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Sample records for profound neuromuscular block

  1. Reversal of profound vecuronium-induced neuromuscular block under sevoflurane anesthesia: sugammadex versus neostigmine.

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    Lemmens Hendrikus JM

    2010-09-01

    Full Text Available Abstract Background Acetylcholinesterase inhibitors cannot rapidly reverse profound neuromuscular block. Sugammadex, a selective relaxant binding agent, reverses the effects of rocuronium and vecuronium by encapsulation. This study assessed the efficacy of sugammadex compared with neostigmine in reversal of profound vecuronium-induced neuromuscular block under sevoflurane anesthesia. Methods Patients aged ≥18 years, American Society of Anesthesiologists class 1-4, scheduled to undergo surgery under general anesthesia were enrolled in this phase III, multicenter, randomized, safety-assessor blinded study. Sevoflurane anesthetized patients received vecuronium 0.1 mg/kg for intubation, with maintenance doses of 0.015 mg/kg as required. Patients were randomized to receive sugammadex 4 mg/kg or neostigmine 70 μg/kg with glycopyrrolate 14 μg/kg at 1-2 post-tetanic counts. The primary efficacy variable was time from start of study drug administration to recovery of the train-of-four ratio to 0.9. Safety assessments included physical examination, laboratory data, vital signs, and adverse events. Results Eighty three patients were included in the intent-to-treat population (sugammadex, n = 47; neostigmine, n = 36. Geometric mean time to recovery of the train-of-four ratio to 0.9 was 15-fold faster with sugammadex (4.5 minutes compared with neostigmine (66.2 minutes; p Conclusions Recovery from profound vecuronium-induced block is significantly faster with sugammadex, compared with neostigmine. Neostigmine did not rapidly reverse profound neuromuscular block (Trial registration number: NCT00473694.

  2. Neuromuscular blocking properties of dioxonium.

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    Tammisto, T; Salmenperä, M

    1980-12-01

    The characteristics of the myoneural block caused by a new neuromuscular blocking agent, dioxonium (Dx), were evaluated in surgical patients. The force and the corresponding electromyogram (EMG) of the thumb adduction evoked by various modes of ulnar nerve stimuli were measured. Onset, maintenance and disappearance of blockade after sequential administration of Dx were compared with results obtained with d-tubocurarine (dTc) or suxamethonium (Sx). Initially the Dx block was shown to be depolarizing with a negligible fade in the 2 Hz train of four stimuli with a single twitch suppression of 90%. On a weight basis, Dx was found to be about 15 times as potent as dTc in suppressing twitch to the 90% level. During maintenance, the block gained nondepolarizing characteristics with profound fades in the 2 and 50 Hz trains. The transition was associated with tachyphylaxis and with a more pronounced suppression of EMG amplitude than that measured in the twitch force. After a total dose of about 100 microgram/kg of Dx, the sensitivity to Dx again increased and the discrepancy between twitch tension and EMG disappeared. This pattern of changes was also seen with Sx. Spontaneous recovery occurred slightly faster than after dTc blocks of corresponding duration. With neostigmine, reversal was hastened and a full recovery with restitution of prerelaxant twitch and disappearance of fades was reached in about 20 min. Some discrepancy in EMG amplitude and twitch force persisted, however.

  3. The undesirable effects of neuromuscular blocking drugs

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    Claudius, C; Garvey, L H; Viby-Mogensen, J

    2009-01-01

    Neuromuscular blocking drugs are designed to bind to the nicotinic receptor at the neuromuscular junction. However, they also interact with other acetylcholine receptors in the body. Binding to these receptors causes adverse effects that vary with the specificity for the cholinergic receptor...... in question. Moreover, all neuromuscular blocking drugs may cause hypersensitivity reactions. Often the symptoms are mild and self-limiting but massive histamine release can cause systematic reactions with circulatory and respiratory symptoms and signs. At the end of anaesthesia, no residual effect...... of a neuromuscular blocking drug should be present. However, the huge variability in response to neuromuscular blocking drugs makes it impossible to predict which patient will suffer postoperative residual curarization. This article discusses the undesirable effects of the currently available neuromuscular blocking...

  4. ATRACURIUM-INDUCED NEUROMUSCULAR BLOCK IN THE ISOLATED ARM

    NARCIS (Netherlands)

    ERIKSSON, LI; VANDENBROM, RHG; LENNMARKEN, C; AGOSTON, S

    1992-01-01

    A modification of the isolated arm technique was applied in 10 females under opioid-based i.v. anaesthesia for comparison of the offset of an atracurium-induced neuromuscular block in an isolated arm to an arm with maintained circulation. The neuromuscular blocking effect of a bolus dose of

  5. Pharmacokinetic studies of neuromuscular blocking agents: Good Clinical Research Practice (GCRP)

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    Viby-Mogensen, J.; Østergaard, D.; Donati, F.

    2000-01-01

    Good Clinical Research Practice (GCRP), neuromuscular blocking agents, pharmacokinetics, pharmacokinetic/pharmacodynamic modeling, population pharmacokinetics, statistics, study design......Good Clinical Research Practice (GCRP), neuromuscular blocking agents, pharmacokinetics, pharmacokinetic/pharmacodynamic modeling, population pharmacokinetics, statistics, study design...

  6. DOSE-RESPONSE RELATION, NEUROMUSCULAR BLOCKING ACTION, INTUBATION CONDITIONS, AND CARDIOVASCULAR EFFECTS OF ORG-9273, A NEW NEUROMUSCULAR BLOCKING-AGENT

    NARCIS (Netherlands)

    VANDENBROEK, L; LAMBALK, LM; RICHARDSON, FJ; WIERDA, JMKH

    The ED50 and the ED90, the time-course of the neuromuscular block, the intubation conditions, and the cardiovascular effects of Org 9273, a new steroidal nondepolarizing neuromuscular blocking agent, have been evaluated in 41 anesthetized patients. From cumulative dose-response curves the ED50 and

  7. Pharmacokinetic studies of neuromuscular blocking agents : Good Clinical Research Practice (GCRP)

    NARCIS (Netherlands)

    Viby-Mogensen, J; Ostergaard, D; Donati, F; Fisher, D; Hunter, J; Kampmann, JP; Kopman, A; Proost, JH; Rasmussen, SN; Skovgaard, LT; Varin, F; Wright, PMC

    2000-01-01

    In September 1997, an international consensus conference on standardization of studies of neuromuscular blocking agents was held in Copenhagen, Denmark. Based on the conference, a set of guidelines fur good clinical research practice (GCRT) in pharmacokinetic studies of neuromuscular blocking agents

  8. [Anaphylaxis during anesthesia--especially from neuromuscular blocking agents].

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    Kageyama, Kyoko; Hayakawa, Yuka; Nakajima, Yasufumi; Hashimoto, Satoru

    2011-01-01

    Among all drugs used for general anesthesia, neuromuscular blocking agents (NMBAs) seem to play a predominant role in the incidence of severe adverse reactions. The overall incidence of perioperative anaphylaxis is estimated at 1 in 10,000-20,000 anesthetic procedures, whereas it is estimated at 1 in 6,500 administrations of NMBAs. After anaphylaxis, allergologic assessment is essential to identify the offending agent and to prevent recurrences. The estimated sensitivity of skin tests for muscle relaxants is approximately 94% to 97%. Prick testing is advised for the diagnosis of the NMBAs responsible for an anaphylactic reaction, and intradermal testing is preferred when investing cross-reaction. The choice of the safest possible anesthetic agents should be based on the result of a rigorously performed allergologic assessment.

  9. CLINICAL-PHARMACOLOGY OF ORG-7617, A SHORT-ACTING NONDEPOLARIZING NEUROMUSCULAR BLOCKING-AGENT

    NARCIS (Netherlands)

    VANDENBROEK, L; WIERDA, JMKH; PROOST, JH; HOMMES, FDM; AGOSTON, S

    The dose-response relationship and the time course of action of Org 7617, a short acting non-depolarizing neuromuscular blocking agent, were evaluated during thiopentone, fentanyl, halothane and N2O anaesthesia. Neuromuscular transmission was monitored mechanomyographically. The ED(50) and ED(90)

  10. The mode of neuromuscular blocking action of chlorpromazine

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    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

  11. Acceleromyography and mechanomyography for establishing potency of neuromuscular blocking agents: a randomized-controlled trial

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    Claudius, C; Viby-Mogensen, J; Skovgaard, Lene Theil

    2009-01-01

    BACKGROUND: Acceleromyography (AMG) is increasingly being used in neuromuscular research, including in studies establishing the potency of neuromuscular blocking and reversal agents. However, AMG is insufficiently validated for use interchangeably with the gold standard, mechanomyography (MMG......) for this purpose. The aim of this study was to compare AMG and MMG for establishing dose-response relationship and potency, using rocuronium as an example. METHODS: We included 40 adult patients in this randomized-controlled single-dose response study. Anaesthesia was induced and maintained with propofol...... difference between AMG and MMG is so small that it justifies AMG to be used for establishing the potency of neuromuscular blocking agents. However, the wide CIs show that we cannot rule out a 13% higher ED(50) and a 26% higher ED(95) for AMG....

  12. Reversal of profound, high-dose rocuronium-induced neuromuscular blockade by sugammadex at two different time points: an international, multicenter, randomized, dose-finding, safety assessor-blinded, phase II trial

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    Pühringer, Friedrich K; Rex, Christopher; Sielenkämper, Andreas W

    2008-01-01

    Sugammadex (Org 25969), a novel, selective relaxant binding agent, was specifically designed to rapidly reverse rocuronium-induced neuromuscular blockade. The efficacy and safety of sugammadex for the reversal of profound, high-dose rocuronium-induced neuromuscular blockade was evaluated....

  13. Reversal of profound, high-dose rocuronium-induced neuromuscular blockade by sugammadex at two different time points - An international, multicenter, randomized, dose-finding, safety assessor-blinded, phase II trial

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    Puhringer, F.K.; Rex, C.; Sielenkamper, A.W.

    2008-01-01

    Background: Sugammadex (Org 25969), a novel, selective relaxant binding agent, was specifically designed to rapidly reverse rocuronium-induced neuromuscular blockade. The efficacy and safety of sugammadex for the reversal of profound, high-dose rocuronium-induced neuromuscular blockade was evalua...

  14. Sugammadex and Reversal of Neuromuscular Block in Adult Patient with Duchenne Muscular Dystrophy

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    Ahmed Abdelgawwad Wefki Abdelgawwad Shousha

    2014-01-01

    Full Text Available Duchenne’s muscular dystrophy (DMD is the most common and severe form of myopathy. Patients with DMD are more sensitive to sedative, anesthetic, and neuromuscular blocking agents which may result in intraoperative and early postoperative cardiovascular and respiratory complications, as well as prolonged recovery from anesthesia. In this case report, we describe a 25-year-old male patient admitted for cholecystectomy under general anesthesia. We induced our anesthesia by oxygen, propofol, fentanyl, and rocuronium bromide. Maintenance was done by fentanyl, rocuronium bromide, sevoflurane, and O2. We report in this case the safety use of sugammadex to antagonize the neuromuscular block and rapid recovery in such category of patients.

  15. [Augmentation by succinylcholine of the neuromuscular blocking effect of vecuronium in children].

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    Yasuda, S; Takeda, J; Sekiguchi, H; Fukushima, K

    1995-07-01

    To evaluate the influence of succinylcholine (Scc) on the neuromuscular blocking effect of subsequently administered vecuronium in children, 30 patients aged 2-14 years scheduled for elective surgery were studied after obtaining the informed consent from the parents. Anesthesia was induced with inhalation of sevoflurane, nitrous oxide and oxygen. T1 of the adductor pollicis muscle to ulnar nerve stimulation elicited by train of four stimulation at 2 Hz was monitored continuously by an acceleration transducer. The patients were divided into two groups; group V (16 patients) received vecuronium (0.03 mg.kg-1) and group SV (14 patients) received vecuronium (0.03 mg.kg-1) after 100% recovery of twitch from neuromuscular blockade induced with Scc (1.0 mg.kg-1). Short onset of action and potentiation of maximal block were demonstrated in group SV. After vecuronium administration, a complete suppression of T1 was observed in 8 patients of group SV and only 1 patient of group V. The present study demonstrates that the neuromuscular blockade of vecuronium can be potentiated with the prior administration of Scc in pediatric patients.

