related neuromuscular changes affecting human vastus lateralis

    PubMed Central

    Piasecki, M.; Ireland, A.; Stashuk, D.; Hamilton‐Wright, A.; Jones, D. A.

    2015-01-01

    Key points Skeletal muscle size and strength decline in older age.The vastus lateralis, a large thigh muscle, undergoes extensive neuromuscular remodelling in healthy ageing, as characterized by a loss of motor neurons, enlargement of surviving motor units and instability of neuromuscular junction transmission.The loss of motor axons and changes to motor unit potential transmission precede a clinically‐relevant loss of muscle mass and function. Abstract The anterior thigh muscles are particularly susceptible to muscle loss and weakness during ageing, although how this is associated with changes to neuromuscular structure and function in terms of motor unit (MU) number, size and MU potential (MUP) stability remains unclear. Intramuscular (I.M.) and surface electromyographic signals were recorded from the vastus lateralis (VL) during voluntary contractions held at 25% maximal knee extensor strength in 22 young (mean ± SD, 25.3 ± 4.8 years) and 20 physically active older men (71.4 ± 6.2 years). MUP size, firing rates, phases, turns and near fibre (NF) jiggle were determined and MU number estimates (MUNEs) were made by comparing average surface MUP with maximal electrically‐evoked compound muscle action potentials. Quadriceps cross‐sectional area was measured by magnetic resonance imaging. In total, 379 individual MUs were sampled in younger men and 346 in older men. Compared to the MU in younger participants, those in older participants had 8% lower firing rates and larger MUP size (+25%), as well as increased complexity, as indicated by phases (+13%), turns (+20%) and NF jiggle (+11%) (all P < 0.0005). The MUNE values (derived from the area of muscle in range of the surface‐electrode) in older participants were ∼70% of those in the young (P < 0.05). Taking into consideration the 30% smaller cross‐sectional area of the VL, the total number of MUs in the older muscles was between 50% and 60% lower compared to in young muscles (P < 0

  10. Neuromuscular Adaptations to Unilateral vs. Bilateral Strength Training in Women.

    PubMed

    Botton, Cíntia E; Radaelli, Regis; Wilhelm, Eurico N; Rech, Anderson; Brown, Lee E; Pinto, Ronei S

    2016-07-01

    Botton, CE, Radaelli, R, Wilhelm, EN, Rech, A, Brown, LE, and Pinto, RS. Neuromuscular adaptations to unilateral vs. bilateral strength training in women. J Strength Cond Res 30(7): 1924-1932, 2016-Considering the bilateral deficit, the sum of forces produced by each limb in a unilateral condition is generally greater than that produced by them in a bilateral condition. Therefore, it can be speculated that performing unilateral strength exercises may allow greater training workloads and subsequently greater neuromuscular adaptations when compared with bilateral training. Hence, the purpose of this study was to compare neuromuscular adaptations with unilateral vs. bilateral training in the knee extensor muscles. Forty-three recreationally active young women were allocated to a control, unilateral (UG) or bilateral (BG) training group, which performed 2 times strength training sessions a week for 12 weeks. Knee extension one repetition maximum (1RM), maximal isometric strength, muscle electrical activity, and muscle thickness were obtained before and after the study period. Muscle strength was measured in unilateral (right + left) and bilateral tests. Both UG and BG increased similarly their unilateral 1RM (33.3 ± 14.3% vs. 24.6 ± 11.9%, respectively), bilateral 1RM (20.3 ± 6.8% vs. 28.5 ± 12.3%, respectively), and isometric strength (14.7 ± 11.3% vs. 13.1 ± 12.5%, respectively). The UG demonstrated greater unilateral isometric strength increase than the BG (21.4 ± 10.5% vs. 10.3 ± 11.1%, respectively) and only the UG increased muscle electrical activity. Muscle thickness increased similarly for both training groups. Neither group exhibited pretesting 1RM bilateral deficit values, but at post-testing, UG showed a significant bilateral deficit (-6.5 ± 7.8%) whereas BG showed a significant bilateral facilitation (5.9 ± 9.0%). Thus, performing unilateral or bilateral exercises was not a decisive factor for improving morphological adaptations and bilateral

  11. A feedback inclusive neuromuscular training program alters frontal plane kinematics.

    PubMed

    Greska, Eric K; Cortes, Nelson; Van Lunen, Bonnie L; Oñate, James A

    2012-06-01

    Anterior cruciate ligament (ACL) neuromuscular training programs have demonstrated beneficial effects in reducing ACL injuries, yet further evaluation of their effects on biomechanical measures across a sports team season is required to elucidate the specific factors that are modifiable. The purpose of this study was to evaluate the effects of a 10-week off-season neuromuscular training program on lower extremity kinematics. Twelve Division I female soccer players (age: 19.2 ± 0.8 years, height: 1.67 ± 0.1 m, weight: 60.2 ± 6.5 kg) performed unanticipated dynamic trials of a running stop-jump task pretraining and posttraining. Data collection was performed using an 8-camera Vicon system (Los Angeles, CA, USA) and 2 Bertec (Columbus, OH, USA) force plates. The 10-week training program consisted of resistance training 2 times per week and field training, consisting of plyometric, agility, and speed drills, 2 times per week. Repeated measures analyses of variance (ANOVAs) were used to assess the differences between pretraining and posttraining kinetics and kinematics of the hip, knee, and ankle at initial contact (IC), peak knee flexion (PKF), and peak stance. Repeated measures ANOVAs were also used to assess isometric strength differences pretraining and posttraining. The alpha level was set at 0.05 a priori. The training program demonstrated significant increases in left hip extension, left and right hip flexion, and right hip adduction isometric strength. At IC, knee abduction angle moved from an abducted to an adducted position (-1.48 ± 3.65° to 1.46 ± 3.86°, p = 0.007), and hip abduction angle increased (-6.05 ± 4.63° to -10.34 ± 6.83°, p = 0.007). Hip abduction angle at PKF increased (-2.23 ± 3.40° to 6.01 ± 3.82°, p = 0.002). The maximum knee extension moment achieved at peak stance increased from pretraining to posttraining (2.02 ± 0.32 to 2.38 ± 0.75 N·m·kg⁻¹, p = 0.027). The neuromuscular training program demonstrated a potential

  12. Kinematic And Neuromuscular Measures Of Intensity During Plyometric Jumps.

    PubMed

    Andrade, David Cristóbal; Manzo, Oscar; Beltrán, Ana Rosa; Álvarez, Cristian; Del Rio, Rodrigo; Toledo, Camilo; Moran, Jason; Ramirez-Campillo, Rodrigo

    2017-08-15

    The aim of this study was to assess jumping performance and neuromuscular activity in lower limb muscles after drop jumps (DJ) from different drop heights (intensity) and during continuous jumping (fatigue), using markers such as reactive strength, jump height, mechanical power and surface electromyography (sEMG). The eccentric (EC) and concentric (CON) sEMG from the medial gastrocnemius (MG), biceps femoris (BF) and rectus (R) muscles were assessed during all tests. In a cross-sectional, randomized study, eleven volleyball players (age 24.4±3.2 years) completed 20 to 90-cm (DJ20 to DJ90) drop jumps and a 60-s continuous jump test. A one-way ANOVA test was used for comparisons, with Sidak post-hoc. The α level was <0.05. Reactive strength was greater for DJ40 compared to DJ90 (p<0.05; ES: 1.27). Additionally jump height was greater for DJ40 and DJ60 compared to DJ20 (p<0.05; ES: 1.26 and 1.27, respectively). No clear pattern of neuromuscular activity appeared during DJ20 to DJ90: some muscles showed greater, lower, or no change with increasing heights for both agonist and antagonist muscles, as well as for eccentric and concentric activity. Mechanical power, but not reactive strength, was reduced in the 60-s jump test (p<0.05; ES: 3.46). No changes were observed in sEMG for any muscle during the eccentric phase nor for the R muscle during the concentric phase of the 60-s jump test. However, for both MG and BF, concentric sEMG was reduced during the 60-s jump test (p<0.05; ES: 5.10 and 4.61, respectively). In conclusion, jumping performance and neuromuscular markers are sensitive to DJ height (intensity), although not in a clear dose-response fashion. In addition, markers such as mechanical power and sEMG are especially sensitive to the effects of continuous jumping (fatigue). Therefore, increasing the drop height during DJ does not ensure a greater training intensity and a combination of different drop heights may be required to elicit adaptations.

  13. Antiepileptic-induced resistance to neuromuscular blockers: mechanisms and clinical significance.

    PubMed

    Soriano, Sulpicio G; Martyn, J A Jeevendra

    2004-01-01

    Prolonged administration of antiepileptic drugs is associated with several drug interactions. In the field of anaesthesia and critical care, patients exhibit both sensitivity and resistance to non-depolarising neuromuscular blockers (NDNMBs) after acute and long-term administration of antiepileptic drugs, respectively. Although antiepileptic therapy alone has only mild neuromuscular effects, acutely administered antiepileptic drugs can potentiate the neuromuscular effects of NDNMBs as a result of direct pre- and post-junctional effects. Resistance to NDNMBs during long-term antiepileptic therapy is due to multiple factors operating alone or in combination, including induction of hepatic drug metabolism, increased protein binding of the NDNMBs and/or upregulation of acetylcholine receptors.

  14. The use of neuromuscular blocking agents in the ICU: where are we now?

    PubMed

    Greenberg, Steven B; Vender, Jeffery

    2013-05-01

    Intensivists use neuromuscular blocking agents for a variety of clinical conditions, including for emergency intubation, acute respiratory distress syndrome, status asthmaticus, elevated intracranial pressure, elevated intra-abdominal pressure, and therapeutic hypothermia after ventricular fibrillation-associated cardiac arrest. The continued creation and use of evidence-based guidelines and protocols could ensure that neuromuscular blocking agents are used and monitored appropriately. A collaborative multidisciplinary approach coupled with constant review of the pharmacology, dosing, drug interactions, and monitoring techniques may reduce the adverse events associated with the use of neuromuscular blocking agents.

  15. Immunohistochemical characterization of capsular cells in neuromuscular spindles of the neck.

    PubMed

    Triantafyllou, Asterios

    2007-09-01

    To identify capsular components of neuromuscular spindles in man by means of immunohistochemistry. Investigation of histologically observed neuromuscular spindles in surgical specimens with the use of markers for sheath cells and basement membranes. Epithelial membrane antigen and CD34 immunoreactivities were found in the outer and inner capsular layers, respectively. S-100 protein was not expressed in the capsules and there was more collagen type IV than laminin. Cells resembling perineurial cells and endoneurial fibroblasts, and basement membrane rich in collagen type IV comprise the capsules of neuromuscular spindles.

  16. Review of Continuous Infusion Neuromuscular Blocking Agents in the Adult Intensive Care Unit.

    PubMed

    Smetana, Keaton S; Roe, Neil A; Doepker, Bruce A; Jones, G Morgan

    The use of continuous infusion neuromuscular blocking agents remains controversial. The clinical benefit of these medications may be overshadowed by concerns of propagating intensive care unit-acquired weakness, which may prolong mechanical ventilation and impair the inability to assess neurologic function or pain. Despite these risks, the use of neuromuscular blocking agents in the intensive care unit is indicated in numerous clinical situations. Understanding pharmacologic nuances and clinical roles of these agents will aid in facilitating safe use in a variety of acute disease processes. This article provides clinicians with information regarding pharmacologic differences, indication for use, adverse effects, recommended doses, ancillary care, and monitoring among agents used for continuous neuromuscular blockade.

  17. Neuromuscular adaptations during the acquisition of muscle strength, power and motor tasks.

    PubMed

    Moritani, T

    1993-01-01

    Neuromuscular performance is determined not only by the size of the involved muscles, but also by the ability of the nervous system to appropriately activate the muscles. Adaptive changes in the nervous system in response to training are referred to as neural adaptation. This article briefly reviews current evidence regarding the neural adaptations during the acquisition of muscle strength power and motor tasks and will be organized under four main topics, namely: (i) muscle strength gain: neural factors versus hypertrophy, (ii) neural adaptations during power training, (iii) neuromuscular adaptations during the acquisition of a motor task, and (iv) neuromuscular adaptations during a ballistic movement.

  18. Neuromuscular medicine competency in physical medicine and rehabilitation residents: a method of development and assessment.

    PubMed

    Lin, Lei; Cuccurullo, Sara J; Innerfield, Caitlin E; Strax, Thomas E; Petagna, Anne

    2013-03-01

    This project endeavored to create an educational module including methodology to instruct physical medicine and rehabilitation residents in the evaluation and appropriate treatment of patients with neuromuscular disorders. It further sought to verify acquired competencies in neuromuscular rehabilitation through objective evaluation methodology. An American Association of Neuromuscular and Electrodiagnostic Medicine board-certified physician with 10 yrs of clinical experience in neuromuscular and general rehabilitation trained 19 residents using a standardized competency-based module. The residents were trained through clinical training, lectures, and review of self-assessment examination concepts from the American Academy of Physical Medicine & Rehabilitation syllabus provided in the Archives of Physical Medicine and Rehabilitation. After delivery of the educational module, knowledge acquisition and skill proficiency were measured in (1) completion of neuromuscular history and physical examination satisfactorily, (2) diagnosis and ability to design a patient care management plan via chart stimulated recall examinations, (3) physician-patient interaction via patient surveys, (4) physician-staff interaction via 360-degree global ratings, and (5) ability to write a comprehensive patient care report and to document a patient care management plan in accordance with Medicare guidelines via written patient reports. Assessment tools developed for this program address the basic competencies outlined by the Accreditation Council for Graduate Medical Education. To test the success of the standardized educational module, data were collected on an ongoing basis. The objective measures compared resident self-assessment examination scores in neuromuscular rehabilitation before and after the institution of the comprehensive neuromuscular competency module in the residency program. Nineteen (100%) of 19 residents successfully demonstrated proficiency in every segment of the

  19. Neuronal activity-dependent membrane traffic at the neuromuscular junction

    PubMed Central

    Miana-Mena, Francisco Javier; Roux, Sylvie; Benichou, Jean-Claude; Osta, Rosario; Brûlet, Philippe

    2002-01-01

    During development and also in adulthood, synaptic connections are modulated by neuronal activity. To follow such modifications in vivo, new genetic tools are designed. The nontoxic C-terminal fragment of tetanus toxin (TTC) fused to a reporter gene such as LacZ retains the retrograde and transsynaptic transport abilities of the holotoxin itself. In this work, the hybrid protein is injected intramuscularly to analyze in vivo the mechanisms of intracellular and transneuronal traffics at the neuromuscular junction (NMJ). Traffic on both sides of the synapse are strongly dependent on presynaptic neural cell activity. In muscle, a directional membrane traffic concentrates β-galactosidase-TTC hybrid protein into the NMJ postsynaptic side. In neurons, the probe is sorted across the cell to dendrites and subsequently to an interconnected neuron. Such fusion protein, sensitive to presynaptic neuronal activity, would be extremely useful to analyze morphological changes and plasticity at the NMJ. PMID:11880654

  20. Calcium imaging at the Drosophila larval neuromuscular junction.

    PubMed

    Macleod, Gregory T

    2012-07-01

    Calcium imaging uses optical imaging techniques to measure the concentration of free calcium [Ca(2+)] in live cells. It is a highly informative technique in neurobiology because Ca(2+) is involved in many neuronal signaling pathways and serves as the trigger for neurotransmitter release. The technique relies on loading Ca(2+) indicators into cells, measuring the quantity and/or wavelength of the photons emitted by the Ca(2+) indicator, and interpreting these data in terms of [Ca(2+)]. There are several possible methods for loading synthetic Ca(2+) indicators into subcellular compartments, for example, topical application of membrane-permeant Ca(2+) indicators, forward-filling of dextran conjugates, and direct injection. These techniques are applicable to calcium imaging at the Drosophila larval neuromuscular junction (NMJ), and are also readily adaptable to Drosophila embryo and adult preparations.

  1. A novel synaptic plasticity rule explains homeostasis of neuromuscular transmission

    PubMed Central

    Ouanounou, Gilles; Baux, Gérard; Bal, Thierry

    2016-01-01

    Excitability differs among muscle fibers and undergoes continuous changes during development and growth, yet the neuromuscular synapse maintains a remarkable fidelity of execution. Here we show in two evolutionarily distant vertebrates (Xenopus laevis cell culture and mouse nerve-muscle ex-vivo) that the skeletal muscle cell constantly senses, through two identified calcium signals, synaptic events and their efficacy in eliciting spikes. These sensors trigger retrograde signal(s) that control presynaptic neurotransmitter release, resulting in synaptic potentiation or depression. In the absence of spikes, synaptic events trigger potentiation. Once the synapse is sufficiently strong to initiate spiking, the occurrence of these spikes activates a negative retrograde feedback. These opposing signals dynamically balance the synapse in order to continuously adjust neurotransmitter release to a level matching current muscle cell excitability. DOI: http://dx.doi.org/10.7554/eLife.12190.001 PMID:27138195

  2. Pulmonary complications of neuromuscular disease: a respiratory mechanics perspective.

    PubMed

    Allen, Julian

    2010-03-01

    Paediatric neuromuscular disease compromises both the gas exchange and pump functions of the respiratory system. This can have profound implications for both growth and development of the respiratory system, as well as morbidity and mortality. Aspiration lung disease is common, and leads to increasingly restrictive pulmonary physiology over time. Abnormal lung and chest wall mechanics, and weak respiratory muscles, can combine to cause respiratory failure. Improving the balance between the work of breathing (by decreasing the respiratory load) and the respiratory pump (by improving respiratory muscle strength and decreasing respiratory muscle fatigue) can help prevent the onset of respiratory failure. Airway clearance techniques and non-invasive ventilation are two important tools in this effort. Better ways of assessing the respiratory pump, mechanical function, control and fatigue are needed especially in children.

  3. Neuromuscular Electrical Stimulation Therapy for Dysphagia Caused by Wilson's Disease

    PubMed Central

    Lee, Seon Yeong; Yang, Hee Seung; Lee, Seung Hwa; Jeung, Hae Won; Park, Young Ok

    2012-01-01

    Wilson's disease is an autosomal recessive disorder of abnormal copper metabolism. Although dysphagia is a common complaint of patients with Wilson's disease and pneumonia is an important cause of death in these patients, management of swallowing function has rarely been reported in the context of Wilson's disease. Hence, we report a case of Wilson's disease presenting with dysphagia. A 33-year-old man visited our hospital with a complaint of difficulty in swallowing, since about last 7 years and which had worsened since the last 2-3 months. He was diagnosed with Wilson's disease about 13 years ago. On the initial VFSS, reduced hyoid bone movement, impaired epiglottic movement and moderate amount of residue in the valleculae during the pharyngeal phase were noted. After 10 sessions of neuromuscular electrical stimulation for 1 hour per day, decreased amount of residue was observed in the valleculae during the pharyngeal phase on the follow-up VFSS. PMID:22837979

  4. Neuromuscular block after intra-arterially injected acetylcholine

    PubMed Central

    Tonali, P.; Gambi, D.

    1973-01-01

    The neuromuscular depolarizing block induced by intra-arterially injected ACh was studied to determine the variability in the same subject and in different subjects without disorders at the motor end-plate. Amplitude of action potentials of the opponens pollicis muscle evoked by intermittent repetitive supramaximal stimulation of the median nerve at the wrist were recorded for one hour from the beginning of ACh injection. The features of prompt and late depression stages after the injection were analysed statistically. Re-testing of the same subjects after a while shows that, in spite of all efforts to maintain the same experimental conditions, variations do occur in late depression. Time course and duration are particularly affected, while the degree of depression is altered but slightly. The presence of such variations limits this test to evaluation of the influence of other factors only within their already established statistical limits. Images PMID:4350703

  5. Isozyme patterns and protein profiles in neuromuscular disorders.

    PubMed

    Edwards, Y H; Tipler, T D; Morgan-Hughes, J A; Neerunjun, J S; Hopkinson, D A

    1982-06-01

    The isozyme patterns of six different enzymes and the polypeptide profiles of soluble proteins have been examined in muscle biopsy specimens from 74 patients with a wide variety of neuromuscular disorders. About half of the samples showed unusual features in at least one, and often several, of the enzymes and proteins tested. The extent of the biochemical abnormalities was roughly proportional to the severity of the disorders. In all cases the unusual isozymes and polypeptide profiles seemed to reflect a reversion to the fetal pattern of gene expression. However, this change appeared to occur in extant muscle and was not dependent on the appearance of new muscle fibres. Among the enzymes, phosphoglycerate mutase followed by creatine kinase appeared to be the most sensitive index of muscle disorder. The extent of the change in the muscle creatine kinase isozyme pattern was not correlated with the levels of serum creatine kinase activity.

  6. Isozyme patterns and protein profiles in neuromuscular disorders.

    PubMed Central

    Edwards, Y H; Tipler, T D; Morgan-Hughes, J A; Neerunjun, J S; Hopkinson, D A

    1982-01-01

    The isozyme patterns of six different enzymes and the polypeptide profiles of soluble proteins have been examined in muscle biopsy specimens from 74 patients with a wide variety of neuromuscular disorders. About half of the samples showed unusual features in at least one, and often several, of the enzymes and proteins tested. The extent of the biochemical abnormalities was roughly proportional to the severity of the disorders. In all cases the unusual isozymes and polypeptide profiles seemed to reflect a reversion to the fetal pattern of gene expression. However, this change appeared to occur in extant muscle and was not dependent on the appearance of new muscle fibres. Among the enzymes, phosphoglycerate mutase followed by creatine kinase appeared to be the most sensitive index of muscle disorder. The extent of the change in the muscle creatine kinase isozyme pattern was not correlated with the levels of serum creatine kinase activity. Images PMID:6286971

  7. Presynaptic elements involved in the maintenance of the neuromuscular junction

    NASA Technical Reports Server (NTRS)

    Burrows, G. H.

    1984-01-01

    Alterations in the neuromuscular junction were observed in rats preceding loss of muscle mass. In view of the possibility that these alterations involve changes in the secretion of myotrophic agents by presynaptic motor neurons, an investigation was undertaken to characterize a neuronall factor which is thought to be involved in the initiation and maintenance of cholinergic synapses. This factor, which is secreted into the incubation medium by NG108-15 neuroblastoma x glioma hybrid cells, induces the aggregation of nicotinic acetylcholine receptors on primary cultures of rat hindlimb myotubes. Previous attempts to purify this factor failed. Extensive washing of the NG108-15 cells with hepes-buffered salt solution followed by short (4 hour) collection times resulted in the collection of incubation medium containing maximal aggregation activity with as little as 5 ug secreted protein per ml of fresh medium. A three-fold increase in specific activity was obtained after anion exchange chromatography.

  8. Neuromuscular electrical stimulation for muscle wasting in heart failure patients.

    PubMed

    Saitoh, Masakazu; Dos Santos, Marcelo Rodrigues; Anker, Markus; Anker, Stefan D; von Haehling, Stephan; Springer, Jochen

    2016-12-15

    Neuromuscular electrical stimulation (NMES) seems to be safe and beneficial in improvement in functional capacity, muscle strength, and quality of life when compared with conventional aerobic exercise, while the change in muscle fiber composition and muscle size was conflicting in patients with heart failure (HF). Moreover, NMES studies seem to have beneficial effects on pro-inflammatory cytokine, oxidative enzyme activity, and protein anabolic and catabolic metabolism that are the key molecular mechanism of muscle wasting in patients with HF. We review specific issues related to the effects of NMES on muscle wasting in patients with HF, whether NMES seems to be an alternative exercise modality preventing or improving in muscle wasting for HF patients who are unable or unwilling to engage in conventional exercise training; however no established strategies currently exist to focus on the patients with HF accompanied by muscle wasting. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  9. Abdominal compartment syndrome successfully treated with neuromuscular blockade

    PubMed Central

    Chiles, Kris T; Feeney, Colin M

    2011-01-01

    A 48 year old male admitted to the intensive care unit after a cardiac arrest complicated by a stroke intra-operatively during automatic implantable cardioverter defibrillator placement. He post-operatively developed a rigid abdomen, elevated peak and plateau pressures, hypoxia and renal insufficiency. He was diagnosed with abdominal compartment syndrome with an intra-abdominal compartment pressure of 40mmHg. The patient was administered 10 mg of intravenous cisatracuriumbesylate in preparation for bedside surgical abdominal decompression. Cisatracurium eliminated the patients need for surgical intervention by reducing his abdominal compartment pressures to normal and improving his hypoxia and renal function. This case illustrates that neuromuscular blockade should be attempted in patients with abdominal compartment syndrome prior to surgical intervention. PMID:22013257

  10. Bone Health and Associated Metabolic Complications in Neuromuscular Diseases

    PubMed Central

    Joyce, Nanette C.; Hache, Lauren P.; Clemens, Paula R.

    2014-01-01

    Synopsis This article reviews the recent literature regarding bone health as it relates to the patient living with neuromuscular disease (NMD). Poor bone health with related morbidity is a significant problem for patients with NMD. Although the evidence addressing issues of bone health and osteoporosis have increased as a result of the Bone and Joint Decade, studies defining the scope of bone-related disease in NMD are scant. The available evidence is discussed focusing on abnormal calcium metabolism, increased fracture risk, and the prevalence of both scoliosis and hypovitaminosis D in Duchenne muscular dystrophy, amyotrophic lateral sclerosis and spinal muscular atrophy. These problems appear common. Osteomalacia often complicates disease-related baseline osteoporosis and may reduce fracture risk if treated. Future directions are discussed, including the urgent need for studies to both determine the nature and extent of poor bone health, and to evaluate the therapeutic effect of available osteoporosis treatments in patients with NMD. PMID:23137737

  11. Presynaptic elements involved in the maintenance of the neuromuscular junction

    NASA Technical Reports Server (NTRS)

    Burrows, G. H.

    1984-01-01

    Alterations in the neuromuscular junction were observed in rats preceding loss of muscle mass. In view of the possibility that these alterations involve changes in the secretion of myotrophic agents by presynaptic motor neurons, an investigation was undertaken to characterize a neuronall factor which is thought to be involved in the initiation and maintenance of cholinergic synapses. This factor, which is secreted into the incubation medium by NG108-15 neuroblastoma x glioma hybrid cells, induces the aggregation of nicotinic acetylcholine receptors on primary cultures of rat hindlimb myotubes. Previous attempts to purify this factor failed. Extensive washing of the NG108-15 cells with hepes-buffered salt solution followed by short (4 hour) collection times resulted in the collection of incubation medium containing maximal aggregation activity with as little as 5 ug secreted protein per ml of fresh medium. A three-fold increase in specific activity was obtained after anion exchange chromatography.

  12. Mechanism of blockade of neuromuscular transmission by pentobarbital.

    PubMed

    Seyama, I; Narahashi, T

    1975-01-01

    The mechanism of block of neuromuscular transmission by pentobarbital has been studied in the frog sciatic nerve-sartorius muscle preparation by means of intracellular microelectrode and voltage clamp techniques. The resting membrane potential was decreased by pentobarbital only to a small extent (less than 15 mV) in both end-plate and non-end-plate regions. Both sodium and potassium components of end-plate current underwent drastic changes after application of pentobarbital. The peak amplitude was decreased with an apparent dissociation constant of 0.9 mM for both currents. The maximum rate of rise of end-plate current was reduced, with apparent dissociation constants of 0.9 and 1.2 mM for sodium and potassium currents, respectively. The times for sodium and potassium end-plate current to reach their peaks were shortened only to a negligible extent. The falling phase of end-plate current was greatly accelerated, sodium current being affected more than potassium current. The transient end-plate depolarization induced by iontophoretic application of acetylcholine was suppressed more effectively than end-plate potential by application of pentobarbital. The falling phase of the former was also shortened. The quantal content of end-plate potential tended to increase at 0.5 mM, but underwent no appreciable change at 1.0 and 1.4 mM. Pentobarbital has a dual action on both quantal content and end-plate membrane depending on the concentration, and the block of neuromuscular transmission is due primarily to a suppression of the end-plate sensitivity to acetylcholine. The differential effect of pentobarbital on sodium and potassium components of end-plate current is compatible with the notion that these two ionic conductances are separate entities.

  13. Influence of resistance load on neuromuscular response to vibration training.

    PubMed

    Luo, Jin; Clarke, Michael; McNamara, Brian; Moran, Kieran

    2009-03-01

    The purpose of this study was to examine the influence of resistance load on the acute and acute residual effects of vibration training, with vibration applied directly to the bicep tendon in a maximal-effort dynamic resistance exercise (3 sets of maximal-effort bicep curls). Eleven participants were exposed to 4 training conditions in random order: exercise with 1 of 2 different loads (40% 1-repetition maximum [RM] or 70% 1RM load) combined with 1 of 2 vibration conditions (vibration [1.2 mm, 65 Hz] or sham vibration). Five minutes before and after the exercise, a set of maximal-effort bicep curls with a load of either 40 or 70% 1RM was performed as the pre- and posttraining test. Concentric elbow joint angular velocity, moment and power, and bicep root mean square electromyography (EMGrms) were measured during training and in the pre- and posttraining tests. The results show that during training (acute effect) and at 5 minutes after training (acute residual effect), vibration did not induce a significant change in EMGrms, mean and peak angular velocities, moment and power, time to peak power, and initial power at 100 milliseconds after the start of the concentric phase for either resistance load. Therefore, in aiming to train neuromuscular output using maximal-effort dynamic contractions (40 and 70% 1RM), there is no benefit in employing direct vibration, at least with a 1.2-mm amplitude and 65-Hz frequency. However, the amplitude of 1.2 mm may be too high to effectively stimulate neuromuscular output in maximal-effort dynamic contractions per se.

  14. Neuromuscular training in construction workers: a longitudinal controlled pilot study.

    PubMed

    Faude, Oliver; Donath, Lars; Bopp, Micha; Hofmann, Sara; Erlacher, Daniel; Zahner, Lukas

    2015-08-01

    Many accidents at construction sites are due to falls. An exercise-based workplace intervention may improve intrinsic fall risk factors. In this pilot study, we aimed at evaluating the effects of neuromuscular exercise on static and functional balance performance as well as on lower limb explosive power in construction workers. Healthy middle-aged construction workers were non-randomly assigned to an intervention [N = 20, age = 40.3 (SD 8.3) years] or a control group [N = 20, age = 41.8 (9.9) years]. The intervention group performed static and dynamic balance and strength exercises (13 weeks, 15 min each day). Before and after the intervention and after an 8-week follow-up, unilateral postural sway, backward balancing (on 3- and 4.5-cm-wide beams) as well as vertical jump height were assessed. We observed a group × time interaction for postural sway (p = 0.002) with a reduction in the intervention group and no relevant change in the control group. Similarly, the number of successful steps while walking backwards on the 3-cm beam increased only in the intervention group (p = 0.047). These effects were likely to most likely practically beneficial from pretest to posttest and to follow-up test for postural sway (+12%, standardized mean difference (SMD) = 0.65 and 17%, SMD = 0.92) and backward balancing on the 3-cm beam (+58%, SMD = 0.59 and 37%, SMD = 0.40). Fifteen minutes of neuromuscular training each day can improve balance performance in construction workers and, thus, may contribute to a decreased fall risk.

  15. THREE-DIMENSIONAL ULTRASTRUCTURE OF THE CRAYFISH NEUROMUSCULAR APPARATUS

    PubMed Central

    Jahromi, S. S.; Atwood, H. L.