  16. Assessment of neuromuscular block: comparison of three clinical methods and evoked electromyography.

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    Tammisto, T; Wirtavuori, K; Linko, K

    1988-01-01

    Three clinical methods, visual, tactile and 'spring', for the assessment of neuromuscular blockade were compared to the EMG recording evoked during enflurane anaesthesia and relaxation with vecuronium in 33 patients. During maintenance of the block, the tactile method, based on the recognition of the strength of movement of the patient's thumb against the observer's fingers, was more accurate than the two other methods. The correlation coefficient compared to the TI of the EMG was 0.77. The tactile method led to over-estimation of muscle strength in only 9-10% of the assessments made by the anaesthetists, while this happened in about one-third of the cases when the visual method was applied. The observers under-estimated the muscle power in about 30% of the assessments made with each of the clinical methods. During the recovery, the mean train-of-four (TOF) ratio of the EMG was less than 40% when the anaesthetists announced that they could detect no fade with the visual or tactile methods. The corresponding value obtained with the spring method (standardized preload with a rubber spring) was significantly higher, 66%. Using the spring, a clinically significant residual fade (TOF less than 0.50) could be detected in nine of the 11 cases. As residual relaxation cannot be ruled out using the clinical methods, quantitative recording of neuromuscular function is recommended in cases where complete recovery from muscle relaxation is of special importance. The spring method is the most reliable clinical method during recovery, while the tactile method is the most accurate during the maintenance of neuromuscular block.

  17. Neuromuscular blocking drugs do not alter the pupillary light reflex of anesthetized humans.

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    Gray, A T; Krejci, S T; Larson, M D

    1997-05-01

    To test the hypothesis that systemically administered neuromuscular blocking drugs acutely alter resting pupil size or the direct reflex response to light in anesthetized humans. Patients were randomized to receive an intravenous injection of saline (0.15 mL/kg), pancuronium bromide (0.1 mg/kg), or vecuronium bromide (0.15 mg/kg) after induction of general anesthesia and tracheal intubation. The University of California, San Francisco, Moffitt-Long Hospitals. Healthy adults (American Society of Anesthesiologists physical status I or II) of either sex scheduled for elective surgery requiring general anesthesia, tracheal intubation, and muscle relaxation of an anticipated duration of 2 or more hours. Measurements of resting pupil size, direct reflex response to light, and constriction velocity were obtained in double-blinded fashion using infrared pupillometry. Pupillary size, reflex amplitude, and constriction velocity were not altered by the presence of either vecuronium or pancuronium. Tetanic stimuli and concomitant isoflurane administration respectively increased and decreased pupillary light reflex amplitude, indicating that pupillary responses were not fixed. We conclude that systemically administered neuromuscular blocking drugs (vecuronium and pancuronium) do not acutely affect the pupillary light reflex in healthy, anesthetized patients.

  18. Anesthesia with Propofol versus Sevoflurane: Does the Longer Neuromuscular Block under Sevoflurane Anesthesia Reduce Laryngeal Injuries?

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    Thomas Mencke

    2013-01-01

    Full Text Available Anesthesia can be maintained with propofol or sevoflurane. Volatile anesthetics increase neuromuscular block of muscle relaxants. We tested the hypothesis, that sevoflurane would cause less vocal cord injuries than an intravenous anesthesia with propofol. In this prospective trial, 65 patients were randomized in 2 groups: SEVO group, anesthesia with sevoflurane, and TIVA group, total intravenous anesthesia with propofol. Intubating and extubating conditions were evaluated. Vocal cord injuries were examined by stroboscopy before and 24 and 72 h after surgery; hoarseness and sore throat were assessed up to 72 h after surgery. Hoarseness and sore throat were comparable between both groups (not significant. Similar findings were observed for vocal cord injuries: 9 (SEVO versus 5 (TIVA patients; ; the overall incidence was 24%. Type of vocal cord injuries: 9 erythema and 5 edema of the vocal folds. Neuromuscular block was significantly longer in the SEVO group compared with the TIVA group: 71 (range: 38–148 min versus 52 (range: 21–74 min; . Five patients (TIVA group versus 11 patients (SEVO group needed neostigmine to achieve a TOF ratio of 1.0 . Under anesthesia with propofol laryngeal injuries were not increased; the risk for residual curarization, however, was lower compared with sevoflurane.

  19. What rules of thumb do clinicians use to decide whether to antagonize nondepolarizing neuromuscular blocking drugs?

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    Videira, Rogerio L R; Vieira, Joaquim E

    2011-11-01

    In anesthesia practice, inadequate antagonism of neuromuscular blocking drugs (NMBD) may lead to frequent prevalence of residual neuromuscular block that is associated with morbidity and death. In this study we analyzed the clinical decision on antagonizing NMBD to generate hypotheses about barriers to the introduction of experts' recommendations into clinical practice. Sequential surveys were conducted among 108 clinical anesthesiologists to elicit the rules of thumb (heuristics) that support their decisions and provide a measurement of the confidence the clinicians have in their own decisions in comparison with their peers' decisions. The 2 most frequently used heuristics for administering reversal were "the interval since the last NMBD dose was short" and "the breathing pattern is inadequate," chosen by 73% and 71% of the clinicians, respectively. Clinicians considered that the prevalence of clinically significant residual block is higher in their colleagues' practices than in their own practice (60% vs 16%, odds ratio=7.8, 95% confidence interval, 3.8 to 16.2, P=0.0001). The clinicians were less likely to use antagonists if >60 minutes had elapsed after a single dose of atracurium (0.5 mg/kg) (31%) in comparison with after rocuronium 0.6 mg/kg (53%) (P=0.0035). In our institution, the clinical decision to antagonize NMBD is mainly based on the pharmacological forecast and a qualitative judgment of the adequacy of the breathing pattern. Clinicians judge themselves as better skilled at avoiding residual block than they do their colleagues, making them overconfident in their capacity to estimate the duration of action of intermediate-acting NMBD. Awareness of these systematic errors related to clinical intuition may facilitate the adoption of experts' recommendations into clinical practice.

  20. A novel skeletal-myosin blocking drug for the study of neuromuscular physiology

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    Dante J Heredia

    2016-12-01

    Full Text Available The failure to transmit neural action potentials (APs into muscle APs is referred to as neuromuscular transmission failure (NTF. Although synaptic dysfunction occurs in a variety of neuromuscular diseases and impaired neurotransmission contributes to muscle fatigue, direct evaluation of neurotransmission by measurement of successfully transduced muscle APs is difficult due to the subsequent movements produced by muscle. Moreover, the voltage-gated sodium channel inhibitor used to study neurotransmitter release at the adult neuromuscular junction is ineffective in embryonic tissue, making it nearly impossible to precisely measure any aspect of neurotransmission in embryonic lethal mouse mutants. In this study we utilized 3-(N-butylethanimidoyl-4-hydroxy-2H-chromen-2-one (BHC, previously identified in a small-molecule screen of skeletal muscle myosin inhibitors, to suppress movements without affecting membrane currents. In contrast to previously characterized drugs from this screen such as BTS, which inhibit skeletal muscle myosin ATPase activity but also block neurotransmission, BHC selectively blocked nerve-evoked muscle contraction without affecting neurotransmitter release. This feature allowed a detailed characterization of neurotransmission in both embryonic and adult mice. In the presence of BHC, neural APs produced by tonic stimulation of the phrenic nerve at rates up to 20 Hz were successfully transmitted into muscle APs. At higher rates of phrenic nerve stimulation, NTF was observed. NTF was intermittent and characterized by successful muscle APs following failed ones, with the percentage of successfully transmitted muscle APs diminishing over time. Nerve stimulation rates that failed to produce NTF in the presence of BHC similarly failed to produce a loss of peak muscle fiber shortening, which was examined using a novel optical method of muscle fatigue, or a loss of peak cytosolic calcium transient intensity, examined in whole

  1. Deep neuromuscular block to optimize surgical space conditions during laparoscopic surgery: a systematic review and meta-analysis

    NARCIS (Netherlands)

    Bruintjes, M.H.D.; Helden, E.V. van; Braat, A.E.; Dahan, A; Scheffer, G.J.; Laarhoven, C.J.H.M. van; Warle, M.C.

    2017-01-01

    Neuromuscular block (NMB) is frequently used in abdominal surgery to improve surgical conditions by relaxation of the abdominal wall and prevention of sudden muscle contractions. The evidence supporting routine use of deep NMB is still under debate. We aimed to provide evidence for the superiority

  2. Differentiating the cellular and humoral components of neuromuscular blocking agent-induced anaphylactic reactions in patients undergoing anaesthesia

    NARCIS (Netherlands)

    Aalberse, R. C.; Kleine Budde, I.; Mulder, M.; Stapel, S. O.; Paulij, W.; Leynadier, F.; Hollmann, M. W.

    2011-01-01

    Background. The significance of IgE antibodies to neuromuscular blocking agent (NMBA)-induced anaphylactic reactions during anaesthesia is unclear. We investigated the relevance of IgE to rocuronium using an in vitro technique. Methods. Serum samples from 61 patients with anaphylactic reactions

  3. Predictors of the variability in neuromuscular block duration following succinylcholine: A prospective, observational study.

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    Dell-Kuster, Salome; Levano, Soledad; Burkhart, Christoph S; Lelais, Frédéric; Zemp, André; Schobinger, Elektra; Hampl, Karl; Kindler, Christoph; Girard, Thierry

    2015-10-01

    The duration of neuromuscular block (NMB) following succinylcholine administration is characterised by a high interindividual variability. However, this has not yet been quantified in a large sample of surgical patients. The significance of underlying clinical factors is unknown. The objective of this study was to profile the variability in NMB duration following a standard dose of succinylcholine and to investigate contributing clinical and genetic factors. A prospective, observational study. Tertiary referral centre. In a total of 1630 surgical patients undergoing a rapid sequence induction and intubation, clinical risk factors for a prolongation in NMB duration following succinylcholine were assessed. In a subset of 202 patients, additional biochemical and molecular genetic investigations of butyrylcholinesterase were performed. A standard 1 mg kg dose of succinylcholine after administration of an induction drug and an opioid. NMB duration measured as the time between administration of succinylcholine until reappearance of palpable muscular response to supramaximal transcutaneous ulnar nerve stimulation. NMB varied from 80 s to 44 min with a median duration of 7.3 min. Sixteen percent of patients had NMB duration in excess of 10 min. A multivariable survival model identified physical status, sex, age, hepatic disease, pregnancy, history of cancer and use of etomidate or metoclopramide as independent risk factors for a prolonged NMB. Three novel butyrylcholinesterase variants were identified: p.Ile5Thr; p.Val178Ile; and p.Try231Ser. Neuromuscular blockade duration in excess of 10 min occurred in 16% of a general surgical population following a single dose of succinylcholine. The multivariable model of clinical risk factors for prolonged NMB revealed a negative predictive value of 87%, thereby indicating that absence of such risk factors may reliably predict a shorter duration of NMB. In patients with clinical risk factors for a prolonged NMB or with

  4. A new approach to anesthesia management in myasthenia gravis: reversal of neuromuscular blockade by sugammadex.

    NARCIS (Netherlands)

    Boer, H.D. de; Egmond, J. van; Driessen, J.J.; Booij, L.H.D.J.

    2010-01-01

    A neuromuscular blocking drug (NMBD) induced neuromuscular blockade (NMB) in patients with myasthenia gravis usually dissipates either spontaneously or by administration of neostigmine. We administered sugammadex to a patient with myasthenia gravis to reverse a rocuronium-induced profound NMB. NMBDs

  5. Vagal sensory evoked potentials disappear under the neuromuscular block - an experimental study.

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    Leutzow, Bianca; Lange, Jörn; Gibb, Andreas; Schroeder, Henry; Nowak, Andreas; Wendt, Michael; Usichenko, Taras I

    2013-09-01

    Transcutaneous vagal nerve stimulation is a promising treatment modality in patients suffering mood disorders and chronic pain, however, the mechanisms are still to be elucidated. A recently developed technique of EEG responses to electrical stimulation of the inner side of the tragus suggests that these responses are far field potentials, generated in the vagal system - Vagal Sensory Evoked Potentials (VSEP). To reproduce the VSEP technique free from myogenic artifacts. Fourteen ASA I-II patients scheduled for elective surgery in standardized Total Intravenous Anesthesia (TIVA) were enrolled. Transcutaneous electrical stimulation was applied to the inner side of the right tragus. Averaged EEG responses were recorded from the electrode positions C4-F4 and T4-O2 before and after induction of TIVA, during the maximal effect of the non-depolarizing muscle relaxing agent, cis-atracurium (C-AR) and after recovery from C-AR under TIVA. Typical response curves with P1, N1 and P2 peaks could be reproduced in all patients before and after anesthesia induction. The response curves disappeared during the C-AR action and re-appeared after recovery from C-AR under TIVA. The disappearance of the scalp responses to electrical tragus stimulation under the neuromuscular block suggests a muscular origin of these potentials. Copyright © 2013 Elsevier Inc. All rights reserved.

  6. Natural polymeric 3-alkylpyridinium salt affects vertebrate skeletal muscle contractility by preferentially blocking neuromuscular transmission.

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    Žužek, Monika Cecilija; Grandič, Marjana; Benoit, Evelyne; Frangež, Robert

    2017-11-05

    The effects of natural polymeric alkylpyridinium salt (nPoly-3-APS), a potent acetylcholinesterase inhibitor isolated from the marine sponge Reniera sarai, were studied on isolated mouse phrenic nerve-hemidiaphragm muscle preparations using electrophysiological approaches. nPoly-3-APS inhibited nerve-evoked isometric muscle twitch and tetanic contraction in a concentration-dependent manner (IC50=29.4μM and 18.5μM, respectively) and produced a 30-44% decrease of directly muscle-elicited twitch and tetanus amplitudes at 54.4μM. Additionally, nPoly-3-APS (9.1-27.2μM) markedly decreased the amplitude of miniature endplate potentials, while their frequency was only affected at the highest concentration used. Endplate potentials were also inhibited by nPoly-3-APS in a concentration-dependent manner (IC50=20.1μM), without significant change in the resting membrane potential of muscle fibers (up to 54.4μM). In conclusion, our results show, for the first time, that nPoly-3-APS preferentially blocks the neuromuscular transmission, in vitro, by a non-depolarizing mechanism. This strongly suggests that the in vivo toxicity of nPoly-3-APS mainly occurs through an antagonist action of the compound on nicotinic acetylcholine receptors of skeletal muscles. Copyright © 2017 Elsevier B.V. All rights reserved.

  7. Avoidance versus use of neuromuscular blocking agents for improving conditions during tracheal intubation or direct laryngoscopy in adults and adolescents

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    Lundstrøm, Lars Hyldborg; Duez, Christophe Hv; Nørskov, Anders Kehlet

    2017-01-01

    . However, no systematic review of randomized trials has evaluated conditions for tracheal intubation, possible adverse effects, and postoperative discomfort. Objectives: To evaluate the effects of avoiding neuromuscular blocking agents (NMBA) versus using NMBA on difficult tracheal intubation (DTI...... and morbidity. Use of neuromuscular blocking agents (NMBA) to facilitate tracheal intubation is a widely accepted practice. However, because of adverse effects, NMBA may be undesirable. Cohort studies have indicated that avoiding NMBA is an independent risk factor for difficult and failed tracheal intubation...... to look for unidentified trials. Selection criteria: We included randomized controlled trials (RCTs) that compared the effects of avoiding versus using NMBA in participants 14 years of age or older. Data collection and analysis: Two review authors extracted data independently. We conducted random...

  8. Pholcodine exposure raises serum IgE in patients with previous anaphylaxis to neuromuscular blocking agents.

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    Harboe, T; Johansson, S G O; Florvaag, E; Oman, H

    2007-12-01

    Neuromuscular blocking agents (NMBAs) can cause anaphylaxis through immunoglobulin E (IgE) antibodies that bind quaternary ammonium ion epitopes. These epitopes are present in numerous common chemicals and drugs, exposure to which, theoretically, could be of importance in the development and maintenance of the IgE sensitization promoting allergic reactions. Pholcodine is one such drug, which in a recent pilot study was shown to induce a remarkable increase in serum IgE levels in two IgE-sensitized individuals. The present study explores the effect of pholcodine exposure on IgE in a population with previously diagnosed IgE-mediated anaphylaxis towards NMBAs. Seventeen patients were randomized to 1 week's exposure with cough syrup containing either pholcodine or guaifenesin. The primary variables serum IgE and IgE antibodies towards pholcodine, morphine and suxamethonium were measured before and 4 and 8 weeks after start of exposure. Patients exposed to pholcodine had a sharp rise in levels of IgE antibodies towards pholcodine, morphine and suxamethonium, the median proportional increases 4 weeks after exposure reaching 39.0, 38.6 and 93.0 times that of the base levels respectively. Median proportional increase of IgE was 19.0. No changes were observed in the guaifenesin group. Serum levels of IgE antibodies associated with allergy towards NMBAs increase significantly in sensitized patients after exposure to cough syrup containing pholcodine. Availability of pholcodine should be restricted by medical authorities because of the potential risk of future allergic reactions to muscle relaxants.

  9. [Deep versus moderate neuromuscular block during one-lung ventilation in lung resection surgery].

    Science.gov (United States)

    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.

  10. Prevalence of IgE against neuromuscular blocking agents in hairdressers and bakers.

    Science.gov (United States)

    Dong, S; Acouetey, D S; Guéant-Rodriguez, R-M; Zmirou-Navier, D; Rémen, T; Blanca, M; Mertes, P M; Guéant, J-L

    2013-11-01

    Allergic IgE-mediated reactions to neuromuscular blocking agents (NMBAs) are the main cause of immediate hypersensitivity reactions in anaesthesia; their predominant occurrence in the absence of previous exposure to NMBAs suggests a risk related to environmental exposure. To investigate the prevalence of specific IgE to quaternary ammonium ions in two populations professionally exposed to quaternary ammonium compounds, in the north-eastern France. The study had a retrospective follow-up design whereby apprentices were assessed after their 2-year training period as apprentices. The professionally exposed hairdresser populations (n = 128) were compared with baker/pastry makers (n = 108) and 'non-exposed' matched control subjects (n = 379). We observed a 4.6-fold higher frequency of positive IgE against quaternary ammonium ions in hairdressers (HD), compared with baker/pastry makers (BP) and control (C) groups. The competitive inhibition of quaternary ammonium Sepharose radioimmunoassay (QAS-IgE RIA) with succinylcholine was significantly higher in HD, compared with BP and C groups, with inhibition percentage of 66.2 ± 7.4, 39.7 ± 6.0 and 43.8 ± 9.9, respectively (P  100 kU/L were the two significant predictors of IgE-sensitization against quaternary ammonium ions in the multivariate analysis of a model that included age, sex, professional exposure, increased concentration of total IgE (IgE > 100 kU/L) and positive IgE against prevalent allergens (Phadiatop(®) ; P = 0.019 and P = 0.001, respectively). The exposure to hairdressing professional occupational factors increases IgE-sensitization to NMBAs and quaternary ammonium ion compounds used in hairdressing. Besides the pholcodine hypothesis, our study suggests that repetitive exposure to quaternary ammonium compounds used in hairdressing is a risk factor for NMBAs sensitization. © 2013 John Wiley & Sons Ltd.

  11. Assessment of the interaction between atracurium and suxamethonium at 50% neuromuscular block using closed-loop feedback control of infusion of atracurium.

    Science.gov (United States)

    Olkkola, K T; Tammisto, T

    1994-08-01

    We have studied the effect of prior administration of suxamethonium on the infusion requirements of atracurium at 50% neuromuscular block in patients undergoing elective general surgery. Anaesthesia was maintained with nitrous oxide in oxygen, propofol and fentanyl. Of 20 patients given atracurium, only 10 were given prior administration of suxamethonium 1 mg kg-1. At the beginning of the infusion, atracurium 0.3 mg kg-1 was given by bolus administration. Interaction between the two drugs was assessed by determining the steady state rate of infusion necessary to produce a constant 50% neuromuscular block. This was accomplished by applying non-linear curve fitting to data on the cumulative dose requirements during anaesthesia. The neuromuscular blocking effect was found to be similar with or without prior administration of suxamethonium. The mean steady-state rate of infusion for atracurium was 0.19 (SD 0.03) mg kg-1 h-1 for patients given suxamethonium and 0.18 (0.09) mg kg-1 h-1 for those who were not given suxamethonium. Thus prior administration of suxamethonium did not affect the infusion requirements of atracurium at 50% neuromuscular block, unlike the situation at constant 90% neuromuscular block.

  12. Pralidoxime inhibits paraoxon-induced depression of rocuronium-neuromuscular block in a time-dependent fashion.

    Science.gov (United States)

    Narimatsu, Eichi; Niiya, Tomohisa; Takahashi, Kazunobu; Yamauchi, Masanori; Yamakage, Michiaki

    2012-07-01

    The composite effects of organophosphorus (OP)-cholinesterase (ChE) inhibitors and oximes on the actions of nondepolarizing neuromuscular blockers in acute OP-ChE inhibitor intoxication have not been evaluated in detail. We investigated the effects of paraoxon (Pox) (an OP-ChE inhibitor) and pralidoxime (PAM) (an oxime) on the nondepolarizing neuromuscular blocking action of rocuronium. Isometric twitch tensions of rat left phrenic nerve-hemidiaphragm preparations elicited by indirect (phrenic nerve) supramaximal stimulation at 0.1 Hz were evaluated. Analysis of variance with post hoc testing was used for statistical comparison, and P fashion. Paraoxon caused a rightward shift in the rocuronium concentration-twitch tension curve (IC(50), 15.48 [15.24-15.72] μM). The rightward shift was completely inhibited by previous copretreatment (IC(50), 9.98 [9.77-10.20] μM) and partially inhibited by simultaneous copretreatment (IC(50), 11.68 [11.45-11.91] μM) with PAM but was not inhibited by subsequent copretreatment (IC(50), 13.69 [13.39-13.99] μM) with PAM (P influence the rightward shift (P < .01, n = 6). Paraoxon depressed rocuronium-induced neuromuscular block by inhibiting ChEs, and the action of Pox was inhibited by PAM. Pralidoxime acts more intensely when applied earlier. The time-dependent effect of PAM indicates that the preceding presence of PAM in proximity to ChEs before Pox is necessary for definite suppression of the Pox-induced ChE inhibition. Copyright © 2012 Elsevier Inc. All rights reserved.

  13. Neuromuscular blockade in cardiac surgery: An update for clinicians

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    Hemmerling Thomas

    2008-01-01

    Full Text Available There have been great advancements in cardiac surgery over the last two decades; the widespread use of off-pump aortocoronary bypass surgery, minimally invasive cardiac surgery, and robotic surgery have also changed the face of cardiac anaesthesia. The concept of "Fast-track anaesthesia" demands the use of nondepolarising neuromuscular blocking drugs with short duration of action, combining the ability to provide (if necessary sufficiently profound neuromuscular blockade during surgery and immediate re-establishment of normal neuromuscular transmission at the end of surgery. Postoperative residual muscle paralysis is one of the major hurdles for immediate or early extubation after cardiac surgery. Nondepolarising neuromuscular blocking drugs for cardiac surgery should therefore be easy to titrate, of rapid onset and short duration of action with a pathway of elimination independent from hepatic or renal dysfunction, and should equally not affect haemodynamic stability. The difference between repetitive bolus application and continuous infusion is outlined in this review, with the pharmacodynamic and pharmacokinetic characteristics of vecuronium, pancuronium, rocuronium, and cisatracurium. Kinemyography and acceleromyography are the most important currently used neuromuscular monitoring methods. Whereas monitoring at the adductor pollicis muscle is appropriate at the end of surgery, monitoring of the corrugator supercilii muscle better reflects neuromuscular blockade at more central, profound muscles, such as the diaphragm, larynx, or thoraco-abdominal muscles. In conclusion, cisatracurium or rocuronium is recommended for neuromuscular blockade in modern cardiac surgery.

  14. A discrete event simulation model of clinical and operating room efficiency outcomes of sugammadex versus neostigmine for neuromuscular block reversal in Canada.

    Science.gov (United States)

    Insinga, Ralph P; Joyal, Cédric; Goyette, Alexandra; Galarneau, André

    2016-11-16

    The objective of this analysis is to explore potential impact on operating room (OR) efficiency and incidence of residual neuromuscular blockade (RNMB) with use of sugammadex (Bridion™, Merck & Co., Inc., Kenilworth, NJ USA) versus neostigmine for neuromuscular block reversal in Canada. A discrete event simulation (DES) model was developed to compare ORs using either neostigmine or sugammadex for NMB reversal over one month. Selected inputs included OR procedure and turnover times, hospital policies for paid staff overtime and procedural cancellations due to OR time over-run, and reductions in RNMB and associated complications with sugammadex use. Trials show sugammadex's impact on OR time and RNMB varies by whether full neuromuscular recovery (train-of-four ratio ≥0.9) is verified prior to extubation in the OR. Scenarios were therefore evaluated reflecting varied assumptions for neuromuscular reversal practices. With use of moderate neuromuscular block, when full neuromuscular recovery is verified prior to extubation (93 procedures performed with sugammadex, 91 with neostigmine), use of sugammadex versus neostigmine avoided 2.4 procedural cancellations due to OR time over-run and 33.5 h of paid staff overtime, while saving an average of 62 min per OR day. No difference was observed between comparators for these endpoints in the scenario when full neuromuscular recovery was not verified prior to extubation, however, per procedure risk of RNMB at extubation was reduced from 60% to 4% (reflecting 51 cases prevented), with associated reductions in risks of hypoxemia (12 cases avoided) and upper airway obstruction (23 cases avoided). Sugammadex impact in reversing deep neuromuscular block was evaluated in an exploratory analysis. When it was hypothetically assumed that 30 min of OR time were saved per procedure, the number of paid hours of staff over-time dropped from 84.1 to 32.0, with a 93% reduction in the per patient risk of residual blockade. In clinical

  15. Thiadiazolodiazepine analogues as a new class of neuromuscular blocking agents: Synthesis, biological evaluation and molecular modeling study.