    1974-01-01

    The synapse-bearing nerve terminals of the opener muscle of the crayfish Procambarus were reconstructed using electron micrographs of regions which had been serially sectioned. The branching patterns of the terminals of excitatory and inhibitory axons and the locations and sizes of neuromuscular and axo-axonal synapses were studied. Excitatory and inhibitory synapses could be distinguished not only on the basis of differences in synaptic vesicles, but also by a difference in density of pre- and postsynaptic membranes. Synapses of both axons usually had one or more sharply localized presynaptic "dense bodies" around which synaptic vesicles appeared to cluster. Some synapses did not have the dense bodies. These structures may be involved in the physiological activity of the synapse. Excitatory axon terminals had more synapses, and a larger percentage of terminal surface area devoted to synaptic contacts, than inhibitory axon terminals. However, the largest synapses of the inhibitory axon exceeded in surface area those of the excitatory axon. Both axons had many side branches coming from the main terminal; often, the side branches were joined to the main terminal by narrow necks. A greater percentage of surface area was devoted to synapses in side branches than in the main terminal. Only a small fraction of total surface area was devoted to axo-axonal synapses, but these were often located at narrow necks or constrictions of the excitatory axon. This arrangement would result in effective blockage of spike invasion of regions of the terminal distal to the synapse, and would allow relatively few synapses to exert a powerful effect on transmitter release from the excitatory axon. A hypothesis to account for the development of the neuromuscular apparatus is presented, in which it is suggested that production of new synapses is more important than enlargement of old ones as a mechanism for allowing the axon to adjust transmitter output to the functional needs of the muscle

  16. Neuromuscular and Metabolic Responses to Three Different Resistance Exercise Methods

    PubMed Central

    Arazi, Hamid; Mirzaei, Bahman; Heidari, Naser

    2013-01-01

    Purpose The aim of this study was to compare the effect of resistance exercise with three different methods on integrated electromyography (IEMG) and metabolic responses in recreational athletes. Methods Twenty four males (mean 23.59±0.87 years) were randomly assigned to three experimental groups. Participants performed knee extension exercises: Slow (SL: 3-3, 3s for each concentric and eccentric action with 50% of 1 RM), Normal (NH: 1-1, 1 s for each concentric and eccentric action 80% of 1 RM) and Traditional (TH: 2-4, 2s for concentric and 4s for eccentric action with 80% of 1 RM). Plasma lactate, glucose and triglyceride concentration and IEMG was measured before and immediately after performing four sets of resistance exercise. Results Each method significantly decreased IEMG (P<0.05), but there was no significant difference between groups. Lactate was increased following TH and NH more than SL method (P<0.05). Each method significantly increased plasma glucose (P<0.05). Work considering time under tension (workTUT) was higher (P<0.05) during TH method than the other methods and during SL it was higher than NH method (P<0.05). Volume load was higher (P<0.05) during NH than the other two methods and during TH it was higher than SL method (P<0.05). Conclusion These results indicate that exercise intensity during the resistance exercise is important for the enhancement of lactate responses, but the slow resistance exercise method could induce acute neuromuscular response as much as high intensity methods. It seems that this method will be advantageous for those who want to increase acute neuromuscular changes with low exercise intensity and volume. PMID:24868429

  17. Combined application of neuromuscular electrical stimulation and voluntary muscular contractions.

    PubMed

    Paillard, Thierry

    2008-01-01

    Electromyostimulation (EMS) and voluntary muscle contraction (VC) constitute different modes of muscle activation and induce different acute physiological effects on the neuromuscular system. Long-term application of each mode of muscle activation can produce different muscle adaptations. It seems theoretically possible to completely or partially cumulate the muscle adaptations induced by each mode of muscle activation applied separately. This work consisted of examining the literature concerning the muscle adaptations induced by long-term application of the combined technique (CT) [i.e. EMS is combined with VC - non-simultaneously] compared with VC and/or EMS alone in healthy subjects and/or athletes and in post-operative knee-injured subjects. In general, CT induced greater muscular adaptations than VC whether in sports training or rehabilitation. This efficiency would be due to the fact that CT can facilitate cumulative effects of training completely or partially induced by VC and EMS practiced alone. CT also provides a greater improvement of the performance of complex dynamic movements than VC. However, EMS cannot improve coordination between different agonistic and antagonistic muscles and thus does not facilitate learning the specific coordination of complex movements. Hence, EMS should be combined with specific sport training to generate neuromuscular adaptations, but also allow the adjustment of motor control during a voluntary movement. Likewise, in a therapeutic context, CT was particularly efficient to accelerate recovery of muscle contractility during a rehabilitation programme. Strength loss and atrophy inherent in a traumatism and/or a surgical operation would be more efficiently compensated with CT than with VC. Furthermore, CT also restored more functional abilities than VC. Finally, in a rehabilitation context, EMS is complementary to voluntary exercise because in the early phase of rehabilitation it elicits a strength increase, which is necessary

  18. Continuous administration of pyridostigmine improves immobilization-induced neuromuscular weakness.

    PubMed

    Frick, Christiane G; Helming, Marc; Martyn, J A Jeevendra; Blobner, Manfred; Fink, Heidrun

    2010-03-01

    To investigate the effects of continuous pyridostigmine infusion on immobilization-induced muscle weakness. Critical illness often results in immobilization of limb and respiratory muscles, leading to muscle atrophy and up-regulation of nicotinic acetylcholine receptors. Pyridostigmine reversibly blocks acetylcholinesterase and has the potential to improve neuromuscular transmission and decrease acetylcholine receptor number. Prospective, randomized, controlled experimental study. Animal laboratory, university hospital. Male Sprague-Dawley rats. A total of 40 rats were immobilized in one hind limb by pinning knee and ankle joints. Rats received either continuous pyridostigmine (15 mg.kg.day) or saline subcutaneously via implanted osmotic pumps. After 7 days and 14 days of immobilization, neuromuscular function, atracurium pharmacodynamics, and expression of acetylcholine receptors were evaluated. At 7 days and 14 days after immobilization, muscle force decreased in all untreated groups, whereas effective doses for paralysis with atracurium and acetylcholine receptor number in the tibialis were significantly increased. Pyridostigmine-treated rats showed a significantly improved muscle force and muscle mass in the immobilized limb. This was associated with an attenuation of acetylcholine receptor up-regulation in the respective leg. At this time, the dose-response curve for atracurium on the immobilized side was shifted to the left in the pyridostigmine group. After 14 days, muscle tension was still less depressed with pyridostigmine infusion, and resistance to the effects of atracurium was still attenuated. However, there were no differences in acetylcholine receptor expression between the immobilized sides of both groups. Continuous pyridostigmine infusion improves muscle weakness after 7 days and 14 days of immobilization. The up-regulation of acetylcholine receptors and the concomitant resistance to atracurium is attenuated in animals treated with pyridostigmine

  19. Cost-effectiveness of shared medical appointments for neuromuscular patients.

    PubMed

    Seesing, Femke M; Groenewoud, Hans J; Drost, Gea; van Engelen, Baziel G M; van der Wilt, Gert Jan

    2015-08-18

    To assess whether shared medical appointments (SMAs) for neuromuscular patients represent a way of using clinicians' time efficiently without compromising quality of care for patients. Patients with a chronic neuromuscular disease (NMD) (n = 272) were randomly allocated to either an SMA or a regular individual annual appointment and followed up for a period of 6 months. Data on resource utilization and quality of life (EQ-5D) were collected prospectively, using a health care perspective. Incremental costs and changes in quality-adjusted life-years (QALYs) were computed using a probabilistic decision model. Factors critical to the incremental cost-effectiveness of SMAs were explored in sensitivity analyses. No substantial differences between SMAs and individual visits in terms of costs per QALY were found (incremental cost-effectiveness ratio €-960.00; 95% confidence interval €-34,600.00, €+36,800.00). Sensitivity analyses showed that the cost-effectiveness ratio was particularly sensitive to SMA group size and proportion of patients seeing their treating neurologist. Cost-effectiveness of SMAs did not show a significant difference vs that of individual appointments based on data from our randomized controlled trial. On the other hand, we were able to show that a minimum of 6 patients per SMA and 75% of patients attending their treating neurologist are specific conditions under which SMAs qualify as a cost-effective alternative. This implies that SMAs may be a means to increase productivity of the physician without compromising quality of care. This study provides Class III evidence that SMAs are not significantly more cost-effective than individual appointments for patients with NMDs. The study lacks the precision to exclude important differences in cost-effectiveness between SMAs and individual appointments. © 2015 American Academy of Neurology.

  20. A Pilot Study of Diagnostic Neuromuscular Ultrasound in Bell's Palsy.

    PubMed

    Tawfik, Eman A; Walker, Francis O; Cartwright, Michael S

    2015-01-01

    Neuromuscular ultrasound of the cranial nerves is an emerging field which may help in the assessment of cranial neuropathies. The aim of this study was to evaluate the role of neuromuscular ultrasound in Bell's palsy. A second objective was to assess the possibility of any associated vagus nerve abnormality. Twenty healthy controls and 12 Bell's palsy patients were recruited. The bilateral facial nerves, vagus nerves, and frontalis muscles were scanned using an 18 MHz linear array transducer. Facial nerve diameter, vagus nerve cross-sectional area, and frontalis thickness were measured. Mean facial nerve diameter was .8 ± .2 mm in controls and 1.1 ± .3 mm in patients group. The facial nerve diameter was significantly larger in patients than controls (P = .006, 95% CI for the difference between groups of .12-.48), with a significant side-to-side difference in patients as well (P = .004, 95% CI for side-to-side difference of .08-.52). ROC curve analysis of the absolute facial nerve diameter revealed a sensitivity of 75% and a specificity of 70%. No significant differences in vagus nerve cross-sectional area or frontalis thickness were detected between patients and controls. Ultrasound can detect facial nerve enlargement in Bell's palsy and may have a role in assessment, or follow-up, of Bell's palsy and other facial nerve disorders. The low sensitivity of the current technique precludes its routine use for diagnosis, however, this study demonstrates its validity and potential for future research. Copyright © 2015 by the American Society of Neuroimaging.

  1. LRP4 Is Critical for Neuromuscular Junction Maintenance

    PubMed Central

    Barik, Arnab; Lu, Yisheng; Sathyamurthy, Anupama; Bowman, Andrew; Shen, Chengyong; Li, Lei; Xiong, Wen-cheng

    2014-01-01

    The neuromuscular junction (NMJ) is a synapse between motor neurons and skeletal muscle fibers, and is critical for control of muscle contraction. Its formation requires neuronal agrin that acts by binding to LRP4 to stimulate MuSK. Mutations have been identified in agrin, MuSK, and LRP4 in patients with congenital myasthenic syndrome, and patients with myasthenia gravis develop antibodies against agrin, LRP4, and MuSK. However, it remains unclear whether the agrin signaling pathway is critical for NMJ maintenance because null mutation of any of the three genes is perinatal lethal. In this study, we generated imKO mice, a mutant strain whose LRP4 gene can be deleted in muscles by doxycycline (Dox) treatment. Ablation of the LRP4 gene in adult muscle enabled studies of its role in NMJ maintenance. We demonstrate that Dox treatment of P30 mice reduced muscle strength and compound muscle action potentials. AChR clusters became fragmented with diminished junctional folds and synaptic vesicles. The amplitude and frequency of miniature endplate potentials were reduced, indicating impaired neuromuscular transmission and providing cellular mechanisms of adult LRP4 deficiency. We showed that LRP4 ablation led to the loss of synaptic agrin and the 90 kDa fragments, which occurred ahead of other prejunctional and postjunctional components, suggesting that LRP4 may regulate the stability of synaptic agrin. These observations demonstrate that LRP4 is essential for maintaining the structural and functional integrity of the NMJ and that loss of muscle LRP4 in adulthood alone is sufficient to cause myasthenic symptoms. PMID:25319686

  2. Neuromuscular Strain Increases Symptom Intensity in Chronic Fatigue Syndrome

    PubMed Central

    Rowe, Peter C.; Fontaine, Kevin R.; Lauver, Megan; Jasion, Samantha E.; Marden, Colleen L.; Moni, Malini; Thompson, Carol B.; Violand, Richard L.

    2016-01-01

    Chronic fatigue syndrome (CFS) is a complex, multisystem disorder that can be disabling. CFS symptoms can be provoked by increased physical or cognitive activity, and by orthostatic stress. In preliminary work, we noted that CFS symptoms also could be provoked by application of longitudinal neural and soft tissue strain to the limbs and spine of affected individuals. In this study we measured the responses to a straight leg raise neuromuscular strain maneuver in individuals with CFS and healthy controls. We randomly assigned 60 individuals with CFS and 20 healthy controls to either a 15 minute period of passive supine straight leg raise (true neuromuscular strain) or a sham straight leg raise. The primary outcome measure was the symptom intensity difference between the scores during and 24 hours after the study maneuver compared to baseline. Fatigue, body pain, lightheadedness, concentration difficulties, and headache scores were measured individually on a 0–10 scale, and summed to create a composite symptom score. Compared to individuals with CFS in the sham strain group, those with CFS in the true strain group reported significantly increased body pain (P = 0.04) and concentration difficulties (P = 0.02) as well as increased composite symptom scores (all P = 0.03) during the maneuver. After 24 hours, the symptom intensity differences were significantly greater for the CFS true strain group for the individual symptom of lightheadedness (P = 0.001) and for the composite symptom score (P = 0.005). During and 24 hours after the exposure to the true strain maneuver, those with CFS had significantly higher individual and composite symptom intensity changes compared to the healthy controls. We conclude that a longitudinal strain applied to the nerves and soft tissues of the lower limb is capable of increasing symptom intensity in individuals with CFS for up to 24 hours. These findings support our preliminary observations that increased mechanical sensitivity may be a

  3. Effects of neuromuscular lags on controlling contact transitions

    PubMed Central

    Venkadesan, Madhusudhan; Valero-Cuevas, Francisco J.

    2009-01-01

    We present a numerical exploration of contact transitions with the fingertip. When picking up objects our fingertips must make contact at specific locations, and—upon contact—maintain posture while producing well-directed force vectors. However, the joint torques for moving the fingertip towards a surface (τm) are different from those for producing static force vectors (τf). We previously described the neural control of such abrupt transitions in humans, and found that unavoidable errors arise because sensorimotor time delays and lags prevent an instantaneous switch between different torques. Here, we use numerical optimization on a finger model to reveal physical bounds for controlling such rapid contact transitions. Resembling human data, it is necessary to anticipatorily switch joint torques to τf at about 30 ms before contact to minimize the initial misdirection of the fingertip force vector. This anticipatory strategy arises in our deterministic model from neuromuscular lags, and not from optimizing for robustness to noise/uncertainties. Importantly, the optimal solution also leads to a trade-off between the speed of force magnitude increase versus the accuracy of initial force direction. This is an alternative to prevailing theories that propose multiplicative noise in muscles as the driver of speed–accuracy trade-offs. We instead find that the speed–accuracy trade-off arises solely from neuromuscular lags. Finally, because our model intentionally uses idealized assumptions, its agreement with human data suggests that the biological system is controlled in a way that approaches the physical boundaries of performance. PMID:19218157

  4. Systemic inflammatory response and neuromuscular involvement in amyotrophic lateral sclerosis

    PubMed Central

    Lu, Ching-Hua; Allen, Kezia; Oei, Felicia; Leoni, Emanuela; Kuhle, Jens; Tree, Timothy; Fratta, Pietro; Sharma, Nikhil; Sidle, Katie; Howard, Robin; Orrell, Richard; Fish, Mark; Greensmith, Linda; Pearce, Neil; Gallo, Valentina

    2016-01-01

    Objective: To evaluate the combined blood expression of neuromuscular and inflammatory biomarkers as predictors of disease progression and prognosis in amyotrophic lateral sclerosis (ALS). Methods: Logistic regression adjusted for markers of the systemic inflammatory state and principal component analysis were carried out on plasma levels of creatine kinase (CK), ferritin, and 11 cytokines measured in 95 patients with ALS and 88 healthy controls. Levels of circulating biomarkers were used to study survival by Cox regression analysis and correlated with disease progression and neurofilament light chain (NfL) levels available from a previous study. Cytokines expression was also tested in blood samples longitudinally collected for up to 4 years from 59 patients with ALS. Results: Significantly higher levels of CK, ferritin, tumor necrosis factor (TNF)–α, and interleukin (IL)–1β, IL-2, IL-8, IL-12p70, IL-4, IL-5, IL-10, and IL-13 and lower levels of interferon (IFN)–γ were found in plasma samples from patients with ALS compared to controls. IL-6, TNF-α, and IFN-γ were the most highly regulated markers when all explanatory variables were jointly analyzed. High ferritin and IL-2 levels were predictors of poor survival. IL-5 levels were positively correlated with CK, as was TNF-α with NfL. IL-6 was strongly associated with CRP levels and was the only marker showing increasing expression towards end-stage disease in the longitudinal analysis. Conclusions: Neuromuscular pathology in ALS involves the systemic regulation of inflammatory markers mostly active on T-cell immune responses. Disease stratification based on the prognostic value of circulating inflammatory markers could improve clinical trials design in ALS. PMID:27308305

  5. Effects of neuromuscular lags on controlling contact transitions.

    PubMed

    Venkadesan, Madhusudhan; Valero-Cuevas, Francisco J

    2009-03-28

    We present a numerical exploration of contact transitions with the fingertip. When picking up objects our fingertips must make contact at specific locations, and-upon contact-maintain posture while producing well-directed force vectors. However, the joint torques for moving the fingertip towards a surface (tau(m)) are different from those for producing static force vectors (tau(f)). We previously described the neural control of such abrupt transitions in humans, and found that unavoidable errors arise because sensorimotor time delays and lags prevent an instantaneous switch between different torques. Here, we use numerical optimization on a finger model to reveal physical bounds for controlling such rapid contact transitions. Resembling human data, it is necessary to anticipatorily switch joint torques to tau(f )at about 30 ms before contact to minimize the initial misdirection of the fingertip force vector. This anticipatory strategy arises in our deterministic model from neuromuscular lags, and not from optimizing for robustness to noise/uncertainties. Importantly, the optimal solution also leads to a trade-off between the speed of force magnitude increase versus the accuracy of initial force direction. This is an alternative to prevailing theories that propose multiplicative noise in muscles as the driver of speed-accuracy trade-offs. We instead find that the speed-accuracy trade-off arises solely from neuromuscular lags. Finally, because our model intentionally uses idealized assumptions, its agreement with human data suggests that the biological system is controlled in a way that approaches the physical boundaries of performance.

  6. Isokinetic testing in patients with neuromuscular diseases: a focused review.

    PubMed

    El Mhandi, Lhassan; Bethoux, François

    2013-02-01

    This literature review aimed to study the use of isokinetic testing in patients with neuromuscular diseases (NMDs) and to identify directions for future research of isokinetic testing. The MEDLINE (January 1, 1965, to July 1, 2010), Cumulative Index to Nursing and Allied Health (1980 to May 2010), and Cochrane Central Register of Controlled Trials (The Cochrane Library Issue 3, 2009) electronic databases were searched. The literature search was conducted using the keywords muscle assessment, muscle strength, evaluation, isokinetic, neuromuscular diseases, muscle fatigue, functional test, rehabilitation, and literature search. Relevant references cited in the selected articles were also considered, regardless of the year of publication. The search strategy yielded 13 articles involving a variety of patients with known NMDs. All studies demonstrated that isokinetic dynamometry is appropriate and safe for ambulatory patients with NMDs. Isokinetic testing has proven to be reliable (intratest/intertest correlation coefficient ranged from 0.65 to 0.98), with the proximal muscles having the highest reliability, and sensitive to disease progression and to the effects of various therapeutic interventions. However, isokinetic testing has never gained wide acceptance, partly because of concerns about stabilizing the dynamometer and the subject during the test and of the lack of standardized protocols for isokinetic strength measurement. Isokinetic testing is an important part of the comprehensive evaluation and rehabilitation of patients with NMD. Research has demonstrated its efficacy in providing clinically relevant information. When integrated with a complete history, physical examination, and functional evaluation, isokinetic testing and exercise can be a valuable tool for the clinician in the assessment, rehabilitation, and performance enhancement of patients with NMD. Such equipment, however, has several disadvantages, rendering it usually impractical in the clinical

  7. Neuromuscular anatomy and evolution of the cetacean forelimb.

    PubMed

    Cooper, Lisa Noelle; Dawson, Susan D; Reidenberg, Joy S; Berta, Annalisa

    2007-09-01

    The forelimb of cetaceans (whales, dolphins, and porpoises) has been radically modified during the limb-to-flipper transition. Extant cetaceans have a soft tissue flipper encasing the manus and acting as a hydrofoil to generate lift. The neuromuscular anatomy that controls flipper movement, however, is poorly understood. This study documents flipper neuromuscular anatomy and tests the hypothesis that antebrachial muscle robustness is related to body size. Data were gathered during dissections of 22 flippers, representing 15 species (7 odontocetes, 15 mysticetes). Results were compared with published descriptions of both artiodactyls and secondarily aquatic vertebrates. Results indicate muscle robustness is best predicted by taxonomic distribution and is not a function of body size. All cetaceans have atrophied triceps muscles, an immobile cubital joint, and lack most connective tissue structures and manus muscles. Forelimbs retain only three muscle groups: triceps (only the scapular head is functional as the humeral heads are vestigal), and antebrachial extensors and flexors. Well-developed flexor and extensor muscles were found in mysticetes and basal odontocetes (i.e., physeterids, kogiids, and ziphiids), whereas later diverging odontocetes (i.e., monodontids, phocoenids, and delphinids) lack or reduce these muscles. Balaenopterid mysticetes (e.g., fin and minke whales) may actively change flipper curvature, while basal odontocetes (e.g., sperm and beaked whales) probably stiffen the flipper through isometric contraction. Later diverging odontocetes lack musculature supporting digital movements and are unable to manipulate flipper curvature. Cetacean forelimbs are unique in that they have lost agility and several soft tissue structures, but retain sensory innervations.

  8. Neural drive preservation after detraining following neuromuscular electrical stimulation training.

    PubMed

    Gondin, Julien; Duclay, Julien; Martin, Alain

    2006-12-06

    The purpose of the study was to investigate the behaviour of the central nervous system when 5 weeks of neuromuscular electrical stimulation (NMES) training was followed by 5 weeks of detraining. Nineteen males were divided into the neuromuscular electrostimulated group (EG, n=12) and the control group (CG, n=7). The training program consisted of 15 sessions of isometric NMES over a 5-week period. The EG subjects were tested before training (PRE), after 5 weeks of NMES training (POST) and after 5 weeks of detraining (DE) while CG subjects were only tested at PRE and at POST. Soleus (SOL) and gastrocnemii (GAS) maximal H-reflex and M-wave potentials were evoked at rest (i.e., H(max) and M(max), respectively) and during maximal voluntary contraction (MVC) (i.e., H(sup) and M(sup), respectively). SOL and GAS V-wave were recorded by supramaximal stimulation delivered during MVC. SOL and GAS electromyographic (EMG) activity as well as muscle activation were also assessed during MVC. After training, plantar flexor MVC increased significantly by 22% (P<0.001). Torque gains were associated with an increase in muscle activation (P<0.05), SOL and GAS normalized EMG activity (P<0.01 and P<0.05, respectively) and V/M(sup) ratios (P<0.01 and P<0.05, respectively). No significant changes occurred in any of these parameters between POST and DE. H(max)/M(max) and H(sup)/M(sup) ratios for both muscles were unchanged after both the training and detraining periods. In conclusion, the NMES training-induced neural adaptations were maintained after detraining, suggesting that neural changes are long-lasting and did not affect the elements of H-reflex pathways.

  9. Reliability and minimal detectable change in scapulothoracic neuromuscular activity.

    PubMed

    Seitz, Amee L; Uhl, Timothy L

    2012-12-01

    Alterations in scapular muscle activity, including excess activation of the upper trapezius (UT) and onset latencies of the lower trapezius (LT) and serratus anterior (SA) muscles, are associated with abnormal scapular motion and shoulder impingement. Limited information exists on the reliability of neuromuscular activity to demonstrate the efficacy of interventions. The purpose of this study was to characterize the reproducibility of scapular muscle activity (mean activity, relative onset timing) over time and establish the minimal detectable change (MDC). Surface electromyography (sEMG) of the UT, LT, SA and anterior deltoid (AD) muscles in 16 adults were captured during an overhead lifting task in two sessions, one-week apart. sEMG data were also normalized to maximum isometric contraction and the relative onset and mean muscle activity during concentric and eccentric phases of the scapular muscles were calculated. Additionally, reliability of the absolute sEMG data during the lifting task and MVIC was evaluated. Both intrasession and intersession reliability of normalized and absolute mean scapular muscle activity, assessed with intraclass correlation coefficients (ICC), ranged from 0.62 to 0.99; MDC values were between 1.3% and 11.7% MVIC and 24 to 135 mV absolute sEMG. Reliability of sEMG during MVIC was ICC = 0.82-0.99, with the exception of intersession upper trapezius reliability (ICC = 0.36). Within session reliability of muscle onset times was ICC = 0.88-0.97, but between session reliability was lower with ICC = 0.43-0.73; MDC were between 39 and 237 ms. Small changes in scapular neuromuscular mean activity (>11.7% MVIC) can be interpreted as meaningful change, while change in muscle onset timing in light of specific processing parameters used in this study is more variable. Copyright © 2012 Elsevier Ltd. All rights reserved.

  10. Age-related neuromuscular function during drop jumps.

    PubMed

    Hoffrén, M; Ishikawa, M; Komi, P V

    2007-10-01

    Muscle- and movement-specific fascicle-tendon interaction affects the performance of the neuromuscular system. This interaction is unknown among elderly and consequently contributes to the lack of understanding the age-related problems on neuromuscular control. The present experiment studied the age specificity of fascicle-tendon interaction of the gastrocnemius medialis (GM) muscle in drop jump (DJ) exercises. Twelve young and thirteen elderly subjects performed maximal squat jumps and DJs with maximal rebound effort on a sledge apparatus. Ankle and knee joint angles, reaction force, and electromyography (EMG) from the soleus (Sol), GM, and tibialis anterior (TA) muscles were measured together with the GM fascicle length by ultrasonography. The results showed that the measured ankle joint stiffness (AJS) during the braking phase correlated positively with the rebound speed in both age groups and that both parameters were significantly lower in the elderly than in young subjects. In both groups, the AJS correlated positively with averaged EMG (aEMG) in Sol during the braking phase and was further associated with GM activation (r = 0.55, P < 0.01) and TA coactivation (TA/GM r = -0.4 P < 0.05) in the elderly subjects. In addition, compared with the young subjects, the elderly subjects showed significantly lower GM aEMG in the braking phase and higher aEMG in the push-off phase, indicating less utilization of tendinous tissue (TT) elasticity. These different activation patterns are in line with the mechanical behavior of GM showing significantly less fascicle shortening and relative TT stretching in the braking phase in the elderly than in the young subjects. These results suggest that age-specific muscle activation patterns as well as mechanical behaviors exist during DJs.

  11. Differentiation of identifiable lobster neuromuscular synapses during development.

    PubMed

    Govind, C K; Meiss, D E; Pearce, J

    1982-04-01

    The ultrastructure of physiologically identified low and high release synapses arising from a single axon on fibres of the distal accessory flexor muscle (DAFM) in a mature lobster was examined by serial section electron microscopy. Low release neuromuscular terminals located only on the proximal fibre were characterized by large synapses (mean area 2.084 micron2), small presynaptic dense bars (mean are 0.021 micron2) and hence a low (2.3%) ratio of dense bar area to synaptic area. In contrast high output terminals located only on the distal fibre had smaller synapses (mean area 0.625 micron2), large dense bars (mean area 0.066 micron2) and a high (23.9%) ratio of bar area to synaptic area. A similar ratio was consistently found for each synaptic type in several other examples of mature lobsters. Hence it was used as a criterion for determining the point at which differentiation occurs during development. In the first larval stage (24 h old) the innervation was localized and undifferentiated. In the fourth (2 week old) and twelfth (1 y old) stage lobsters, the innervation had proliferated to small bundles of proximal and distal fibres. During development synapses increase in their mean surface area in the proximal fibre while remaining constant in the distal fibre. The mean surface area of the dense bars is similar in all stages except for the proximal fibres of the twelfth stage where it is smaller by 50%. Similarly the ratio fo dense bar area to synaptic area is not significantly different for all stages except for the twelfth stage proximal fibres where it is half the value. Consequently differentiation of low and high release neuromuscular terminals occurs by the twelfth stage with an increase in the mean surface area of synapses and a decrease in the mean surface area of dense bars. This morphological differentiation is enhanced in the mature lobster.

  12. [THE VIBRATION TRAINING AS SARCOPENIA INTERVENTION: IMPACT ON THE NEUROMUSCULAR SYSTEM OF THE ELDERLY].

    PubMed

    Palop Montoro, María Victoria; Párraga Montilla, Juan Antonio; Lozano Aguilera, Emilio; Arteaga Checa, Milagros

    2015-10-01

    Introducción: el envejecimiento se acompaña de una reducción progresiva de la masa muscular que contribuye al desarrollo de limitaciones funcionales, donde el entrenamiento vibratorio puede ser una opción de intervención óptima en la prevención y tratamiento de la sarcopenia. Objetivo: comprobar la efectividad del entrenamiento de vibraciones de cuerpo completo en el sistema neuromuscular de los adultos mayores. Métodos: revisión sistemática en las bases de datos Medline, CINAHL, WOS y PEDro, mediante la combinación de los descriptores del Medical Subjects Headings (MeSH) referentes a entrenamiento vibratorio, fuerza muscular, masa muscular y personas mayores. Resultados: fueron encontrados un total de 214 estudios sobre el entrenamiento vibratorio en personas mayores, bien como única intervención o en combinación con otros ejercicios, de los cuales 45 cumplían los criterios de selección. De ellos, 30 artículos fueron eliminados por no superar los 5 puntos según la escala de PEDro. Se incluyeron para el análisis final 15 ensayos clínicos. Conclusión: el entrenamiento con plataformas vibratorias demuestra ser un método de entrenamiento de la fuerza seguro, adecuado y eficaz para la población de mayor edad, pero con resultados similares al ejercicio de resistencia convencional, en la prevención y tratamiento de la sarcopenia.

  13. Impaired voluntary neuromuscular activation limits muscle power in mobility-limited older adults

    USDA-ARS?s Scientific Manuscript database

    Background. Age-related alterations of neuromuscular activation may contribute to deficits in muscle power and mobility function. This study assesses whether impaired activation of the agonist quadriceps and antagonist hamstrings, including amplitude- and velocity-dependent characteristics of activa...

  14. Impaired neuromuscular transmission and response to acetylcholinesterase inhibitors in centronuclear myopathies.

    PubMed

    Robb, Stephanie A; Sewry, Caroline A; Dowling, James J; Feng, Lucy; Cullup, Tom; Lillis, Sue; Abbs, Stephen; Lees, Melissa M; Laporte, Jocelyn; Manzur, Adnan Y; Knight, Ravi K; Mills, Kerry R; Pike, Michael G; Kress, Wolfram; Beeson, David; Jungbluth, Heinz; Pitt, Matthew C; Muntoni, Francesco

    2011-06-01

    Many clinical features of autosomal centronuclear myopathies (CNM) and X-linked myotubular myopathy (XLMTM) are common to congenital myasthenic syndromes (CMS). We describe three children whose clinical and electrophysiological findings originally suggested CMS, in whom CNM was diagnosed pathologically, though not yet genetically characterised. A fourth case, with XLMTM, also showed electrophysiological features of a neuromuscular transmission defect. Three (including the XLMTM case) showed improved strength with acetylcholinesterase inhibitor treatment. We also studied neuromuscular junction structure and function in the MTM1 knockdown zebrafish model of XLMTM, demonstrating abnormal neuromuscular junction organization; anticholinesterase therapy resulted in marked clinical response. These observations suggest that a neuromuscular transmission defect may accompany CNM and contribute to muscle weakness. Muscle biopsy should be considered in infants suspected to have CMS, especially if treatment response is incomplete, or no CMS gene mutation is identified. Treatment with acetylcholinesterase inhibitors may benefit some CNM patients. This warrants further confirmation. Copyright © 2011 Elsevier B.V. All rights reserved.

  15. The Dutch neuromuscular database CRAMP (Computer Registry of All Myopathies and Polyneuropathies): development and preliminary data.

    PubMed

    van Engelen, B G M; van Veenendaal, H; van Doorn, P A; Faber, C G; van der Hoeven, J H; Janssen, N G; Notermans, N C; van Schaik, I N; Visser, L H; Verschuuren, J J G M

    2007-01-01

    Each of the various neuromuscular diseases is rare. Consequently, solid epidemiological data are not available and it is often difficult to find sufficient patients for studies. For this reason, the Dutch neuromuscular database, CRAMP (Computer Registry of All Myopathies and Polyneuropathies), was developed in 2004 by the Dutch Neuromuscular Research Support Centre, to store information on patient characteristics and diagnoses (based on Rowland and McLeod's classification) in a uniform and easily retrievable manner. Care was taken to preserve data confidentiality. It is envisaged that CRAMP will prove particularly useful for studies in which multicentre collaboration is needed to recruit a sufficiently large number of patients. More than 10,000 patients with neuromuscular diseases (4,837 female, 5,476 male) have been registered since 2004, half of whom (n=5059) have peripheral nerve disorders.

  16. British Thoracic Society guideline for respiratory management of children with neuromuscular weakness: commentary.

    PubMed

    Hull, Jeremy

    2012-07-01

    The British Thoracic Society guideline for respiratory management of children with neuromuscular weakness summarises the available evidence in this field and provides recommendations that will aid healthcare professionals in delivering good quality patient care.

  17. Using foot pressure to maintain neuromuscular function during long-duration spaceflight

    NASA Astrophysics Data System (ADS)

    Layne, Charles S.; Mulavara, Ajitkumar P.; McDonald, P. Vernon; Pruett, Casey J.; Bloomberg, Jacob J.; Kozlovskaya, Inessa B.

    2000-01-01

    It is proposed that the application of foot pressure can result in enhanced lower limb neuromuscular activation over the course of a long-duration spaceflight. If confirmed, a device that generates increased foot sensory input can be developed for use on the International Space Station as a countermeasure to neuromuscular degradation. In the present experiment, surface EMG was collected from six subjects during freefloating arm raises performed with or without foot pressure. Two subjects performed the experiment multiple times during their flight. The results indicate that foot pressure results in significantly increased magnitudes of lower limb neuromuscular activation when compared to movements performed without foot pressure. Furthermore, repeated application of pressure throughout a flight continues to produce enhanced neuromuscular activation without significant modification of the phasic activation features. These findings suggest that the development of a countermeasure utilizing foot pressure merits continued exploration. .

  18. Ultrasound of oral and masticatory muscles: Why every neuromuscular swallow team should have an ultrasound machine.

    PubMed

    Van Den Engel-Hoek, Lenie; Lagarde, Marloes; Van Alfen, Nens

    2017-03-01

    Patients with neuromuscular disorders often present with swallowing difficulties due to oral phase problems and pharyngeal residue after swallow. It is important to assess the underlying pathology and cause of the swallowing disturbance in this patient group, such as dystrophic changes in oral and masticatory muscles. This allows for more patient-tailored recommendations, for example optimal compensation strategies to maintain function for longer. Ultrasound can show structural changes caused by dystrophy or denervation of muscles, detect involuntary movements such as fasciculations, and provide dynamic video images of tongue motion during swallowing attempts. This article, based on the authors' extensive experience with ultrasound in neuromuscular disease, explains the concepts of oral muscle ultrasound and its proven value in assessing neuromuscular mastication and swallowing problems. As a patient-friendly and portable technique, we advocate its use as a standard tool for analyzing neuromuscular dysphagia. Clin. Anat. 30:183-193, 2017. © 2017 Wiley Periodicals, Inc.

  19. Robotics, assistive technology, and occupational therapy management to improve upper limb function in pediatric neuromuscular diseases.

    PubMed

    Rahman, Tariq; Basante, Joseph; Alexander, Michael

    2012-08-01

    This article presents an overview of occupational therapy assessments and treatment options for individuals with neuromuscular disabilities, with a particular focus on children with neuromuscular disorders. The discussion includes descriptions of standard treatments, commercial adaptive equipment, and homemade adaptive solutions. The state of the art in therapeutic and assistive robots and orthoses for the upper and lower extremity is also provided. Copyright © 2012. Published by Elsevier Inc.

  20. Proprioceptive Neuromuscular Facilitation (PNF): Its Mechanisms and Effects on Range of Motion and Muscular Function.

    PubMed

    Hindle, Kayla B; Whitcomb, Tyler J; Briggs, Wyatt O; Hong, Junggi

    2012-03-01

    Proprioceptive neuromuscular facilitation (PNF) is common practice for increasing range of motion, though little research has been done to evaluate theories behind it. The purpose of this study was to review possible mechanisms, proposed theories, and physiological changes that occur due to proprioceptive neuromuscular facilitation techniques. Four theoretical mechanisms were identified: autogenic inhibition, reciprocal inhibition, stress relaxation, and the gate control theory. The studies suggest that a combination of these four mechanisms enhance range of motion. When completed prior to exercise, proprioceptive neuromuscular facilitation decreases performance in maximal effort exercises. When this stretching technique is performed consistently and post exercise, it increases athletic performance, along with range of motion. Little investigation has been done regarding the theoretical mechanisms of proprioceptive neuromuscular facilitation, though four mechanisms were identified from the literature. As stated, the main goal of proprioceptive neuromuscular facilitation is to increase range of motion and performance. Studies found both of these to be true when completed under the correct conditions. These mechanisms were found to be plausible; however, further investigation needs to be conducted. All four mechanisms behind the stretching technique explain the reasoning behind the increase in range of motion, as well as in strength and athletic performance. Proprioceptive neuromuscular facilitation shows potential benefits if performed correctly and consistently.

  1. Effects of neuromuscular training on knee joint stability after anterior cruciate ligament reconstruction

    PubMed Central

    Shim, Jae-Kwang; Choi, Ho-Suk; Shin, Jun-Ho

    2015-01-01

    [Purpose] This study examined the effects of neuromuscular training on knee joint stability after anterior cruciate ligament reconstruction. [Subjects and Methods] The subjects were 16 adults who underwent arthroscopic anterior cruciate reconstruction and neuromuscular training. The Lysholm scale was used to assess functional disorders on the affected knee joint. A KT-2000 arthrometer was used to measure anterior displacement of the tibia against the femur. Surface electromyography was used to detect the muscle activation of the vastus medialis oblique, vastus lateralis, biceps femoris, and semitendinosus before and after neuromuscular training. [Results] There was significant relaxation in tibial anterior displacement of the affected and sound sides in the supine position before neuromuscular training. Furthermore, the difference in the tibial anterior displacement of the affected knee joints in the standing position was reduced after neuromuscular training. Moreover, the variation of the muscle activation evoked higher muscle activation of the vastus medialis oblique, vastus lateralis, biceps femoris, and semitendinosus. [Conclusion] Neuromuscular training may improve functional joint stability in patients with orthopedic musculoskeletal injuries in the postoperative period. PMID:26834316

  2. Appropriate dosing of sugammadex to reverse deep rocuronium-induced neuromuscular blockade in morbidly obese patients.