    Science.gov (United States)

    El-Subbagh, Hussein I; El-Azab, Adel S; Hassan, Ghada S; El-Messery, Shahenda M; Abdel-Aziz, Alaa A-M; El-Taher, Kamal E H

    2017-01-27

    The synthesis, biological evaluation and molecular modeling study of 6,7-dihydro-[1,3,4] thiadiazolo[3,2-a][1,3]diazepine analogues as new class of neuromuscular blocking agents are described. The new compounds act via competitive mechanism with ACh which could be reversed by the anticholinesterase - Physostigmine. Compounds GS-53 (30) and AAH1 (33) induced dose-dependent neuromuscular blockade with onset time of 3 and 10 min, ED50 0.15 and 0.36 mmol/kg i.p., respectively, in rats. Compound 30 proved to be as twice as potent as 33 with rapid onset and shorter duration (P Molecular modeling analysis indicated that hydrogen bonding to Thr120 and Thr124 beside hydrophobic interactions play effective role incorporating the active ligands to nAChR. The obtained model could be useful for further development of new skeletal muscle relaxants. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  16. Dependence of the adequacy of muscle relaxation on the degree of neuromuscular block and depth of enflurane anesthesia during abdominal surgery.

    Science.gov (United States)

    Tammisto, T; Olkkola, K T

    1995-03-01

    We studied the intensity of neuromuscular block that is adequate for surgical relaxation at different end-tidal levels of enflurane during N2O-O2-fentanyl anesthesia in 30 patients undergoing upper abdominal surgery. After induction of anesthesia with thiopental 4-6 mg/kg and vecuronium 0.07 mg/kg intravenously (i.v.), patients were randomly assigned to receive nitrous oxide-oxygen (2:1) and enflurane at 0.3% (Group I), 0.6% (Group II), or 1.2% (Group III) end-tidal level throughout anesthesia. The initial neuromuscular block was allowed to terminate and additional increments of 1 mg vecuronium were given when indicated by clinical signs or by spontaneous electromyography of neck muscles. In Group I additional vecuronium had to be given 62 times and in Groups II and III, 33, and 16 times, respectively. The mean (SD) neuromuscular block at the time of additional vecuronium was 75.9% +/- 20.7%, 62.5% +/- 20.1%, and 39.3% +/- 21.1% in Groups I to III, respectively. We conclude that there was a clear linear relationship between the end-tidal concentration of enflurane and the degree of neuromuscular block necessary to produce adequate surgical muscle relaxation (P < 0.001).

  17. Neuromuscular blocking agents in patients with acute respiratory distress syndrome: a summary of the current evidence from three randomized controlled trials

    NARCIS (Netherlands)

    Serpa Neto, Ary; Pereira, Victor Galvão Moura; Espósito, Daniel Crepaldi; Damasceno, Maria Cecília Toledo; Schultz, Marcus J.

    2012-01-01

    Background: Acute respiratory distress syndrome (ARDS) is a potentially fatal disease with high mortality. Our aim was to summarize the current evidence for use of neuromuscular blocking agents (NMBA) in the early phase of ARDS. Methods: Systematic review and meta-analysis of publications between

  18. Effects of avoiding neuromuscular blocking agents during maintenance of anaesthesia on recovery characteristics in patients undergoing craniotomy for supratentorial lesions: A randomised controlled study

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    Ruchi A Jain

    2017-01-01

    Full Text Available Background and Aims: Neuromuscular blocking agents have been one of the cornerstones of anaesthesia. With the advent of newer surgical, anaesthetic and neurological monitoring techniques, their utility in neuroanaesthesia practice seems dispensable. The aim of this prospective, comparative, randomised study was to determine whether neuromuscular blocking agents are required in patients undergoing supratentorial surgery when balanced anaesthesia with desflurane, dexmedetomidine and scalp block is used. Methods: Sixty patients with the American Society of Anesthesiologists physical status I or II, aged between 18 and 60 years were included in the study. All patients received anaesthesia including desflurane, dexmedetomidine and scalp block. The patients were randomly allocated to receive no neuromuscular blocking agent (Group A or atracurium infusion to keep train-of-four count 2 (Group B. The two groups were compared with respect to haemodynamic stability, brain relaxation scores and recovery characteristics. Haemodynamic parameters and time taken to achieve Aldrete score >9 and other secondary outcomes were analysed using Student's t-test. Non-parametric data were analysed using the Mann–Whitney test. Results: The mean arterial pressure was comparable between the groups. The intraoperative heart rate was comparable; however, in the post-operative period, it remained higher in Group B for 30 min after extubation (P = 0.02. The brain relaxation scores were comparable among the two groups (P = 0.27. Tracheal extubation time, time taken for orientation and time required to reach Aldrete score ≥9 were comparable among the two groups. Conclusion: The present study suggests that balanced anaesthesia using desflurane, dexmedetomidine and scalp block can preclude the use of neuromuscular blocking agents in patients undergoing supratentorial surgery under intense haemodynamic monitoring.

  19. Edrophonium antagonism of intense mivacurium-induced neuromuscular block in children.

    Science.gov (United States)

    Abdulatif, M; Al-Ghamdi, A; Al-Sanabary, M; Abdel-Gaffar, M E

    1996-02-01

    We have studied the time course of recovery after administration of edrophonium during intense mivacurium block in children aged 2-10 yr, using thumb acceleration in response to train-of-four (TOF) stimulation. Forty-three children receiving alfentanil, propofol, nitrous oxide, isoflurane anaesthesia and mivacurium 0.2 mg kg-1 were allocated randomly to one of three groups. Patients in group 1 (n = 15) received edrophonium 1 mg kg-1, 2 min after maximum block (intense block group). At the time of administration of edrophonium in this group, there was no response to TOF stimulation (100% block) and the post-tetanic count was 10.7 (range 0-20). Patients in group 2 received the same dose of edrophonium after 10% recovery of the first twitch (T1) in the TOF (conventional reversal). Patients in group 3 (n = 13) recovered spontaneously. All patients developed complete suppression of twitch height in response to the bolus dose of mivacurium. All recovery times were measured from the point of maximum block after mivacurium. Mean time for 25% recovery of T1 (clinical duration) was 3.8 (SD 1.1) min in the intense block group. This was significantly shorter than the conventional reversal (8.3 (2.4) min) and spontaneous recovery (9.2 (3.5) min) groups (P < 0.001). The times for 75% and 90% recovery of T1 were shorter in the intense block group (9.4 (2.8), 12.3 (4.2) min) compared with the conventional (13.1 (3.8), 17.3 (4.8) min) and spontaneous recovery (14.9 (4.5), 17.9 (5.2) min) groups (P < 0.01). Total recovery time required for 70% recovery of the TOF ratio (T4/T1) was 8.8 (2.4) min in the intense block group. This was significantly shorter than the conventional reversal (11.9 (3.2) min) (P < 0.05) and spontaneous recovery (17.1 (4.0) min) groups (P < 0.001). Conventional reversal was associated with a shorter total recovery time compared with spontaneous recovery (P < 0.01). The recovery index (time interval between T1 25% and 75%) was comparable in groups 1-3 (5.5 (2

  20. Association between intraoperative non-depolarising neuromuscular blocking agent dose and 30-day readmission after abdominal surgery.

    Science.gov (United States)

    Thevathasan, T; Shih, S L; Safavi, K C; Berger, D L; Burns, S M; Grabitz, S D; Glidden, R S; Zafonte, R D; Eikermann, M; Schneider, J C

    2017-10-01

    We hypothesised that intraoperative non-depolarising neuromuscular blocking agent (NMBA) dose is associated with 30-day hospital readmission. Data from 13,122 adult patients who underwent abdominal surgery under general anaesthesia at a tertiary care hospital were analysed by multivariable regression, to examine the effects of intraoperatively administered NMBA dose on 30-day readmission (primary endpoint), hospital length of stay, and hospital costs. Clinicians used cisatracurium (mean dose [SD] 0.19 mg kg-1 [0.12]), rocuronium (0.83 mg kg-1 [0.53]) and vecuronium (0.14 mg kg-1 [0.07]). Intraoperative administration of NMBAs was dose-dependently associated with higher risk of 30-day hospital readmission (adjusted odds ratio 1.89 [95% Confidence Interval (CI) 1.26-2.84] for 5th quintile vs 1st quintile; P for trend: Psurgery) significantly modified the risk (interaction term: aOR 1.31 [95% CI 1.05-1.63], P=0.02), and the adjusted odds of readmission in patients undergoing ambulatory surgical procedures who received high-dose NMBAs vs low-dose NMBAs amounted to 2.61 [95% CI 1.11-6.17], P for trend: Phigh doses of NMBAs given during abdominal surgery was associated with an increased risk of 30-day readmission, particularly in patients undergoing ambulatory surgery.

  1. Deep neuromuscular block to optimize surgical space conditions during laparoscopic surgery: a systematic review and meta-analysis.

    Science.gov (United States)

    Bruintjes, M H; van Helden, E V; Braat, A E; Dahan, A; Scheffer, G J; van Laarhoven, C J; Warlé, M C

    2017-06-01

    Neuromuscular block (NMB) is frequently used in abdominal surgery to improve surgical conditions by relaxation of the abdominal wall and prevention of sudden muscle contractions. The evidence supporting routine use of deep NMB is still under debate. We aimed to provide evidence for the superiority of routine use of deep NMB during laparoscopic surgery. We performed a systematic review and meta-analysis of studies comparing the influence of deep vs moderate NMB during laparoscopic procedures on surgical space conditions and clinical outcomes. Trials were identified from Medline, Embase, and Central databases from inception to December 2016. We included randomized trials, crossover studies, and cohort studies. Our search yielded 12 studies on the effect of deep NMB on the surgical space conditions. Deep NMB during laparoscopic surgeries improves the surgical space conditions when compared with moderate NMB, with a mean difference of 0.65 (95% confidence interval (CI): 0.47-0.83) on a scale of 1-5, and it facilitates the use of low-pressure pneumoperitoneum. Furthermore, deep NMB reduces postoperative pain scores in the postanaesthesia care unit, with a mean difference of - 0.52 (95% CI: -0.71 to - 0.32). Deep NMB improves surgical space conditions during laparoscopic surgery and reduces postoperative pain scores in the postanaesthesia care unit. Whether this leads to fewer intraoperative complications, an improved quality of recovery, or both after laparoscopic surgery should be pursued in future studies. The review methodology was specified in advance and registered at Prospero on July 27, 2016, registration number CRD42016042144. © The Author 2017. Published by Oxford University Press on behalf of the British Journal of Anaesthesia. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  2. Antibiotic-induced immediate type hypersensitivity is a risk factor for positive allergy skin tests for neuromuscular blocking agents.

    Science.gov (United States)

    Hagau, Natalia; Gherman, Nadia; Cocis, Mihaela; Petrisor, Cristina

    2016-01-01

    Skin tests for neuromuscular blocking agents (NMBAs) are not currently recommended for the general population undergoing general anaesthesia. In a previous study we have reported a high incidence of positive allergy tests for NMBAs in patients with a positive history of non-anaesthetic drug allergy, a larger prospective study being needed to confirm those preliminary results. The objective of this study was to compare the skin tests results for patients with a positive history of antibiotic-induced immediate type hypersensitivity reactions to those of controls without drug allergies. Ninety eight patients with previous antibiotic hypersensitivity and 72 controls were prospectively included. Skin tests were performed for atracurium, pancuronium, rocuronium, and suxamethonium. We found 65 positive skin tests from the 392 tests performed in patients with a positive history of antibiotic hypersensitivity (1 6.58%) and 23 positive skin tests from the 288 performed in controls (7.98%), the two incidences showing significant statistical difference (p = 0.0011). The relative risk for having a positive skin test for NMBAs for patients versus controls was 1.77 (1.15-2.76). For atracurium, skin tests were more often positive in patients with a positive history of antibiotic hypersensitivity versus controls (p = 0.02). For pancuronium, rocuronium and suxamethonium the statistical difference was not attained (p-values 0.08 for pancuronium, 0.23 for rocuronium, and 0.26 for suxamethonium). Patients with a positive history of antibiotic hypersensitivity seem to have a higher incidence of positive skin tests for NMBAs. They might represent a group at higher risk for developing intraoperative anaphylaxis compared to the general population. Copyright © 2015 The Authors. Production and hosting by Elsevier B.V. All rights reserved.

  3. Static balance and function in children with cerebral palsy submitted to neuromuscular block and neuromuscular electrical stimulation: study protocol for prospective, randomized, controlled trial.

    Science.gov (United States)

    Kazon, Soráia; Grecco, Luanda A C; Pasini, Hugo; Corrêa, João C F; Christovão, Thaluanna C L; de Carvalho, Paulo de Tarsocamillo; Giannasi, Lilian Chrystiane; Lucareli, Paulo R G; de Oliveira, Luis Vicente Franco; Salgado, Afonso Shiguemi Inoue; Sampaio, Luciana M M; Oliveira, Claudia S

    2012-05-16

    The use of botulinum toxin A (BT-A) for the treatment of lower limb spasticity is common in children with cerebral palsy (CP). Following the administration of BT-A, physical therapy plays a fundamental role in potentiating the functionality of the child. The balance deficit found in children with CP is mainly caused by muscle imbalance (spastic agonist and weak antagonist). Neuromuscular electrical stimulation (NMES) is a promising therapeutic modality for muscle strengthening in this population. The aim of the present study is to describe a protocol for a study aimed at analyzing the effects of NMES on dorsiflexors combined with physical therapy on static and functional balance in children with CP submitted to BT- A. Protocol for a prospective, randomized, controlled trial with a blinded evaluator. Eligible participants will be children with cerebral palsy (Levels I, II and III of the Gross Motor Function Classification System) between five and 12 years of age, with independent gait with or without a gait-assistance device. All participants will receive BT-A in the lower limbs (triceps surae). The children will then be randomly allocated for either treatment with motor physical therapy combined with NMES on the tibialis anterior or motor physical therapy alone. The participants will be evaluated on three occasions: 1) one week prior to the administration of BT-A; 2) one week after the administration of BT-A; and 3) four months after the administration of BT-A (end of intervention). Spasticity will be assessed by the Modified Ashworth Scale and Modified Tardieu Scale. Static balance will be assessed using the Medicapteurs Fusyo pressure platform and functional balance will be assessed using the Berg Balance Scale. The aim of this protocol study is to describe the methodology of a randomized, controlled, clinical trial comparing the effect of motor physical therapy combined with NMES on the tibialis anterior muscle or motor physical therapy alone on static and

  4. Static balance and function in children with cerebral palsy submitted to neuromuscular block and neuromuscular electrical stimulation: Study protocol for prospective, randomized, controlled trial

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    Kazon Soráia

    2012-05-01

    Full Text Available Abstract Background The use of botulinum toxin A (BT-A for the treatment of lower limb spasticity is common in children with cerebral palsy (CP. Following the administration of BT-A, physical therapy plays a fundamental role in potentiating the functionality of the child. The balance deficit found in children with CP is mainly caused by muscle imbalance (spastic agonist and weak antagonist. Neuromuscular electrical stimulation (NMES is a promising therapeutic modality for muscle strengthening in this population. The aim of the present study is to describe a protocol for a study aimed at analyzing the effects of NMES on dorsiflexors combined with physical therapy on static and functional balance in children with CP submitted to BT- A. Methods/Design Protocol for a prospective, randomized, controlled trial with a blinded evaluator. Eligible participants will be children with cerebral palsy (Levels I, II and III of the Gross Motor Function Classification System between five and 12 years of age, with independent gait with or without a gait-assistance device. All participants will receive BT-A in the lower limbs (triceps surae. The children will then be randomly allocated for either treatment with motor physical therapy combined with NMES on the tibialis anterior or motor physical therapy alone. The participants will be evaluated on three occasions: 1 one week prior to the administration of BT-A; 2 one week after the administration of BT-A; and 3 four months after the administration of BT-A (end of intervention. Spasticity will be assessed by the Modified Ashworth Scale and Modified Tardieu Scale. Static balance will be assessed using the Medicapteurs Fusyo pressure platform and functional balance will be assessed using the Berg Balance Scale. Discussion The aim of this protocol study is to describe the methodology of a randomized, controlled, clinical trial comparing the effect of motor physical therapy combined with NMES on the tibialis anterior

  5. Influência do sevoflurano e do isoflurano na recuperação do bloqueio neuromuscular produzido pelo cisatracúrio Influencia del sevoflurano y del isoflurano en la recuperación del bloqueo neuromuscular producido por el cisatracúrio The influence of sevoflurane and isoflurane on the recovery from cisatracurium-induced neuromuscular block

    Directory of Open Access Journals (Sweden)

    Angélica de Fátima de Assunção Braga

    2002-09-01

    índice de recuperación (T1(25-75% fueron respectivamente: Grupo I (66,2 ± 13,42 min y 23,6 ± 5,02 min, Grupo II (54,4 ± 6,58 min y 14,9 ± 3,82 min y Grupo III (47,2 ± 7,43 y 16,2 ± 2,93. En relación a la duración clínica hubo diferencia significante entre los grupos I y II, I y III, y II y III. Para el índice de recuperación hubo diferencia significante entre el grupo I y los demás grupos. CONCLUSIONES: La recuperación del bloqueo neuromuscular producido por el cisatracúrio fue más lenta durante la anestesia con los agentes volátiles de que con el propofol, siendo el efecto más pronunciado con el sevoflurano.BACKGROUND AND OBJECTIVES: The effects of neuromuscular blockers on the neuromuscular junction are potentiated by volatile anesthetics. This study aimed at evaluating the influence of sevoflurane and isoflurane on the recovery of cisatracurium- induced neuromuscular block. METHODS: Ninety ASA I and II patients undergoing elective surgeries under general anesthesia were included in this study. Patients were allocated in three groups: Group I (sevoflurane, Group II (isoflurane and Group III (propofol. All patients were premedicated with intramuscular midazolam (0.1 mg.kg-1 30 min before surgery. Anesthesia was induced with alfentanil (50 µg.kg-1, propofol (2.5 mg.kg-1 and cisatracurium (0.15 mg.kg-1. Patients were then ventilated under mask with 100% O2 until disappearance of all TOF responses when laryngoscopy and tracheal intubation were performed. Volatile agents for anesthetic maintenance were introduced immediately after tracheal intubation in 2% and 1% concentrations, respectively, for sevoflurane and isoflurane, as well as the propofol continuous infusion (7 to 10 mg.kg-1.h-1 for Group III. All patients received a 50% mixture of O2 and N2O. Neuromuscular function was monitored by adductor pollicis muscle acceleromyography with TOF stimulation at 15-second intervals. Clinical duration of neuromuscular block (T1(25% and recovery index (RI

  6. PHARMACODYNAMICS AND PHARMACOKINETICS OF AN INFUSION OF ORG-9487, A NEW SHORT-ACTING STEROIDAL NEUROMUSCULAR BLOCKING-AGENT

    NARCIS (Netherlands)

    VANDENBROEK, L; WIERDA, JMKH; SMEULERS, NJ; PROOST, JH

    We have evaluated in 10 anaesthetized patients the time course of action, infusion requirements, reversibility and pharmacokinetics of Org 9487. Org 9487 was administered as a bolus dose of 1.5 mg kg(-1), followed by an infusion to maintain a block of 75-85 % for 60 min. After recovery from the

  7. Influência da lidocaína no bloqueio neuromuscular produzido pelo rocurônio: estudo em preparação nervo frênico-diafragma de rato Influencia de la lidocaína en el bloqueo neuromuscular producido por el rocuronio: estudio en preparación nervio frénico-diafragma de ratón Influence of lidocaine on the neuromuscular block produced by rocuronium: study in rat phrenic-diaphragmatic nerve preparation

    Directory of Open Access Journals (Sweden)

    Yolanda Christina S. Loyola

    2006-04-01

    el antagonismo parcial de neostigmina.BACKGROUND AND OBJECTIVES: The action mechanism of local anesthetics (LA on neuromuscular junction motivated several studies. When administered at low doses, they do not interfere on neuromuscular transmission. But high doses may compromise neuromuscular transmission and increase the effects of neuromuscular blockers. The objective of this study was to evaluate lidocaine interaction with rocuronium on rat diaphragm through its influence on neuromuscular block degree. METHODS: Rats, weighing between 250 and 300 g, were used. Preparation was set according to the technique described by Bulbring. Groups were formed (n = 5 according to the drug being studied: lidocaine - 20 µg.mL-1 (Group I; rocuronium - 4 µg.mL-1 (Group II, and rocuronium - 4 µg.mL-1 with lidocaine - 20 µg.mL-1 (Group III. The following items were assessed: 1 the extent of diaphragm muscle responses to indirect stimulation, both before and 60 minutes after adding lidocaine and a neuromuscular blocker; 2 membrane potentials (MP and miniature end-plate potentials (MEPP; 3 the effectiveness of neostigmine, and 4 aminopyridine on neuromuscular blockage reversal. RESULTS: When administered separately, lidocaine did not alter the extent of muscular responses. With the previous use of lidocaine, rocuronium neuromuscular blockage was 82.8% ± 1.91%, with a significant difference (p = 0.0079 when compared to the group with isolated rocuronium (57.8% ± 1.9%. Blockage was both partially and fully reverted by neostigmine and 4-aminopyridine, respectively. Lidocaine did not alter membrane potential and caused an initial increase on MEPP, followed by a blockage. CONCLUSIONS: Lidocaine increases the neuromuscular blocking produced by rocuronium. MEPP modifications identify a presynaptic action. The complete antagonism of 4-aminopyridine indicates a presynaptic component. This idea is supported by the partial antagonism through neostigmine.

  8. Efeito da administração do atracúrio sobre a recuperação do bloqueio neuromuscular induzido pelo pancurônio Efecto de la administración del atracúrio sobre la recuperación del bloqueo neuromuscular inducido por el pancuronio Effect of atracurium on pancuronium-induced neuromuscular block recovery

    Directory of Open Access Journals (Sweden)

    Luís Fernando Rodrigues Maria

    2004-06-01

    complementación con atracúrio no promovió alteración en la recuperación espontanea inicial del bloqueo neuromuscular inducido por el pancuronio y promovió diminución de 20% en el tiempo de recuperación total.BACKGROUND AND OBJECTIVES: Additional neuromuscular blocker doses are in general needed during wall closing after abdominal surgeries. This study aimed at determining during partial pancuronium-induced neuromuscular block recovery, the effect of additional atracurium dose on spontaneous neuromuscular block recovery. METHODS: Participated in this study 30 patients divided in two groups: pancuronium group (n = 14 and atracurium group (n = 16. Neuromuscular function was continuously monitored by accelerometry of abductor pollicis muscle using TOF to supramaximally stimulate ulnar nerve. Anesthesia was induced with propofol, fentanyl and 0.08 mg.kg-1 pancuronium, and was maintained with 60% N2O in oxygen and 0.5% isoflurane expired concentration. When T1 returned to 25% of control, 0.025 mg.kg-1 pancuronium or 0.20 mg.kg-1 atracurium were administered to pancuronium or atracurium group, respectively. Time for spontaneous T1 recovery = 10%, 25%, 75% of recovery index (RI 25-75% and time to T4/T1 equal 0.8 after complementary dose, were recorded. RESULTS: There were no differences between groups on T1 spontaneous recovery to 10% (45.00 ± 15.50 vs. 49.69 ± 9.41, 25% (61.64 ± 18.58 vs. 64.25 ± 12.51 and 75% (94.00 ± 28.52 vs. 84.69 ± 16.50. Recovery index (RI 25-75% and time to T4/T1= 0.8 were shorter in the atracurium group. CONCLUSIONS: In this study, atracurium complementation has made no difference in initial spontaneous recovery of pancuronium-induced neuromuscular block, but has decreased total recovery time in 20%.

  9. Double-blind comparison of the variability in spontaneous recovery of cisatracurium- and vecuronium-induced neuromuscular block in adult and elderly patients.