    PubMed

    Loupec, T; Frasca, D; Rousseau, N; Faure, J-P; Mimoz, O; Debaene, B

    2016-03-01

    In morbidly obese patients, the speed of reversal of neuromuscular blockade with sugammadex based on ideal body weight is still matter of debate. In this single-center, randomised, double-blinded study, neuromuscular blockade was monitored in 50 patients using acceleromyography at the adductor pollicis. At the end of surgery with deep rocuronium-induced neuromuscular blockade, patients randomly received sugammadex 4 mg.kg(-1) (high dose group), 2 mg.kg(-1) (middle dose group), or 1 mg.kg(-1) (low dose group) of ideal body weight. After administration of the first dose of sugammadex, the mean (SD) recovery time (censored at 600 s) from deep neuromuscular blockade was significantly shorter (p < 0.001) in the high-dose group (n = 14; 255 (63) s) vs the middle-dose group (n = 13; 429 (102) s), or low-dose group (n = 4; 581 (154) s). Success rate from neuromuscular blockade reversal defined by a train-of-four ≥ 0.9 within 10 min after sugammadex administration, were 93%, 77% and 22% for these high, middle and low-dose groups respectively (p < 0.05 vs low-dose group). In morbidly obese patients, 4 mg.kg(-1) of ideal body weight of sugammadex allows suitable reversal of deep rocuronium-induced neuromuscular blockade. Monitoring remains essential to detect residual curarisation or recurarisation.

  3. Sugammadex given for rocuronium-induced neuromuscular blockade in infants: a retrospectıve study.

    PubMed

    Ozmete, Ozlem; Bali, Cagla; Cok, Oya Yalcin; Turk, Hatice Evren Eker; Ozyilkan, Nesrın Bozdogan; Civi, Soner; Aribogan, Anıs

    2016-12-01

    To evaluate the efficacy and safety of sugammadex in reversing profound neuromuscular block induced by rocuronium in infant patients. Retrospective observational study. University teaching hospital. Twenty-six infants (2-12 months of age; 3-11 kg) with an American Society of Anesthesiologists classification I, II, or III who were scheduled to undergo neurosurgical procedures were included in the study. Anesthesia was induced with 5 mg/kg thiopental, 1 μg/kg fentanyl and 0.6 mg/kg rocuronium. Sevoflurane was administered to all patients after intubation. The neuromuscular block was monitored with acceleromyography using train-of-four (TOF) stimuli. Patients received additional doses of rocuronium to maintain a deep block during surgery. If profound neuromuscular block (TOF, 0) persisted at the end of the surgery, 3mg/kg sugammadex was administered. The demographic data, surgeries, and anesthetic agents were recorded. The time from sugammadex administration to recovery of neuromuscular function (TOF ratio, >0.9) and complications during and after extubation were also recorded. Twenty-six infants who had a deep neuromuscular block (TOF, 0) at the end of surgery received 3 mg/kg sugammadex. The mean recovery time of the T4/T1 ratio of 0.9 was 112 seconds. No clinical evidence of recurarization or residual curarization was observed. The efficacy and safety of sugammadex were confirmed in infant surgical patients for reversal of deep neuromuscular block induced by rocuronium. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. Proprioceptive Neuromuscular Facilitation (PNF): Its Mechanisms and Effects on Range of Motion and Muscular Function

    PubMed Central

    Hindle, Kayla B.; Whitcomb, Tyler J.; Briggs, Wyatt O.; Hong, Junggi

    2012-01-01

    Proprioceptive neuromuscular facilitation (PNF) is common practice for increasing range of motion, though little research has been done to evaluate theories behind it. The purpose of this study was to review possible mechanisms, proposed theories, and physiological changes that occur due to proprioceptive neuromuscular facilitation techniques. Four theoretical mechanisms were identified: autogenic inhibition, reciprocal inhibition, stress relaxation, and the gate control theory. The studies suggest that a combination of these four mechanisms enhance range of motion. When completed prior to exercise, proprioceptive neuromuscular facilitation decreases performance in maximal effort exercises. When this stretching technique is performed consistently and post exercise, it increases athletic performance, along with range of motion. Little investigation has been done regarding the theoretical mechanisms of proprioceptive neuromuscular facilitation, though four mechanisms were identified from the literature. As stated, the main goal of proprioceptive neuromuscular facilitation is to increase range of motion and performance. Studies found both of these to be true when completed under the correct conditions. These mechanisms were found to be plausible; however, further investigation needs to be conducted. All four mechanisms behind the stretching technique explain the reasoning behind the increase in range of motion, as well as in strength and athletic performance. Proprioceptive neuromuscular facilitation shows potential benefits if performed correctly and consistently. PMID:23487249

  5. Impaired neuromuscular transmission and skeletal muscle fiber necrosis in mice lacking Na/Ca exchanger 3

    PubMed Central

    Sokolow, Sophie; Manto, Mario; Gailly, Philippe; Molgó, Jordi; Vandebrouck, Clarisse; Vanderwinden, Jean-Marie; Herchuelz, Andre; Schurmans, Stéphane

    2004-01-01

    We produced and analyzed mice deficient for Na/Ca exchanger 3 (NCX3), a protein that mediates cellular Ca2+ efflux (forward mode) or Ca2+ influx (reverse mode) and thus controls intracellular Ca2+ concentration. NCX3-deficient mice (Ncx3–/–) present a skeletal muscle fiber necrosis and a defective neuromuscular transmission, reflecting the absence of NCX3 in the sarcolemma of the muscle fibers and at the neuromuscular junction. The defective neuromuscular transmission is characterized by the presence of electromyographic abnormalities, including low compound muscle action potential amplitude, a decremental response at low-frequency nerve stimulation, an incremental response, and a prominent postexercise facilitation at high-frequency nerve stimulation, as well as neuromuscular blocks. The analysis of quantal transmitter release in Ncx3–/– neuromuscular junctions revealed an important facilitation superimposed on the depression of synaptic responses and an elevated delayed release during high-frequency nerve stimulation. It is suggested that Ca2+ entering nerve terminals is cleared relatively slowly in the absence of NCX3, thereby enhancing residual Ca2+ and evoked and delayed quantal transmitter release during repetitive nerve stimulation. Our findings indicate that NCX3 plays an important role in vivo in the control of Ca2+ concentrations in the skeletal muscle fibers and at the neuromuscular junction. PMID:14722618

  6. Neuromuscular disorder in response to anterior cruciate ligament creep.

    PubMed

    Chu, Derrick; LeBlanc, Robby; D'Ambrosia, Peter; D'Ambrosia, Renee; Baratta, Richard V; Solomonow, Moshe

    2003-03-01

    To determine the effect of creep developed in the anterior cruciate ligament and other viscoelastic knee structures on the function of the flexor and extensor muscles of males and females. Static load applied to the proximal tibia of young healthy male and female subjects in a laboratory setting with maximal voluntary knee flexion and extension performed before and after the load application. Static loads applied to various joints during occupational and sports activities are epidemiologically linked to higher than normal rates of disability reports. The physiological and biomechanical processes active in the development of such a neuromuscular disorder are not known. We hypothesize that creep developed in the anterior cruciate ligament due to prolonged static load will have pronounced impact on the reflexive activation of the associated musculature in a manner that may increase the risk of injury. Females are expected to be exposed to higher risk than males. Male and female groups performed maximal voluntary knee flexion and extension before and after applying 200 and 150 N, respectively, to the proximal tibia for a 10 min period. Flexion and extension forces as well as electromyograph from agonist and antagonist muscles were measured at 35 degrees and 90 degrees knee flexion. Data was analyzed through repeated measures of analysis of variance. It was found that in extension, quadriceps electromyographic activity increased significantly after anterior cruciate ligament creep while hamstrings co-activation did not change. There was also a trend towards increased extension force after creep was developed, with significant effect of gender (larger increase in females). Similarly, significant increase in hamstrings electromyographic activity and a trend towards increased force during knee flexion was observed but with no effect of gender. Electromyographic spasms from the flexors and extensors were recorded in 30% of the subjects during the 10 min static loading

  7. TMEM184b Promotes Axon Degeneration and Neuromuscular Junction Maintenance.

    PubMed

    Bhattacharya, Martha R C; Geisler, Stefanie; Pittman, Sara K; Doan, Ryan A; Weihl, Conrad C; Milbrandt, Jeffrey; DiAntonio, Aaron

    2016-04-27

    Complex nervous systems achieve proper connectivity during development and must maintain these connections throughout life. The processes of axon and synaptic maintenance and axon degeneration after injury are jointly controlled by a number of proteins within neurons, including ubiquitin ligases and mitogen activated protein kinases. However, our understanding of these molecular cascades is incomplete. Here we describe the phenotype resulting from mutation of TMEM184b, a protein identified in a screen for axon degeneration mediators. TMEM184b is highly expressed in the mouse nervous system and is found in recycling endosomes in neuronal cell bodies and axons. Disruption of TMEM184b expression results in prolonged maintenance of peripheral axons following nerve injury, demonstrating a role for TMEM184b in axon degeneration. In contrast to this protective phenotype in axons, uninjured mutant mice have anatomical and functional impairments in the peripheral nervous system. Loss of TMEM184b causes swellings at neuromuscular junctions that become more numerous with age, demonstrating that TMEM184b is critical for the maintenance of synaptic architecture. These swellings contain abnormal multivesicular structures similar to those seen in patients with neurodegenerative disorders. Mutant animals also show abnormal sensory terminal morphology. TMEM184b mutant animals are deficient on the inverted screen test, illustrating a role for TMEM184b in sensory-motor function. Overall, we have identified an important function for TMEM184b in peripheral nerve terminal structure, function, and the axon degeneration pathway. Our work has identified both neuroprotective and neurodegenerative roles for a previously undescribed protein, TMEM184b. TMEM184b mutation causes delayed axon degeneration following peripheral nerve injury, indicating that it participates in the degeneration process. Simultaneously, TMEM184b mutation causes progressive structural abnormalities at neuromuscular

  8. Neuromuscular strategies contributing to faster multidirectional agility performance.

    PubMed

    Spiteri, Tania; Newton, Robert U; Nimphius, Sophia

    2015-08-01

    The aim of this study was to first determine differences in neuromuscular strategy between a faster and slower agility performance, and second compare differences in muscle activation strategy employed when performing two closely executed agility movements. Participants recruited from an elite female basketball team completed an ultrasound to determine quadriceps muscle-cross sectional area; reactive isometric mid-thigh pull to determine the rate of muscle activation, rate of force development, pre-motor time and motor time; and multidirectional agility tests completing two directional changes in response to a visual stimulus. Peak and average relative muscle activation of the rectus femoris, vastus medialis, vastus lateralis, biceps femoris, semitendinosus and gastrocnemius were measured 100ms prior to heel strike (pre-heel strike) and across stance phase for both directional changes. Faster agility performance was characterized by greater pre-heel strike muscle activity and greater anterior muscle activation during stance phase resulting in greater hip and knee extension increasing propulsive impulse. Differences between directional changes appear to result from processing speed, where a greater delay in refractory times during the second directional change resulted in greater anterior muscle activation, decelerating the body while movement direction was determined. Copyright © 2015 Elsevier Ltd. All rights reserved.

  9. Assisted vital capacity to assess recruitment level in neuromuscular diseases.

    PubMed

    Santos, Dante B; Boré, Aurélien; Castrillo, Lorena Del Amo; Lacombe, Matthieu; Falaize, Line; Orlikowski, David; Lofaso, Frédéric; Prigent, Hélène

    2017-09-01

    Respiratory muscle weakness and chest wall abnormalities in neuromuscular diseases (NMD) may lead to decreased pulmonary volumes. We assessed the reversibility of vital capacity (VC) reduction with mechanical In-Exsufflation (MI-E). We evaluated the effects of positive inspiratory and negative expiratory pressures on spirometric variables under passive (without patients' participation) and active (with active participation) application in 47 NMD patients. VC, inspiratory capacity (IC), expiratory reserve volume (ERV) were measured during maneuvers without and with MI-E assistance, delivering inspiratory assistance (+40cmH2O), expiratory assistance (-40cmH2O) and both (±40cmH2O). Passive and active assistance improved significantly VC and IC compared to baseline (P<0.0001 for both). ERV improved only with active assistance which normalized VC in 10, IC in 18 and ERV in 6 patients, mainly in patients with late-onset NMD. MI-E assistance produced greater increases in IC than in ERV, resulting in a VC increase enhanced by patients' active participation. This type of evaluation may help to evaluate the potential reversibility of restrictive ventilatory pattern in NMDs. Copyright © 2017 Elsevier B.V. All rights reserved.

  10. Role of analgesics, sedatives, neuromuscular blockers, and delirium.

    PubMed

    Hall, Jesse B; Schweickert, William; Kress, John P

    2009-10-01

    A major focus on critical care medicine concerns the institution of life-support therapies, such as mechanical ventilation, during periods of organ failure to permit a window of opportunity to diagnose and treat underlying disorders so that patients may be returned to their prior functional status upon recovery. With the growing success of these intensive care unit-based therapies and longer-term follow-up of patients, severe weakness involving the peripheral nervous system and muscles has been identified in many recovering patients, often confounding the time course or magnitude of recovery. Mechanical ventilation is often accompanied by pharmacologic treatments including analgesics, sedatives, and neuromuscular blockers. These drugs and the encephalopathies accompanying some forms of critical illness result in a high prevalence of delirium in mechanically ventilated patients. These drug effects likely contribute to an impaired ability to assess the magnitude of intensive care unit-acquired weakness, to additional time spent immobilized and mechanically ventilated, and to additional weakness from the patient's relative immobility and bedridden state. This review surveys recent literature documenting these relationships and identifying approaches to minimize pharmacologic contributions to intensive care unit-acquired weakness.

  11. Neuromuscular fiber segmentation through particle filtering and discrete optimization

    NASA Astrophysics Data System (ADS)

    Dietenbeck, Thomas; Varray, François; Kybic, Jan; Basset, Olivier; Cachard, Christian

    2014-03-01

    We present an algorithm to segment a set of parallel, intertwined and bifurcating fibers from 3D images, targeted for the identification of neuronal fibers in very large sets of 3D confocal microscopy images. The method consists of preprocessing, local calculation of fiber probabilities, seed detection, tracking by particle filtering, global supervised seed clustering and final voxel segmentation. The preprocessing uses a novel random local probability filtering (RLPF). The fiber probabilities computation is performed by means of SVM using steerable filters and the RLPF outputs as features. The global segmentation is solved by discrete optimization. The combination of global and local approaches makes the segmentation robust, yet the individual data blocks can be processed sequentially, limiting memory consumption. The method is automatic but efficient manual interactions are possible if needed. The method is validated on the Neuromuscular Projection Fibers dataset from the Diadem Challenge. On the 15 first blocks present, our method has a 99.4% detection rate. We also compare our segmentation results to a state-of-the-art method. On average, the performances of our method are either higher or equivalent to that of the state-of-the-art method but less user interactions is needed in our approach.

  12. Oxidative stress induces overgrowth of the Drosophila neuromuscular junction

    PubMed Central

    Milton, Valerie J.; Jarrett, Helen E.; Gowers, Kate; Chalak, Salma; Briggs, Laura; Robinson, Iain M.; Sweeney, Sean T.

    2011-01-01

    Synaptic terminals are known to expand and contract throughout an animal's life. The physiological constraints and demands that regulate appropriate synaptic growth and connectivity are currently poorly understood. In previous work, we identified a Drosophila model of lysosomal storage disease (LSD), spinster (spin), with larval neuromuscular synapse overgrowth. Here we identify a reactive oxygen species (ROS) burden in spin that may be attributable to previously identified lipofuscin deposition and lysosomal dysfunction, a cellular hallmark of LSD. Reducing ROS in spin mutants rescues synaptic overgrowth and electrophysiological deficits. Synapse overgrowth was also observed in mutants defective for protection from ROS and animals subjected to excessive ROS. ROS are known to stimulate JNK and fos signaling. Furthermore, JNK and fos in turn are known potent activators of synapse growth and function. Inhibiting JNK and fos activity in spin rescues synapse overgrowth and electrophysiological deficits. Similarly, inhibiting JNK, fos, and jun activity in animals with excessive oxidative stress rescues the overgrowth phenotype. These data suggest that ROS, via activation of the JNK signaling pathway, are a major regulator of synapse overgrowth. In LSD, increased autophagy contributes to lysosomal storage and, presumably, elevated levels of oxidative stress. In support of this suggestion, we report here that impaired autophagy function reverses synaptic overgrowth in spin. Our data describe a previously unexplored link between oxidative stress and synapse overgrowth via the JNK signaling pathway. PMID:21987827

  13. The rapid evolution of molecular genetic diagnostics in neuromuscular diseases.

    PubMed

    Volk, Alexander E; Kubisch, Christian

    2017-10-01

    The development of massively parallel sequencing (MPS) has revolutionized molecular genetic diagnostics in monogenic disorders. The present review gives a brief overview of different MPS-based approaches used in clinical diagnostics of neuromuscular disorders (NMDs) and highlights their advantages and limitations. MPS-based approaches like gene panel sequencing, (whole) exome sequencing, (whole) genome sequencing, and RNA sequencing have been used to identify the genetic cause in NMDs. Although gene panel sequencing has evolved as a standard test for heterogeneous diseases, it is still debated, mainly because of financial issues and unsolved problems of variant interpretation, whether genome sequencing (and to a lesser extent also exome sequencing) of single patients can already be regarded as routine diagnostics. However, it has been shown that the inclusion of parents and additional family members often leads to a substantial increase in the diagnostic yield in exome-wide/genome-wide MPS approaches. In addition, MPS-based RNA sequencing just enters the research and diagnostic scene. Next-generation sequencing increasingly enables the detection of the genetic cause in highly heterogeneous diseases like NMDs in an efficient and affordable way. Gene panel sequencing and family-based exome sequencing have been proven as potent and cost-efficient diagnostic tools. Although clinical validation and interpretation of genome sequencing is still challenging, diagnostic RNA sequencing represents a promising tool to bypass some hurdles of diagnostics using genomic DNA.

  14. Development of neuromuscular organization in the ctenophore Pleurobrachia bachei.

    PubMed

    Norekian, Tigran P; Moroz, Leonid L

    2016-01-01

    The phylogenetic position of the phylum Ctenophora and the nature of ctenphore nervous systems are highly debated topics in modern evolutionary biology. However, very little is known about the organization of ctenophore neural and muscular systems, and virtually nothing has been reported about their embryogenesis. Here we have characterized the neural and muscular development of the sea gooseberry, Pleurobrachia bachei, starting from the cleavage stages to posthatching larvae. Scanning electron microscopy and immunochemistry were used to describe the formation of the embryonic mouth, tentacles, combs, aboral organ, and putative sensory cells. The muscles started their specification at the end of the first day of Pleurobrachia development. In contrast, neurons appeared 2 days after myogenesis, just before the hatching of fully formed cydippid larvae. The first tubulin-immunoreactive neurons, a small group of four to six cells with neuronal processes, was initially recognized at the aboral pole during the third day of development. Surprisingly, this observed neurogenesis occurred after the emergence of distinct behavioral patterns in the embryos. Thus, the embryonic behavior associated with comb cilia beatings and initial muscle organization does not require morphologically defined neurons and their elongated neurites. This study provides the first description of neuromuscular development in the enigmatic ctenophores and establishes the foundation for future research using emerging genomic tools and resources. © 2015 Wiley Periodicals, Inc.

  15. Peripheral nerve and neuromuscular junction pathology in Pompe disease

    PubMed Central

    Falk, Darin J.; Todd, Adrian Gary; Lee, Sooyeon; Soustek, Meghan S.; ElMallah, Mai K.; Fuller, David D.; Notterpek, Lucia; Byrne, Barry J.

    2015-01-01

    Pompe disease is a systemic metabolic disorder characterized by lack of acid-alpha glucosidase (GAA) resulting in ubiquitous lysosomal glycogen accumulation. Respiratory and ambulatory dysfunction are prominent features in patients with Pompe yet the mechanism defining the development of muscle weakness is currently unclear. Transgenic animal models of Pompe disease mirroring the patient phenotype have been invaluable in mechanistic and therapeutic study. Here, we demonstrate significant pathological alterations at neuromuscular junctions (NMJs) of the diaphragm and tibialis anterior muscle as prominent features of disease pathology in Gaa knockout mice. Postsynaptic defects including increased motor endplate area and fragmentation were readily observed in Gaa−/− but not wild-type mice. Presynaptic neuropathic changes were also evident, as demonstrated by significant reduction in the levels of neurofilament proteins, and alterations in axonal fiber diameter and myelin thickness within the sciatic and phrenic nerves. Our data suggest the loss of NMJ integrity is a primary contributor to the decline in respiratory and ambulatory function in Pompe and arises from both pre- and postsynaptic pathology. These observations highlight the importance of systemic phenotype correction, specifically restoration of GAA to skeletal muscle and the nervous system for treatment of Pompe disease. PMID:25217571

  16. Safe neuromuscular electrical stimulator designed for the elderly.

    PubMed

    Krenn, Matthias; Haller, Michael; Bijak, Manfred; Unger, Ewald; Hofer, Christian; Kern, Helmut; Mayr, Winfried

    2011-03-01

    A stimulator for neuromuscular electrical stimulation (NMES) was designed, especially suiting the requirements of elderly people with reduced cognitive abilities and diminished fine motor skills. The aging of skeletal muscle is characterized by a progressive decline in muscle mass, force, and condition. Muscle training with NMES reduces the degradation process. The discussed system is intended for evoked muscle training of the anterior and posterior thigh. The core of the stimulator is based on a microcontroller with two modular output stages. The system has two charge-balanced biphasic voltage-controlled stimulation channels. Additionally, the evoked myoelectric signal (M-wave) and the myokinematic signal (surface acceleration) are measured. A central controller unit allows using the stimulator as a stand-alone device. To set up the training sequences and to evaluate the compliance data, a personal computer is connected to the stimulator via a universal serial bus. To help elderly people handle the stimulator by themselves, the user interface is kept very simple. For safety reasons, the electrode impedance is monitored during stimulation. A comprehensive compliance management with included measurements of muscle activity and stimulation intensity enables a scientific use of the stimulator in clinical trials. © 2011, Copyright the Authors. Artificial Organs © 2011, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.

  17. Noninvasive Respiratory Management of Patients With Neuromuscular Disease.

    PubMed

    Bach, John R

    2017-08-01

    This review article describes definitive noninvasive respiratory management of respiratory muscle dysfunction to eliminate need to resort to tracheotomy. In 2010 clinicians from 22 centers in 18 countries reported 1,623 spinal muscular atrophy type 1 (SMA1), Duchenne muscular dystrophy (DMD), and amyotrophic lateral sclerosis users of noninvasive ventilatory support (NVS) of whom 760 required it continuously (CNVS). The CNVS sustained their lives by over 3,000 patient-years without resort to indwelling tracheostomy tubes. These centers have now extubated at least 74 consecutive ventilator unweanable patients with DMD, over 95% of CNVS-dependent patients with SMA1, and hundreds of others with advanced neuromuscular disorders (NMDs) without resort to tracheotomy. Two centers reported a 99% success rate at extubating 258 ventilator unweanable patients without resort to tracheotomy. Patients with myopathic or lower motor neuron disorders can be managed noninvasively by up to CNVS, indefinitely, despite having little or no measurable vital capacity, with the use of physical medicine respiratory muscle aids. Ventilator-dependent patients can be decannulated of their tracheostomy tubes.

  18. Obesity-related differences in neuromuscular fatigue in adolescent girls.

    PubMed

    Garcia-Vicencio, Sebastian; Martin, Vincent; Kluka, Virginie; Cardenoux, Charlotte; Jegu, Anne-Gaëlle; Fourot, Anne-Véronique; Coudeyre, Emmanuel; Ratel, Sébastien

    2015-11-01

    The aim of this study was to investigate the effect of obesity on neuromuscular fatigue in adolescent girls. Twelve lean (13.6 ± 0.8 years) and 12 obese (13.9 ± 0.9 years) girls repeated 5-s maximal voluntary contractions (MVC) of the knee extensors until the generated torque reached 55 % of its initial value. Magnetic stimulations were delivered to the femoral nerve every five MVCs to follow the course of voluntary activation (VA) and potentiated twitch torque (Qtwpot). Torque reached 55 % of its initial value after 52.6 ± 20.4 and 74.9 ± 22.8 repetitions in obese and lean girls, respectively (p < 0.01). Furthermore, the decline of VA was smaller in obese girls (p < 0.001). In contrast, Qtwpot decreased to a greater extent in obese girls (p < 0.05). Obese girls fatigue faster than their lean counterparts. The peripheral factors mainly account for fatigue in obese girls, whereas central factors are mainly involved in lean girls.

  19. Effects of vision and lumbar posture on trunk neuromuscular control.

    PubMed

    Maaswinkel, Erwin; van Drunen, Paul; Veeger, Dirk-Jan H E J; van Dieën, Jaap H

    2015-01-21

    The goal of this study was to determine the effects of vision and lumbar posture on trunk neuromuscular control. Torso perturbations were applied with a pushing device while the subjects were restrained at the pelvis in a kneeling-seated position. Torso kinematics and the muscle activity of the lumbar part of the M. Longissimus were recorded for 14 healthy subjects. Four conditions were included: a flexion, extension and neutral lumbar posture with eyes closed and the neutral posture with eyes open. Frequency response functions of the admittance and reflexes showed that there was no significant difference between the eyes open and eyes closed conditions, thereby confirming that vision does not play a role in the stabilization of the trunk during small-amplitude trunk perturbations. In contrast, manipulating posture did lead to significant differences. In particular, the flexed condition led to a lower admittance and lower reflex contribution compared to the neutral condition. Furthermore, the muscle pre-activation (prior to the onset of the perturbation) was significantly lower in the flexed posture compared to neutral. This confirms that flexing the lumbar spine increases the passive tissue stiffness and decreases the contribution of reflex activity to trunk control.

  20. Applications of Shape Memory Alloys for Neurology and Neuromuscular Rehabilitation

    PubMed Central

    Pittaccio, Simone; Garavaglia, Lorenzo; Ceriotti, Carlo; Passaretti, Francesca

    2015-01-01

    Shape memory alloys (SMAs) are a very promising class of metallic materials that display interesting nonlinear properties, such as pseudoelasticity (PE), shape memory effect (SME) and damping capacity, due to high mechanical hysteresis and internal friction. Our group has applied SMA in the field of neuromuscular rehabilitation, designing some new devices based on the mentioned SMA properties: in particular, a new type of orthosis for spastic limb repositioning, which allows residual voluntary movement of the impaired limb and has no predetermined final target position, but follows and supports muscular elongation in a dynamic and compliant way. Considering patients in the sub-acute phase after a neurological lesion, and possibly bedridden, the paper presents a mobiliser for the ankle joint, which is designed exploiting the SME to provide passive exercise to the paretic lower limb. Two different SMA-based applications in the field of neuroscience are then presented, a guide and a limb mobiliser specially designed to be compatible with diagnostic instrumentations that impose rigid constraints in terms of electromagnetic compatibility and noise distortion. Finally, the paper discusses possible uses of these materials in the treatment of movement disorders, such as dystonia or hyperkinesia, where their dynamic characteristics can be advantageous. PMID:26023790

  1. The mode of neuromuscular blocking action of chlorpromazine

    PubMed Central

    Su, C.; Lee, C. Y.

    1960-01-01

    The inhibitory action of chlorpromazine on skeletal muscle has been studied with isolated preparations. In the nerve-muscle preparations of the frog sartorius and the rat diaphragm, the twitch responses to indirect stimulation are much more strongly depressed by chlorpromazine than those to direct stimulation. The conductivity of the nerve trunk is unaffected. The contractures of the frog rectus abdominis muscle caused by acetylcholine are depressed by chlorpromazine,but the contractures due to KCl are not influenced. Larger doses of chlorpromazine cause contracture by themselves, and this cannot be prevented by tubocurarine. In the sartorius muscle of the toad, the depolarization due to acetylcholine is reduced by chlorpromazine. The paralysing action of chlorpromazine adds to that of tubocurarine, and is antagonized to some extent by eserine or neostigmine. Muscles treated with chlorpromazine do not completely recover on washing. High concentrations of chlorpromazine depress the release of acetylcholine by motor-nerve stimulation, although they do not affect the enzymic synthesis of acetylcholine by acetone-dried powder of guinea-pig brain. The differences between the neuromuscular block produced by chlorpromazine and that by tubocurarine are discussed. PMID:13835490

  2. Frontotemporal dementia: a bridge between dementia and neuromuscular disease

    PubMed Central

    Ng, Adeline SL; Rademakers, Rosa; Miller, BL

    2015-01-01

    The concept that frontotemporal dementia (FTD) is a purely “cortical” dementia has largely been refuted by the recognition of its close association with motor neuron disease, and the identification of transactive response DNA-binding protein 43 (TDP-43) as a major pathological substrate underlying both diseases. Genetic findings have transformed this field and revealed connections between disorders that were previous thought clinically unrelated. The discovery of the C9ORF72 locus as responsible for majority of hereditary FTD, ALS and FTD-ALS cases and the understanding that repeat-containing RNA plays a crucial role in pathogenesis of both disorders has paved the way for development of potential biomarkers and therapeutic targets for these devastating diseases. In this review, we summarize the historical aspects leading up to our current understanding of the genetic, clinical and neuropathological overlap between FTD and ALS, and include brief discussions on chronic traumatic encephalopathy (CTE) given its association with TDP-43 pathology, increased dementia risk and reports of ALS in CTE patients. Additionally we describe other genetic associations between dementia and neuromuscular disease, such as inclusion body myositis with Paget’s disease and frontotemporal dementia (IBMPFD). PMID:25557955

  3. Effect of movement velocity during resistance training on neuromuscular performance.

    PubMed

    Pareja-Blanco, F; Rodríguez-Rosell, D; Sánchez-Medina, L; Gorostiaga, E M; González-Badillo, J J

    2014-10-01

    This study aimed to compare the effect on neuromuscular performance of 2 isoinertial resistance training programs that differed only in actual repetition velocity: maximal intended (MaxV) vs. half-maximal (HalfV) concentric velocity. 21 resistance-trained young men were randomly assigned to a MaxV (n=10) or HalfV (n=11) group and trained for 6 weeks using the full squat exercise. A complementary study (n=8) described the acute metabolic and mechanical response to the protocols used. MaxV training resulted in a likely more beneficial effect than HalfV on squat performance: maximum strength (ES: 0.94 vs. 0.54), velocity developed against all (ES: 1.76 vs. 0.88), light (ES: 1.76 vs. 0.75) and heavy (ES: 2.03 vs. 1.64) loads common to pre- and post-tests, and CMJ height (ES: 0.63 vs. 0.15). The effect on 20-m sprint was unclear, however. Both groups attained the greatest improvements in squat performance at their training velocities. Movement velocity seemed to be of greater importance than time under tension for inducing strength adaptations. Slightly higher metabolic stress (blood lactate and ammonia) and CMJ height loss were found for MaxV vs. HalfV, while metabolite levels were low to moderate for both conditions. MaxV may provide a superior stimulus for inducing adaptations directed towards improving athletic performance.

  4. Time course of neuromuscular adaptations to knee extensor eccentric training.

    PubMed

    Baroni, B M; Rodrigues, R; Franke, R A; Geremia, J M; Rassier, D E; Vaz, M A

    2013-10-01

    This study investigated the chronology of neural and morphological adaptations to knee extensor eccentric training and their contribution to strength gains in isometric, concentric and eccentric muscle actions. 20 male healthy subjects performed a 12-week eccentric training program on an isokinetic dynamometer, and neuromuscular evaluations of knee extensors were performed every 4 weeks. After 12 training weeks, significant increases were observed for: isometric (24%), concentric (15%) and eccentric (29%) torques; isometric (29%) and eccentric (33%) electromyographic activity; muscle thickness (10%) and anatomical cross-sectional area (19%). Eccentric and isometric torques increased progressively until the end of the program. Concentric torque and muscle mass parameters increased until the eighth training week, but did not change from this point to the twelfth training week. Eccentric and isometric activation increased at 4 and 8 training weeks, respectively, while no change was found in concentric activation. These results suggest that: 1) the relative increment in concentric strength was minor and does not relate to neural effects; 2) eccentric and isometric strength gains up to 8 training weeks are explained by the increased neural activation and muscle mass, whereas the increments in the last 4 training weeks seem to be associated with other mechanisms.

  5. Some aspects of the neuromuscular system of Ascaris.

    PubMed

    del Castillo, J; Rivera, A; Solórzano, S; Serrato, J

    1989-12-01

    1. The structure and physiological properties of the somatic neuromuscular system of the nematode Ascaris lumbricoides var. suum are discussed taking into account early work from this laboratory, the results of other workers, and experiments in progress. 2. Anatomically, the central feature of the somatic musculature is the presence of a specialized muscular, but not contractile, structure: the syncytium or sarcopile, formed by the terminal processes of the muscle cell arms that contact the nerve cord. In this region, they are electrically coupled to one another and form chemical synapses with the nerve fibres. 3. The syncytium serves, primarily, as a nerve-muscle manifold, or device which allows the neural control of the muscle fields with great economy in the amount of nervous tissue, numbers of chemical junctions and quantities of synaptic transmitters. 4. The structure of the syncytium is being studied with the scanning electron microscope. The results reveal the convergence of the arms on discrete sites as they approach the cords, as well as a longitudinal arrangement of the terminal processes as they course over the cords, at least in the posterior region of the animal. 5. The characteristics of the electrical coupling between the muscle cells are being investigated. A pattern has been observed that is dependent on the position of the cell pairs with respect to the longitudinal axis of the animal. 6. A study of signal propagation in the arms has revealed that the morphology of the muscle cells contributes to the extent and preferential direction of propagation.

  6. Toward a better understanding of gastrointestinal nitrergic neuromuscular transmission.

    PubMed

    Lies, B; Groneberg, D; Friebe, A

    2014-07-01

    Nitric oxide (NO) is an important inhibitory neurotransmitter in the gastrointestinal (GI) tract. The majority of nitrergic effects are transduced by NO-sensitive guanylyl cyclase (NO-GC) as the receptor for NO, and, thus, mediated by cGMP-dependent mechanisms. Work carried out during the past years has demonstrated NO to be largely involved in GI smooth muscle relaxation and motility. However, detailed investigation of nitrergic signaling has turned out to be complicated as NO-GC was identified in several different GI cell types such as smooth muscle cells, interstitial cells of Cajal and fibroblast-like cells. With regards to nitrergic neurotransmission, special focus has been placed on the role of interstitial cells of Cajal using mutant mice with reduced populations of ICC. Recently, global and cell-specific knockout mice for enzymes participating in nitrergic signaling have been generated providing a suitable approach to further examine the role of NO-mediated signaling in GI smooth muscle. This review discusses the current knowledge on nitrergic mechanisms in gastrointestinal neuromuscular transmission with a focus on genetic models and outlines possible further investigations to gain better understanding on NO-mediated effects in the GI tract. © 2014 John Wiley & Sons Ltd.

  7. Lim kinase regulates the development of olfactory and neuromuscular synapses.

    PubMed

    Ang, Lay-Hong; Chen, Weitao; Yao, Ying; Ozawa, Rie; Tao, Enxiang; Yonekura, Junichiro; Uemura, Tadashi; Keshishian, Haig; Hing, Huey

    2006-05-01

    Lim Kinase (Limk) belongs to a phylogenetically conserved family of serine/threonine kinases, which have been shown to be potent regulators of the actin cytoskeleton. Despite accumulating evidence of its biochemical actions, its in vivo function has remained poorly understood. The association of the Limk1 gene with Williams Syndrome indicates that proteins of this family play a role in the nervous system. To unravel the cellular and molecular functions of Limk, we have either knocked out or activated the Limk gene in Drosophila. At the neuromuscular junction, loss of Limk leads to enlarged terminals, while increasing the activity of Limk leads to stunted terminals with fewer synaptic boutons. In the antennal lobe, loss of Limk abolishes the ability of p21-activated kinase (Pak) to alter glomerular development. In contrast, increase in Limk function leads to ectopic glomeruli, a phenotype suppressible by the coexpression of a hyperactive Cofilin gene. These results establish Limk as a critical regulator of Cofilin function and synapse development, and a downstream effector of Pak in vivo.

  8. Neuromuscular fatigue during dynamic maximal strength and hypertrophic resistance loadings.

    PubMed

    Walker, Simon; Davis, Lisa; Avela, Janne; Häkkinen, Keijo

    2012-06-01

    The purpose of this study was to compare the acute neuromuscular fatigue during dynamic maximal strength and hypertrophic loadings, known to cause different adaptations underlying strength gain during training. Thirteen healthy, untrained males performed two leg press loadings, one week apart, consisting of 15 sets of 1 repetition maximum (MAX) and 5 sets of 10 repetition maximums (HYP). Concentric load and muscle activity, electromyography (EMG) amplitude and median frequency, was assessed throughout each set. Additionally, maximal bilateral isometric force and muscle activity was assessed pre-, mid-, and up to 30 min post-loading. Concentric load during MAX was decreased after set 10 (P<0.05), while the load was maintained throughout HYP. Both loadings caused large reductions in maximal isometric force (MAX=-30±6.4% vs. HYP=-48±9.7%, P<0.001). The decreased concentric and isometric strength during MAX loading was accompanied by reduced EMG amplitude (P<0.05). Conversely, hypertrophic loading caused decreased median frequency only during isometric contractions (P<0.01). During concentric contractions, EMG amplitude increased and median frequency decreased in HYP (P<0.01). Our results indicate reduced neural drive during MAX loading and more complex changes in muscle activity during HYP loading. Copyright © 2011 Elsevier Ltd. All rights reserved.

  9. Mechanisms of elimination, remodeling, and competition at frog neuromuscular junctions.

    PubMed

    Herrera, A A; Werle, M J

    1990-01-01

    Mechanisms governing synapse elimination, synaptic remodeling, and polyneuronal innervation were examined in anatomical and electrophysiological studies of frog neuromuscular junctions. There was a substantial level of polyneuronal innervation in adult junctions and this varied seasonally. Nerve terminal retraction and synapse elimination occurred during normal growth and following reinnervation. Synapse elimination was not inevitable, however. Repeated in vivo observations of some identified junctions showed that polyneuronal innervation could persist for over a year, while at other junctions it arose de novo by terminal sprouting. We concluded that polyneuronal innervation in adult muscles was governed by an equilibrium between processes of retraction and elimination on one hand, and sprouting and synaptogenesis on the other. Other observations revealed that structural remodeling was a common feature of adult junctions. Most often, remodeling involved the simultaneous growth and retraction of different parts of the same junction. The net result was usually junctional growth that, in small frogs, appeared to provide a good match between synaptic size and the electrical demands of transmission. In larger animals, pre- and postsynaptic sizes were not as well matched, providing morphological evidence for a growth-associated decline in synaptic efficacy. Finally, electrophysiology was used to describe some of the functional correlates and consequences of competitive interactions between the terminals of different axons. These results are explained by a hypothetical mechanism that involves trophic support provided by the muscle to the motoneuron, the overall level of nerve-muscle activity, and the synchrony of pre- and postsynaptic activity.

  10. Interleaved neuromuscular electrical stimulation after spinal cord injury.

    PubMed

    Bergquist, Austin J; Wiest, Matheus J; Okuma, Yoshino; Collins, David F

    2017-02-28

    Neuromuscular electrical stimulation (NMES) over a muscle belly (mNMES) recruits superficial motor units (MUs) preferentially, whereas NMES over a nerve trunk (nNMES) recruits MUs evenly throughout the muscle. We performed tests to determine whether "interleaving" pulses between the mNMES and nNMES sites (iNMES) reduces the fatigability of contractions for people experiencing paralysis because of chronic spinal cord injury. Plantar flexion torque and soleus electromyography (M-waves) were recorded from 8 participants. A fatigue protocol (75 contractions; 2 s on/2 s off for 5 min) was delivered by iNMES. The results were compared with previously published data collected with mNMES and nNMES in the same 8 participants. Torque declined ∼40% more during mNMES than during nNMES or iNMES. M-waves declined during mNMES but not during nNMES or iNMES. To reduce fatigability of electrically evoked contractions of paralyzed plantar flexors, iNMES is equivalent to nNMES, and both are superior to mNMES. Muscle Nerve, 2017. © 2017 Wiley Periodicals, Inc.

  11. Functional neuromuscular stimulation: outcomes in young people with tetraplegia.

    PubMed

    Mulcahey, M J; Smith, B T; Betz, R R; Triolo, R J; Peckham, P H

    1994-01-01

    Percutaneous intramuscular functional neuromuscular stimulation (FNS) systems were fitted to the forearms of five adolescents with tetraplegia in an effort to provide active grasp and release. Two assessments designed at Case Western Reserve University to evaluate functional outcomes of FNS in adults were employed. The common object test (COT) was used to assess hand function during five activities of daily living (ADLs): eating, drinking, writing, brushing teeth and applying toothpaste. A usage survey provided information on the frequency of FNS use in environments outside of the laboratory. In addition, interviews were employed using open-ended questions to gain a deeper understanding of the perceptions of FNS in the adolescents' own environments. Based on the COT results, each adolescent was able to perform ADLs with and without FNS. However, FNS allowed unilateral function so that the extremity without FNS was freed to assist in balance or participate in bilateral tasks. Also, FNS reduced the need for multiple devices, providing users with the potential to perform activities in a variety of environments without transporting adaptive equipment. Those who reported using FNS most often obtained hard-bound school books, held pens during classroom and homework assignments, engaged in leisure activities and performed hygiene tasks. FNS was also used as a means to communicate and socialize through hand gestures. Well-known factors that influence the independence of people with tetraplegia also appeared to affect FNS use.

  12. Electrostimulation Training Effects on diurnal Fluctuations of Neuromuscular Performance.

    PubMed

    Gueldich, H; Zarrouk, N; Chtourou, H; Zghal, F; Sahli, S; Rebai, H

    2017-01-01

    This study examined the effects of electrostimulation (ES) strength training at the same time-of-day on the diurnal fluctuations of maximal voluntary isometric contractions (MVIC) and surface electromyography (EMG). 20 male performed 3 MVICs of knee extension coupled with surface EMG before and after 5 weeks of 3 ES training sessions per week. Each ES training session consisted in 45 isometric contractions. The participants were randomly assigned to either a morning (MTG, 07:00-08:00 h) or an evening (ETG, 17:00-18:00 h) training group. Both groups performed the evaluation tests at 07:00 and 17:00 h. Before ES training, MVIC was significantly higher in the evening compared to the morning for all groups, but there was no significant difference between groups for all EMG parameters. After the ES training, the diurnal variations in MVIC were blunted in the MTG and persisted in the ETG. Significant time-of-day effect was noticed for all EMG parameters but there was no group effect. The elimination of the diurnal fluctuations of MVIC and the appearance of EMG variations by training in the morning hours suggest that neural adaptations are the main source of temporal specificity of neuromuscular performance after ES strength training.

  13. Using factor analysis to identify neuromuscular synergies during treadmill walking

    NASA Technical Reports Server (NTRS)

    Merkle, L. A.; Layne, C. S.; Bloomberg, J. J.; Zhang, J. J.

    1998-01-01

    Neuroscientists are often interested in grouping variables to facilitate understanding of a particular phenomenon. Factor analysis is a powerful statistical technique that groups variables into conceptually meaningful clusters, but remains underutilized by neuroscience researchers presumably due to its complicated concepts and procedures. This paper illustrates an application of factor analysis to identify coordinated patterns of whole-body muscle activation during treadmill walking. Ten male subjects walked on a treadmill (6.4 km/h) for 20 s during which surface electromyographic (EMG) activity was obtained from the left side sternocleidomastoid, neck extensors, erector spinae, and right side biceps femoris, rectus femoris, tibialis anterior, and medial gastrocnemius. Factor analysis revealed 65% of the variance of seven muscles sampled aligned with two orthogonal factors, labeled 'transition control' and 'loading'. These two factors describe coordinated patterns of muscular activity across body segments that would not be evident by evaluating individual muscle patterns. The results show that factor analysis can be effectively used to explore relationships among muscle patterns across all body segments to increase understanding of the complex coordination necessary for smooth and efficient locomotion. We encourage neuroscientists to consider using factor analysis to identify coordinated patterns of neuromuscular activation that would be obscured using more traditional EMG analyses.

  14. Three-dimensional neuron-muscle constructs with neuromuscular junctions.

    PubMed

    Morimoto, Yuya; Kato-Negishi, Midori; Onoe, Hiroaki; Takeuchi, Shoji

    2013-12-01

    This paper describes a fabrication method of muscle tissue constructs driven by neurotransmitters released from activated motor neurons. The constructs consist of three-dimensional (3D) free-standing skeletal muscle fibers co-cultured with motor neurons. We differentiated mouse neural stem cells (mNSCs) cultured on the skeletal muscle fibers into neurons that extend their processes into the muscle fibers. We found that acetylcholine receptors (AChRs) were formed at the connection between the muscle fibers and the neurons. The neuron-muscle constructs consist of highly aligned, long and matured muscle fibers that facilitate wide contractions of muscle fibers in a single direction. The contractions of the neuron-muscle construct were observed after glutamic acid activation of the neurons. The contraction was stopped by treatment with curare, an neuromuscular junction (NMJ) antagonist. These results indicate that our method succeeded in the formation of NMJs in the neuron-muscle constructs. The neuron-muscle construct system can potentially be used in pharmacokinetic assays related to NMJ disease therapies and in soft-robotic actuators.

  15. Transsynaptic Teneurin Signaling in Neuromuscular Synapse Organization and Target Choice

    PubMed Central

    Mosca, Timothy J.; Hong, Weizhe; Dani, Vardhan S.; Favaloro, Vincenzo; Luo, Liqun

    2012-01-01

    Synapse assembly requires transsynaptic signals between the pre- and postsynapse1, but the understanding of essential organizational molecules remains incomplete2. Teneurins are conserved, EGF-repeat containing transmembrane proteins with large extracellular domains3. Here we show that two Drosophila Teneurins, Ten-m and Ten-a, are required for neuromuscular synapse organization and target selection. Ten-a is presynaptic while Ten-m is mostly postsynaptic; neuronal Ten-a and muscle Ten-m form a complex in vivo. Pre- or postsynaptic Teneurin perturbations cause severe synapse loss and impair many facets of organization transsynaptically and cell-autonomously. These include defects in active zone apposition, release sites, membrane and vesicle organization, and synaptic transmission. Moreover, the presynaptic microtubule and postsynaptic spectrin cytoskeletons are severely disrupted, suggesting a mechanism whereby Teneurins organize the cytoskeleton, which in turn affects other aspects of synapse development. Supporting this, Ten-m physically interacts with α-spectrin. Genetic analyses of teneurin and neuroligin reveal their differential roles that synergize to promote synapse assembly. Finally, at elevated endogenous levels, Ten-m regulates specific motoneuron-muscle target selection. Our study identifies the Teneurins as a key bi-directional transsynaptic signal in general synapse organization, and demonstrates that such a molecule can also regulate target selection. PMID:22426000

  16. The neuromuscular features of acromegaly: a clinical and pathological study.

    PubMed Central

    Khaleeli, A A; Levy, R D; Edwards, R H; McPhail, G; Mills, K R; Round, J M; Betteridge, D J

    1984-01-01

    A study of the neuromuscular features of acromegaly was performed in six patients. Clinical assessment was supplemented by quadriceps force measurements, plasma creatine kinase (CK) activities, electromyography (EMG) and nerve conduction studies. Muscle mass was measured by urinary creatinine/height indices (CHI) and cross sectional area (CSA) of thighs and calves on computed tomography. Quadriceps force/unit cross sectional area was derived. Needle biopsies of vastus lateralis were studied by histochemical and ultrastructural methods. Mean fibre area (MFA) and fibre type proportions were measured. Most of the subjects studied had muscle pain and proximal muscle weakness confirmed by quadriceps force measurements. This occurred in the absence of muscle wasting, as shown by cross sectional area measurements and normal or raised creatinine/height indices. "Myopathic" features were demonstrated by needle biopsy in half the patients and occasionally by electromyography and raised plasma creatine kinase activity. Abnormalities on needle biopsy included variation in fibre size, type 2 fibre atrophy and large type 1 MFA relative to type 2 MFA. Electronmicroscopy showed the non-specific findings of increased glycogen accumulation, excess lipofuscin pigment and myofilament loss. Images PMID:6481369

  17. Verifax: Biometric instruments measuring neuromuscular disorders/performance impairments

    NASA Astrophysics Data System (ADS)

    Morgenthaler, George W.; Shrairman, Ruth; Landau, Alexander

    1998-01-01

    VeriFax, founded in 1990 by Dr. Ruth Shrairman and Mr. Alex Landau, began operations with the aim of developing a biometric tool for the verification of signatures from a distance. In the course of developing this VeriFax Autograph technology, two other related applications for the technologies under development at VeriFax became apparent. The first application was in the use of biometric measurements as clinical monitoring tools for physicians investigating neuromuscular diseases (embodied in VeriFax's Neuroskill technology). The second application was to evaluate persons with critical skills (e.g., airline pilots, bus drivers) for physical and mental performance impairments caused by stress, physiological disorders, alcohol, drug abuse, etc. (represented by VeriFax's Impairoscope prototype instrument). This last application raised the possibility of using a space-qualified Impairoscope variant to evaluate astronaut performance with respect to the impacts of stress, fatigue, excessive workload, build-up of toxic chemicals within the space habitat, etc. The three applications of VeriFax's patented technology are accomplished by application-specific modifications of the customized VeriFax software. Strong commercial market potentials exist for all three VeriFax technology applications, and market progress will be presented in more detail below.

  18. Neuromuscular disorders: genes, genetic counseling and therapeutic trials

    PubMed Central

    Zatz, Mayana; Passos-Bueno, Maria Rita; Vainzof, Mariz

    2016-01-01

    Abstract Neuromuscular disorders (NMD) are a heterogeneous group of genetic conditions, with autosomal dominant, recessive, or X-linked inheritance. They are characterized by progressive muscle degeneration and weakness. Here, we are presenting our major contributions to the field during the past 30 years. We have mapped and identified several novel genes responsible for NMD. Genotype-phenotype correlations studies enhanced our comprehension on the effect of gene mutations on related proteins and their impact on clinical findings. The search for modifier factors allowed the identification of a novel "protective"; variant which may have important implication on therapeutic developments. Molecular diagnosis was introduced in the 1980s and new technologies have been incorporated since then. Next generation sequencing greatly improved our capacity to identify disease-causing mutations with important benefits for research and prevention through genetic counseling of patients' families. Stem cells researches, from and for patients, have been used as tools to study human genetic diseases mechanisms and for therapies development. The clinical effect of preclinical trials in mice and canine models for muscular dystrophies are under investigation. Finally, the integration of our researches and genetic services with our post-graduation program resulted in a significant output of new geneticists, spreading out this expertise to our large country. PMID:27575431

  19. Using factor analysis to identify neuromuscular synergies during treadmill walking

    NASA Technical Reports Server (NTRS)

    Merkle, L. A.; Layne, C. S.; Bloomberg, J. J.; Zhang, J. J.

    1998-01-01

    Neuroscientists are often interested in grouping variables to facilitate understanding of a particular phenomenon. Factor analysis is a powerful statistical technique that groups variables into conceptually meaningful clusters, but remains underutilized by neuroscience researchers presumably due to its complicated concepts and procedures. This paper illustrates an application of factor analysis to identify coordinated patterns of whole-body muscle activation during treadmill walking. Ten male subjects walked on a treadmill (6.4 km/h) for 20 s during which surface electromyographic (EMG) activity was obtained from the left side sternocleidomastoid, neck extensors, erector spinae, and right side biceps femoris, rectus femoris, tibialis anterior, and medial gastrocnemius. Factor analysis revealed 65% of the variance of seven muscles sampled aligned with two orthogonal factors, labeled 'transition control' and 'loading'. These two factors describe coordinated patterns of muscular activity across body segments that would not be evident by evaluating individual muscle patterns. The results show that factor analysis can be effectively used to explore relationships among muscle patterns across all body segments to increase understanding of the complex coordination necessary for smooth and efficient locomotion. We encourage neuroscientists to consider using factor analysis to identify coordinated patterns of neuromuscular activation that would be obscured using more traditional EMG analyses.

  20. Prolonged administration of pyridostigmine impairs neuromuscular function with and without down-regulation of acetylcholine receptors.

    PubMed

    Richtsfeld, Martina; Yasuhara, Shingo; Fink, Heidrun; Blobner, Manfred; Martyn, J A Jeevendra

    2013-08-01

    The acetylcholinesterase inhibitor, pyridostigmine, is prophylactically administered to mitigate the toxic effects of nerve gas poisoning. The authors tested the hypothesis that prolonged pyridostigmine administration can lead to neuromuscular dysfunction and even down-regulation of acetylcholine receptors. Pyridostigmine (5 or 25 mg·kg·day) or saline was continuously administered via osmotic pumps to rats, and infused for either 14 or 28 days until the day of neuromuscular assessment (at day 14 or 28), or discontinued 24 h before neuromuscular assessment. Neurotransmission and muscle function were examined by single-twitch, train-of-four stimulation and 100-Hz tetanic stimulation. Sensitivity to atracurium and acetylcholine receptor number (quantitated by I-α-bungarotoxin) provided additional measures of neuromuscular integrity. Specific tetanic tensions (Newton [N]/muscle weight [g]) were significantly (P < 0.05) decreased at 14 (10.3 N/g) and 28 (11.1 N/g) days of 25 mg·kg·day pyridostigmine compared with controls (13.1-13.6 N/g). Decreased effective dose (0.81-1.05 vs. 0.16-0.45 mg/kg; P < 0.05) and decreased plasma concentration (3.02-3.27 vs. 0.45-1.37 μg/ml; P < 0.05) of atracurium for 50% paralysis (controls vs. 25 mg·kg·day pyridostigmine, respectively), irrespective of discontinuation of pyridostigmine, confirmed the pyridostigmine-induced altered neurotransmission. Pyridostigmine (25 mg·kg·day) down-regulated acetylcholine receptors at 28 days. Prolonged administration of pyridostigmine (25 mg·kg·day) leads to neuromuscular impairment, which can persist even when pyridostigmine is discontinued 24 h before assessment of neuromuscular function. Pyridostigmine has the potential to down-regulate acetylcholine receptors, but induces neuromuscular dysfunction even in the absence of receptor changes.

  1. Pilates and Proprioceptive Neuromuscular Facilitation Methods Induce Similar Strength Gains but Different Neuromuscular Adaptations in Elderly Women.

    PubMed

    Teixeira de Carvalho, Fabiana; de Andrade Mesquita, Laiana Sepúlveda; Pereira, Rafael; Neto, Osmar Pinto; Amaro Zangaro, Renato

    2017-09-26

    Background/Study Context: The aging process is associated with a decline in muscle mass, strength, and conditioning. Two training methods that may be useful to improve muscle function are Pilates and proprioceptive neuromuscular facilitation (PNF). Thus, the present study aimed to compare the influence of training programs using Pilates and PNF methods with elderly women. Sixty healthy elderly women were randomly divided into three groups: Pilates group, PNF group, and control group. Pilates and PNF groups underwent 1-month training programs with Pilates and PNF methods, respectively. The control group received no intervention during the 1 month. The maximal isometric force levels from knee extension and flexion, as well as the electromyography (EMG) signals from quadriceps and biceps femoris, were recorded before and after the 1-month intervention period. A two-way analysis of variance revealed that the Pilates and PNF methods induced similar strength gains from knee flexors and extensors, but Pilates exhibited greater low-gamma drive (i.e., oscillations in 30-60 Hz) in the EMG power spectrum after the training period. These results support use of both Pilates and PNF methods to enhance lower limb muscle strength in older groups, which is very important for gait, postural stability, and performance of daily life activities.

  2. Zebrafish calls for reinterpretation for the roles of P/Q calcium channels in neuromuscular transmission.

    PubMed

    Wen, Hua; Linhoff, Michael W; Hubbard, Jeffrey M; Nelson, Nathan R; Stensland, Donald; Dallman, Julia; Mandel, Gail; Brehm, Paul

    2013-04-24

    A long-held tenet of neuromuscular transmission is that calcium-dependent neurotransmitter release is mediated by N-type calcium channels in frog but P/Q-type channels in mammals. The N-type assignment in frog is based principally on pharmacological sensitivity to ω-conotoxin GVIA. Our studies show that zebrafish neuromuscular transmission is also sensitive to ω-conotoxin GVIA. However, positional cloning of a mutant line with compromised neuromuscular function identified a mutation in a P/Q- rather than N-type channel. Cloning and heterologous expression of this P/Q-type channel confirmed a block by ω-conotoxin GVIA raising the likelihood that all vertebrates, including frog, use the P/Q-type calcium channel for neuromuscular transmission. In addition, our P/Q defective mutant line offered a means of testing the ability of roscovitine, known to potentiate frog neuromuscular transmission, to mediate behavioral and functional rescue. Acute treatment led to rapid improvement of both, pointing to potential therapeutic benefit for myasthenic disorders involving calcium channel dysfunction.

  3. Ryanodine and inositol triphosphate receptors modulate facilitation and tetanic depression at the frog neuromuscular junction.

    PubMed

    Silveira, Priscila E; Lima, Ricardo F; Guimarães, Jennifer D S; Molgó, Jordi; Naves, Ligia A; Kushmerick, Christopher

    2015-10-01

    Short-term plasticity of synaptic function is an important physiological control of transmitter release. Short-term plasticity can be regulated by intracellular calcium released by ryanodine and inositol triphosphate (IP3) receptors, but the role of these receptors at the neuromuscular junction is understood incompletely. We measured short-term plasticity of evoked endplate potential (EPP) amplitudes from frog neuromuscular junctions treated with ryanodine, 2-aminoethoxydiphenylborane (2-APB), or 1-[6-[[(17β)-3-methoxyestra-1,3,5(10)-trien-17-yl]amino]hexyl]-1H-pyrrole-2,5-dione (U- 73122). Ryanodine decreases paired-pulse facilitation for intervals <20 ms and markedly decreases tetanic depression. Treatment with 2-APB reduces EPP amplitude, increases paired-pulse facilitation for intervals of <20 ms, and significantly reduces tetanic depression. U-73122 decreases EPP amplitude and decreases paired-pulse depression for intervals <20 ms. Ryanodine, IP3 receptors, and phospholipase C modulate short-term plasticity of transmitter release at the neuromuscular junction. These results suggest possible targets for improving the safety factor of neuromuscular transmission during repetitive activity of the neuromuscular junction. © 2015 Wiley Periodicals, Inc.

  4. Functional Connectivity under Optogenetic Control Allows Modeling of Human Neuromuscular Disease.

    PubMed

    Steinbeck, Julius A; Jaiswal, Manoj K; Calder, Elizabeth L; Kishinevsky, Sarah; Weishaupt, Andreas; Toyka, Klaus V; Goldstein, Peter A; Studer, Lorenz

    2016-01-07

    Capturing the full potential of human pluripotent stem cell (PSC)-derived neurons in disease modeling and regenerative medicine requires analysis in complex functional systems. Here we establish optogenetic control in human PSC-derived spinal motorneurons and show that co-culture of these cells with human myoblast-derived skeletal muscle builds a functional all-human neuromuscular junction that can be triggered to twitch upon light stimulation. To model neuromuscular disease we incubated these co-cultures with IgG from myasthenia gravis patients and active complement. Myasthenia gravis is an autoimmune disorder that selectively targets neuromuscular junctions. We saw a reversible reduction in the amplitude of muscle contractions, representing a surrogate marker for the characteristic loss of muscle strength seen in this disease. The ability to recapitulate key aspects of disease pathology and its symptomatic treatment suggests that this neuromuscular junction assay has significant potential for modeling of neuromuscular disease and regeneration. Copyright © 2016 Elsevier Inc. All rights reserved.

  5. Influence of whole body vibration platform frequency on neuromuscular performance of community-dwelling older adults.

    PubMed

    Furness, Trentham P; Maschette, Wayne E

    2009-08-01

    The purpose of this study was to progressively overload vibration platform frequency to describe sea-saw whole body vibration influence on neuromuscular performance of community-dwelling older adults. Seventy-three community-dwelling older adults (aged 72 +/- 8 years) were randomly assigned to 4 groups (zero, one, 2, and 3 whole body vibration sessions per week). Quantifiers of neuromuscular performance such as the 5-Chair Stands test, the Timed Up and Go (TUG) test, and the Tinetti test were recorded. Furthermore, Health-related quality of life was qualified with the SF-36 Health Survey. A 6-week whole body vibration intervention significantly improved the quantifiers of neuromuscular performance in a community-dwelling older adult sample. Whole body vibration was shown to significantly reduce time taken to complete the 5-Chair Stands test (p < 0.05) and the TUG test (p < 0.05). Tinetti test scores significantly improved (p < 0.05). as did all components of health-related quality of life (p < 0.05). Overall, progressively overloaded frequency elicited more beneficial improvement for the 3 whole body vibration sessions per week group. It was concluded that progressively overloaded frequency was effective in improving quantifiable measures of neuromuscular performance in the sample and that practitioners may confidently prescribe 3 whole body vibration sessions per week with more precise knowledge of the effects of whole body vibration on neuromuscular performance and health-related quality-of-life effects.

  6. Neuromuscular scoliosis and pelvic fixation in 2015: Where do we stand?

    PubMed

    Anari, Jason B; Spiegel, David A; Baldwin, Keith D

    2015-09-18

    Neuromuscular scoliosis is a challenging problem to treat in a heterogeneous patient population. When the decision is made for surgery the surgeon must select a technique employed to correct the curve and achieve the goals of surgery, namely a straight spine over a level pelvis. Pre-operatively the surgeon must ask if pelvic fixation is worth the extra complications and infection risk it introduces to an already compromised host. Since the advent of posterior spinal fusion the technology used for instrumentation has changed drastically. However, many of the common problems seen with the unit rod decades ago we are still dealing with today with pedicle screw technology. Screw cut out, pseudoarthrosis, non-union, prominent hardware, wound complications, and infection are all possible complications when extending a spinal fusion construct to the pelvis in a neuromuscular scoliosis patient. Additionally, placing pelvic fixation in a neuromuscular patient results in extra blood loss, greater surgical time, more extensive dissection with creation of a deep dead space, and an incision that extends close to the rectum in patients who are commonly incontinent. Balancing the risk of placing pelvic fixation when the benefit, some may argue, is limited in non-ambulating patients is difficult when the literature is so mottled. Despite frequent advancements in technology issues with neuromuscular scoliosis remain the same and in the next 10 years we must do what we can to make safe neuromuscular spine surgery a reality.

  7. Regulation and Restoration of Motoneuronal Synaptic Transmission During Neuromuscular Regeneration in the Pulmonate Snail Helisoma trivolvis

    PubMed Central

    Turner, M. B.; Szabo-Maas, T. M.; Poyer, J. C.; Zoran, M. J.

    2015-01-01

    Regeneration of motor systems involves reestablishment of central control networks, reinnervation of muscle targets by motoneurons, and reconnection of neuromodulatory circuits. Still, how these processes are integrated as motor function is restored during regeneration remains ill defined. Here, we examined the mechanisms underlying motoneuronal regeneration of neuromuscular synapses related to feeding movements in the pulmonate snail Helisoma trivolvis. Neurons B19 and B110, although activated during different phases of the feeding pattern, innervate similar sets of muscles. However, the percentage of muscle fibers innervated, the efficacy of excitatory junction potentials, and the strength of muscle contractions were different for each cell’s specific connections. After peripheral nerve crush, a sequence of transient electrical and chemical connections formed centrally within the buccal ganglia. Neuromuscular synapse regeneration involved a three-phase process: the emergence of spontaneous synaptic transmission (P1), the acquisition of evoked potentials of weak efficacy (P2), and the establishment of functional reinnervation (P3). Differential synaptic efficacy at muscle contacts was recapitulated in cell culture. Differences in motoneuronal presynaptic properties (i.e., quantal content) were the basis of disparate neuromuscular synapse function, suggesting a role for retrograde target influences. We propose a homeostatic model of molluscan motor system regeneration. This model has three restoration events: (1) transient central synaptogenesis during axonal outgrowth, (2) intermotoneuronal inhibitory synaptogenesis during initial neuromuscular synapse formation, and (3) target-dependent regulation of neuromuscular junction formation. PMID:21876114

  8. [Deep versus moderate neuromuscular block during one-lung ventilation in lung resection surgery].

    PubMed

    Casanova, Javier; Piñeiro, Patricia; De La Gala, Francisco; Olmedilla, Luis; Cruz, Patricia; Duque, Patricia; Garutti, Ignacio

    Neuromuscular relaxants are essential during general anesthesia for several procedures. Classical anesthesiology literature indicates that the use of neuromuscular blockade in thoracic surgery may be deleterious in patients in lateral decubitus position in one-lung ventilation. The primary objective of our study was to compare respiratory function according to the degree of patient neuromuscular relaxation. Secondary, we wanted to check that neuromuscular blockade during one-lung ventilation is not deleterious. A prospective, longitudinal observational study was made in which each patient served as both treated subject and control. 76 consecutive patients programmed for lung resection surgery in Gregorio Marañon Hospital along the year of 2013 who required one-lung ventilation in lateral decubitus were included. Ventilator data, hemodynamic parameters were registered in different moments according to train-of-four response (intense, deep and moderate blockade) during one-lung ventilation. Peak, plateau and mean pressures were significantly lower during the intense and deep blockade. Besides, compliance and peripheral oxygen saturation were significantly higher in those moments. Heart rate was significantly higher during deep blockade. No mechanical ventilation parameters were modified during measurements. Deep neuromuscular blockade attenuates the poor lung mechanics observed during one-lung ventilation. Copyright © 2016 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.

  9. Neuromuscular disease and respiratory physiology in children: putting lung function into perspective.

    PubMed

    Fauroux, Brigitte; Khirani, Sonia

    2014-08-01

    Neuromuscular diseases represent a heterogeneous group of disorders of the muscle, nerve or neuromuscular junction. The respiratory muscles are rarely spared in neuromuscular diseases even if the type of muscle involvement, severity and time course greatly varies among the different diseases. Diagnosis of respiratory muscle weakness is crucial because of the importance of respiratory morbidity and mortality. Presently, routine respiratory evaluation is based on non-invasive volitional tests, such as the measurement of lung volumes, spirometry and the maximal static pressures, which may be difficult or impossible to obtain in some young children. Other tools or parameters are thus needed to assess the respiratory muscle weakness and its consequences in young children. The measurement of oesogastric pressures can be helpful as they allow the diagnosis and quantification of paradoxical breathing, as well as the assessment of the strength of the inspiratory and expiratory muscles by means of the oesophageal pressure during a maximal sniff and of the gastric pressure during a maximal cough. Sleep assessment should also be part of the respiratory evaluation of children with neuromuscular disease with at least the recording of nocturnal gas exchange if polysomnography is not possible or unavailable. This improvement in the assessment of respiratory muscle performance may increase our understanding of the respiratory pathophysiology of the different neuromuscular diseases, improve patient care, and guide research and innovative therapies by identifying and validating respiratory parameters.

  10. Wnt4 Participates in the Formation of Vertebrate Neuromuscular Junction

    PubMed Central

    Strochlic, Laure; Falk, Julien; Goillot, Evelyne; Sigoillot, Séverine; Bourgeois, Francine; Delers, Perrine; Rouvière, Jérôme; Swain, Amanda; Castellani, Valérie; Schaeffer, Laurent; Legay, Claire

    2012-01-01

    Neuromuscular junction (NMJ) formation requires the highly coordinated communication of several reciprocal signaling processes between motoneurons and their muscle targets. Identification of the early, spatially restricted cues in target recognition at the NMJ is still poorly documented, especially in mammals. Wnt signaling is one of the key pathways regulating synaptic connectivity. Here, we report that Wnt4 contributes to the formation of vertebrate NMJ in vivo. Results from a microarray screen and quantitative RT-PCR demonstrate that Wnt4 expression is regulated during muscle cell differentiation in vitro and muscle development in vivo, being highly expressed when the first synaptic contacts are formed and subsequently downregulated. Analysis of the mouse Wnt4−/− NMJ phenotype reveals profound innervation defects including motor axons overgrowing and bypassing AChR aggregates with 30% of AChR clusters being unapposed by nerve terminals. In addition, loss of Wnt4 function results in a 35% decrease of the number of prepatterned AChR clusters while Wnt4 overexpression in cultured myotubes increases the number of AChR clusters demonstrating that Wnt4 directly affects postsynaptic differentiation. In contrast, muscle structure and the localization of several synaptic proteins including acetylcholinesterase, MuSK and rapsyn are not perturbed in the Wnt4 mutant. Finally, we identify MuSK as a Wnt4 receptor. Wnt4 not only interacts with MuSK ectodomain but also mediates MuSK activation. Taken together our data reveal a new role for Wnt4 in mammalian NMJ formation that could be mediated by MuSK, a key receptor in synaptogenesis. PMID:22253844

  11. Effects of several antibiotics on the neuromuscular junction: Part II.

    PubMed

    Yamada, S; Kuno, Y; Iwanaga, H

    1986-04-01

    The effects of various kinds of antibiotics including tetracycline (TC), chloramphenicol (CP), sodium cephalothin (CET), sodium cefazolin (CEZ), colistin sulfate (CL), colistin sodium methanesulfonate (CL-M), bacitracin (BC), gramicidin HCl (GR), rifampicin sulfate (RFP) and lincomycin (LCM), on the neuromuscular junction (NMJ) were studied by in vitro and in vivo experiments. In in vitro experiments, CL and LCM exhibited a blocking effect on the NMJ in rat phrenic nerve diaphragm preparations, and GR caused a marked increase in muscle contraction. This effect was not affected by administration of eserine or CaCl2. In in vitro experiments with frog sciatica nerve and musculus sartorius preparations, CL and GR induced the appearance of endplate potentials, suggesting blockade of the NMJ. No blocking effect of other antibiotics was observed. In in vitro experiments with the preparations from Rana catesbiana frogs, TC and LCM induced a decrease in the frequency of miniature endplate potentials. In in vivo experiments with rabbit musculus tibialis anterior preparations, CL, TC and LCM exerted a blocking effect soon after administration, but GR and RFP had a late blocking effect. CL, GR, BC and RFP were found not to compete with eserine or CaCl2 in terms of the blocking effect on the NMJ. From the fact that TC did not compete with eserine but did compete with CaCl2 and with KCl as blockers at the NMJ, this blocking effect of TC seems to be due to inhibition of release of acetylcholine (ACh). The fact that LCM competes with eserine indicates that this antibiotic has the same type of action as curare on ACh receptors of the NMJ.(ABSTRACT TRUNCATED AT 250 WORDS)

  12. Effects of hindlimb unloading on neuromuscular development of neonatal rats

    NASA Technical Reports Server (NTRS)

    Huckstorf, B. L.; Slocum, G. R.; Bain, J. L.; Reiser, P. M.; Sedlak, F. R.; Wong-Riley, M. T.; Riley, D. A.