    Science.gov (United States)

    Pühringer, F K; Heier, T; Dodgson, M; Erkola, O; Goonetilleke, P; Hofmockel, R; Gaetke, M R; Mortensen, C R; Upadhyaya, B; Eriksson, L I

    2002-04-01

    This study was designed to compare variability in the offset of two neuromuscular blocking agents with different elimination pathways. The spontaneous recovery profiles of cisatracurium and vecuronium were compared in adult (18-64 years) and elderly (> or =65 years) patients receiving N2O/O2/fentanyl/propofol anaesthesia. Patients were randomised to receive an initial bolus dose and maintenance doses of 3xED95, respectively, 0.6xED95 for cisatracurium (0.15 and 0.03 mg.kg-1) or 2xED95, respectively, and 0.4xED95 for vecuronium (0.1 and 0.02 mg.kg(-1)), as recommended in their prescribing information. Administration of the study drugs was double-blinded, and neuromuscular transmission was monitored using mechanomyography of the evoked response of the adductor pollicis, following ulnar nerve stimulation. The clinically effective duration (minutes) of the initial bolus dose, defined as the mean time to 25% T1 recovery (+/-SD), for the adult and elderly patients was 53.5+/-9.8 and 57.3+/-11.5 for cisatracurium, respectively, and 34.1+/-9.0 and 47.5+/-14.4 for vecuronium, respectively. The duration of spontaneous sufficient recovery (SSR), defined as the mean (+/-SD) time interval in minutes from 25% T1 recovery to a T4:T1 ratio > or =0.8 after the last bolus dose, for the adult, respectively, elderly patients was 28.3+/-8.0 and 31.7+/-10.0 for cisatracurium and 38.5+/-13.2 and 60.3+/-26.1 for vecuronium. Whereas both the clinically effective duration and the duration of SSR are comparable between the adult and the elderly patients receiving cisatracurium, they differ substantially between these two age groups for vecuronium. Furthermore, the variability in offset is significantly lower in patients receiving cisatracurium, especially in the elderly, which may be of particular clinical interest.

  10. What is profound?

    DEFF Research Database (Denmark)

    Sobisch, Jan-Ulrich

    2013-01-01

    Discussing the question, which elements on the path are to be considered profound. While a general view is that the most subtle practises are also the most profound, 'Jig-rten-mgon-po maintains that the most fundamental one's are to be considered the most profound.......Discussing the question, which elements on the path are to be considered profound. While a general view is that the most subtle practises are also the most profound, 'Jig-rten-mgon-po maintains that the most fundamental one's are to be considered the most profound....

  11. Reclassifying Anaphylaxis to Neuromuscular Blocking Agents Based on the Presumed Patho-Mechanism: IgE-Mediated, Pharmacological Adverse Reaction or “Innate Hypersensitivity”?

    Directory of Open Access Journals (Sweden)

    David Spoerl

    2017-06-01

    Full Text Available Approximately 60% of perioperative anaphylactic reactions are thought to be immunoglobulin IgE mediated, whereas 40% are thought to be non-IgE mediated hypersensitivity reactions (both considered non-dose-related type B adverse drug reactions. In both cases, symptoms are elicited by mast cell degranulation. Also, pharmacological reactions to drugs (type A, dose-related may sometimes mimic symptoms triggered by mast cell degranulation. In case of hypotension, bronchospasm, or urticarial rash due to mast cell degranulation, identification of the responsible mechanism is complicated. However, determination of the type of the underlying adverse drug reaction is of paramount interest for the decision of whether the culprit drug may be re-administered. Neuromuscular blocking agents (NMBA are among the most frequent cause of perioperative anaphylaxis. Recently, it has been shown that NMBA may activate mast cells independently from IgE antibodies via the human Mas-related G-protein-coupled receptor member X2 (MRGPRX2. In light of this new insight into the patho-mechanism of pseudo-allergic adverse drug reactions, in which as drug-receptor interaction results in anaphylaxis like symptoms, we critically reviewed the literature on NMBA-induced perioperative anaphylaxis. We challenge the dogma that NMBA mainly cause IgE-mediated anaphylaxis via an IgE-mediated mechanism, which is based on studies that consider positive skin test to be specific for IgE-mediated hypersensitivity. Finally, we discuss the question whether MRGPRX2 mediated pseudo-allergic reactions should be re-classified as type A adverse reactions.

  12. Influência da nifedipina no bloqueio neuromuscular produzido pelo atracúrio e pelo cisatracúrio: estudo em preparações nervo frênico-diafragma de rato Influencia de la nifedipina en el bloqueo neuromuscular producido por atracurio y cisatracurio: estudio en preparación nervio frénico diafragma de ratón Influence of nifedipine on the neuromuscular block produced by atracurium and cistracurium: study in rat phrenic-diaphragmatic nerve preparation

    Directory of Open Access Journals (Sweden)

    Silmara Rodrigues de Sousa

    2006-04-01

    significantly increase the neuromuscular blocking activity of atracurium and cistracurium. Nifedipine did not alter the membrane potential and caused an initial increase on MEPP frequencies, followed by a blockage. CONCLUSIONS: Nifedipine, on the employed concentration, increased the neuromuscular blockage produced by atracurium and cistracurium. Electrophysiological studies demonstrate the existence of presynaptic action and absence of depolarizing action over the muscle fiber.

  13. Neuromuscular disorders

    OpenAIRE

    Reed, Umbertina C.

    2002-01-01

    Objetivo: apresentar os dados essenciais para o diagnóstico diferencial entre as principais doenças neuromusculares, denominação genérica sob a qual agrupam-se diferentes afecções, decorrentes do acometimento primário da unidade motora (motoneurônio medular, raiz nervosa, nervo periférico, junção mioneural e músculo). Fontes dos dados: os aspectos clínicos fundamentais para estabelecer o diagnóstico diferencial entre as diferentes doenças neuromusculares, bem como entre estas e as causas de h...

  14. Sugammadex Improves Neuromuscular Function in Patients ...

    African Journals Online (AJOL)

    2018-02-23

    Feb 23, 2018 ... with a modified gamma-cyclodextrin structure offers a viable alternative to the traditional decurarization by cholinesterase inhibitors in the context of the use of steroidal neuromuscular blocking agents. Sugammadex shows its effects through encapsulation of the steroidal neuromuscular blockers, its effects ...

  15. Neuromuscular Scoliosis

    Science.gov (United States)

    ... to the optimal care of patients with spinal deformity Patients and Families Professionals About SRS Türkçe español ... unable to maintain appropriate balance / alignment of the spine and trunk. Neuromuscular curves are often associated with ...

  16. Influência dos Hipnóticos no bloqueio neuromuscular produzido pelo cisatracúrio: emprego da aceleromiografia Influencia de los hipnóticos en el bloqueo neuromuscular producido por el cisatracurio: uso de la aceleromiografía Influence of hypnotics on cisatracurium-induced neuromuscular block: use of acceleromyograhpy

    OpenAIRE

    Angélica de Fátima de Assunção Braga; Franklin Sarmento da Silva Braga; Glória Maria Braga Potério; José Aristeu Fachini Frias; Fernanda Maria Silva Pedro; Derli Conceição Munhoz

    2013-01-01

    JUSTIFICATIVA E OBJETIVOS: Os efeitos farmacodinâmicos dos bloqueadores neuromusculares (BNM) podem ser influenciados por diferentes drogas, entre elas os hipnóticos. O objetivo deste estudo foi avaliar a influência do propofol e do etomidato sobre o bloqueio neuromuscular produzido pelo cisatracúrio. MÉTODO: Foram incluídos 60 pacientes, ASA I e II, submetidos a cirurgias eletivas sob anestesia geral, distribuídos aleatoriamente em dois grupos de acordo com o hipnótico empregado: GI (propofo...

  17. Avoidance of neuromuscular blocking agents may increase the risk of difficult tracheal intubation: a cohort study of 103,812 consecutive adult patients recorded in the Danish Anaesthesia Database

    DEFF Research Database (Denmark)

    Lundstrøm, L H; Møller, A M; Rosenstock, Charlotte Vallentin

    2009-01-01

    BACKGROUND: Previous studies indicate that avoiding neuromuscular blocking agents (NMBAs) may be a risk factor for difficult tracheal intubation (DTI). We investigated whether avoiding NMBA was associated with DTI. METHODS: A cohort of 103,812 consecutive patients planned for tracheal intubation...... by direct laryngoscopy was retrieved from the Danish Anaesthesia Database. We used an intubation score based upon the number of attempts, change from direct laryngoscopy to a more advanced technique, or intubation by a different operator. We retrieved data on age, sex, ASA physical status classification......, priority of surgery, time of surgery, previous DTI, modified Mallampati score, BMI, and the use of NMBA. Using logistic regression, we assessed whether avoiding NMBA was associated with DTI. RESULTS: The frequency of DTI was 5.1 [95% confidence interval (CI): 5.0-5.3]%. In a univariate analysis, avoiding...

  18. Influência da freqüência de estímulos na instalação do bloqueio neuromuscular produzido pelo rocurônio e pancurônio: avaliação pelo método acelerográfico Influencia de la frecuencia de estímulos en la instalación del bloqueo neuromuscular producido por el rocuronio y pancuronio: evaluación por el método acelerográfico Influence of stimulation frequency on rocuronium and pancuronium-induced neuromuscular block onset: acceleromyography evaluation

    Directory of Open Access Journals (Sweden)

    Derli da Conceição Munhóz

    2004-02-01

    condiciones de intubación traqueal. RESULTADOS: Los tiempos medios (segundos para el inicio de acción e instalación de bloqueo neuromuscular total producido por el pancuronio fueron: Grupo I (159,33 ± 35,22 y 222 ± 46,56 y Grupo II (77,83 ± 9,52 y 105,96 ± 15,58; para el rocuronio: Grupo I (83 ± 17,25 y 125,33 ± 20,12 y Grupo II (48,96 ± 10,16 y 59,83 ± 10,36 con diferencia significativa entre los grupos. Las condiciones de intubación traqueal fueron satisfactorias en 117 pacientes (97,5% e insatisfactorias en 3 (2,5%. CONCLUSIONES: El inicio de acción y el tiempo para la obtención del bloqueo neuromuscular total en el músculo aductor del pulgar, producidos por el rocuronio y por el pancuronio, son más cortos cuando hay empleo de mayores frecuencias de estímulos.BACKGROUND AND OBJECTIVES: Factors associated to patients and neuromuscular blockers (NMB, as well as others inherent to neuromuscular function monitoring, may affect neuromuscular block onset. This study aimed at the influence of two different stimulation frequencies on rocuronium and pancuronium-induced neuromuscular block. METHODS: Participated in this study 120 patients, physical status ASA I and II, submitted to elective procedures under general anesthesia, who were randomly allocated in two groups, according to the stimulation frequency employed to monitor neuromuscular block: Group I - 0.1 Hz (n = 60 and Group II - 1 Hz (n = 60. Two subgroups were formed within each group (n = 30, according to the neuromuscular blocker: Subgroup P (pancuronium and Subgroup R (rocuronium. Patients were premedicated with muscular midazolam (0.1 mg.kg-1, 30 minutes before surgery. Anesthesia was induced with propofol (2.5 mg.kg-1 preceded by alfentanil (50 µg.kg-1 and followed by pancuronium or rocuronium. Patients were ventilated under mask with 100% oxygen until 75% or more decrease in adductor pollicis muscle response, when laryngoscopy and tracheal intubation were performed. Neuromuscular function was

  19. Influência dos Hipnóticos no bloqueio neuromuscular produzido pelo cisatracúrio: emprego da aceleromiografia Influencia de los hipnóticos en el bloqueo neuromuscular producido por el cisatracurio: uso de la aceleromiografía Influence of hypnotics on cisatracurium-induced neuromuscular block: use of acceleromyograhpy

    Directory of Open Access Journals (Sweden)

    Angélica de Fátima de Assunção Braga

    2013-06-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: Os efeitos farmacodinâmicos dos bloqueadores neuromusculares (BNM podem ser influenciados por diferentes drogas, entre elas os hipnóticos. O objetivo deste estudo foi avaliar a influência do propofol e do etomidato sobre o bloqueio neuromuscular produzido pelo cisatracúrio. MÉTODO: Foram incluídos 60 pacientes, ASA I e II, submetidos a cirurgias eletivas sob anestesia geral, distribuídos aleatoriamente em dois grupos de acordo com o hipnótico empregado: GI (propofol e GII (etomidato. As pacientes receberam midazolam (0,1 mg.kg-1 por via muscular como medicação pré-anestésica, a indução foi com propofol (2,5 mg.kg-1 ou etomidato (0,3 mg.kg-1 precedido de fentanil (250 µg e seguido de cisatracúrio (0,1 mg.kg-1. Os pacientes foram ventilados com oxigênio a 100% até a obtenção de redução de 95% ou mais na amplitude da resposta do adutor do polegar, quando foi feita a laringoscopia e a intubação traqueal. A função neuromuscular foi monitorizada com aceleromiografia. Avaliaram-se o início de ação do cisatracúrio, as condições de intubação traqueal e as repercussões hemodinâmicas. RESULTADOS: Os tempos médios e os desvios padrão para o início de ação do cisatracúrio foram: GI (86,6 ± 14,3" e GII (116,9 ± 11,6", com diferença significativa (p JUSTIFICATIVA Y OBJETIVOS: Los efectos farmacodinámicos de los bloqueantes neuromusculares (BNM pueden estar influenciados por diferentes fármacos, entre ellos los hipnóticos. El objetivo de este estudio, fue evaluar la influencia del propofol y del etomidato sobre el bloqueo neuromuscular producido por el cisatracurio. MÉTODO: Se incluyeron en el estudio 60 pacientes, con ASA I y II, sometidos a cirugías electivas bajo anestesia general, distribuidos aleatoriamente en dos grupos de acuerdo con el hipnótico usado: GI (propofol y GII (etomidato. Las pacientes recibieron midazolam (0,1 mg.kg-1 por vía muscular como medicación preanest