    2000-01-01

    We hypothesized that hindlimb suspension unloading of 8-day-old neonatal rats would disrupt the normal development of muscle fiber types and the motor innervation of the antigravity (weightbearing) soleus muscles but not extensor digitorum longus (EDL) muscles. Five rats were suspended 4.5 h and returned 1.5 h to the dam for nursing on a 24 h cycle for 9 days. To control for isolation from the dam, the remaining five littermates were removed on the same schedule but not suspended. Another litter of 10 rats housed in the same room provided a vivarium control. Fibers were typed by myofibrillar ATPase histochemistry and immunostaining for embryonic, slow, fast IIA and fast IIB isomyosins. The percentage of multiple innervation and the complexity of singly-innervated motor terminal endings were assessed in silver/cholinesterase stained sections. Unique to the soleus, unloading accelerated production of fast IIA myosin, delayed expression of slow myosin and retarded increases in standardized muscle weight and fiber size. Loss of multiple innervation was not delayed. However, fewer than normal motor nerve endings achieved complexity. Suspended rats continued unloaded hindlimb movements. These findings suggest that motor neurons resolve multiple innervation through nerve impulse activity, whereas the postsynaptic element (muscle fiber) controls endplate size, which regulates motor terminal arborization. Unexpectedly, in the EDL of unloaded rats, transition from embryonic to fast myosin expression was retarded. Suspension-related foot drop, which stretches and chronically loads EDL, may have prevented fast fiber differentiation. These results demonstrate that neuromuscular development of both weightbearing and non-weightbearing muscles in rats is dependent upon and modulated by hindlimb loading.

  13. Time course of neuromuscular alterations during a prolonged running exercise.

    PubMed

    Place, Nicolas; Lepers, Romuald; Deley, Gaëlle; Millet, Guillaume Y

    2004-08-01

    This study investigated the time course of contractile and neural alterations of knee extensor (KE) muscles during a long-duration running exercise. Nine well-trained triathletes and endurance runners sustained 55% of their maximal aerobic velocity (MAV) on a motorized treadmill for a period of 5 h. Maximal voluntary contraction (MVC), maximal voluntary activation level (%VA), and electrically evoked contractions (single and tetanic stimulations) of KE muscles were evaluated before, after each hour of exercise during short (10 min) interruptions, and at the end of the 5-h period. Oxygen uptake was also measured at regular intervals during the exercise. Reductions of MVC and %VA were significant after the 4th hour of exercise and reached -28% (P < 0.001) and -16% (P < 0.01) respectively at the end of the exercise. The reduction in MVC was highly correlated with the decline of %VA (r = 0.98, P < 0.001). M-wave was also altered after the fourth hour of exercise (P < 0.05) in both vastus lateralis and rectus femoris muscles. Peak twitch was potentiated at the end of the exercise (+18%, P = 0.01); 20- and 80-Hz maximal tetanic forces were not altered by the exercise. Oxygen uptake increased linearly during the running period (+18% at 5 h, P < 0.001). These findings suggest that KE maximal voluntary force generating capability is depressed in the final stages of a 5-h running exercise. Central activation failure and alterations in muscle action potential transmission were important mechanisms contributing to the impairment of the neuromuscular function during prolonged running.

  14. Lower Limb Neuromuscular Asymmetry in Volleyball and Basketball Players

    PubMed Central

    Fort-Vanmeerhaeghe, Azahara; Gual, Gabriel; Romero-Rodriguez, Daniel; Unnitha, Viswanath

    2016-01-01

    Abstract The primary objective of the present study was to evaluate the agreement between the dominant leg (DL) (determined subjectively) and the stronger leg (SL) (determined via a functional test) in a group of basketball and volleyball players. The secondary objective was to calculate lower limb neuromuscular asymmetry when comparing the DL vs the non-dominant leg (NDL) and the SL vs the weaker (WL) leg in the whole group and when differentiating by sex. Seventy-nine male and female volleyball and basketball players (age: 23.7 ± 4.5 years) performed three single-leg vertical countermovement jumps (SLVCJ) on a contact mat. Vertical jump height and an inter-limb asymmetry index (ASI) were determined. Only 32 (40%) of the subjects had a concordance between the perception of their dominant leg and the limb reaching the highest jump height. Using the DL as the discriminating variable, significant (p<0.05) inter-limb differences were found in the total group of players. When comparing between sexes, significant differences (p<0.05) arose in the female group only. With regard to the WL vs. the SL, significant (p<0.05) differences were noted in the whole group and when stratified into males and females. The mean ASI ranged from 9.31% (males) to 12.84% (females) and from 10.49% (males) to 14.26% (females), when comparing the DL vs. the NDL and the SL vs. the WL, respectively. Subjective expression of leg dominance cannot be used as a predictor of limb jump performance. Vertical jump asymmetry of 10-15% exists and this can be considered as a reference value for male and female basketball and volleyball players. PMID:28149351

  15. Embryonic ethanol exposure alters synaptic properties at zebrafish neuromuscular junctions.

    PubMed

    Sylvain, Nicole J; Brewster, Daniel L; Ali, Declan W

    2011-01-01

    Pre-natal alcohol exposure induces delays in fine and gross motor skills, and deficiencies in reflex development via mechanisms that remain to be elucidated. The purpose of the present study was to investigate the effect of embryonic ethanol exposure (16-hour exposure window with 1.5%, 2% or 2.5% EtOH) on synaptic properties at the neuromuscular junction (NMJ) in 3 day post fertilization (dpf) zebrafish larvae. Immunohistochemical studies show that exposure of embryos to 2.5% ethanol for 16 h results in motor neuron axons that display abnormal branching patterns. Co-labelling embryos with pre-synaptic markers such as SV-2 or 3A10, and the post-synaptic marker, α-bungarotoxin, which irreversibly binds to nicotinic acetylcholine receptors (nAChRs), indicates that pre- and post-synaptic sites are properly aligned even when motor neuron axons display abnormal morphology. Miniature endplate currents (mEPCs) recorded from muscle fibers revealed the presence of two types of mEPCs that we dubbed fast and slow. Ethanol treated fish experienced significant changes in the frequencies of fast and slow mEPCs, and an increase in the rise time of slow mEPCs recorded from red muscle fibers. Additionally, embryonic exposure to ethanol resulted in a significant increase in the decay time of fast mEPCs recorded from white fibers. Mean mEPC amplitude was unaffected by ethanol treatment. Together, these results indicate that zebrafish embryos exposed to ethanol may experience altered synaptic properties at the NMJ.

  16. Caenorhabditis elegans Neuromuscular Junction: GABA Receptors and Ivermectin Action

    PubMed Central

    Hernando, Guillermina; Bouzat, Cecilia

    2014-01-01

    The prevalence of human and animal helminth infections remains staggeringly high, thus urging the need for concerted efforts towards this area of research. GABA receptors, encoded by the unc-49 gene, mediate body muscle inhibition in Caenorhabditis elegans and parasitic nematodes and are targets of anthelmintic drugs. Thus, the characterization of nematode GABA receptors provides a foundation for rational anti-parasitic drug design. We therefore explored UNC-49 channels from C. elegans muscle cultured cells of the first larval stage at the electrophysiological and behavioral levels. Whole-cell recordings reveal that GABA, muscimol and the anthelmintic piperazine elicit macroscopic currents from UNC-49 receptors that decay in their sustained presence, indicating full desensitization. Single-channel recordings show that all drugs elicit openings of ∼2.5 pA (+100 mV), which appear either as brief isolated events or in short bursts. The comparison of the lowest concentration required for detectable channel opening, the frequency of openings and the amplitude of macroscopic currents suggest that piperazine is the least efficacious of the three drugs. Macroscopic and single-channel GABA-activated currents are profoundly and apparently irreversibly inhibited by ivermectin. To gain further insight into ivermectin action at C. elegans muscle, we analyzed its effect on single-channel activity of the levamisol-sensitive nicotinic receptor (L-AChR), the excitatory receptor involved in neuromuscular transmission. Ivermectin produces a profound inhibition of the frequency of channel opening without significant changes in channel properties. By revealing that ivermectin inhibits C. elegans muscle GABA and L-AChR receptors, our study adds two receptors to the already known ivermectin targets, thus contributing to the elucidation of its pleiotropic effects. Behavioral assays in worms show that ivermectin potentiates piperazine-induced paralysis, thus suggesting that their

  17. Mediators of burn-induced neuromuscular changes in mice.

    PubMed Central

    Tomera, J. F.; Martyn, J.

    1989-01-01

    1. Muscle paresis and aberrant pharmacological responses are two important pathophysiological changes that have been observed at the neuromuscular junction following thermal injury. By use of the mouse model of 20%, 30% and 50% total body surface area (BSA) burn, we examined the significance of intracellular mediators, adenosine 3':5'-cyclic monophosphate (cyclic AMP) and prostaglandin E2 (PGE2) in perturbing the physiological function of tension development and the pharmacological response to (+)-tubocurarine (+)-Tc at day 21 post-burn. 2. Cyclic AMP levels increased with the size of burn. The relationship between mean cyclic AMP levels and burn size was significant (R2 = 0.96, r = 0.98). Significant (P less than 0.05) reductions in tension development (g) were observed for the 30% and 50% BSA burn group compared to controls (30.3 +/- 8.3 and 34.1 +/- 5.9 vs 59.1 +/- 1.0, respectively). Tension alterations were associated with increased cyclic AMP levels; the relationship between increased cyclic AMP levels and tension decrease was significant (R2 = 0.82, r = 0.91). The dose of (+)-Tc required to inhibit twitch tension increased in proportion to burn size and was statistically significant in the 50% BSA burn group compared to controls (0.3320 +/- 0.09 vs 0.1093 +/- 0.11 mg kg-1, P less than 0.05). The alterations in the effective dose of (+)-Tc were significantly correlated to increases in cyclic AMP levels (R2 = 0.70, r = 0.83). Although PGE2 levels were elevated in the 30% and 50% burn groups, no relation was seen to either tension or (+)-Tc doses.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:2556207

  18. Aging influences adaptations of the neuromuscular junction to endurance training.

    PubMed

    Deschenes, M R; Roby, M A; Glass, E K

    2011-09-08

    This investigation sought to determine if aging affected adaptations of the neuromuscular junction (NMJ) to exercise training. Twenty young adult (8 months) and 20 aged (24 months) rats were assigned to either a program of treadmill exercise, or sedentary conditions. Following the 10-week experimental period, rats were euthanized, and soleus and plantaris muscles were removed and frozen. Longitudinal sections of the muscles were fluorescently stained to visualize pre-synaptic nerve terminals and post-synaptic endplates on both slow- and fast-twitch fibers. Images were collected with confocal microscopy and quantified. Muscle cross-sections were histochemically stained to assess muscle fiber profiles (size and fiber type). Our analysis of NMJs revealed a high degree of specificity and sensitivity to aging, exercise training, and their interaction. In the soleus, slow-twitch NMJs demonstrated significant (P ≤ 0.05) training-induced adaptations in young adult, but not aged rats. In the fast-twitch NMJs of the soleus, aging, but not training, was associated with remodeling. In the plantaris, aging, but not training, remodeled the predominant fast-twitch NMJs, but only pre-synaptically. In contrast, the slow-twitch NMJs of the plantaris displayed morphologic adaptations to both aging and exercise in pre- and post-synaptic components. Muscle fiber profiles indicated that changes in NMJ size were unrelated to adaptations of their fibers. Our data show that aging interferes with the ability of NMJs to adapt to exercise training. Results also reveal complexity in the coordination of synaptic responses among different muscles, and different fiber types within muscles, in their adaptation to aging and exercise training.

  19. Recovery of central and peripheral neuromuscular fatigue after exercise.

    PubMed

    Carroll, Timothy John; Taylor, Janet L; Gandevia, Simon C

    2016-12-08

    Sustained physical exercise leads to a reduced capacity to produce voluntary force that typically outlasts the exercise bout. This "fatigue" can be due both to impaired muscle function, termed "peripheral fatigue", and a reduction in the capacity of the central nervous system to activate muscles, termed "central fatigue". In this mini-review we consider the factors that determine the recovery of voluntary force generating capacity after various types of exercise. After brief, high-intensity exercise there is typically a rapid restitution of force that is due to recovery of central fatigue (typically within 2min) and aspects of peripheral fatigue associated with excitation-contraction coupling and re-perfusion of muscles (typically within 3-5 min). Complete recovery of muscle function may be incomplete for some hours, however, due to prolonged impairment in intracellular Ca(2+) release or sensitivity. After low-intensity exercise of long duration, voluntary force typically shows rapid, partial, recovery within the first few minutes, due largely to recovery of the central, neural component. However, this ability to voluntarily activate muscles may not recover completely within 30 minutes after exercise. Recovery of peripheral fatigue contributes comparatively little to the fast initial force restitution, and is typically incomplete for at least 20-30 minutes. Work remains to identify what factors underlie the prolonged central fatigue that usually accompanies long-duration single joint and locomotor exercise, and to document how the time-course of neuromuscular recovery is affected by exercise intensity and duration in locomotor exercise. Such information could be useful to enhance rehabilitation and sports performance.

  20. Are Females More Resistant to Extreme Neuromuscular Fatigue?

    PubMed

    Temesi, John; Arnal, Pierrick J; Rupp, Thomas; Féasson, Léonard; Cartier, Régine; Gergelé, Laurent; Verges, Samuel; Martin, Vincent; Millet, Guillaume Y

    2015-07-01

    Despite interest in the possibility of females outperforming males in ultraendurance sporting events, little is known about the sex differences in fatigue during prolonged locomotor exercise. This study investigated possible sex differences in central and peripheral fatigue in the knee extensors and plantar flexors resulting from a 110-km ultra-trail-running race. Neuromuscular function of the knee extensors and plantar flexors was evaluated via transcranial magnetic stimulation (TMS) and electrical nerve stimulation before and after an ultra-trail-running race in 20 experienced ultraendurance trail runners (10 females and 10 males matched by percent of the winning time by sex) during maximal and submaximal voluntary contractions and in relaxed muscle. Maximal voluntary knee extensor torque decreased more in males than in females (-38% vs -29%, P = 0.006) although the reduction in plantar flexor torque was similar between sexes (-26% vs -31%). Evoked mechanical plantar flexor responses decreased more in males than in females (-23% vs -8% for potentiated twitch amplitude, P = 0.010), indicating greater plantar flexor peripheral fatigue in males. Maximal voluntary activation assessed by TMS and electrical nerve stimulation decreased similarly in both sexes for both muscle groups. Indices of knee extensor peripheral fatigue and corticospinal excitability and inhibition changes were also similar for both sexes. Females exhibited less peripheral fatigue in the plantar flexors than males did after a 110-km ultra-trail-running race and males demonstrated a greater decrease in maximal force loss in the knee extensors. There were no differences in the magnitude of central fatigue for either muscle group or TMS-induced outcomes. The lower level of fatigue in the knee extensors and peripheral fatigue in the plantar flexors could partly explain the reports of better performance in females in extreme duration running races as race distance increases.

  1. Extubation of patients with neuromuscular weakness: a new management paradigm.

    PubMed

    Bach, John Robert; Gonçalves, Miguel R; Hamdani, Irram; Winck, Joao Carlos

    2010-05-01

    Successful extubation conventionally necessitates the passing of spontaneous breathing trials (SBTs) and ventilator weaning parameters. We report successful extubation of patients with neuromuscular disease (NMD) and weakness who could not pass them. NMD-specific extubation criteria and a new extubation protocol were developed. Data were collected on 157 consecutive "unweanable" patients, including 83 transferred from other hospitals who refused tracheostomies. They could not pass the SBTs before or after extubation. Once the pulse oxyhemoglobin saturation (Spo(2)) was maintained at > or = 95% in ambient air, patients were extubated to full noninvasive mechanical ventilation (NIV) support and aggressive mechanically assisted coughing (MAC). Rather than oxygen, NIV and MAC were used to maintain or return the Spo(2) to > or = 95%. Extubation success was defined as not requiring reintubation during the hospitalization and was considered as a function of diagnosis, preintubation NIV experience, and vital capacity and assisted cough peak flows (CPF) at extubation. Before hospitalization 96 (61%) patients had no experience with NIV, 41 (26%) used it < 24 h per day, and 20 (13%) were continuously NIV dependent. The first-attempt protocol extubation success rate was 95% (149 patients). All 98 extubation attempts on patients with assisted CPF > or = 160 L/m were successful. The dependence on continuous NIV and the duration of dependence prior to intubation correlated with extubation success (P < .005). Six of eight patients who initially failed extubation succeeded on subsequent attempts, so only two with no measurable assisted CPF underwent tracheotomy. Continuous volume-cycled NIV via oral interfaces and masks and MAC with oximetry feedback in ambient air can permit safe extubation of unweanable patients with NMD.

  2. Modular neuromuscular control of human locomotion by central pattern generator.

    PubMed

    Haghpanah, Seyyed Arash; Farahmand, Farzam; Zohoor, Hassan

    2017-02-28

    The central pattern generators (CPG) in the spinal cord are thought to be responsible for producing the rhythmic motor patterns during rhythmic activities. For locomotor tasks, this involves much complexity, due to a redundant system of muscle actuators with a large number of highly nonlinear muscles. This study proposes a reduced neural control strategy for the CPG, based on modular organization of the co-active muscles, i.e., muscle synergies. Four synergies were extracted from the EMG data of the major leg muscles of two subjects, during two gait trials each, using non-negative matrix factorization algorithm. A Matsuoka׳s four-neuron CPG model with mutual inhibition, was utilized to generate the rhythmic activation patterns of the muscle synergies, using the hip flexion angle and foot contact force information from the sensory afferents as inputs. The model parameters were tuned using the experimental data of one gait trial, which resulted in a good fitting accuracy (RMSEs between 0.0491 and 0.1399) between the simulation and experimental synergy activations. The model׳s performance was then assessed by comparing its predictions for the activation patterns of the individual leg muscles during locomotion with the relevant EMG data. Results indicated that the characteristic features of the complex activation patterns of the muscles were well reproduced by the model for different gait trials and subjects. In general, the CPG- and muscle synergy-based model was promising in view of its simple architecture, yet extensive potentials for neuromuscular control, e.g., resolving redundancies, distributed and fast control, and modulation of locomotion by simple control signals.

  3. Neuromuscular consequences of an extreme mountain ultra-marathon.

    PubMed

    Millet, Guillaume Y; Tomazin, Katja; Verges, Samuel; Vincent, Christopher; Bonnefoy, Régis; Boisson, Renée-Claude; Gergelé, Laurent; Féasson, Léonard; Martin, Vincent

    2011-02-22

    We investigated the physiological consequences of one of the most extreme exercises realized by humans in race conditions: a 166-km mountain ultra-marathon (MUM) with 9500 m of positive and negative elevation change. For this purpose, (i) the fatigue induced by the MUM and (ii) the recovery processes over two weeks were assessed. Evaluation of neuromuscular function (NMF) and blood markers of muscle damage and inflammation were performed before and immediately following (n = 22), and 2, 5, 9 and 16 days after the MUM (n = 11) in experienced ultra-marathon runners. Large maximal voluntary contraction decreases occurred after MUM (-35% [95% CI: -28 to -42%] and -39% [95% CI: -32 to -46%] for KE and PF, respectively), with alteration of maximal voluntary activation, mainly for KE (-19% [95% CI: -7 to -32%]). Significant modifications in markers of muscle damage and inflammation were observed after the MUM as suggested by the large changes in creatine kinase (from 144 ± 94 to 13,633 ± 12,626 UI L(-1)), myoglobin (from 32 ± 22 to 1,432 ± 1,209 µg L(-1)), and C-Reactive Protein (from <2.0 to 37.7 ± 26.5 mg L(-1)). Moderate to large reductions in maximal compound muscle action potential amplitude, high-frequency doublet force, and low frequency fatigue (index of excitation-contraction coupling alteration) were also observed for both muscle groups. Sixteen days after MUM, NMF had returned to initial values, with most of the recovery process occurring within 9 days of the race. These findings suggest that the large alterations in NMF after an ultra-marathon race are multi-factorial, including failure of excitation-contraction coupling, which has never been described after prolonged running. It is also concluded that as early as two weeks after such an extreme running exercise, maximal force capacities have returned to baseline.

  4. Caenorhabditis elegans neuromuscular junction: GABA receptors and ivermectin action.

    PubMed

    Hernando, Guillermina; Bouzat, Cecilia

    2014-01-01

    The prevalence of human and animal helminth infections remains staggeringly high, thus urging the need for concerted efforts towards this area of research. GABA receptors, encoded by the unc-49 gene, mediate body muscle inhibition in Caenorhabditis elegans and parasitic nematodes and are targets of anthelmintic drugs. Thus, the characterization of nematode GABA receptors provides a foundation for rational anti-parasitic drug design. We therefore explored UNC-49 channels from C. elegans muscle cultured cells of the first larval stage at the electrophysiological and behavioral levels. Whole-cell recordings reveal that GABA, muscimol and the anthelmintic piperazine elicit macroscopic currents from UNC-49 receptors that decay in their sustained presence, indicating full desensitization. Single-channel recordings show that all drugs elicit openings of ∼2.5 pA (+100 mV), which appear either as brief isolated events or in short bursts. The comparison of the lowest concentration required for detectable channel opening, the frequency of openings and the amplitude of macroscopic currents suggest that piperazine is the least efficacious of the three drugs. Macroscopic and single-channel GABA-activated currents are profoundly and apparently irreversibly inhibited by ivermectin. To gain further insight into ivermectin action at C. elegans muscle, we analyzed its effect on single-channel activity of the levamisol-sensitive nicotinic receptor (L-AChR), the excitatory receptor involved in neuromuscular transmission. Ivermectin produces a profound inhibition of the frequency of channel opening without significant changes in channel properties. By revealing that ivermectin inhibits C. elegans muscle GABA and L-AChR receptors, our study adds two receptors to the already known ivermectin targets, thus contributing to the elucidation of its pleiotropic effects. Behavioral assays in worms show that ivermectin potentiates piperazine-induced paralysis, thus suggesting that their

  5. Lower Limb Neuromuscular Asymmetry in Volleyball and Basketball Players.

    PubMed

    Fort-Vanmeerhaeghe, Azahara; Gual, Gabriel; Romero-Rodriguez, Daniel; Unnitha, Viswanath

    2016-04-01

    The primary objective of the present study was to evaluate the agreement between the dominant leg (DL) (determined subjectively) and the stronger leg (SL) (determined via a functional test) in a group of basketball and volleyball players. The secondary objective was to calculate lower limb neuromuscular asymmetry when comparing the DL vs the non-dominant leg (NDL) and the SL vs the weaker (WL) leg in the whole group and when differentiating by sex. Seventy-nine male and female volleyball and basketball players (age: 23.7 ± 4.5 years) performed three single-leg vertical countermovement jumps (SLVCJ) on a contact mat. Vertical jump height and an inter-limb asymmetry index (ASI) were determined. Only 32 (40%) of the subjects had a concordance between the perception of their dominant leg and the limb reaching the highest jump height. Using the DL as the discriminating variable, significant (p<0.05) inter-limb differences were found in the total group of players. When comparing between sexes, significant differences (p<0.05) arose in the female group only. With regard to the WL vs. the SL, significant (p<0.05) differences were noted in the whole group and when stratified into males and females. The mean ASI ranged from 9.31% (males) to 12.84% (females) and from 10.49% (males) to 14.26% (females), when comparing the DL vs. the NDL and the SL vs. the WL, respectively. Subjective expression of leg dominance cannot be used as a predictor of limb jump performance. Vertical jump asymmetry of 10-15% exists and this can be considered as a reference value for male and female basketball and volleyball players.

  6. Neuromuscular fatigue following isometric contractions with similar torque time integral.

    PubMed

    Rozand, V; Cattagni, T; Theurel, J; Martin, A; Lepers, R

    2015-01-01

    Torque time integral (TTI) is the combination of intensity and duration of a contraction. The aim of this study was to compare neuromuscular alterations following different isometric sub-maximal contractions of the knee extensor muscles but with similar TTI. Sixteen participants performed 3 sustained contractions at different intensities (25%, 50%, and 75% of Maximal Voluntary Contraction (MVC) torque) with different durations (68.5±33.4 s, 35.1±16.8 s and 24.8±12.9 s, respectively) but similar TTI value. MVC torque, maximal voluntary activation level (VAL), M-wave characteristics and potentiated doublet amplitude were assessed before and immediately after the sustained contractions. EMG activity of the vastus lateralis (VL) and -rectus femoris (RF) muscles was recorded during the sustained contractions. MVC torque reduction was similar in the 3 conditions after the exercise (-23.4±2.7%). VAL decreased significantly in a similar extent (-3.1±1.3%) after the 3 sustained contractions. Potentiated doublet amplitude was similarly reduced in the 3 conditions (-19.7±1.5%), but VL and RF M-wave amplitudes remained unchanged. EMG activity of VL and RF muscles increased in the same extent during the 3 contractions (VL: 54.5±40.4%; RF: 53.1±48.7%). These results suggest that central and peripheral alterations accounting for muscle fatigue are similar following isometric contractions with similar TTI. TTI should be considered in the exploration of muscle fatigue during sustained isometric contractions.

  7. Whistle and cough pressures in children with neuromuscular disorders.

    PubMed

    Aloui, Sabrina; Khirani, Sonia; Ramirez, Adriana; Colella, Marina; Louis, Bruno; Amaddeo, Alessandro; Fauroux, Brigitte

    2016-04-01

    Expiratory muscle strength is a determinant of cough function. Maximal static expiratory pressure (PEmax) manoeuvres are widely used but are limited by patient motivation and technique. The study hypothesized that whistle mouth (PmW) and cough gastric (PgasCough) pressures might provide additional tests of expiratory muscle strength in children and young adults with neuromuscular disease (NMD). We retrospectively reviewed the data of lung function and respiratory muscle tests of all the patients with NMD followed in our centre between November 2001 and December 2013. PmW and PgasCough were compared to other common tests. Three hundred and four respiratory evaluations were performed in 143 patients, aged 3-29 years old. Seventy-two patients had 2 to 8 evaluations. Median [interquartiles] PEmax (38 [28-54] cmH2O) did not differ significantly from PgasCough (45 [30-60] cmH2O) and both were significantly greater than PmW (30 [19-44] cmH2O). Significant good correlations were observed between all the expiratory muscle parameters. The best correlation was observed between PEmax and PmW (r = 0.812, p < 0.001). Moreover, good correlations were found between the percentage of predicted forced vital capacity and PmW (r = 0.619, p < 0.001) and PgasCough (r = 0.568, p < 0.001). Concerning the whistle test, the non-invasive measurement highly correlated with invasive measurements. PmW and PgasCough are simple and valuable tests to assess expiratory muscle strength in children and young adults with NMD. These tests are particularly useful in children having difficulties to perform PEmax manoeuvre. They have the great advantage of their simplicity, but PgasCough is limited by its invasiveness. Copyright © 2016 Elsevier Ltd. All rights reserved.

  8. A Murine Model of Muscle Training by Neuromuscular Electrical Stimulation

    PubMed Central

    Ambrosio, Fabrisia; Fitzgerald, G. Kelley; Ferrari, Ricardo; Distefano, Giovanna; Carvell, George

    2012-01-01

    Neuromuscular electrical stimulation (NMES) is a common clinical modality that is widely used to restore1, maintain2 or enhance3-5 muscle functional capacity. Transcutaneous surface stimulation of skeletal muscle involves a current flow between a cathode and an anode, thereby inducing excitement of the motor unit and the surrounding muscle fibers. NMES is an attractive modality to evaluate skeletal muscle adaptive responses for several reasons. First, it provides a reproducible experimental model in which physiological adaptations, such as myofiber hypertophy and muscle strengthening6, angiogenesis7-9, growth factor secretion9-11, and muscle precursor cell activation12 are well documented. Such physiological responses may be carefully titrated using different parameters of stimulation (for Cochrane review, see 13). In addition, NMES recruits motor units non-selectively, and in a spatially fixed and temporally synchronous manner14, offering the advantage of exerting a treatment effect on all fibers, regardless of fiber type. Although there are specified contraindications to NMES in clinical populations, including peripheral venous disorders or malignancy, for example, NMES is safe and feasible, even for those who are ill and/or bedridden and for populations in which rigorous exercise may be challenging. Here, we demonstrate the protocol for adapting commercially available electrodes and performing a NMES protocol using a murine model. This animal model has the advantage of utilizing a clinically available device and providing instant feedback regarding positioning of the electrode to elicit the desired muscle contractile effect. For the purpose of this manuscript, we will describe the protocol for muscle stimulation of the anterior compartment muscles of a mouse hindlimb. PMID:22617846

  9. A murine model of muscle training by neuromuscular electrical stimulation.

    PubMed

    Ambrosio, Fabrisia; Fitzgerald, G Kelley; Ferrari, Ricardo; Distefano, Giovanna; Carvell, George

    2012-05-09

    Neuromuscular electrical stimulation (NMES) is a common clinical modality that is widely used to restore (1), maintain (2) or enhance (3-5) muscle functional capacity. Transcutaneous surface stimulation of skeletal muscle involves a current flow between a cathode and an anode, thereby inducing excitement of the motor unit and the surrounding muscle fibers. NMES is an attractive modality to evaluate skeletal muscle adaptive responses for several reasons. First, it provides a reproducible experimental model in which physiological adaptations, such as myofiber hypertophy and muscle strengthening (6), angiogenesis (7-9), growth factor secretion (9-11), and muscle precursor cell activation (12) are well documented. Such physiological responses may be carefully titrated using different parameters of stimulation (for Cochrane review, see (13)). In addition, NMES recruits motor units non-selectively, and in a spatially fixed and temporally synchronous manner (14), offering the advantage of exerting a treatment effect on all fibers, regardless of fiber type. Although there are specified contraindications to NMES in clinical populations, including peripheral venous disorders or malignancy, for example, NMES is safe and feasible, even for those who are ill and/or bedridden and for populations in which rigorous exercise may be challenging. Here, we demonstrate the protocol for adapting commercially available electrodes and performing a NMES protocol using a murine model. This animal model has the advantage of utilizing a clinically available device and providing instant feedback regarding positioning of the electrode to elicit the desired muscle contractile effect. For the purpose of this manuscript, we will describe the protocol for muscle stimulation of the anterior compartment muscles of a mouse hindlimb.

  10. Maturation and Sex Differences in Neuromuscular Characteristics of Youth Athletes.

    PubMed

    DiStefano, Lindsay J; Martinez, Jessica C; Crowley, Elizabeth; Matteau, Erin; Kerner, Michael S; Boling, Michelle C; Nguyen, Anh-Dung; Trojian, Thomas H

    2015-09-01

    Understanding how neuromuscular factors that are associated with lower extremity injury risk, such as landing kinematics, muscle strength, and flexibility, change as children mature may enhance age-specific recommendations for injury prevention programs. The purpose of this study was to compare these factors in prepubertal, pubertal, and postpubertal male and female athletes. Subjects were classified on maturation stage (prepubertal: 16 males, 15 females, age: 9 ± 1 years; pubertal: 13 males, 12 females, age: 12 ± 3 years; postpubertal: 30 males, 27 females, age: 16 ± 2 years). Researchers measured lower extremity isometric muscle strength and flexibility and evaluated kinematics and vertical ground reaction forces (VGRFs) during a jump-landing task. Three-dimensional kinematics at initial contact (IC), joint displacements, and peak VGRF were calculated. Separate multivariate analyses of variance were performed to evaluate sex and maturation differences (α ≤ 0.05). Postpubertal females landed with less knee flexion at IC (p = 0.006) and demonstrated lower knee extension strength (p = 0.01) than prepubertal and pubertal females. Postpubertal males landed with less hip adduction displacement (postpubertal males = 12.53 ± 6.15°, prepubertal males = 18.84 ± 7.47°; p = 0.04) and less peak VGRF (postpubertal males = 1.53 ± 0.27% body weight [BW], prepubertal males = 1.99 ± 0.32% BW; p = 0.03) compared with prepubertal males. These findings suggest encouraging sagittal plane absorption and decreasing frontal plane motion at the hip, whereas maintaining quadriceps strength may be important for reducing injury risk in postpubertal athletes.

  11. Analgesia, sedation, and neuromuscular blockade during targeted temperature management after cardiac arrest.

    PubMed

    Riker, Richard R; Gagnon, David J; May, Teresa; Seder, David B; Fraser, Gilles L

    2015-12-01

    The approach to sedation, analgesia, and neuromuscular blockade during targeted temperature management (TTM) remains largely unstudied, forcing clinicians to adapt previous research from other patient environments. During TTM, very little data guide drug selection, doses, and specific therapeutic goals. Sedation should be deep enough to prevent awareness during neuromuscular blockade, but titration is complex as metabolism and clearance are delayed for almost all drugs during hypothermia. Deeper sedation is associated with prolonged intensive care unit (ICU) and ventilator therapy, increased delirium and infection, and delayed wakening which can confound early critical neurological assessments, potentially resulting in erroneous prognostication and inappropriate withdrawal of life support. We review the potential therapeutic goals for sedation, analgesia, and neuromuscular blockade during TTM; the adverse events associated with that treatment; data suggesting that TTM and organ dysfunction impair drug metabolism; and controversies and potential benefits of specific monitoring. We also highlight the areas needing better research to guide our therapy.

  12. Reversal of neuromuscular block with a selective relaxant-binding agent: sugammadex.

    PubMed

    Ren, Wendy H P; Jahr, Jonathan S

    2009-01-01

    Muscle relaxants are used in the perioperative period to aid in endotracheal intubation, facilitate surgical exposure, and in the critical care setting for prolonged relaxation. Until now, the only mechanism to reverse their effect is acetylcholinesterase inhibitors that result in excess parasympathetic activity and require a second drug to prevent this side effect. Additionally, the onset and degree of neuromuscular antagonism are often unpredictable and unreliable. Sugammadex is the first of the cyclodextrins to be used as a therapeutic agent. It quickly, effectively, and safely reverses steroidal neuromuscular blockers by encapsulating the muscle relaxant and rendering it inactive. Sugammadex may be considered the ideal reversal agent and the first drug in its class, which will likely change the practice of anesthesia and clinical neuromuscular pharmacology.

  13. Acetylcholinesterase of human erythrocytes and neuromuscular junctions: homologies revealed by monoclonal antibodies.

    PubMed Central

    Fambrough, D M; Engel, A G; Rosenberry, T L

    1982-01-01

    Human erythrocyte acetylcholinesterase was used to immunize mice, and hybridomas were generated by fusion of mouse spleen cells with cells of the Sp 2/0 myeloma cell line. Five independently derived hybridoma clones produced antibodies that bound to purified erythrocyte acetylcholinesterase. All of these antibodies crossreacted with human and monkey neuromuscular junctions; immunocytochemical staining patterns corresponded to the distribution of junctional acetylcholinesterase. The monoclonal antibodies fell into at least four categories based on differences in crossreactivity with neuromuscular acetylcholinesterase of rabbit, dog, calf, and guinea pig, and competition tests indicated that the antibodies defined five different antigenic sites on the acetylcholinesterase molecule. It is concluded that there is a high level of homology between the acetylcholinesterases of erythrocytes and neuromuscular junctions. Images PMID:6175961

  14. Local identifiability and sensitivity analysis of neuromuscular blockade and depth of hypnosis models.

    PubMed

    Silva, M M; Lemos, J M; Coito, A; Costa, B A; Wigren, T; Mendonça, T

    2014-01-01

    This paper addresses the local identifiability and sensitivity properties of two classes of Wiener models for the neuromuscular blockade and depth of hypnosis, when drug dose profiles like the ones commonly administered in the clinical practice are used as model inputs. The local parameter identifiability was assessed based on the singular value decomposition of the normalized sensitivity matrix. For the given input signal excitation, the results show an over-parameterization of the standard pharmacokinetic/pharmacodynamic models. The same identifiability assessment was performed on recently proposed minimally parameterized parsimonious models for both the neuromuscular blockade and the depth of hypnosis. The results show that the majority of the model parameters are identifiable from the available input-output data. This indicates that any identification strategy based on the minimally parameterized parsimonious Wiener models for the neuromuscular blockade and for the depth of hypnosis is likely to be more successful than if standard models are used.

  15. A pivoting elliptical training system for improving pivoting neuromuscular control and rehabilitating musculoskeletal injuries.

    PubMed

    Ren, Yupeng; Lee, Song Joo; Park, Hyung-Soon; Zhang, Li-Qun

    2013-09-01

    Knee injuries often occur in pivoting activities but most existing training and rehabilitation devices mainly involve sagittal movements. A pivoting elliptical training system (PETS) was developed to train and evaluate neuromuscular control in pivoting for the purposes of prevention and rehabilitation of musculoskeletal injuries. The PETS have capabilities of controlling two footplates individually or simultaneously through servomotor control so that the footplates behave like two torsional springs with adjustable offset and stiffness, slippery surface, or under external perturbations. Feasibility of the PETS in improving pivoting neuromuscular control and pivoting neuromechanical properties was demonstrated through experiments on healthy subjects, with reduced pivoting instability and reaction time, and improved proprioceptive acuity following training. The PETS can potentially be used as a therapeutic and research tool to investigate mechanisms underlying pivoting-related injuries and train human subjects for improving neuromuscular control during risky pivoting activities.

  16. [Structure of the neuromuscular spindles in the tongue of human fetuses].

    PubMed

    Maĭboroda, Iu N; Mokin, Iu N

    1983-10-01

    The dynamics in development of some components--of the neuromuscular spindles in the human fetus and newborn tongues have been studied by means of certain general and neurohistological methods with elements of morphometry. During the whole prenatal period of the human life, there is a certain synchronism in the development of the lingual proper muscles and the neuro-muscular spindles. Certain integration in the development of the neuro-muscular spindles is observed in 4-6-month-old fetuses; in 8-9-month-old fetuses and in newborns it is substituted for heterochronicity. By the time of birth, the muscle spindle contractile elements are supplied with a more differentiated efferent innervation. The latter actively effects the morphological state of the intrafusal muscle fibers and forms a base for functional activity of the tongue.

  17. Erbb2 regulates neuromuscular synapse formation and is essential for muscle spindle development.

    PubMed

    Leu, Marco; Bellmunt, Elena; Schwander, Martin; Fariñas, Isabel; Brenner, Hans Rudolf; Müller, Ulrich

    2003-06-01

    Neuregulins and their Erbb receptors have been implicated in neuromuscular synapse formation by regulating gene expression in subsynaptic nuclei. To analyze the function of Erbb2 in this process, we have inactivated the Erbb2 gene in developing muscle fibers by Cre/Lox-mediated gene ablation. Neuromuscular synapses form in the mutant mice, but the synapses are less efficient and contain reduced levels of acetylcholine receptors. Surprisingly, the mutant mice also show proprioceptive defects caused by abnormal muscle spindle development. Sensory Ia afferent neurons establish initial contact with Erbb2-deficient myotubes. However, functional spindles never develop. Taken together, our data suggest that Erbb2 signaling regulates the formation of both neuromuscular synapses and muscle spindles.

  18. Sympathetic innervation controls homeostasis of neuromuscular junctions in health and disease.

    PubMed

    Khan, Muzamil Majid; Lustrino, Danilo; Silveira, Willian A; Wild, Franziska; Straka, Tatjana; Issop, Yasmin; O'Connor, Emily; Cox, Dan; Reischl, Markus; Marquardt, Till; Labeit, Dittmar; Labeit, Siegfried; Benoit, Evelyne; Molgó, Jordi; Lochmüller, Hanns; Witzemann, Veit; Kettelhut, Isis C; Navegantes, Luiz C C; Pozzan, Tullio; Rudolf, Rüdiger

    2016-01-19

    The distribution and function of sympathetic innervation in skeletal muscle have largely remained elusive. Here we demonstrate that sympathetic neurons make close contact with neuromuscular junctions and form a network in skeletal muscle that may functionally couple different targets including blood vessels, motor neurons, and muscle fibers. Direct stimulation of sympathetic neurons led to activation of muscle postsynaptic β2-adrenoreceptor (ADRB2), cAMP production, and import of the transcriptional coactivator peroxisome proliferator-activated receptor γ-coactivator 1α (PPARGC1A) into myonuclei. Electrophysiological and morphological deficits of neuromuscular junctions upon sympathectomy and in myasthenic mice were rescued by sympathicomimetic treatment. In conclusion, this study identifies the neuromuscular junction as a target of the sympathetic nervous system and shows that sympathetic input is crucial for synapse maintenance and function.

  19. Reduced gap junctional coupling leads to uncorrelated motor neuron firing and precocious neuromuscular synapse elimination.

    PubMed

    Personius, Kirkwood E; Chang, Qiang; Mentis, George Z; O'Donovan, Michael J; Balice-Gordon, Rita J

    2007-07-10

    During late embryonic and early postnatal life, neuromuscular junctions undergo synapse elimination that is modulated by patterns of motor neuron activity. Here, we test the hypothesis that reduced spinal neuron gap junctional coupling decreases temporally correlated motor neuron activity that, in turn, modulates neuromuscular synapse elimination, by using mutant mice lacking connexin 40 (Cx40), a developmentally regulated gap junction protein expressed in motor and other spinal neurons. In Cx40-/- mice, electrical coupling among lumbar motor neurons, measured by whole-cell recordings, was reduced, and single motor unit recordings in awake, behaving neonates showed that temporally correlated motor neuron activity was also reduced. Immunostaining and intracellular recording showed that the neuromuscular synapse elimination was accelerated in muscles from Cx40-/- mice compared with WT littermates. Our work shows that gap junctional coupling modulates neuronal activity patterns that, in turn, mediate synaptic competition, a process that shapes synaptic circuitry in the developing brain.

  20. Reduced gap junctional coupling leads to uncorrelated motor neuron firing and precocious neuromuscular synapse elimination

    PubMed Central

    Personius, Kirkwood E.; Chang, Qiang; Mentis, George Z.; O'Donovan, Michael J.; Balice-Gordon, Rita J.

    2007-01-01

    During late embryonic and early postnatal life, neuromuscular junctions undergo synapse elimination that is modulated by patterns of motor neuron activity. Here, we test the hypothesis that reduced spinal neuron gap junctional coupling decreases temporally correlated motor neuron activity that, in turn, modulates neuromuscular synapse elimination, by using mutant mice lacking connexin 40 (Cx40), a developmentally regulated gap junction protein expressed in motor and other spinal neurons. In Cx40−/− mice, electrical coupling among lumbar motor neurons, measured by whole-cell recordings, was reduced, and single motor unit recordings in awake, behaving neonates showed that temporally correlated motor neuron activity was also reduced. Immunostaining and intracellular recording showed that the neuromuscular synapse elimination was accelerated in muscles from Cx40−/− mice compared with WT littermates. Our work shows that gap junctional coupling modulates neuronal activity patterns that, in turn, mediate synaptic competition, a process that shapes synaptic circuitry in the developing brain. PMID:17609378

  1. Chronic exposure to a 60-Hz electric field: effects on neuromuscular function in the rat

    SciTech Connect

    Jaffe, R.A.; Laszewski, B.L.; Carr, D.B.

    1981-01-01

    Neuromuscular function in adult male rats was studied following 30 days of exposure to a 60-Hz electric field at 100 kV/m (unperturbed field strength). Isometric force transducters were attached to the tendons of the plantaris (predominantly fast twitch), and soleus (predominantly slow twitch) muscles in the urethan-anesthetized rat. Square-wave stimuli were delivered to the distal stump of the transected sciatic nerve. Several measurements were used to characterize neuromuscular function, including twitch characteristics, chronaxie, tetanic and posttetanic potentiation, and fatigue and recovery. The results from three independent series of experiments are reported. Only recovery from fatigue in slow-twitch muscles was consistently and significantly affected (enhanced) by electric-field exposure. This effect does not appear to be mediated by field-induced changes in either neuromuscular transmission, or in the contractile mechanism itself. It is suggested that the effect may be mediated secondary to an effect on mechanisms regulating muscle blood flow or metabolism.

  2. Chronic exposure to a 60-Hz electric field: effects on neuromuscular function in the rat.

    PubMed

    Jaffe, R A; Laszewski, B L; Carr, D B

    1981-01-01

    Neuromuscular function in adult male rats was studied following 30 days of exposure to a 60-Hz electric field at 100 kV/m (unperturbed field strength). Isometric force transducers were attached to the tendons of the plantaris (predominantly fast twitch), and soleus (predominantly slow twitch) muscles in the urethan-anesthetized rat. Square-wave stimuli were delivered to the distal stump of the transected sciatic nerve. Several measurements were used to characterized neuromuscular function, including twitch characteristics, chronaxie, tetanic and posttetanic potentiation, and fatigue and recovery. The results from three independent series of experiments are reported. Only recovery from fatigue in slow-twitch muscles was consistently and significantly affected (enhanced) by electric-field exposure. This effect does not appear to be mediated by field-induced changes in either neuromuscular transmission, or in the contractile mechanism itself. It is suggested that the effect may be mediated secondary to an effect on mechanisms regulating muscle blood flow or metabolism.

  3. Placebo response in pain, fatigue, and performance: possible implications for neuromuscular disorders.

    PubMed

    Shaibani, Aziz; Frisaldi, Elisa; Benedetti, Fabrizio

    2017-03-01

    The placebo response in neuromuscular disorders is not well understood. The only available data regarding the underlying mechanisms are related to neuropathic pain. In this review, we describe the factors that contribute to improved outcomes in the placebo arm, with specific attention to pain and fatigue, as well as some of the most important psychobiological mechanisms that may explain such a response. This approach might also improve our insight into the symptomatology and therapeutic responses of other neuromuscular disorders. The fact that more than 90% of tested analgesics for neuropathic pain have failed in advanced phases of clinical trials should prompt a greater investment of effort and resources into understanding the mechanisms and impact of placebos in clinical research. Such an endeavor will help improve the design of clinical trials and will provide information that informs clinical neuromuscular practice. This article is protected by copyright. All rights reserved.

  4. Sympathetic innervation controls homeostasis of neuromuscular junctions in health and disease

    PubMed Central

    Khan, Muzamil Majid; Lustrino, Danilo; Silveira, Willian A.; Wild, Franziska; Straka, Tatjana; Issop, Yasmin; O’Connor, Emily; Cox, Dan; Reischl, Markus; Marquardt, Till; Labeit, Dittmar; Labeit, Siegfried; Benoit, Evelyne; Molgó, Jordi; Lochmüller, Hanns; Witzemann, Veit; Kettelhut, Isis C.; Navegantes, Luiz C. C.; Pozzan, Tullio; Rudolf, Rüdiger

    2016-01-01

    The distribution and function of sympathetic innervation in skeletal muscle have largely remained elusive. Here we demonstrate that sympathetic neurons make close contact with neuromuscular junctions and form a network in skeletal muscle that may functionally couple different targets including blood vessels, motor neurons, and muscle fibers. Direct stimulation of sympathetic neurons led to activation of muscle postsynaptic β2-adrenoreceptor (ADRB2), cAMP production, and import of the transcriptional coactivator peroxisome proliferator-activated receptor γ-coactivator 1α (PPARGC1A) into myonuclei. Electrophysiological and morphological deficits of neuromuscular junctions upon sympathectomy and in myasthenic mice were rescued by sympathicomimetic treatment. In conclusion, this study identifies the neuromuscular junction as a target of the sympathetic nervous system and shows that sympathetic input is crucial for synapse maintenance and function. PMID:26733679

  5. Design and evaluation of artificial receptors for the reversal of neuromuscular block.

    PubMed

    Sohajda, Tamás; Fábián, Ákos; Tuza, Kata; Malanga, Milo; Benkovics, Gábor; Fülesdi, Béla; Tassonyi, Edömér; Szente, Lajos

    2017-03-30

    Applying patient friendly and cost-efficient medications in healthcare will be a real challenge in the 21st century. Sugammadex is a selective, yet expensive agent used for the post-surgical reversal of neuromuscular block since 2008. A wide library of cyclodextrin-based follow-ups, having potentially similar affinity towards target aminosteroid type neuromuscular blocking agents has been established. Almost 20 compounds were assessed with respect to in vitro affinity against three commonly applied drugs. Based on the capillary electrophoretic screening, carboxymethylated and sulfobutylated gamma-cyclodextrin derivatives have the potential to be promising lead molecules for their affinity towards pipecuronium was identical or even superior to Sugammadex. Carboxymethylated gamma-cyclodextrin showed efficient and complete reversal of the pipecuronium induced neuromuscular block in an ex vivo rat diaphragm experiment.

  6. Neuromuscular electrical stimulation in critically ill patients in the intensive care unit: a systematic review

    PubMed Central

    Ferreira, Lucas Lima; Vanderlei, Luiz Carlos Marques; Valenti, Vitor Engrácia

    2014-01-01

    Objective To analyze the outcomes enabled by the neuromuscular electric stimulation in critically ill patients in intensive care unit assisted. Methods A systematic review of the literature by means of clinical trials published between 2002 and 2012 in the databases LILACS, SciELO, MEDLINE and PEDro using the descriptors “intensive care unit”, “physical therapy”, “physiotherapy”, “electric stimulation” and “randomized controlled trials”. Results We included four trials. The sample size varied between 8 to 33 individuals of both genders, with ages ranging between 52 and 79 years, undergoing invasive mechanical ventilation. Of the articles analyzed, three showed significant benefits of neuromuscular electrical stimulation in critically ill patients, such as improvement in peripheral muscle strength, exercise capacity, functionality, or loss of thickness of the muscle layer. Conclusion The application of neuromuscular electrical stimulation promotes a beneficial response in critically patients in intensive care. PMID:25295458

  7. Femoral quadriceps neuromuscular electrical stimulation after total knee arthroplasty: a systematic review

    PubMed Central

    Volpato, Helena Bruna Bettoni; Szego, Paulo; Lenza, Mario; Milan, Silvia Lefone; Talerman, Claudia; Ferretti, Mario

    2016-01-01

    ABSTRACT The purpose of this study was to evaluate the effects of neuromuscular electrical stimulation in patients submitted to total knee arthroplasty. This was a systematic review with no language or publication status restriction. Our search was made in Cochrane Library, MEDLINE, Embase and LILACS. Randomized or quasi-randomized clinical trials evaluating neuromuscular electrical stimulation after total knee arthroplasty were included. Four studies with moderate risk of bias and low statistical power were included, totalizing 376 participants. There was no statistically significant difference in knee function, pain and range of motion during 12 month follow-up. This review concluded that neuromuscular electrical stimulation was less effective than traditional rehabilitation in function, muscular strength and range of motion. However, this technique was useful for quadriceps activation during the first days after surgery. PMID:26537511

  8. Neuromuscular scoliosis as a sequelae of Guillain-Barré syndrome.

    PubMed

    Edwards, Max R; Panteliadis, Pavlos; Lucas, Jonathan D

    2010-01-01

    The neuromuscular sequaelae of Guillain-Barré syndrome are well documented in the literature. Persistent distal muscular weakness and loss of peripheral limb reflexes are common in those affected. We report a case of a 14-year-old boy who developed the Miller-Fisher variant of Guillain-Barré syndrome at the age of 8 years. Six years after the acute episode, he had persistent lower limb areflexia and mild weakness. He had also developed a neuromuscular scoliosis. The scoliosis was successfully treated with posterior instrumentation and fusion surgery. Neuromuscular scoliosis is rare following Guillain-Barré syndrome, with no previous reports associated with the Miller-Fisher variant that we are aware of. When evaluating patients post Guillain-Barré syndrome, structural spinal examination is essential to identify rare deformity that may need surgical correction.

  9. Neuromuscular electrical stimulation in critically ill patients in the intensive care unit: a systematic review.

    PubMed

    Ferreira, Lucas Lima; Vanderlei, Luiz Carlos Marques; Valenti, Vitor Engrácia

    2014-09-01

    To analyze the outcomes enabled by the neuromuscular electric stimulation in critically ill patients in intensive care unit assisted. A systematic review of the literature by means of clinical trials published between 2002 and 2012 in the databases LILACS, SciELO, MEDLINE and PEDro using the descriptors "intensive care unit", "physical therapy", "physiotherapy", "electric stimulation" and "randomized controlled trials". We included four trials. The sample size varied between 8 to 33 individuals of both genders, with ages ranging between 52 and 79 years, undergoing invasive mechanical ventilation. Of the articles analyzed, three showed significant benefits of neuromuscular electrical stimulation in critically ill patients, such as improvement in peripheral muscle strength, exercise capacity, functionality, or loss of thickness of the muscle layer. The application of neuromuscular electrical stimulation promotes a beneficial response in critically patients in intensive care.

  10. The effects of neuromuscular training on knee joint motor control during sidecutting in female elite soccer and handball players.

    PubMed

    Zebis, Mette K; Bencke, Jesper; Andersen, Lars L; Døssing, Simon; Alkjaer, Tine; Magnusson, S Peter; Kjaer, Michael; Aagaard, Per

    2008-07-01

    The project aimed to implement neuromuscular training during a full soccer and handball league season and to experimentally analyze the neuromuscular adaptation mechanisms elicited by this training during a standardized sidecutting maneuver known to be associated with non-contact anterior cruciate ligament (ACL) injury. The players were tested before and after 1 season without implementation of the prophylactic training and subsequently before and after a full season with the implementation of prophylactic training. A total of 12 female elite soccer players and 8 female elite team handball players aged 26 +/- 3 years at the start of the study. The subjects participated in a specific neuromuscular training program previously shown to reduce non-contact ACL injury. Neuromuscular activity at the knee joint, joint angles at the hip and knee, and ground reaction forces were recorded during a sidecutting maneuver. Neuromuscular activity in the prelanding phase was obtained 10 and 50 ms before foot strike on a force plate and at 10 and 50 ms after foot strike on a force plate. Neuromuscular training markedly increased before activity and landing activity electromyography (EMG) of the semitendinosus (P < 0.05), while quadriceps EMG activity remained unchanged. Neuromuscular training increased EMG activity for the medial hamstring muscles, thereby decreasing the risk of dynamic valgus. This observed neuromuscular adaptation during sidecutting could potentially reduce the risk for non-contact ACL injury.

  11. The role of neuromuscular changes in aging and knee osteoarthritis on dynamic postural control.

    PubMed

    Takacs, Judit; Carpenter, Mark G; Garland, S Jayne; Hunt, Michael A

    2013-04-01

    Knee osteoarthritis (OA) is a chronic joint condition, with 30% of those over the age of 75 exhibiting severe radiographic disease. Nearly 50% of those with knee OA have experienced a fall in the past year. Falls are a considerable public health concern, with a high risk of serious injury and a significant socioeconomic impact. The ability to defend against a fall relies on adequate dynamic postural control, and alterations in dynamic postural control are seen with normal aging. Neuromuscular changes associated with aging may be responsible for some of these alterations in dynamic postural control. Even greater neuromuscular deficits, which may impact dynamic postural control and the ability to defend against a fall, are seen in people with knee OA. There is little evidence to date on how knee OA affects the ability to respond to and defend against falls and the neuromuscular changes that contribute to balance deficits. As a result, this review will: summarize the key characteristics of postural responses to an external perturbation, highlight the changes in dynamic postural control seen with normal aging, review the neuromuscular changes associated with aging that have known and possible effects on dynamic postural control, and summarize the neuromuscular changes and balance problems in knee OA. Future research to better understand the role of neuromuscular changes in knee OA and their effect on dynamic postural control will be suggested. Such an understanding is critical to the successful creation and implementation of fall prevention and treatment programs, in order to reduce the excessive risk of falling in knee OA.

  12. A preferential delivery method to investigate direct neuromuscular blockade effect of inhaled anesthetics on skeletal muscle.

    PubMed

    Ye, Ling; Yang, Pingliang

    2017-04-01

    Inhaled anesthetics can enhance the effect of neuromuscular blocker, but whether inhaled anesthetics such as sevoflurane have a direct effect on skeletal muscle contractility is unknown. Selectively blocking skeletal muscle may prevent the interference effect of central nervous system. So we decided to evaluate a local application of neuromuscular blocker (NMB) atracurium to prevent the general effect on skeletal muscle. In part 1, sevoflurane (a inhaled anesthetic) minimum alveolar concentrations (MAC) of 1.0, 1.5 and 2.0 would be applied in succession. Neuromuscular function was assessed at each MAC. In part 2, patients are randomized into four groups: group1 (propofol+NMB, sevoflurane 0 MAC), and groups 2 to 4 (NMB+sevoflurane 1.0, 1.5 and 2.0 MAC respectively). In group 1, patients were anesthetized by propofol, then 0.01mg/kg atracurium was injected into the tested arm intravenously after the arterial blood flow was blocked using a tourniquet. For the other 3 groups, patients inhaled 1.0 MAC, 1.5 MAC, or 2.0 MAC of sevoflurane. Then 0.01mg/kg atracurium was injected. Neuromuscular function was recorded for the 4 groups. Neuromuscular function was assessed by acceleromyography measurement of evoked responses to train-of four (TOF) stimuli (2Hz for 2s applied every 12s) at the adductor pollicis using a TOF-Guard™ neuromuscular transmission monitor. If proven, our hypothesis would demonstrate the inhaled anesthetics have no direct effect on contractility but only by increasing the skeletal muscle sensitivity to NMB.

  13. The Role of Neuromuscular Changes in Aging and Knee Osteoarthritis on Dynamic Postural Control

    PubMed Central

    Takacs, Judit; Carpenter, Mark G.; Garland, S. Jayne; Hunt, Michael A.

    2013-01-01

    Knee osteoarthritis (OA) is a chronic joint condition, with 30% of those over the age of 75 exhibiting severe radiographic disease. Nearly 50% of those with knee OA have experienced a fall in the past year. Falls are a considerable public health concern, with a high risk of serious injury and a significant socioeconomic impact. The ability to defend against a fall relies on adequate dynamic postural control, and alterations in dynamic postural control are seen with normal aging. Neuromuscular changes associated with aging may be responsible for some of these alterations in dynamic postural control. Even greater neuromuscular deficits, which may impact dynamic postural control and the ability to defend against a fall, are seen in people with knee OA. There is little evidence to date on how knee OA affects the ability to respond to and defend against falls and the neuromuscular changes that contribute to balance deficits. As a result, this review will: summarize the key characteristics of postural responses to an external perturbation, highlight the changes in dynamic postural control seen with normal aging, review the neuromuscular changes associated with aging that have known and possible effects on dynamic postural control, and summarize the neuromuscular changes and balance problems in knee OA. Future research to better understand the role of neuromuscular changes in knee OA and their effect on dynamic postural control will be suggested. Such an understanding is critical to the successful creation and implementation of fall prevention and treatment programs, in order to reduce the excessive risk of falling in knee OA. PMID:23696951

  14. Neuromuscular and psychomotor abnormalities in patients with schizophrenia and their first-degree relatives.

    PubMed

    Flyckt, L; Wiesel, F A; Borg, J; Edman, G; Ansved, T; Sydow, O; Borg, K

    2000-01-01

    In previous studies of schizophrenic patients, neuromuscular (histopathological and electrophysiological) and psychomotor (finger tapping) abnormalities were found. The present study was designed to investigate relationships between these abnormalities and a family history of psychosis in 14 schizophrenic patients and 25 unaffected first-degree relatives compared to 14 healthy controls. Muscle biopsies were performed in either m. tibialis anterior or m. lateralis. Macro EMG recordings were made from m. tibialis anterior. A finger tapping test was used to investigate psychomotor performance. Neuromuscular abnormalities (muscle biopsies and/or macro EMG) and/or aberrant psychomotor performance (finger tapping test) were found in 13 (93%) patients, 14 (56%) first-degree relatives and in three (21%) controls. A statistically significant relationship for the psychomotor, but not neuromuscular changes to a family history of psychosis was found using a logistic regression method. The percentage of patients, relatives and healthy controls exhibiting were 36/40/7% in the muscle biopsy, 50/20/0% in the macro EMG, and 71/82/14% in the finger tapping investigations. A higher frequency of neuromuscular and psychomotor abnormalities was found in patients with schizophrenia and their first-degree relatives compared to healthy controls. The relationship between psychomotor findings and a family history of psychosis indicate that central aspects of motor aberrations are associated with a hereditary disposition of psychosis. The neuromuscular as well as psychomotor changes indicate that schizophrenia may be a systemic disease involving the central nervous system as well as peripheral organs. An altered cell membrane is suggested to be an underlying factor based on the type of neuromuscular findings.

  15. Neuromuscular adaptations to sprint interval training and the effect of mammalian omega-3 fatty acid supplementation.

    PubMed

    Lewis, Evan J H; Stucky, Frédéric; Radonic, Peter W; Metherel, Adam H; Wolever, Thomas M S; Wells, Greg D

    2017-03-01

    Sprint interval training (SIT) stimulates rapid metabolic adaptations within skeletal muscle but the nature of neuromuscular adaptions is unknown. Omega-3 polyunsaturated fatty acids (N-3 PUFA) are suggested to enhance neuromuscular adaptations to exercise. We measured the neuromuscular adaptations to SIT (Study-1) and conducted a placebo-controlled randomized double blinded study to determine the effect of N-3 PUFA supplementation on neuromuscular adaptations to SIT (Study-2). In Study-1, seven active men (24.4 ± 2.6 years, VO2 peak 43.8 ± 8.7 ml kg min(-1)) completed 2-weeks of SIT with pre- and post-training 10 km cycling time trials (TT). In Study-2, 30 active men (24.5 ± 4.2 years, VO2 peak 41.0 ± 5.1 ml kg min(-1)) were randomly assigned to receive N-3 PUFA (2330 mg day(-1)) (n = 14) or olive oil (n = 16) during 2-weeks of SIT with pre- and post-training TTs. Four week post-training, a SIT session and TT were also performed. Change in neuromuscular function was assessed from resting twitches, quadriceps maximal voluntary contraction (MVC) force, and potentiated twitch force (Q tw). Study-1 showed that SIT did not elicit significant neuromuscular adaptations. Study-2 showed that N-3 PUFA supplementation had no significant effect on neuromuscular adaptations. Training caused lower MVC force [mean ± SD; N-3 PUFA -9 ± 11%, placebo -9 ± 13% (p < 0.05 time)] and Q tw peripheral fatigue [N-3 PUFA -10 ± 19%, placebo -14 ± 13% (p < 0.05 time)]. TT time was lower after training in all groups [Study-1 -10%, Study-2 N-3 PUFA -8%, placebo -12% (p < 0.05 time)]. Two weeks of SIT improved TT performance in the absence of measurable neuromuscular adaptations. N-3 PUFA supplementation had no significant effect on SIT training adaptations.

  16. Prolonged neuromuscular block in a preeclamptic patient induced by magnesium sulfate

    PubMed Central

    Berdai, Mohamed Adnane; Labib, Smael; Harandou, Mustapha

    2016-01-01

    Recent large use of magnesium in the obstetric population should incite anesthesiologists to control its side effects and drugs interactions. We report a case of a 30-year-old woman, with severe preeclampsia and HELLP syndrome, receiving sulfate magnesium and nicardipine, who underwent a cesarean section under general anesthesia. She developed a prolonged and deep neuromuscular blockade, which was antagonized three hours later with neostigmine. In case of therapeutic hypermagnesaemia, non-depolarizing relaxants must be used in reduced doses, and at increased time intervals, with appropriate neuromuscular monitoring. PMID:28154698

  17. Prolonged neuromuscular block in a preeclamptic patient induced by magnesium sulfate.

    PubMed

    Berdai, Mohamed Adnane; Labib, Smael; Harandou, Mustapha

    2016-01-01

    Recent large use of magnesium in the obstetric population should incite anesthesiologists to control its side effects and drugs interactions. We report a case of a 30-year-old woman, with severe preeclampsia and HELLP syndrome, receiving sulfate magnesium and nicardipine, who underwent a cesarean section under general anesthesia. She developed a prolonged and deep neuromuscular blockade, which was antagonized three hours later with neostigmine. In case of therapeutic hypermagnesaemia, non-depolarizing relaxants must be used in reduced doses, and at increased time intervals, with appropriate neuromuscular monitoring.

  18. Using Surface Electromyography To Assess Sex Differences in Neuromuscular Response Characteristics

    PubMed Central

    Shultz, Sandra J.; Perrin, David H.

    1999-01-01

    Objective: To provide an overview of the continuum of muscular responses that typically occur with joint perturbation. The applications and limitations of surface electromyography (sEMG) in evaluating these responses are also addressed. Research applications assessing sex differences in these neuromuscular response characteristics are discussed along with suggestions for future research. Data Sources: MEDLINE was searched from 1969 through 1998. Sport DISCUS was searched from 1975 through 1998. Terms searched included “anterior cruciate ligament,” “epidemiology,” “neuromuscular control,” “neuromuscular performance,” “electromyography,” “latency,” “reflex,” “electromechanical delay,” “dynamic stability,” “intrinsic stiffness,” “short-range stiffness,” “muscle,” “mechanoreceptors,” and “reaction time.” Data Synthesis: It is widely accepted that efficient neuromuscular control is essential to dynamic joint stability and protection. Many studies have established the significant role of the muscles, and particularly the hamstrings, in providing knee stability. By observing the timing, phasing, and recruitment of reflexive muscular activation after a loading stress to the knee, we can better understand the coordinative mechanisms necessary to protect the joint and prevent ligament injury. A number of research models have employed the use of sEMG to evaluate neuromuscular responses at the knee after joint loading or perturbation. However, very few studies have specifically addressed potential sex differences in these response characteristics. Conclusions/Recommendations: From the limited research available, it appears that a sex difference may exist in some aspects of neuromuscular responses. However, further research is needed to explore these differences at the knee and their potential role as predisposing factors to the higher incidence of anterior cruciate ligament injuries in females. Future studies should

  19. Feeding and Swallowing Disorders in Pediatric Neuromuscular Diseases: An Overview

    PubMed Central

    van den Engel-Hoek, Lenie; de Groot, Imelda J.M.; de Swart, Bert J.M.; Erasmus, Corrie E.

    2015-01-01

    Feeding and swallowing problems in infants and children have a great impact on health and wellbeing. The aim of this study was to provide an overview of recognized feeding and swallowing problems in different groups of children with neuromuscular diseases, based on relevant literature and expert opinion, and to propose recommendations for the assessment and treatment of these problems. Almost all pediatric neuromuscular diseases are accompanied by feeding and swallowing problems during the different phases of deglutition, problems that give rise to a wide variety of signs and symptoms, which emphasizes the importance of a comprehensive feeding and swallowing assessment by a speech and language therapist. PMID:27858755

  20. [Rocuronium and sugammadex use for the management of neuromuscular blockade in urgent abdominal surgery in a patient with Landouzy-Dejerine myopathy].

    PubMed

    Hélaine, L; Le Cocq, C; Saadi, H; Abdelkrim, N; Atti, A

    2014-11-01

    In patients with neuromuscular diseases, the use of rocuronium in the general anesthesia rapid sequence induction provides safety intubation conditions, but induces a deep and prolonged neuromuscular blockade. We report dose reduction to 0.8mg/kg for a 47-year-old female with Landouzy-Dejerine myopathy. Therefore, less dose of sugammadex was given to reverse the neuromuscular block.

  1. Neuromuscular function during knee extension exercise after cold water immersion.

    PubMed

    Wakabayashi, Hitoshi; Wijayanto, Titis; Tochihara, Yutaka

    2017-06-23

    Human adaptability to cold environment has been focused on in the physiological anthropology and related research area. Concerning the human acclimatization process in the natural climate, it is necessary to conduct a research assessing comprehensive effect of cold environment and physical activities in cold. This study investigated the effect of cold water immersion on the exercise performance and neuromuscular function during maximal and submaximal isometric knee extension. Nine healthy males participated in this study. They performed maximal and submaximal (20, 40, and 60% maximal load) isometric knee extension pre- and post-immersion in 23, 26, and 34 °C water. The muscle activity of the rectus femoris (RF) and vastus lateralis (VL) was measured using surface electromyography (EMG). The percentages of the maximum voluntary contraction (%MVC) and mean power frequency (MPF) of EMG data were analyzed. The post-immersion maximal force was significantly lower in 23 °C than in 26 and 34 °C conditions (P < 0.05). The post-immersion %MVC of RF was significantly higher than pre-immersion during 60% maximal exercise in 23 and 26 °C conditions (P < 0.05). In the VL, the post-immersion %MVC was significantly higher than pre-immersion in 23 and 26 °C conditions during 20% maximal exercise and in 26 °C at 40 and 60% maximal intensities (P < 0.05). The post-immersion %MVC of VL was significantly higher in 26 °C than in 34 °C at 20 and 60% maximal load (P < 0.05). The post-immersion MPF of RF during 20% maximal intensity was significantly lower in 23 °C than in 26 and 34 °C conditions (P < 0.05), and significantly different between three water temperature conditions at 40 and 60% maximal intensities (P < 0.05). The post-immersion MPF of VL during three submaximal trials were significantly lower in 23 and 26 °C than in 34 °C conditions (P < 0.05). The lower shift of EMG frequency would be connected with the decrease in the

  2. On facilitation of transmitter release at the toad neuromuscular junction

    PubMed Central

    Balnave, Ron J.; Gage, Peter W.