  20. Influence of intense neuromuscular blockade on surgical conditions during laparotomy

    DEFF Research Database (Denmark)

    Madsen, Matias Vested; Donatsky, Anders Meller; Jensen, Bente Rona

    2015-01-01

    endotracheally intubated, mechanically ventilated, anesthetized with propofol and fentanyl, and randomized into two groups in a cross-over assessor-blinded design. Neuromuscular block was established with rocuronium. Artificial laparotomy for ileus was performed. We investigated the influence of intense......PURPOSE: Intense neuromuscular block may improve surgical conditions in ileus laparotomies; however, it is difficult to evaluate. The aim of this study was to investigate if neuromuscular block improved surgical conditions in pigs with artificial ileus laparotomy. METHODS: Six pigs were...... neuromuscular block on surgical conditions with a subjective rating scale, force needed to close the fascia, incidences of abdominal contractions while suctioning the lungs, width of the wound diastase and operating time as outcome parameters. RESULTS: In all six pigs no abdominal contractions occurred while...

  1. Profound Olfactory Dysfunction in Myasthenia Gravis

    Science.gov (United States)

    Leon-Sarmiento, Fidias E.; Bayona, Edgardo A.; Bayona-Prieto, Jaime; Osman, Allen; Doty, Richard L.

    2012-01-01

    In this study we demonstrate that myasthenia gravis, an autoimmune disease strongly identified with deficient acetylcholine receptor transmission at the post-synaptic neuromuscular junction, is accompanied by a profound loss of olfactory function. Twenty-seven MG patients, 27 matched healthy controls, and 11 patients with polymiositis, a disease with peripheral neuromuscular symptoms analogous to myasthenia gravis with no known central nervous system involvement, were tested. All were administered the University of Pennsylvania Smell Identification Test (UPSIT) and the Picture Identification Test (PIT), a test analogous in content and form to the UPSIT designed to control for non-olfactory cognitive confounds. The UPSIT scores of the myasthenia gravis patients were markedly lower than those of the age- and sex-matched normal controls [respective means (SDs) = 20.15 (6.40) & 35.67 (4.95); p<0.0001], as well as those of the polymiositis patients who scored slightly below the normal range [33.30 (1.42); p<0.0001]. The latter finding, along with direct monitoring of the inhalation of the patients during testing, implies that the MG-related olfactory deficit is unlikely due to difficulties sniffing, per se. All PIT scores were within or near the normal range, although subtle deficits were apparent in both the MG and PM patients, conceivably reflecting influences of mild cognitive impairment. No relationships between performance on the UPSIT and thymectomy, time since diagnosis, type of treatment regimen, or the presence or absence of serum anti-nicotinic or muscarinic antibodies were apparent. Our findings suggest that MG influences olfactory function to the same degree as observed in a number of neurodegenerative diseases in which central nervous system cholinergic dysfunction has been documented. PMID:23082113

  2. Doenças neuromusculares Neuromuscular disorders

    Directory of Open Access Journals (Sweden)

    Umbertina C. Reed

    2002-08-01

    Full Text Available Objetivo: apresentar os dados essenciais para o diagnóstico diferencial entre as principais doenças neuromusculares, denominação genérica sob a qual agrupam-se diferentes afecções, decorrentes do acometimento primário da unidade motora (motoneurônio medular, raiz nervosa, nervo periférico, junção mioneural e músculo. Fontes dos dados: os aspectos clínicos fundamentais para estabelecer o diagnóstico diferencial entre as diferentes doenças neuromusculares, bem como entre estas e as causas de hipotonia muscular secundária ao comprometimento do sistema nervoso central ou a doenças sistêmicas não-neurológicas, são enfatizados, com base na experiência clínica vinda do atendimento a crianças com doenças neuromusculares durante os últimos 12 anos, no ambulatório de doenças neuromusculares do Hospital das Clínicas da Faculdade de Medicina, da Universidade de São Paulo. A revisão bibliográfica foi efetuada através do Medline e do periódico Neuromuscular Disorders, publicação oficial da World Muscle Society. Síntese dos dados: nas crianças, a maior parte destas afecções é geneticamente determinada, sendo as mais comuns a distrofia muscular progressiva ligada ao sexo, de Duchenne, a amiotrofia espinal infantil, a distrofia muscular congênita, a distrofia miotônica de Steinert, e as miopatias congênitas, estruturais e não estruturais. Polineuropatias hereditárias, síndrome miastênica congênita e miopatias metabólicas são menos comuns, porém mostram correlação geno-fenotípica cada vez mais precisa. Conclusões: na década passada, inúmeros avanços da genética molecular facilitaram imensamente o diagnóstico e o aconselhamento genético das doenças neuromusculares mais comuns das crianças, inclusive possibilitando diagnóstico fetal e, adicionalmente, vieram permitir melhor caracterização fenotípica e classificação mais objetiva.Objective: to discuss the most important aspects for performing a

  3. Learn About Neuromuscular Disease

    Science.gov (United States)

    ... for MDA Blog Donate Search MDA.org Close Learn About Neuromuscular Disease Muscular dystrophy, ALS and related ... power of a multiple-disease approach, MDA leverages learnings from one disease to accelerate progress in others ...

  4. Neuromuscular blockade in children Bloqueadores neuromusculares em crianças

    Directory of Open Access Journals (Sweden)

    João Fernando Lourenço de Almeida

    2000-06-01

    Full Text Available Neuromuscular blocking agents (NMBAs have been widely used to control patients who need to be immobilized for some kind of medical intervention, such as an invasive procedure or synchronism with mechanical ventilation. The purpose of this monograph is to review the pharmacology of the NMBAs, to compare the main differences between the neuromuscular junction in neonates, infants, toddlers and adults, and moreover to discuss their indications in critically ill pediatric patients. Continuous improvement of knowledge about NMBAs pharmacology, adverse effects, and the many other remaining unanswered questions about neuromuscular junction and neuromuscular blockade in children is essential for the correct use of these drugs. Therefore, the indication of these agents in pediatrics is determined with extreme judiciousness. Computorized (Medline 1990-2000 and active search of articles were the mechanisms used in this review.Os bloqueadores neuromusculares têm sido amplamente utilizados para controlar pacientes que necessitem imobilidade para algum tipo de intervenção médica, desde a realização de procedimentos invasivos até a obtenção de sincronismo com a ventilação mecânica. O objetivo básico desta monografia é revisar a farmacologia dos principais bloqueadores neuromusculares, analisar as diferenças existentes na junção neuromuscular de neonatos, lactentes, pré-escolares e adultos, além de discutir suas indicações em pacientes criticamente enfermos internados em unidade de terapia intensiva pediátrica. Revisão computadorizada da literatura (Medline 1990-2000 associado a busca ativa de artigos compuseram o mecanismo de busca dos dados desta revisão.

  5. Hereditary neuromuscular diseases

    Energy Technology Data Exchange (ETDEWEB)

    Oezsarlak, O. E-mail: ozkan.ozsarlak@uza.be; Schepens, E.; Parizel, P.M.; Goethem, J.W. van; Vanhoenacker, F.; Schepper, A.M. de; Martin, J.J

    2001-12-01

    This article presents the actual classification of neuromuscular diseases based on present expansion of our knowledge and understanding due to genetic developments. It summarizes the genetic and clinical presentations of each disorder together with CT findings, which we studied in a large group of patients with neuromuscular diseases. The muscular dystrophies as the largest and most common group of hereditary muscle diseases will be highlighted by giving detailed information about the role of CT and MRI in the differential diagnosis. The radiological features of neuromuscular diseases are atrophy, hypertrophy, pseudohypertrophy and fatty infiltration of muscles on a selective basis. Although the patterns and distribution of involvement are characteristic in some of the diseases, the definition of the type of disease based on CT scan only is not always possible.

  6. Neuromuscular blockade for optimising surgical conditions during abdominal and gynaecological surgery

    DEFF Research Database (Denmark)

    Madsen, M V; Staehr-Rye, A K; Gätke, M R

    2015-01-01

    BACKGROUND: The level of neuromuscular blockade (NMB) that provides optimal surgical conditions during abdominal surgery has not been well established. The aim of this systematic review was to evaluate current evidence on the use of neuromuscular blocking agents in order to optimise surgical...

  7. When Lightning Strikes Twice: Profoundly Gifted, Profoundly Accomplished.

    Science.gov (United States)

    Makel, Matthew C; Kell, Harrison J; Lubinski, David; Putallaz, Martha; Benbow, Camilla P

    2016-07-01

    The educational, occupational, and creative accomplishments of the profoundly gifted participants (IQs ⩾ 160) in the Study of Mathematically Precocious Youth (SMPY) are astounding, but are they representative of equally able 12-year-olds? Duke University's Talent Identification Program (TIP) identified 259 young adolescents who were equally gifted. By age 40, their life accomplishments also were extraordinary: Thirty-seven percent had earned doctorates, 7.5% had achieved academic tenure (4.3% at research-intensive universities), and 9% held patents; many were high-level leaders in major organizations. As was the case for the SMPY sample before them, differential ability strengths predicted their contrasting and eventual developmental trajectories-even though essentially all participants possessed both mathematical and verbal reasoning abilities far superior to those of typical Ph.D. recipients. Individuals, even profoundly gifted ones, primarily do what they are best at. Differences in ability patterns, like differences in interests, guide development along different paths, but ability level, coupled with commitment, determines whether and the extent to which noteworthy accomplishments are reached if opportunity presents itself. © The Author(s) 2016.

  8. [Characteristics of neuromuscular scoliosis].

    Science.gov (United States)

    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.

  9. Autoantibodies in neuromuscular transmission disorders

    National Research Council Canada - National Science Library

    Vincent, Angela

    2008-01-01

    .... The neuromuscular junction (NMJ) is the site of a number of different autoimmune and genetic disorders, and it is also the target of many neurotoxins from venomous snakes, spiders, scorpions and other species...

  10. Neuromuscular disease classification system

    Science.gov (United States)

    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.

  11. A randomized, dose-response study of sugammadex given for the reversal of deep rocuronium- or vecuronium-induced neuromuscular blockade under sevoflurane anesthesia

    DEFF Research Database (Denmark)

    Duvaldestin, Philippe; Kuizenga, Karel; Saldien, Vera

    2010-01-01

    Sugammadex is the first of a new class of selective muscle relaxant binding drugs developed for the rapid and complete reversal of neuromuscular blockade induced by rocuronium and vecuronium. Many studies have demonstrated a dose-response relationship with sugammadex for reversal of neuromuscular...... blockade in patients induced and maintained under propofol anesthesia. However, sevoflurane anesthesia, unlike propofol, can prolong the effect of neuromuscular blocking drugs (NMBDs) such as rocuronium and vecuronium....

  12. Neuromuscular ultrasound of cranial nerves.

    Science.gov (United States)

    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.

  13. Doenças neuromusculares

    OpenAIRE

    Reed,Umbertina C.

    2002-01-01

    Objetivo: apresentar os dados essenciais para o diagnóstico diferencial entre as principais doenças neuromusculares, denominação genérica sob a qual agrupam-se diferentes afecções, decorrentes do acometimento primário da unidade motora (motoneurônio medular, raiz nervosa, nervo periférico, junção mioneural e músculo). Fontes dos dados: os aspectos clínicos fundamentais para estabelecer o diagnóstico diferencial entre as diferentes doenças neuromusculares, bem como entre estas e as causas de h...