    1974-01-01

    1. The time dependence of the increase in amplitude (facilitation) of a second end-plate potential (e.p.p.) elicited within 10-100 msec of a preceding e.p.p. was examined at neuromuscular junctions in sartorius muscles of toads. Facilitation was defined by two characteristics, initial facilitation and the time constant of its exponential decay. 2. The time constant of decay of facilitation was longer at lower temperatures and the Q10 was 4·3 in the range 10-25° C. There was no significant effect of temperature on initial facilitation. 3. Ouabain (10-4-10-3 M), lithium substitution for sodium, sodium azide (5 mM) and N-ethylmaleimide (NEM, 0·1 mM) initially had no effect on initial facilitation or the decay of facilitation. After some time, they all caused a longer time constant of decay of facilitation and a depression of initial facilitation. 4. It was concluded that the decay of facilitation is not directly dependent on active transport of sodium ions, calcium efflux, ATP-dependent movements of calcium or mitochondrial uptake of calcium following an action potential. 5. Ouabain, lithium, sodium azide, and NEM all caused an increase in transmitter release. This effect, and the late effects on facilitation, were thought to be due to an increase in intracellular calcium concentration in nerve terminals. 6. No relationship was found between the quantal content of e.p.p.s (range, 0·8-100) and initial facilitation, or the time constant of decay of facilitation. 7. Substitution of strontium for calcium ions caused a marked prolongation of the time constant of decay of facilitation, and a depression of initial facilitation. 8. The results were consistent with the hypothesis that the time constant of decay of facilitation is related to the rate of disappearance of an `active' complex of calcium (CaA) which, of itself, is not sufficient for transmitter release. It is suggested that an action potential produces CaA which decays with the time constant of facilitation and

  3. Genetic and evolutionary analysis of the Drosophila larval neuromuscular junction

    NASA Astrophysics Data System (ADS)

    Campbell, Megan

    Although evolution of brains and behaviors is of fundamental biological importance, we lack comprehensive understanding of the general principles governing these processes or the specific mechanisms and molecules through which the evolutionary changes are effected. Because synapses are the basic structural and functional units of nervous systems, one way to address these problems is to dissect the genetic and molecular pathways responsible for morphological evolution of a defined synapse. I have undertaken such an analysis by examining morphology of the larval neuromuscular junction (NMJ) in wild caught D. melanogaster as well as in over 20 other species of Drosophila. Whereas variation in NMJ morphology within a species is limited, I discovered a surprisingly extensive variation among different species. Compared with evolution of other morphological traits, NMJ morphology appears to be evolving very rapidly. Moreover, my data indicate that natural selection rather than genetic drift is primarily responsible for evolution of NMJ morphology. To dissect underlying molecular mechanisms that may govern NMJ growth and evolutionary divergence, I focused on a naturally occurring variant in D. melanogaster that causes NMJ overgrowth. I discovered that the variant mapped to Mob2, a gene encoding a kinase adapter protein originally described in yeast as a member of the Mitotic Exit Network (MEN). I have subsequently examined mutations in the Drosophila orthologs of all the core components of the yeast MEN and found that all of them function as part of a common pathway that acts presynaptically to negatively regulate NMJ growth. As in the regulation of yeast cytokinesis, these components of the MEN appear to act ultimately by regulating actin dynamics during the process of bouton growth and division. These studies have thus led to the discovery of an entirely new role for the MEN---regulation of synaptic growth---that is separate from its function in cell division. This work

  4. Neuromuscular responses to incremental caffeine doses: performance and side effects.

    PubMed

    Pallarés, Jesús G; Fernández-Elías, Valentín E; Ortega, Juan F; Muñoz, Gloria; Muñoz-Guerra, Jesús; Mora-Rodríguez, Ricardo

    2013-11-01

    The purpose of this study was to determine the oral dose of caffeine needed to increase muscle force and power output during all-out single multijoint movements. Thirteen resistance-trained men underwent a battery of muscle strength and power tests in a randomized, double-blind, crossover design, under four different conditions: (a) placebo ingestion (PLAC) or with caffeine ingestion at doses of (b) 3 mg · kg(-1) body weight (CAFF 3mg), (c) 6 mg · kg(-1) (CAFF 6mg), and (d) 9 mg · kg(-1) (CAFF 9mg). The muscle strength and power tests consisted in the measurement of bar displacement velocity and muscle power output during free-weight full-squat (SQ) and bench press (BP) exercises against four incremental loads (25%, 50%, 75%, and 90% one-repetition maximum [1RM]). Cycling peak power output was measured using a 4-s inertial load test. Caffeine side effects were evaluated at the end of each trial and 24 h later. Mean propulsive velocity at light loads (25%-50% 1RM) increased significantly above PLAC for all caffeine doses (5.4%-8.5%, P = 0.039-0.003). At the medium load (75% 1RM), CAFF 3mg did not improve SQ or BP muscle power or BP velocity. CAFF 9mg was needed to enhance BP velocity and SQ power at the heaviest load (90% 1RM) and cycling peak power output (6.8%-11.7%, P = 0.03-0.05). The CAFF 9mg trial drastically increased the frequency of the adverse side effects (15%-62%). The ergogenic dose of caffeine required to enhance neuromuscular performance during a single all-out contraction depends on the magnitude of load used. A dose of 3 mg · kg(-1) is enough to improve high-velocity muscle actions against low loads, whereas a higher caffeine dose (9 mg · kg(-1)) is necessary against high loads, despite the appearance of adverse side effects.

  5. Deep neuromuscular blockade and low insufflation pressure during laparoscopic hysterectomy.

    PubMed

    Madsen, Matias Vested; Istre, Olav; Springborg, Henrik Halvor; Staehr-Rye, Anne Kathrine; Rosenberg, Jacob; Lund, Jørgen; Gätke, Mona Ring

    2017-05-01

    Establishment of sufficient muscle relaxation is essential in laparoscopic surgery. During laparoscopy, surgeons can experience abdominal contractions in their patients. Deep neuromuscular block (NMB) has the potential to prevent such episodes. In this study, we explored if deep NMB reduces the incidence of sudden abdominal contractions as compared with standard NMB. This was a pre-planned secondary analysis of a randomized, controlled study. A total of 110 patients scheduled for laparoscopic hysterectomy were randomized to either deep NMB and 8 mmHg pneumoperitoneum (deep NMB group) or single-bolus NMB and 12 mmHg pneumoperitoneum (standard NMB group). NMB was established with rocuronium and reversed with sugammadex. Two gynaecologists registered episodes of sudden abdominal contractions, alarms from the insufflator due to increased intraabdominal pressure and incidences with tightness of the abdominal wall. No sudden abdominal contractions were detected in the deep NMB group as compared with 12 episodes in the standard NMB group (p < 0.001). The insufflator alarmed in no versus ten procedures (p = 0.001) in the deep and standard NMB group, respectively. The gynaecologists registered increasing abdominal tensions in no versus eight procedures (p = 0.006) in the deep and standard NMB group, respectively. Deep NMB in combination with 8 mmHg pneumoperitoneum prevented sudden abdominal contractions during laparoscopic hysterectomy. This work was funded in part by a research grant from the Investigator Initiated Studies Program of Merck Sharp & Dohme Corp, USA. The opinions expressed in this paper are those of the authors and do not necessarily represent those of Merck Sharp & Dohme Corp. The study was assigned with EudraCT number 2012-003787-51 and registered with clinicaltrials.gov (NCT01722097). Articles published in the DMJ are “open access”. This means that the articles are distributed under the terms of the Creative Commons Attribution Non-commercial License

  6. Neuromuscular Electrical Stimulation for Mobility Support of Elderly

    PubMed Central

    2015-01-01

    The stimulator for neuromuscular electrical stimulation for mobility support of elderly is not very complicated, but for application within “MOBIL” we have some additional demands to fulfill. First we have specific safety issues for this user group. A powerful compliance management system is crucial not only to guide daily application, but for creating hard data for the scientific outcome. We also need to assure easy handling of the stimulator, because the subjects are generally not able to cope with too difficult and complex motor skills. So, we developed five generations of stimulators and optimizing solutions after field tests. We are already planning the sixth generation with wireless control of the stimulation units by the central main handheld control unit. In a prototype, we have implemented a newly available high capacity memory, a breakthrough in “compliance data storage” as they offer the necessary high storage capacity and fast data handling for an affordable prize. The circuit also contains a 3D accelerometer sensor which acts as a further important safety features: if the control unit drops, this event is detected automatically by the sensor and activates an emergency switch-off that disables the stimulation to avoid associated risks. Further, we have implemented a hardware emergence shutdown and other safety measures. Finally, in the last example muscle torque measurements are referenced with compliance data. In the study normalized maximum voluntary contraction (MVC) and maximum stimulation induced contraction (MSC) were assessed in regular check-ups along the training period. With additional consideration of adjusted stimulation intensity for training out of the compliance data records we are able to estimate the induced contraction strength, which turned out to amount in average 11% of MVC. This value may seem on a first sight rather low, and ought to be considered in relation to the results at the end of the training period. Therefore the

  7. Qualitative Neuromuscular Monitoring: How to Optimize the Use of a Peripheral Nerve Stimulator to Reduce the Risk of Residual Neuromuscular Blockade.

    PubMed

    Thilen, Stephan R; Bhananker, Sanjay M

    This review provides recommendations for anesthesia providers who may not yet have quantitative monitoring and sugammadex available and thus are providing care within the limitations of a conventional peripheral nerve stimulator (PNS) and neostigmine. In order to achieve best results, the provider needs to understand the limitations of the PNS. The PNS should be applied properly and early. All overdosing of neuromuscular blocking drugs should be avoided and the intraoperative neuromuscular blockade should be maintained only as deep as necessary. The adductor pollicis is the gold standard site and must be used for the pre-reversal assessment, also when the ulnar nerve and thumb were not accessible intraoperatively. Spontaneous recovery should be maximized and neostigmine should be administered after a TOF count of 4 has been confirmed at the adductor pollicis. Extubation should not occur within 10 min after administration of an appropriate dose of neostigmine.

  8. Critical components of neuromuscular training to reduce ACL injury risk in female athletes: meta-regression analysis.

    PubMed

    Sugimoto, Dai; Myer, Gregory D; Barber Foss, Kim D; Pepin, Michael J; Micheli, Lyle J; Hewett, Timothy E

    2016-10-01

    The aim of this study was to determine key components in neuromuscular training that optimise ACL injury reduction in female athletes using meta-regression analyses. Systematic review and meta-regression. The literature search was performed in PubMed and EBSCO. Inclusion criteria for the current analysis were: (1) documented the number of ACL injuries, (2) employed a neuromuscular training intervention that aimed to reduce ACL injuries, (3) had a comparison group, (4) used a prospective control study design and (5) recruited female athletes as participants. Two independent reviewers extracted studies which met the inclusion criteria. Methodological quality of included study and strength of recommendation were evaluated. Number of ACL injuries and participants in control and intervention groups, age of participants, dosage of neuromuscular training, exercise variations within neuromuscular training and status of verbal feedback were extracted. The meta-regression analyses identified age of participants, dosage of neuromuscular training, exercise variations within neuromuscular training and utilisation of verbal feedback as significant predictors of ACL injury reduction (p=0.01 in fixed-effects model, p=0.03 in random-effects model). Inclusion of 1 of the 4 components in neuromuscular training could reduce ACL injury risk by 17.2-17.7% in female athletes. No significant heterogeneity and publication bias effects were detected. Strength of recommendation was rated as A (recommendation based on consistent and good-quality patient-oriented study evidence). Age of participants, dosage of neuromuscular training, exercise variations within neuromuscular training and utilisation of verbal feedback are predictors that influence the optimisation of prophylactic effects of neuromuscular training and the resultant ACL injury reduction in female athletes. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to

  9. Critical components of neuromuscular training to reduce ACL injury risk in female athletes: meta-regression analysis

    PubMed Central

    Sugimoto, Dai; Myer, Gregory D; Barber Foss, Kim D; Pepin, Michael J; Micheli, Lyle J; Hewett, Timothy E

    2017-01-01

    Objective The aim of this study was to determine key components in neuromuscular training that optimise ACL injury reduction in female athletes using meta-regression analyses. Design Systematic review and meta-regression. Data sources The literature search was performed in PubMed and EBSCO. Eligibility criteria Inclusion criteria for the current analysis were: (1) documented the number of ACL injuries, (2) employed a neuromuscular training intervention that aimed to reduce ACL injuries, (3) had a comparison group, (4) used a prospective control study design and (5) recruited female athletes as participants. Two independent reviewers extracted studies which met the inclusion criteria. Methodological quality of included study and strength of recommendation were evaluated. Number of ACL injuries and participants in control and intervention groups, age of participants, dosage of neuromuscular training, exercise variations within neuromuscular training and status of verbal feedback were extracted. Results The meta-regression analyses identified age of participants, dosage of neuromuscular training, exercise variations within neuromuscular training and utilisation of verbal feedback as significant predictors of ACL injury reduction (p=0.01 in fixed-effects model, p=0.03 in random-effects model). Inclusion of 1 of the 4 components in neuromuscular training could reduce ACL injury risk by 17.2–17.7% in female athletes. No significant heterogeneity and publication bias effects were detected. Strength of recommendation was rated as A (recommendation based on consistent and good-quality patient-oriented study evidence). Conclusions Age of participants, dosage of neuromuscular training, exercise variations within neuromuscular training and utilisation of verbal feedback are predictors that influence the optimisation of prophylactic effects of neuromuscular training and the resultant ACL injury reduction in female athletes. PMID:27251898

  10. Neuromuscular adaptations to training, injury and passive interventions: implications for running economy.

    PubMed

    Bonacci, Jason; Chapman, Andrew; Blanch, Peter; Vicenzino, Bill

    2009-01-01

    Performance in endurance sports such as running, cycling and triathlon has long been investigated from a physiological perspective. A strong relationship between running economy and distance running performance is well established in the literature. From this established base, improvements in running economy have traditionally been achieved through endurance training. More recently, research has demonstrated short-term resistance and plyometric training has resulted in enhanced running economy. This improvement in running economy has been hypothesized to be a result of enhanced neuromuscular characteristics such as improved muscle power development and more efficient use of stored elastic energy during running. Changes in indirect measures of neuromuscular control (i.e. stance phase contact times, maximal forward jumps) have been used to support this hypothesis. These results suggest that neuromuscular adaptations in response to training (i.e. neuromuscular learning effects) are an important contributor to enhancements in running economy. However, there is no direct evidence to suggest that these adaptations translate into more efficient muscle recruitment patterns during running. Optimization of training and run performance may be facilitated through direct investigation of muscle recruitment patterns before and after training interventions. There is emerging evidence that demonstrates neuromuscular adaptations during running and cycling vary with training status. Highly trained runners and cyclists display more refined patterns of muscle recruitment than their novice counterparts. In contrast, interference with motor learning and neuromuscular adaptation may occur as a result of ongoing multidiscipline training (e.g. triathlon). In the sport of triathlon, impairments in running economy are frequently observed after cycling. This impairment is related mainly to physiological stress, but an alteration in lower limb muscle coordination during running after cycling

  11. Electromyographic and Neuromuscular Force Patterns Associated with Unexpectedly Loaded Rapid Limb Movements.

    ERIC Educational Resources Information Center

    Richardson, Charles; Simmons, Roger W.

    Bi-articular, unidirectional arm movements were studied to evaluate the electromyographic (EMG) and neuromuscular force patterns that occur when a limb is unexpectedly perturbed. A series of training trials were continued with a control load spring attached to the apparatus until a pre-specified criterion for learning was attained. The limb was…

  12. Neurophysiological Strategies for the Diagnosis of Disorders of the Neuromuscular Junction in Children

    ERIC Educational Resources Information Center

    Pitt, Matthew

    2008-01-01

    The disorders of the neuromuscular junction seen in children, the congenital myasthenic syndromes and autoimmune myasthenia gravis, are very rare. Their clinical symptoms and signs may be variable, most notably in the neonate and infant. They should enter the differential diagnosis of many different clinical presentations, such as "floppy infant"…

  13. Whole-body vibration does not influence knee joint neuromuscular function or proprioception.

    PubMed

    Hannah, R; Minshull, C; Folland, J P

    2013-02-01

    This study examined the acute effects of whole-body vibration (WBV) on knee joint position sense and indices of neuromuscular function, specifically strength, electromechanical delay and the rate of force development. Electromyography and electrically evoked contractions were used to investigate neural and contractile responses to WBV. Fourteen healthy males completed two treatment conditions on separate occasions: (1) 5 × 1 min of unilateral isometric squat exercise on a synchronous vibrating platform [30 Hz, 4 mm peak-to-peak amplitude] (WBV) and (2) a control condition (CON) of the same exercise without WBV. Knee joint position sense (joint angle replication task) and quadriceps neuromuscular function were assessed pre-, immediately-post and 1 h post-exercise. During maximum voluntary knee extensions, the peak force (PF(V)), electromechanical delay (EMD(V)), rate of force development (RFD(V)) and EMG of the quadriceps were measured. Twitch contractions of the knee extensors were electrically evoked to assess EMD(E) and RFD(E). The results showed no influence of WBV on knee joint position, EMD(V), PF(V) and RFD(V) during the initial 50, 100 or 150 ms of contraction. Similarly, electrically evoked neuromuscular function and neural activation remained unchanged following the vibration exercise. A single session of unilateral WBV did not influence any indices of thigh muscle neuromuscular performance or knee joint proprioception.

  14. Reversal of profound neuromuscular blockade with sugammadex in an infant after bronchial foreign body removal.

    PubMed

    Azizoglu, Mustafa; Birbicer, Handan; Memis, Suleyman; Taşkınlar, Hakan

    2016-09-01

    Sugammadex is a selective chemical agent that can reverse neuromuscular blockade induced by vecuronium and rocuronium. The aim of this report is to discuss the effectiveness of sugammadex in the reversal of neuromuscular blockade in children younger than 2 years. A 16-month-old boy, weighing 10 kg, was admitted to the pediatric emergency department due to choking, cyanosis, and severe respiratory distress that occurred while he was eating peanuts. In the emergency department, the patient's condition deteriorated, and he went into respiratory arrest. He was immediately intubated and taken to the operating room. A rigid bronchoscopy was performed under general anesthesia, with administration of intravenous pentothal (5 mg/kg), rocuronium (0.6 mg/kg), and fentanyl (0.5 μg/kg) in the operating room. The foreign body was removed within 6 minutes, and the profound neuromuscular blockade was reversed with a dose of 2 mg/kg sugammadex. He was extubated successfully after obtaining the spontaneous respiratory activity, and adequate breathing was restored. Clinical use of sugammadex in children younger than 2 years is not recommended because of the lack of clinical studies. In this case report, the profound neuromuscular blockade was successfully reversed with a dose of 2 mg/kg sugammadex in a 16-month-old boy. However, more prospective clinical studies are required for the safe use of this agent in children.

  15. Effect of magnesium sulphate on sugammadex reversal time for neuromuscular blockade: a randomised controlled study.

    PubMed

    Germano Filho, P A; Cavalcanti, I L; Barrucand, L; Verçosa, N

    2015-08-01

    Magnesium potentiates neuromuscular blockade. Sugammadex reverses rocuronium-induced blockade. The aim of this study was to determine the effect of pre-treatment with magnesium sulphate on sugammadex reversal time for neuromuscular blockade. Seventy-three patients were randomly assigned to receive magnesium sulphate (40 mg.kg(-1) ) or saline intravenously. After anaesthetic induction, continuous train-of-four monitoring was performed and rocuronium was administered (0.6 mg.kg(-1) ). When a second twitch appeared, the patients received sugammadex (2 mg.kg(-1) ). The median (IQR [range]) reversal time of moderate neuromuscular blockade to a train-of-four ratio of 0.9 facilitated by sugammadex was 115 (93-177.5 [68-315]) s in the magnesium group and 120 (105-140 [70-298]) s in the saline group (p = 0.79). The median (IQR [range]) clinical duration was 45 (35.5-53 [22-102]) min in the magnesium group and 37 (31-43 [19-73]) min in the saline group (p = 0.031). Pre-treatment with magnesium did not significantly affect sugammadex reversal time of moderate neuromuscular blockade induced by rocuronium.

  16. Deep neuromuscular blockade leads to a larger intraabdominal volume during laparoscopy.

    PubMed

    Lindekaer, Astrid Listov; Halvor Springborg, Henrik; Istre, Olav

    2013-06-25

    Shoulder pain is a commonly reported symptom following laparoscopic procedures such as myomectomy or hysterectomy, and recent studies have shown that lowering the insufflation pressure during surgery may reduce the risk of post-operative pain. In this pilot study, a method is presented for measuring the intra-abdominal space available to the surgeon during laproscopy, in order to examine whether the relaxation produced by deep neuromuscular blockade can increase the working surgical space sufficiently to permit a reduction in the CO2 insufflation pressure. Using the laproscopic grasper, the distance from the promontory to the skin is measured at two different insufflation pressures: 8 mm Hg and 12 mm Hg. After the initial measurements, a neuromuscular blocking agent (rocuronium) is administered to the patient and the intra-abdominal volume is measured again. Pilot data collected from 15 patients shows that the intra-abdominal space at 8 mm Hg with blockade is comparable to the intra-abdominal space measured at 12 mm Hg without blockade. The impact of neuromuscular blockade was not correlated with patient height, weight, BMI, and age. Thus, using neuromuscular blockade to maintain a steady volume while reducing insufflation pressure may produce improved patient outcomes.

  17. Current Methodological Issues in the Study of Children with Inherited Neuromuscular Disorders

    ERIC Educational Resources Information Center

    Mercuri, Eugenio; Messina, Sonia; Pane, Marika; Bertini, Enrico

    2008-01-01

    Several clinical trials assessing children with hereditary neuromuscular disorders have been performed over the last decade. These studies highlighted issues related to design and performance of clinical studies assessing children with this group of disorders. This article reviews recent literature and clinical experience in this area,…

  18. Ultrastructural comparison of the Drosophila larval and adult ventral abdominal neuromuscular junction.

    PubMed

    Wagner, Nicole

    2017-07-01

    Drosophila melanogaster has recently emerged as model system for studying synaptic transmission and plasticity during adulthood, aging and neurodegeneration. However, still little is known about the basic neuronal mechanisms of synaptic function in the adult fly. Per se, adult Drosophila neuromuscular junctions should be highly suited for studying these aspects as they allow for genetic manipulations in combination with ultrastructural and electrophysiological analyses. Although different neuromuscular junctions of the adult fly have been described during the last years, a direct ultrastructural comparison with their larval counterpart is lacking. The present study was designed to close this gap by providing a detailed ultrastructural comparison of the larval and the adult neuromuscular junction of the ventrolongitudinal muscle. Assessment of several parameters revealed similarities but also major differences in the ultrastructural organisation of the two model neuromuscular junctions. While basic morphological parameters are retained from the larval into the adult stage, the analysis discovered major differences of potential functional relevance in the adult: The electron-dense membrane apposition of the presynaptic and postsynaptic membrane is shorter, the subsynaptic reticulum is less elaborated and the number of synaptic vesicles at a certain distance of the presynaptic membrane is higher. © 2017 Wiley Periodicals, Inc.

  19. Neurophysiological Strategies for the Diagnosis of Disorders of the Neuromuscular Junction in Children

    ERIC Educational Resources Information Center

    Pitt, Matthew

    2008-01-01

    The disorders of the neuromuscular junction seen in children, the congenital myasthenic syndromes and autoimmune myasthenia gravis, are very rare. Their clinical symptoms and signs may be variable, most notably in the neonate and infant. They should enter the differential diagnosis of many different clinical presentations, such as "floppy infant"…

  20. Modulation of pain-induced neuromuscular trunk responses by pain expectations: a single group study.

    PubMed

    Tétreau, Charles; Dubois, Jean-Daniel; Piché, Mathieu; Descarreaux, Martin

    2012-10-01

    The purpose of this study was to investigate the alteration of pain-induced neuromuscular trunk responses by expectations in healthy volunteers. Twenty-three asymptomatic participants performed series of flexion-extension movements in 3 different experimental conditions: innocuous heat stimulation (control) and noxious heat stimulation associated with expectations of low or high pain intensity. These stimuli were administered by a contact thermode placed over the lumbar region (L4 and L5) to assess the modulation of neuromuscular responses and kinematics during the flexion-extension task. Surface electromyography (EMG) of lumbar erector spinae at L2 and L3 and L4 and L5 as well as lumbopelvic kinematic variables were compared across conditions. Noxious stimulation significantly altered EMG responses but only in full trunk flexion. Interestingly, this alteration was significant only for muscles where noxious stimulation was applied (L4 and L5) and not for the other segment (L2 and L3). Conversely, expectations significantly altered EMG activity at L2 and L3 but not at the segment where noxious stimulation was applied. These results confirm previous findings and indicate that experimental pain can alter neuromuscular responses during a trunk flexion-extension task. Furthermore, this study suggests that expectations can alter some of these alterations. Future studies should determine whether neuromuscular changes induced by expectations may contribute to the transition from acute to chronic low-back pain. Copyright © 2012 National University of Health Sciences. Published by Mosby, Inc. All rights reserved.

  1. Man-machine interface system for neuromuscular training and evaluation based on EMG and MMG signals.

    PubMed

    de la Rosa, Ramon; Alonso, Alonso; Carrera, Albano; Durán, Ramon; Fernández, Patricia

    2010-01-01

    This paper presents the UVa-NTS (University of Valladolid Neuromuscular Training System), a multifunction and portable Neuromuscular Training System. The UVa-NTS is designed to analyze the voluntary control of severe neuromotor handicapped patients, their interactive response, and their adaptation to neuromuscular interface systems, such as neural prostheses or domotic applications. Thus, it is an excellent tool to evaluate the residual muscle capabilities in the handicapped. The UVa-NTS is composed of a custom signal conditioning front-end and a computer. The front-end electronics is described thoroughly as well as the overall features of the custom software implementation. The software system is composed of a set of graphical training tools and a processing core. The UVa-NTS works with two classes of neuromuscular signals: the classic myoelectric signals (MES) and, as a novelty, the myomechanic signals (MMS). In order to evaluate the performance of the processing core, a complete analysis has been done to classify its efficiency and to check that it fulfils with the real-time constraints. Tests were performed both with healthy and selected impaired subjects. The adaptation was achieved rapidly, applying a predefined protocol for the UVa-NTS set of training tools. Fine voluntary control was demonstrated to be reached with the myoelectric signals. And the UVa-NTS demonstrated to provide a satisfactory voluntary control when applying the myomechanic signals.

  2. Metabolic, cardiorespiratory, and neuromuscular fitness performance in children with cerebral palsy: A comparison with healthy youth

    PubMed Central

    García, Claudia Cardona; Alcocer-Gamboa, Alberto; Ruiz, Margarita Pérez; Caballero, Ignacio Martínez; Faigenbaum, Avery D.; Esteve-Lanao, Jonathan; Saiz, Beatriz Moral; Lorenzo, Teresa Martín; Lara, Sergio Lerma

    2016-01-01

    The aim of this study was to assess metabolic, cardiorespiratory, and neuromuscular fitness parameters in children with spastic cerebral palsy (CP) and to compare these findings with typically developing children. 40 children with CP (21 males, 19 females; mean age, 11.0±3.3 yr; range, 6.5–17.1 yr; Gross Motor Function Classification System levels 1 or 2) and 40 healthy, age- and sex-matched children completed a test battery that consisted of 8 tests and 28 measures that assessed cardio-respiratory fitness, energy expenditure, anaerobic endurance, muscle strength, agility, stability and flexibility. Children with CP had significantly lower performance (P<0.05) on most cardiorespiratory and metabolic tests than those of healthy children, Differences in neuromuscular measures of muscular strength, speed, agility, anaerobic endurance, and flexibility between groups were most apparent. Grouped differences in cardiorespiratory variables revealed a 25% difference in performance, whereas grouped differences in metabolic and neuromuscular measures were 43% and 60%, respectively. The physical fitness of contemporary children with CP is significantly less than healthy, age-matched children. Significant differences in neuromuscular measures between groups can aid in the identification of specific fitness abilities in need of improvement in this population. PMID:27162775

  3. Efficacy of brain-computer interface-driven neuromuscular electrical stimulation for chronic paresis after stroke.

    PubMed

    Mukaino, Masahiko; Ono, Takashi; Shindo, Keiichiro; Fujiwara, Toshiyuki; Ota, Tetsuo; Kimura, Akio; Liu, Meigen; Ushiba, Junichi

    2014-04-01

    Brain computer interface technology is of great interest to researchers as a potential therapeutic measure for people with severe neurological disorders. The aim of this study was to examine the efficacy of brain computer interface, by comparing conventional neuromuscular electrical stimulation and brain computer interface-driven neuromuscular electrical stimulation, using an A-B-A-B withdrawal single-subject design. A 38-year-old male with severe hemiplegia due to a putaminal haemorrhage participated in this study. The design involved 2 epochs. In epoch A, the patient attempted to open his fingers during the application of neuromuscular electrical stimulation, irrespective of his actual brain activity. In epoch B, neuromuscular electrical stimulation was applied only when a significant motor-related cortical potential was observed in the electroencephalogram. The subject initially showed diffuse functional magnetic resonance imaging activation and small electro-encephalogram responses while attempting finger movement. Epoch A was associated with few neurological or clinical signs of improvement. Epoch B, with a brain computer interface, was associated with marked lateralization of electroencephalogram (EEG) and blood oxygenation level dependent responses. Voluntary electromyogram (EMG) activity, with significant EEG-EMG coherence, was also prompted. Clinical improvement in upper-extremity function and muscle tone was observed. These results indicate that self-directed training with a brain computer interface may induce activity- dependent cortical plasticity and promote functional recovery. This preliminary clinical investigation encourages further research using a controlled design.

  4. Evidence-based guideline: neuromuscular ultrasound for the diagnosis of carpal tunnel syndrome.

    PubMed

    Cartwright, Michael S; Hobson-Webb, Lisa D; Boon, Andrea J; Alter, Katharine E; Hunt, Christopher H; Flores, Victor H; Werner, Robert A; Shook, Steven J; Thomas, T Darrell; Primack, Scott J; Walker, Francis O

    2012-08-01

    The purpose of this study was to develop an evidence-based guideline for the use of neuromuscular ultrasound in the diagnosis of carpal tunnel syndrome (CTS). Two questions were asked: (1) What is the accuracy of median nerve cross-sectional area enlargement as measured with ultrasound for the diagnosis of CTS? (2) What added value, if any, does neuromuscular ultrasound provide over electrodiagnostic studies alone for the diagnosis of CTS? A systematic review was performed, and studies were classified according to American Academy of Neurology criteria for rating articles of diagnostic accuracy (question 1) and for screening articles (question 2). Neuromuscular ultrasound measurement of median nerve cross-sectional area at the wrist is accurate and may be offered as a diagnostic test for CTS (Level A). Neuromuscular ultrasound probably adds value to electrodiagnostic studies when diagnosing CTS and should be considered in screening for structural abnormalities at the wrist in those with CTS (Level B). Copyright © 2012 Wiley Periodicals, Inc.

  5. Role of Extracellular Matrix Proteins and Their Receptors in the Development of the Vertebrate Neuromuscular Junction

    PubMed Central

    Singhal, Neha; Martin, Paul T.

    2012-01-01

    The vertebrate neuromuscular junction remains the best-studied model for understanding the mechanisms involved in synaptogenesis, due to its relatively large size, its simplicity of patterning and its unparalleled experimental accessibility. During neuromuscular development, each skeletal myofiber secretes and deposits around its extracellular surface an assemblage of extracellular matrix (ECM) proteins that ultimately form a basal lamina. This is also the case at the neuromuscular junction, where the motor nerve contributes additional factors. Before most of the current molecular components were known, it was clear that the synaptic ECM of adult skeletal muscles was unique in composition and contained factors sufficient to induce the differentiation of both pre- and postsynaptic membranes. Biochemical, genetic and microscopy studies have confirmed that agrin, laminin (221, 421, and 521), collagen IV (α3-α6), collagen XIII, perlecan and the ColQ-bound form of acetylcholinesterase are all synaptic ECM proteins with important roles in neuromuscular development. The roles of their many potential receptors and/or binding proteins has been more difficult to assess at the genetic level due to the complexity of membrane interactions with these large proteins, but roles for MuSK-LRP4 in agrin signaling and for integrins, dystroglycan, and voltage-gated calcium channels in laminin-dependent phenotypes have been identified. Synaptic extracellular matrix proteins and their receptors are involved in almost all aspects of synaptic development, including synaptic initiation, topography, ultrastructure, maturation, stability and transmission. PMID:21766463

  6. Sugammadex as a reversal agent for neuromuscular block: an evidence-based review

    PubMed Central

    Schaller, Stefan Josef; Fink, Heidrun

    2013-01-01

    Sugammadex is the first clinical representative of a new class of drugs called selective relaxant binding agents. It has revolutionized the way anesthesiologists think about drug reversal. Sugammadex selectively binds rocuronium or vecuronium, thereby reversing their neuromuscular blocking action. Due to its 1:1 binding of rocuronium or vecuronium, it is able to reverse any depth of neuromuscular block. So far, it has been approved for use in adult patients and for pediatric patients over 2 years. Since its approval in Europe, Japan, and Australia, further insight on its use in special patient populations and specific diseases have become available. Due to its pharmacodynamic profile, sugammadex, in combination with rocuronium, may have the potential to displace succinylcholine as the “gold standard” muscle relaxant for rapid sequence induction. The use of rocuronium or vecuronium, with the potential of reverse of their action with sugammadex, seems to be safe in patients with impaired neuromuscular transmission, ie, neuromuscular diseases, including myasthenia gravis. Data from long-term use of sugammadex is not yet available. Evidence suggesting an economic advantage of using sugammadex and justifying its relatively high cost for an anesthesia-related drug, is missing. PMID:24098155

  7. Muscle motor point identification is essential for optimizing neuromuscular electrical stimulation use.

    PubMed

    Gobbo, Massimiliano; Maffiuletti, Nicola A; Orizio, Claudio; Minetto, Marco A

    2014-02-25

    Transcutaneous neuromuscular electrical stimulation applied in clinical settings is currently characterized by a wide heterogeneity of stimulation protocols and modalities. Practitioners usually refer to anatomic charts (often provided with the user manuals of commercially available stimulators) for electrode positioning, which may lead to inconsistent outcomes, poor tolerance by the patients, and adverse reactions. Recent evidence has highlighted the crucial importance of stimulating over the muscle motor points to improve the effectiveness of neuromuscular electrical stimulation. Nevertheless, the correct electrophysiological definition of muscle motor point and its practical significance are not always fully comprehended by therapists and researchers in the field. The commentary describes a straightforward and quick electrophysiological procedure for muscle motor point identification. It consists in muscle surface mapping by using a stimulation pen-electrode and it is aimed at identifying the skin area above the muscle where the motor threshold is the lowest for a given electrical input, that is the skin area most responsive to electrical stimulation. After the motor point mapping procedure, a proper placement of the stimulation electrode(s) allows neuromuscular electrical stimulation to maximize the evoked tension, while minimizing the dose of the injected current and the level of discomfort. If routinely applied, we expect this procedure to improve both stimulation effectiveness and patient adherence to the treatment.The aims of this clinical commentary are to present an optimized procedure for the application of neuromuscular electrical stimulation and to highlight the clinical implications related to its use.

  8. Evidence-Based Systematic Review: Effects of Neuromuscular Electrical Stimulation on Swallowing and Neural Activation

    ERIC Educational Resources Information Center

    Clark, Heather; Lazarus, Cathy; Arvedson, Joan; Schooling, Tracy; Frymark, Tobi

    2009-01-01

    Purpose: To systematically review the literature examining the effects of neuromuscular electrical stimulation (NMES) on swallowing and neural activation. The review was conducted as part of a series examining the effects of oral motor exercises (OMEs) on speech, swallowing, and neural activation. Method: A systematic search was conducted to…

  9. Evidence-Based Systematic Review: Effects of Neuromuscular Electrical Stimulation on Swallowing and Neural Activation

    ERIC Educational Resources Information Center

    Clark, Heather; Lazarus, Cathy; Arvedson, Joan; Schooling, Tracy; Frymark, Tobi

    2009-01-01

    Purpose: To systematically review the literature examining the effects of neuromuscular electrical stimulation (NMES) on swallowing and neural activation. The review was conducted as part of a series examining the effects of oral motor exercises (OMEs) on speech, swallowing, and neural activation. Method: A systematic search was conducted to…

  10. Pharyngeal neuromuscular dysfunction associated with bilateral guttural pouch tympany in a foal

    PubMed Central

    Bell, Chris

    2007-01-01

    A 2-month-old warmblood filly was presented for a 1-week history of a large, nonpainful, fluctuant swelling of the parotid and laryngeal area. Bilateral guttural pouch tympany was diagnosed. Surgical correction resolved the guttural pouch tympany; however, postoperative pharyngeal neuromuscular dysfunction developed. PMID:17334035

  11. Fetal costello syndrome with neuromuscular spindles excess and p.Gly12Val HRAS mutation.

    PubMed

    Quélin, Chloé; Loget, Philippe; Rozel, Céline; D'Hervé, Dominique; Fradin, Mélanie; Demurger, Florence; Odent, Sylvie; Pasquier, Laurent; Cavé, Hélène; Marcorelles, Pascale

    2017-07-01

    Costello syndrome (CS) is a rare multiple congenital disorder caused by activating germline mutations in HRAS gene and is characterized by coarse facial features, severe feeding difficulties, failure to thrive, mild to severe intellectual disability, severe postnatal growth retardation, cardiac abnormalities or cancer predisposition. Phenotypic spectrum associated with HRAS mutations is broad, ranging from attenuated CS phenotype to neonatal and lethal forms with limited genotype-phenotype correlations. Congenital myopathy with neuromuscular spindle excess has been rarely described in the literature. We report a new severe fetal case of CS with distal arthrogryposis due to neuromuscular spindle excess, confirmed by the detection of the p.Gly12Val mutation in HRAS gene. This case emphasizes the fact that HRAS is the only gene responsible for neuromuscular spindle excess, underlines a correlation between p.Gly12Val mutation and severe CS phenotype and points out the importance of a muscle biopsy performed according to the suitable procedure in neuromuscular disorders for any fetal arthrogryposis. Copyright © 2017. Published by Elsevier Masson SAS.

  12. A system for studying mechanisms of neuromuscular junction development and maintenance.

    PubMed

    Vilmont, Valérie; Cadot, Bruno; Ouanounou, Gilles; Gomes, Edgar R

    2016-07-01

    The neuromuscular junction (NMJ), a cellular synapse between a motor neuron and a skeletal muscle fiber, enables the translation of chemical cues into physical activity. The development of this special structure has been subject to numerous investigations, but its complexity renders in vivo studies particularly difficult to perform. In vitro modeling of the neuromuscular junction represents a powerful tool to delineate fully the fine tuning of events that lead to subcellular specialization at the pre-synaptic and post-synaptic sites. Here, we describe a novel heterologous co-culture in vitro method using rat spinal cord explants with dorsal root ganglia and murine primary myoblasts to study neuromuscular junctions. This system allows the formation and long-term survival of highly differentiated myofibers, motor neurons, supporting glial cells and functional neuromuscular junctions with post-synaptic specialization. Therefore, fundamental aspects of NMJ formation and maintenance can be studied using the described system, which can be adapted to model multiple NMJ-associated disorders. © 2016. Published by The Company of Biologists Ltd.

  13. Synaptic Defects in the Spinal and Neuromuscular Circuitry in a Mouse Model of Spinal Muscular Atrophy

    PubMed Central

    Zingg, Brian; Feng, Zhihua; Ko, Chien-Ping

    2010-01-01

    Spinal muscular atrophy (SMA) is a major genetic cause of death in childhood characterized by marked muscle weakness. To investigate mechanisms underlying motor impairment in SMA, we examined the spinal and neuromuscular circuitry governing hindlimb ambulatory behavior in SMA model mice (SMNΔ7). In the neuromuscular circuitry, we found that nearly all neuromuscular junctions (NMJs) in hindlimb muscles of SMNΔ7 mice remained fully innervated at the disease end stage and were capable of eliciting muscle contraction, despite a modest reduction in quantal content. In the spinal circuitry, we observed a ∼28% loss of synapses onto spinal motoneurons in the lateral column of lumbar segments 3–5, and a significant reduction in proprioceptive sensory neurons, which may contribute to the 50% reduction in vesicular glutamate transporter 1(VGLUT1)-positive synapses onto SMNΔ7 motoneurons. In addition, there was an increase in the association of activated microglia with SMNΔ7 motoneurons. Together, our results present a novel concept that synaptic defects occur at multiple levels of the spinal and neuromuscular circuitry in SMNΔ7 mice, and that proprioceptive spinal synapses could be a potential target for SMA therapy. PMID:21085654

  14. A Distinct Perisynaptic Glial Cell Type Forms Tripartite Neuromuscular Synapses in the Drosophila Adult

    PubMed Central

    Strauss, Alexandra L.; Kawasaki, Fumiko; Ordway, Richard W.

    2015-01-01

    Previous studies of Drosophila flight muscle neuromuscular synapses have revealed their tripartite architecture and established an attractive experimental model for genetic analysis of glial function in synaptic transmission. Here we extend these findings by defining a new Drosophila glial cell type, designated peripheral perisynaptic glia (PPG), which resides in the periphery and interacts specifically with fine motor axon branches forming neuromuscular synapses. Identification and specific labeling of PPG was achieved through cell type-specific RNAi-mediated knockdown (KD) of a glial marker, Glutamine Synthetase 2 (GS2). In addition, comparison among different Drosophila neuromuscular synapse models from adult and larval developmental stages indicated the presence of tripartite synapses on several different muscle types in the adult. In contrast, PPG appear to be absent from larval body wall neuromuscular synapses, which do not exhibit a tripartite architecture but rather are imbedded in the muscle plasma membrane. Evolutionary conservation of tripartite synapse architecture and peripheral perisynaptic glia in vertebrates and Drosophila suggests ancient and conserved roles for glia-synapse interactions in synaptic transmission. PMID:26053860

  15. Heterogeneity in synaptic vesicle release at neuromuscular synapses of mice expressing synaptopHluorin.

    PubMed

    Wyatt, Ryan M; Balice-Gordon, Rita J

    2008-01-02

    Mammalian neuromuscular junctions are useful model synapses to study the relationship between synaptic structure and function, although these have rarely been studied together at the same synapses. To do this, we generated transgenic lines of mice in which the thy1.2 promoter drives expression of synaptopHluorin (spH) as a means of optically measuring synaptic vesicle distribution and release. SpH is colocalized with other synaptic vesicle proteins in presynaptic terminals and does not alter normal synaptic function. Nerve stimulation leads to readily detectable and reproducible fluorescence changes in motor axon terminals that vary with stimulus frequency and, when compared with electrophysiological recordings, are reliable indicators of neurotransmitter release. Measurements of fluorescence intensity changes reveal a surprising amount of heterogeneity in synaptic vesicle release throughout individual presynaptic motor axon terminals. Some discrete terminal regions consistently displayed a greater rate and extent of release than others, regardless of stimulation frequency. The amount of release at a particular site is highly correlated to the relative abundance of synaptic vesicles there, indicating that a relatively constant fraction of the total vesicular pool, approximately 30%, is released in response to activity. These studies reveal previously unknown relationships between synaptic structure and function at mammalian neuromuscular junctions and demonstrate the usefulness of spH expressing mice as a tool for studying neuromuscular synapses in adults, as well as during development and diseases that affect neuromuscular synaptic function.

  16. A Distinct Perisynaptic Glial Cell Type Forms Tripartite Neuromuscular Synapses in the Drosophila Adult.

    PubMed

    Strauss, Alexandra L; Kawasaki, Fumiko; Ordway, Richard W

    2015-01-01

    Previous studies of Drosophila flight muscle neuromuscular synapses have revealed their tripartite architecture and established an attractive experimental model for genetic analysis of glial function in synaptic transmission. Here we extend these findings by defining a new Drosophila glial cell type, designated peripheral perisynaptic glia (PPG), which resides in the periphery and interacts specifically with fine motor axon branches forming neuromuscular synapses. Identification and specific labeling of PPG was achieved through cell type-specific RNAi-mediated knockdown (KD) of a glial marker, Glutamine Synthetase 2 (GS2). In addition, comparison among different Drosophila neuromuscular synapse models from adult and larval developmental stages indicated the presence of tripartite synapses on several different muscle types in the adult. In contrast, PPG appear to be absent from larval body wall neuromuscular synapses, which do not exhibit a tripartite architecture but rather are imbedded in the muscle plasma membrane. Evolutionary conservation of tripartite synapse architecture and peripheral perisynaptic glia in vertebrates and Drosophila suggests ancient and conserved roles for glia-synapse interactions in synaptic transmission.

  17. Synaptic defects in the spinal and neuromuscular circuitry in a mouse model of spinal muscular atrophy.

    PubMed

    Ling, Karen K Y; Lin, Ming-Yi; Zingg, Brian; Feng, Zhihua; Ko, Chien-Ping

    2010-11-11

    Spinal muscular atrophy (SMA) is a major genetic cause of death in childhood characterized by marked muscle weakness. To investigate mechanisms underlying motor impairment in SMA, we examined the spinal and neuromuscular circuitry governing hindlimb ambulatory behavior in SMA model mice (SMNΔ7). In the neuromuscular circuitry, we found that nearly all neuromuscular junctions (NMJs) in hindlimb muscles of SMNΔ7 mice remained fully innervated at the disease end stage and were capable of eliciting muscle contraction, despite a modest reduction in quantal content. In the spinal circuitry, we observed a ∼28% loss of synapses onto spinal motoneurons in the lateral column of lumbar segments 3-5, and a significant reduction in proprioceptive sensory neurons, which may contribute to the 50% reduction in vesicular glutamate transporter 1(VGLUT1)-positive synapses onto SMNΔ7 motoneurons. In addition, there was an increase in the association of activated microglia with SMNΔ7 motoneurons. Together, our results present a novel concept that synaptic defects occur at multiple levels of the spinal and neuromuscular circuitry in SMNΔ7 mice, and that proprioceptive spinal synapses could be a potential target for SMA therapy.

  18. MicroRNA-206 delays ALS progression and promotes regeneration of neuromuscular synapses in mice.

    PubMed

    Williams, Andrew H; Valdez, Gregorio; Moresi, Viviana; Qi, Xiaoxia; McAnally, John; Elliott, Jeffrey L; Bassel-Duby, Rhonda; Sanes, Joshua R; Olson, Eric N

    2009-12-11

    Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by loss of motor neurons, denervation of target muscles, muscle atrophy, and paralysis. Understanding ALS pathogenesis may require a fuller understanding of the bidirectional signaling between motor neurons and skeletal muscle fibers at neuromuscular synapses. Here, we show that a key regulator of this signaling is miR-206, a skeletal muscle-specific microRNA that is dramatically induced in a mouse model of ALS. Mice that are genetically deficient in miR-206 form normal neuromuscular synapses during development, but deficiency of miR-206 in the ALS mouse model accelerates disease progression. miR-206 is required for efficient regeneration of neuromuscular synapses after acute nerve injury, which probably accounts for its salutary effects in ALS. miR-206 mediates these effects at least in part through histone deacetylase 4 and fibroblast growth factor signaling pathways. Thus, miR-206 slows ALS progression by sensing motor neuron injury and promoting the compensatory regeneration of neuromuscular synapses.

  19. Neuromuscular Adaptations to Eccentric Strength Training in Children and Adolescents with Cerebral Palsy

    ERIC Educational Resources Information Center

    Reid, Siobhan; Hamer, Peter; Alderson, Jacqueline; Lloyd, David

    2010-01-01

    Aim: To determine the neuromuscular outcomes of an eccentric strength-training programme for children and adolescents with cerebral palsy (CP). Method: In this randomised, parallel-group trial with waiting control, 14 participants with CP (six males, eight females; mean age 11y, SD 2y range 9-15y), diagnosed with upper-limb spasticity were…

  20. Family Stress with Chronic Childhood Illness: Cystic Fibrosis, Neuromuscular Disease, and Renal Disease.

    ERIC Educational Resources Information Center

    Holroyd, Jean; Guthrie, Donald

    1986-01-01

    Parents of children with neuromuscular disease, cystic fibrosis, and renal disease were compared with parents of control subjects matched by age to the clinical cases. The three clinical groups exhibited different patterns of stressful response, consistent with the nature of their illnesses and the requirements for care imposed on the families.…

  1. Non-invasive neuromuscular electrical stimulation in patients with central nervous system lesions: an educational review.

    PubMed

    Schuhfried, Othmar; Crevenna, Richard; Fialka-Moser, Veronika; Paternostro-Sluga, Tatjana

    2012-02-01

    The aim of this educational review is to provide an overview of the clinical application of transcutaneous electrical stimulation of the extremities in patients with upper motor neurone lesions. In general two methods of electrical stimulation can be distinguished: (i) therapeutic electrical stimulation, and (ii) functional electrical stimulation. Therapeutic electrical stimulation improves neuromuscular functional condition by strengthening muscles, increasing motor control, reducing spasticity, decreasing pain and increasing range of motion. Transcutaneous electrical stimulation may be used for neuromuscular electrical stimulation inducing repetitive muscle contraction, electromyography-triggered neuromuscular electrical stimulation, position-triggered electrical stimulation and subsensory or sensory transcutaneous electric stimulation. Functional electrical stimulation provokes muscle contraction and thereby produces a functionally useful movement during stimulation. In patients with spinal cord injuries or stroke, electrical upper limb neuroprostheses are applied to enhance upper limb and hand function, and electrical lower limb neuroprostheses are applied for restoration of standing and walking. For example, a dropped foot stimulator is used to trigger ankle dorsiflexion to restore gait function. A review of the literature and clinical experience of the use of therapeutic electrical stimulation as well as of functional electrical stimulation in combination with botulinum toxin, exercise therapy and/or splinting are presented. Although the evidence is limited we conclude that neuromuscular electrical stimulation in patients with central nervous system lesions can be an effective modality to improve function, and that combination with other treatments has an additive therapeutic effect.

  2. Family Stress with Chronic Childhood Illness: Cystic Fibrosis, Neuromuscular Disease, and Renal Disease.

    ERIC Educational Resources Information Center

    Holroyd, Jean; Guthrie, Donald

    1986-01-01

    Parents of children with neuromuscular disease, cystic fibrosis, and renal disease were compared with parents of control subjects matched by age to the clinical cases. The three clinical groups exhibited different patterns of stressful response, consistent with the nature of their illnesses and the requirements for care imposed on the families.…

  3. Mirror Visual Feedback Induces Lower Neuromuscular Activity in Children with Spastic Hemiparetic Cerebral Palsy

    ERIC Educational Resources Information Center

    Feltham, Max G.; Ledebt, Annick; Deconinck, Frederik J. A.; Savelsbergh, Geert J. P.

    2010-01-01

    The study examined the effects of mirror feedback information on neuromuscular activation during bimanual coordination in eight children with spastic hemiparetic cerebral palsy (SHCP) and a matched control group. The "mirror box" creates a visual illusion, which gives rise to a visual perception of a zero lag, symmetric movement between the two…

  4. Mirror Visual Feedback Induces Lower Neuromuscular Activity in Children with Spastic Hemiparetic Cerebral Palsy

    ERIC Educational Resources Information Center

    Feltham, Max G.; Ledebt, Annick; Deconinck, Frederik J. A.; Savelsbergh, Geert J. P.

    2010-01-01

    The study examined the effects of mirror feedback information on neuromuscular activation during bimanual coordination in eight children with spastic hemiparetic cerebral palsy (SHCP) and a matched control group. The "mirror box" creates a visual illusion, which gives rise to a visual perception of a zero lag, symmetric movement between the two…

  5. Proprioceptive Neuromuscular Facilitation Flexibility Techniques: Acute Effects on Arterial Blood Pressure.

    ERIC Educational Resources Information Center

    Cornelius, William L.; Craft-Hamm, Kelley

    1988-01-01

    The effects of stretching techniques on arterial blood pressure (ABP) were studied in three groups of 20 men each. Each group performed one of three proprioceptive neuromuscular facilitation (PNF) techniques. Results are presented. The study indicates that the benefits of stretching may outweigh the risk of elevated ABP. (JL)

  6. Enhancement of neuromuscular dynamics and strength behavior using extremely low magnitude mechanical signals in mice.

    PubMed

    Mettlach, Gabriel; Polo-Parada, Luis; Peca, Lauren; Rubin, Clinton T; Plattner, Florian; Bibb, James A

    2014-01-03

    Exercise in general, and mechanical signals in particular, help ameliorate the neuromuscular symptoms of aging and possibly other neurodegenerative disorders by enhancing muscle function. To better understand the salutary mechanisms of such physical stimuli, we evaluated the potential for low intensity mechanical signals to promote enhanced muscle dynamics. The effects of daily brief periods of low intensity vibration (LIV) on neuromuscular functions and behavioral correlates were assessed in mice. Physiological analysis revealed that LIV increased isometric force production in semitendinosus skeletal muscle. This effect was evident in both young and old mice. Isometric force recordings also showed that LIV reduced the fatiguing effects of intensive synaptic muscle stimulation. Furthermore, LIV increased evoked neurotransmitter release at neuromuscular synapses but had no effect on spontaneous end plate potential amplitude or frequency. In behavioral studies, LIV increased mouse grip strength and potentiated initial motor activity in a novel environment. These results provide evidence for the efficacy of LIV in producing changes in the neuromuscular system that translate into performance gains at a behavioral scale. © 2013 Published by Elsevier Ltd.

  7. A system for studying mechanisms of neuromuscular junction development and maintenance

    PubMed Central

    Vilmont, Valérie; Ouanounou, Gilles

    2016-01-01

    The neuromuscular junction (NMJ), a cellular synapse between a motor neuron and a skeletal muscle fiber, enables the translation of chemical cues into physical activity. The development of this special structure has been subject to numerous investigations, but its complexity renders in vivo studies particularly difficult to perform. In vitro modeling of the neuromuscular junction represents a powerful tool to delineate fully the fine tuning of events that lead to subcellular specialization at the pre-synaptic and post-synaptic sites. Here, we describe a novel heterologous co-culture in vitro method using rat spinal cord explants with dorsal root ganglia and murine primary myoblasts to study neuromuscular junctions. This system allows the formation and long-term survival of highly differentiated myofibers, motor neurons, supporting glial cells and functional neuromuscular junctions with post-synaptic specialization. Therefore, fundamental aspects of NMJ formation and maintenance can be studied using the described system, which can be adapted to model multiple NMJ-associated disorders. PMID:27226316

  8. Motor function measure: validation of a short form for young children with neuromuscular diseases.

    PubMed

    de Lattre, Capucine; Payan, Christine; Vuillerot, Carole; Rippert, Pascal; de Castro, Denis; Bérard, Carole; Poirot, Isabelle

    2013-11-01

    To validate a useful version of the Motor Function Measure (MFM) in children with neuromuscular diseases aged <7 years old. Two prospective cohort studies that documented the MFM completion of children aged between 2 and 7 years old. French-speaking rehabilitation departments from France, Belgium, and Switzerland. Healthy children (n=194) and children with a neuromuscular disease (n=88). Patients were rated by the MFM either once or twice by trained medical professionals, with a delay between the 2 MFMs ranging between 8 and 30 days. Intra- and interrater reliability of the MFM. The subtests making up the MFM-32, a scale monitoring severity and progression of motor function in patients with a neuromuscular disease in 3 functional domains, were carried out in healthy children aged 2 to 7 years. Twenty items of the MFM-32 were successfully completed by these children and were used to constitute the MFM-20. Principal component analysis of the MFM-20 confirmed the 3 functional domains. Inter- and intrarater reliability of the 3 subscores and total score were high (intraclass correlation coefficient >.90), and discriminant validity was good. The MFM-20 can be used as an outcome measure for assessment of motor function in young children with neuromuscular disease. Copyright © 2013 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

  9. Current Methodological Issues in the Study of Children with Inherited Neuromuscular Disorders

    ERIC Educational Resources Information Center

    Mercuri, Eugenio; Messina, Sonia; Pane, Marika; Bertini, Enrico

    2008-01-01

    Several clinical trials assessing children with hereditary neuromuscular disorders have been performed over the last decade. These studies highlighted issues related to design and performance of clinical studies assessing children with this group of disorders. This article reviews recent literature and clinical experience in this area,…

  10. Medical and psychosocial considerations in rehabilitation care of childhood neuromuscular diseases.

    PubMed

    Vanasse, Michel; Paré, Hélène; Zeller, Reinhard

    2013-01-01

    Most neuromuscular diseases (NMD) are rare inherited conditions presenting a relentlessly progressive course. Some NMD are associated with intellectual impairment and many are complicated by musculoskeletal, cardiac, or respiratory problems. Rehabilitation care of NMD is thus best provided by a team of medical specialists and health professionals working closely together in an interdisciplinary fashion. Rehabilitation for patients with NMD should include physical evaluation but also assessment of their ability to manage their daily activities or work and their need for assistive devices. With appropriate physical, psychological, and social support, those patients can maintain a good quality of life and an adequate level of well-being. Surgical management of musculoskeletal problems in children related to neuromuscular disorders remains a highly controversial subject. This review is based mainly on the author's personal experience with conservative and periopeporative rehabilitation care for neuromuscular diseases. A multidisciplinary approach is advocated. The importance of proactive respiratory care is emphasized. Rehabilitation after surgical treatment of spinal deformities is analyzed outlining the particular aspects in spinal muscular atrophy, Duchenne's muscular dystrophy, congenital muscular dystrophy, and congenital myopathies. A disease- specific approach is recommended as the wide variety of different neuromuscular pathologies requires careful planning of treatment strategies best managed in a team approach.

  11. Loss of neurofilaments in the neuromuscular junction in a rat model of proximal axonopathy.

    PubMed

    Soler-Martín, C; Vilardosa, U; Saldaña-Ruíz, S; Garcia, N; Llorens, J

    2012-02-01

    Rodents exposed to 3,3'-iminodipropionitrile (IDPN) develop an axonopathy similar to that observed in amyotrophic lateral sclerosis motor neurones, in which neurofilaments accumulate in swollen proximal axon segments. This study addressed the hypotheses that this proximal axonopathy is associated with loss of neurofilament proteins in the neuromuscular junctions and a progressive loss of neurofilaments advancing in a distal-proximal direction from the distal motor nerve. Adult male Long-Evans rats were exposed to 0 or 15 mM of IDPN in drinking water for 1, 3 or 5 weeks, and their distal axons and neuromuscular junction organization studied by immunohistochemistry. Quantitative data were obtained by confocal microscopy on whole mounts of the Levator auris longus. Muscles showed no change in the distribution of acetylcholine receptor labelling in the neuromuscular junctions after IDPN. In contrast, the amount of neurofilament labelling in the junctions was significantly reduced by IDPN, assessed with two different anti-neurofilament antibodies. In preterminal axons and in more proximal axon levels, no statistically significant reductions in neurofilament content were observed. The proximal neurofilamentous axonopathy induced by IDPN is associated with an abnormally low content of neurofilaments in the motor terminals, with a potential impact in the function or stability of the neuromuscular junction. In contrast, neurofilaments are significantly maintained in the distal axon. © 2011 The Authors. Neuropathology and Applied Neurobiology © 2011 British Neuropathological Society.

  12. Adaptation of neuromuscular activation patterns during treadmill walking after long-duration space flight

    NASA Astrophysics Data System (ADS)

    Layne, C. S.; Lange, G. W.; Pruett, C. J.; McDonald, P. V.; Merkle, L. A.; Mulavara, A. P.; Smith, S. L.; Kozlovskaya, I. B.; Bloomberg, J. J.

    The precise neuromuscular control needed for optimal locomotion, particularly around heel strike and toe off, is known to be compromised after short duration (8- to 15-day) space flight. We hypothesized here that longer exposure to weightlessness would result in maladaptive neuromuscular activation during postflight treadmill walking. We also hypothesized that space flight would affect the ability of the sensory-motor control system to generate adaptive neuromuscular activation patterns in response to changes in visual target distance during postflight treadmill walking. Seven crewmembers, who completed 3- to 6-month missions, walked on a motorized treadmill while visually fixating on a target placed 30 cm (NEAR) or 2 m (FAR) from the subject's eyes. Electronic foot switch data and surface electromyography were collected from selected muscles of the right lower limb. Results indicate that the phasic features of neuromuscular activation were moderately affected and the relative amplitude of activity in the tibialis anterior and rectus femoris around toe off changed after space flight. Changes also were evident after space flight in how these muscles adapted to the shift in visual target distance.

  13. Rocuronium-induced neuromuscular block and sugammadex in pediatric patient with duchenne muscular dystrophy

    PubMed Central

    Kim, Ji Eun; Chun, Hea Rim

    2017-01-01

    Abstract Introduction: Anesthetic management of patients with Duchenne muscular dystrophy (DMD) is complicated because these patients are more sensitive to nondepolarizing neuromuscular blocking agents (NMBAs) and are vulnerable to postoperative complications, such as postoperative residual curarization and respiratory failure. Sugammadex is a new reversal agent for aminosteroidal NMBAs, but its safety in children is controversial. Clinical features: An 11-year-old boy with DMD underwent general anesthesia for a percutaneous nephrolithotomy. We used rocuronium bromide and sugammadex to reverse the deep neuromuscular block. Reversal of neuromuscular block was done 15 minutes after administration of 2 mg/kg of sugammadex. The patient's recovery from anesthesia was uneventful, and he was discharged to the postoperative recovery ward. Conclusion: A delayed recovery was achieved, but no adverse events were observed, such as recurarization or hypersensitivity to sugammadex. We report safe use of 2 mg/kg of sugammadex to reverse a deep neuromuscular block in a child with DMD. PMID:28353578

  14. Neuromuscular Adaptations to Eccentric Strength Training in Children and Adolescents with Cerebral Palsy

    ERIC Educational Resources Information Center

    Reid, Siobhan; Hamer, Peter; Alderson, Jacqueline; Lloyd, David

    2010-01-01

    Aim: To determine the neuromuscular outcomes of an eccentric strength-training programme for children and adolescents with cerebral palsy (CP). Method: In this randomised, parallel-group trial with waiting control, 14 participants with CP (six males, eight females; mean age 11y, SD 2y range 9-15y), diagnosed with upper-limb spasticity were…

  15. Man-Machine Interface System for Neuromuscular Training and Evaluation Based on EMG and MMG Signals

    PubMed Central

    de la Rosa, Ramon; Alonso, Alonso; Carrera, Albano; Durán, Ramon; Fernández, Patricia

    2010-01-01

    This paper presents the UVa-NTS (University of Valladolid Neuromuscular Training System), a multifunction and portable Neuromuscular Training System. The UVa-NTS is designed to analyze the voluntary control of severe neuromotor handicapped patients, their interactive response, and their adaptation to neuromuscular interface systems, such as neural prostheses or domotic applications. Thus, it is an excellent tool to evaluate the residual muscle capabilities in the handicapped. The UVa-NTS is composed of a custom signal conditioning front-end and a computer. The front-end electronics is described thoroughly as well as the overall features of the custom software implementation. The software system is composed of a set of graphical training tools and a processing core. The UVa-NTS works with two classes of neuromuscular signals: the classic myoelectric signals (MES) and, as a novelty, the myomechanic signals (MMS). In order to evaluate the performance of the processing core, a complete analysis has been done to classify its efficiency and to check that it fulfils with the real-time constraints. Tests were performed both with healthy and selected impaired subjects. The adaptation was achieved rapidly, applying a predefined protocol for the UVa-NTS set of training tools. Fine voluntary control was demonstrated to be reached with the myoelectric signals. And the UVa-NTS demonstrated to provide a satisfactory voluntary control when applying the myomechanic signals. PMID:22163515

  16. Progressive adaptation in physical activity and neuromuscular performance during 520d confinement.

    PubMed

    Belavý, Daniel L; Gast, Ulf; Daumer, Martin; Fomina, Elena; Rawer, Rainer; Schießl, Hans; Schneider, Stefan; Schubert, Harald; Soaz, Cristina; Felsenberg, Dieter

    2013-01-01

    To understand whether prolonged confinement results in reductions in physical activity and adaptation in the musculoskeletal system, six subjects were measured during 520 d isolation in the Mars500 study. We tested the hypothesis that physical activity reduces in prolonged confinement and that this would be associated with decrements of neuromuscular performance. Physical activity, as measured by average acceleration of the body's center of mass ("activity temperature") using the actibelt® device, decreased progressively over the course of isolation (p<0.00001). Concurrently, countermovement jump power and single-leg hop force decreased during isolation (p<0.001) whilst grip force did not change (p≥0.14). Similar to other models of inactivity, greater decrements of neuromuscular performance occurred in the lower-limb than in the upper-limb. Subject motivational state increased non-significantly (p = 0.20) during isolation, suggesting reductions in lower-limb neuromuscular performance were unrelated to motivation. Overall, we conclude that prolonged confinement is a form of physical inactivity and is associated with adaptation in the neuromuscular system.

  17. Progressive Adaptation in Physical Activity and Neuromuscular Performance during 520d Confinement

    PubMed Central

    Belavý, Daniel L.; Gast, Ulf; Daumer, Martin; Fomina, Elena; Rawer, Rainer; Schießl, Hans; Schneider, Stefan; Schubert, Harald; Soaz, Cristina; Felsenberg, Dieter

    2013-01-01

    To understand whether prolonged confinement results in reductions in physical activity and adaptation in the musculoskeletal system, six subjects were measured during 520 d isolation in the Mars500 study. We tested the hypothesis that physical activity reduces in prolonged confinement and that this would be associated with decrements of neuromuscular performance. Physical activity, as measured by average acceleration of the body’s center of mass (“activity temperature”) using the actibelt® device, decreased progressively over the course of isolation (p<0.00001). Concurrently, countermovement jump power and single-leg hop force decreased during isolation (p<0.001) whilst grip force did not change (p≥0.14). Similar to other models of inactivity, greater decrements of neuromuscular performance occurred in the lower-limb than in the upper-limb. Subject motivational state increased non-significantly (p = 0.20) during isolation, suggesting reductions in lower-limb neuromuscular performance were unrelated to motivation. Overall, we conclude that prolonged confinement is a form of physical inactivity and is associated with adaptation in the neuromuscular system. PMID:23555896

  18. The use of an online support group for neuromuscular disorders: a thematic analysis of message postings.

    PubMed

    Meade, Oonagh; Buchanan, Heather; Coulson, Neil

    2017-06-08

    People affected by neuromuscular disorders can experience adverse psychosocial consequences and difficulties accessing information and support. Online support groups provide new opportunities for peer support. The aim of this study was to understand how contributors used the message board function of a newly available neuromuscular disorders online support group. Message postings (n = 1951) from the first five months of the message board of a newly formed online support group for neuromuscular disorders hosted by a charitable organization were analyzed using inductive thematic analysis. Members created a sense of community through disclosing personal information, connecting with people with similar illness experiences or interests, welcoming others and sharing aspirations for the development of a resourceful community. Experiences, emotional reactions and support were shared in relation to: delayed diagnosis; symptom interpretation; illness management and progression; the isolating impact of rare disorders; and the influence of social and political factors on illness experiences. This study provided a novel insight into individuals' experiences of accessing a newly available online support group for rare conditions hosted by a charitable organization. The findings highlight how the online support group provided an important peer support environment for members to connect with others, exchange information and support and engender discussion on political and social issues unique to living with often-rare neuromuscular disorders. Online support groups may therefore provide an important and easily accessible support outlet for people with neuromuscular disorders as well as a platform for empowering members to raise awareness about the impact of living with these conditions. Further research is needed to examine member motivations for using such groups and any effects of participation in greater detail. Implications for rehabilitation Online support groups may

  19. The Influence of Robotic Assistance on Reducing Neuromuscular Effort and Fatigue during Extravehicular Activity Glove Use

    NASA Technical Reports Server (NTRS)

    Madden, Kaci E.; Deshpande, Ashish D.; Peters, Benjamin J.; Rogers, Jonathan M.; Laske, Evan A.; McBryan, Emily R.

    2017-01-01

    The three-layered, pressurized space suit glove worn by Extravehicular Activity (EVA) crew members during missions commonly causes hand and forearm fatigue. The Spacesuit RoboGlove (SSRG), a Phase VI EVA space suit glove modified with robotic grasp-assist capabilities, has been developed to augment grip strength in order to improve endurance and reduce the risk of injury in astronauts. The overall goals of this study were to i) quantify the neuromuscular modulations that occur in response to wearing a conventional Phase VI space suit glove (SSG) during a fatiguing task, and ii) determine the efficacy of Spacesuit RoboGlove (SSRG) in reversing the adverse neuromuscular modulations and restoring altered muscular activity to barehanded levels. Six subjects performed a fatigue sequence consisting of repetitive dynamic-gripping interspersed with isometric grip-holds under three conditions: barehanded, wearing pressurized SSG, and wearing pressurized SSRG. Surface electromyography (sEMG) from six forearm muscles (flexor digitorum superficialis (FDS), flexor carpi radialis (FCR), flexor carpi ulnaris (FCU), extensor digitorum (ED), extensor carpi radialis longus (ECRL), and extensor carpi ulnaris (ECU)) and subjective fatigue ratings were collected during each condition. Trends in amplitude and spectral distributions of the sEMG signals were used to derive metrics quantifying neuromuscular effort and fatigue that were compared across the glove conditions. Results showed that by augmenting finger flexion, the SSRG successfully reduced the neuromuscular effort needed to close the fingers of the space suit glove in more than half of subjects during two types of tasks. However, the SSRG required more neuromuscular effort to extend the fingers compared to a conventional SSG in many subjects. Psychologically, the SSRG aided subjects in feeling less fatigued during short periods of intense work compared to the SSG. The results of this study reveal the promise of the SSRG as a

  20. Reversal of neuromuscular blockade by sugammadex in laparoscopic bariatric surgery: In support of dose reduction.

    PubMed

    Badaoui, Rachid; Cabaret, Aurélie; Alami, Youssef; Zogheib, Elie; Popov, Ivan; Lorne, Emmanuel; Dupont, Hervé

    2016-02-01

    Sugammadex is the first molecule able to antagonize steroidal muscle relaxants with few adverse effects. Doses are adjusted to body weight and the level of neuromuscular blockade. Sleeve gastrectomy is becoming a very popular form of bariatric surgery. It requires deep muscle relaxation followed by complete and rapid reversal to decrease postoperative and especially post-anaesthetic morbidity. Sugammadex is therefore particularly indicated in this setting. The objective of this study was to evaluate the deep neuromuscular blockade reversal time after administration of various doses of sugammadex (based on real weight or at lower doses). Secondary endpoints were the interval between the sugammadex injection and extubation and transfer from the operating room to the recovery room. We then investigated any complications observed in the recovery room. This pilot, prospective, observational, clinical practice evaluation study was conducted in the Amiens University Hospital. Neuromuscular blockade was induced by rocuronium. At the end of the operation, deep neuromuscular blockade was reversed by sugammadex at the dose of 4mg/kg. Sixty-four patients were included: 31 patients received sugammadex at a dosage based on their real weight (RW) and 33 patients received a lower dose (based on ideal weight [IW]). For identical rocuronium doses calculated based on IBW, sugammadex doses were significantly lower in the IW group: 349 (± 65) mg versus 508 (± 75) mg (P<0.0001). Despite this dose reduction, neuromuscular blockade reversal took 115 (± 69) s in the IW group versus 87 (± 40) s in the RW group, but with no significant difference between the two groups (P=0.08). The intervals between injection of sugammadex and extubation (P=0.07) and transfer from the operating room to the recovery room (P=0.68) were also non-significantly longer in the IW group. The mean dose of sugammadex used by anaesthetists in the IW group was 4mg/kg of ideal weight increased by 35% to 50% (n