  14. REHABILITATION PROGRAMS FOR PEOPLE WITH NEUROMUSCULAR DISORDERS

    OpenAIRE

    Anton Zupan

    2004-01-01

    Neuromuscular diseases are inherited, chronic, degenerative and progressive. The main characteristics of neuromuscular diseases are: muscular weakness, contractures, scoliosis, respiratory insufficiency, cardiac affection, nutrition disturbances, dependence on the help of others, possible social isolation and physiological problems. Appropriate rehabilitation programs should influence all mentioned characteristics. A special unit for rehabilitation of patients with neuromuscular diseases with...

  15. Profound thrombocytopenia after primary exposure to eptifibatide

    Directory of Open Access Journals (Sweden)

    Nicholas B Norgard

    2010-09-01

    Full Text Available Nicholas B Norgard, Brian T BadgleyUniversity at Buffalo, School of Pharmacy and Pharmaceutical Sciences, Buffalo, NY, USAAbstract: Eptifibatide is a glycoprotein IIb/IIIa receptor antagonist used to reduce the incidence of ischemic events in patients with acute coronary syndromes and those undergoing percutaneous coronary intervention. A minority of patients given eptifibatide develop acute, profound thrombocytopenia (<20,000 cells/mm3 within a few hours of receiving the drug. This case report discusses a patient who developed profound thrombocytopenia within hours of receiving eptifibatide for the first time. The Naranjo algorithm classified the likelihood that this patient’s thrombocytopenia was related to eptifibatide as probable. Profound thrombocytopenia is an uncommon but clinically important complication of eptifibatide. This case report emphasizes the importance of monitoring platelet counts routinely at baseline and within 2–6 hours of eptifibatide administration.Keywords: drug-induced thrombocytopenia, glycoprotein IIb/IIIa antagonists, eptifibatide, thrombocytopenia

  16. Total Spinal Block after Thoracic Paravertebral Block.

    Science.gov (United States)

    Beyaz, Serbülent Gökhan; Özocak, Hande; Ergönenç, Tolga; Erdem, Ali Fuat; Palabıyık, Onur

    2014-02-01

    Thoracic paravertebral block (TPVB) can be performed with or without general anaesthesia for various surgical procedures. TPVB is a popular anaesthetic technique due to its low side effect profile and high analgesic potency. We used 20 mL of 0.5% levobupivacaine for a single injection of unilateral TPVB at the T7 level with neurostimulator in a 63 year old patient with co-morbid disease who underwent cholecystectomy. Following the application patient lost consciousness, and was intubated. Haemodynamic instability was normalised with rapid volume replacement and vasopressors. Anaesthetic drugs were stopped at the end of the surgery and muscle relaxant was antagonised. Return of mucle strenght was shown with neuromuscular block monitoring. Approximately three hours after TPVB, spontaneous breathing started and consciousness returned. A total spinal block is a rare and life-threatening complication. A total spinal block is a complication of spinal anaesthesia, and it can also occur after peripheral blocks. Clinical presentation is characterised by hypotension, bradicardia, apnea, and cardiac arrest. An early diagnosis and appropriate treatment is life saving. In this case report, we want to present total spinal block after TPVB.

  17. Profound thrombocytopenia after primary exposure to eptifibatide.

    Science.gov (United States)

    Norgard, Nicholas B; Badgley, Brian T

    2010-01-01

    Eptifibatide is a glycoprotein IIb/IIIa receptor antagonist used to reduce the incidence of ischemic events in patients with acute coronary syndromes and those undergoing percutaneous coronary intervention. A minority of patients given eptifibatide develop acute, profound thrombocytopenia (eptifibatide for the first time. The Naranjo algorithm classified the likelihood that this patient's thrombocytopenia was related to eptifibatide as probable. Profound thrombocytopenia is an uncommon but clinically important complication of eptifibatide. This case report emphasizes the importance of monitoring platelet counts routinely at baseline and within 2-6 hours of eptifibatide administration.

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

    Science.gov (United States)

    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.

  19. Neuromuscular dose-response studies: determining sample size.

    Science.gov (United States)

    Kopman, A F; Lien, C A; Naguib, M

    2011-02-01

    Investigators planning dose-response studies of neuromuscular blockers have rarely used a priori power analysis to determine the minimal sample size their protocols require. Institutional Review Boards and peer-reviewed journals now generally ask for this information. This study outlines a proposed method for meeting these requirements. The slopes of the dose-response relationships of eight neuromuscular blocking agents were determined using regression analysis. These values were substituted for γ in the Hill equation. When this is done, the coefficient of variation (COV) around the mean value of the ED₅₀ for each drug is easily calculated. Using these values, we performed an a priori one-sample two-tailed t-test of the means to determine the required sample size when the allowable error in the ED₅₀ was varied from ±10-20%. The COV averaged 22% (range 15-27%). We used a COV value of 25% in determining the sample size. If the allowable error in finding the mean ED₅₀ is ±15%, a sample size of 24 is needed to achieve a power of 80%. Increasing 'accuracy' beyond this point requires increasing greater sample sizes (e.g. an 'n' of 37 for a ±12% error). On the basis of the results of this retrospective analysis, a total sample size of not less than 24 subjects should be adequate for determining a neuromuscular blocking drug's clinical potency with a reasonable degree of assurance.

  20. Muscle ultrasound in neuromuscular disorders.

    NARCIS (Netherlands)

    Pillen, S.; Arts, I.M.P.; Zwarts, M.J.

    2008-01-01

    Muscle ultrasound is a useful tool in the diagnosis of neuromuscular disorders, as these disorders result in muscle atrophy and intramuscular fibrosis and fatty infiltration, which can be visualized with ultrasound. Several prospective studies have reported high sensitivities and specificities in

  1. Palliative care in neuromuscular diseases

    NARCIS (Netherlands)

    de Visser, Marianne; Oliver, David J.

    2017-01-01

    Purpose of review Palliative care is an approach that improves the quality of life of patients and their families facing the problem associated with life-threatening illness. Neuromuscular disorders (NMDs) are characterized by progressive muscle weakness, leading to pronounced and incapacitating

  2. Vocational perspectives and neuromuscular disorders

    NARCIS (Netherlands)

    Andries, F.; Wevers, C. W.; Wintzen, A. R.; Busch, H. F.; Höweler, C. J.; de Jager, A. E.; Padberg, G. W.; de Visser, M.; Wokke, J. H.

    1997-01-01

    The present study analyses the actual occupational situation, vocational handicaps and past labour career of a group of about 1000 Dutch patients suffering from a neuromuscular disorder (NMD). On the basis of the likelihood of a substantial employment history and sufficient numbers of patients, four

  3. Neuromuscular paralysis for newborn infants receiving mechanical ventilation.

    Science.gov (United States)

    Cools, F; Offringa, M

    2005-04-18

    Ventilated newborn infants breathing in asynchrony with the ventilator are at risk for complications during mechanical ventilation, such as pneumothorax or intraventricular hemorrhage, and are exposed to more severe barotrauma, which consequently could impair their clinical outcome. Neuromuscular paralysis, which eliminates spontaneous breathing efforts of the infant, has potential advantages in this respect. However, a number of complications have been reported with muscle relaxation in infants, so that concerns exist regarding the safety of prolonged neuromuscular paralysis in newborn infants. To determine whether routine neuromuscular paralysis of newborn infants receiving mechanical ventilation compared with no routine paralysis results in clinically important benefits or harms. The Cochrane Central Register of Controlled Trials (CENTRAL, The Cochrane Library, Issue 1, 2004), MEDLINE (from 1966 to April 2004) and EMBASE (from 1988 to April 2004) were searched. References of review articles were hand searched. Language restriction was not imposed. All trials using random or quasi-random patient allocation, in which the routine use of neuromuscular blocking agents during mechanical ventilation was compared to no paralysis or selective paralysis in newborn infants. Methodological quality was assessed blindly and independently by the two authors. Data were abstracted using standard methods of the Cochrane Collaboration and its Neonatal Review Group, with independent evaluation of trial quality, and abstraction and synthesis of data by both authors. Treatment effect was analysed using relative risk, risk difference and weighted mean difference. Ten possibly eligible trials were identified, of which six were included in the review. All the included trials studied preterm infants ventilated for respiratory distress syndrome, and used pancuronium as the neuromuscular blocking agent. In the analysis of the results of all trials, no significant difference was found in

  4. Effect of 50% enantiomeric excess bupivacaine mixture combined with pancuronium on neuromuscular transmission in rat phrenic nerve-diaphragm preparation; a pilot study.

    Science.gov (United States)

    de Assunção Braga, Angelica de Fátima; Carvalho, Vanessa Henriques; da Silva Braga, Franklin Sarmento; Potério, Gloria Maria Braga; Santos, Filipe Nadir Caparica; Junqueira, Fernando Eduardo Féres

    2015-11-01

    Local anaesthetics are drugs that are widely used in clinical practice. However, the effects of these drugs on the neuromuscular junction and their influence on the blockade produced by non-depolarising neuromuscular blocking drugs are still under investigation. The aim of this study was to evaluate, in vitro, the influence of a 50% enantiomeric excess bupivacaine mixture on neuromuscular transmission and neuromuscular block produced by pancuronium. Rats were distributed into three groups (n = 5) according to the drug studied namely, 50% enantiomeric excess bupivacaine mixture (5 μg/mL); pancuronium (2 μg/mL); 50% enantiomeric excess bupivacaine mixture + pancuronium. The following parameters were evaluated: (1) Effects of a 50% enantiomeric excess bupivacaine mixture on membrane potential (MP) and miniature endplate potentials (MEPPs); (2) amplitude of diaphragmatic response before and 60 min after the addition of a 50% enantiomeric excess bupivacaine mixture; the degree of neuromuscular block with pancuronium and pancuronium combined with a 50% enantiomeric excess bupivacaine mixture. A 50% enantiomeric excess bupivacaine mixture did not alter the amplitude of muscle response (MP) but decreased the frequency and amplitude of MEPP. The block produced by pancuronium was potentiated by a 50% enantiomeric excess bupivacaine mixture. A 50% enantiomeric excess bupivacaine mixture used alone did not affect neuromuscular transmission, but potentiated the neuromuscular block produced by pancuronium. No action was shown on the muscle fibre, and alterations on MEPPs demonstrated a presynaptic action.

  5. Partial neuromuscular blockade in humans enhances muscle blood flow during exercise independently of muscle oxygen uptake and acetylcholine receptor blockade

    DEFF Research Database (Denmark)

    Hellsten, Ylva; Krustrup, Peter; Iaia, F Marcello

    2009-01-01

    This study examined the role of acetylcholine for skeletal muscle blood flow during exercise by use of the competitive neuromuscular blocking agent cisatracurium in combination with the acetylcholine receptor blocker glycopyrrone. Nine healthy male subjects performed a 10-min bout of one-legged k......This study examined the role of acetylcholine for skeletal muscle blood flow during exercise by use of the competitive neuromuscular blocking agent cisatracurium in combination with the acetylcholine receptor blocker glycopyrrone. Nine healthy male subjects performed a 10-min bout of one....... The enhanced exercise hyperemia during partial neuromuscular blockade may be related to a greater recruitment of fast-twitch muscle fibres. Key words: blood flow, neuromuscular blockade, exercise, skeletal muscle....

  6. Profound thrombocytopenia after primary exposure to eptifibatide

    OpenAIRE

    Norgard, Nicholas; Badgley,Brian

    2010-01-01

    Nicholas B Norgard, Brian T BadgleyUniversity at Buffalo, School of Pharmacy and Pharmaceutical Sciences, Buffalo, NY, USAAbstract: Eptifibatide is a glycoprotein IIb/IIIa receptor antagonist used to reduce the incidence of ischemic events in patients with acute coronary syndromes and those undergoing percutaneous coronary intervention. A minority of patients given eptifibatide develop acute, profound thrombocytopenia (<20,000 cells/mm3) within a few hours of receiving the drug. This c...

  7. [Respiratory treatments in neuromuscular disease].

    Science.gov (United States)

    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.

  8. Neuromuscular fatigue in racquet sports.

    Science.gov (United States)

    Girard, Olivier; Millet, Grégoire P

    2008-02-01

    This article describes the physiologic and neural mechanisms that cause neuromuscular fatigue in racquet sports: table tennis, tennis, squash, and badminton. In these intermittent and dual activities, performance may be limited as a match progresses because of a reduced central activation, linked to changes in neurotransmitter concentration or in response to afferent sensory feedback. Alternatively, modulation of spinal loop properties may occur because of changes in metabolic or mechanical properties within the muscle. Finally, increased fatigue manifested by mistimed strokes, lower speed, and altered on-court movements may be caused by ionic disturbances and impairments in excitation-contraction coupling properties. These alterations in neuromuscular function contribute to decrease in racquet sports performance observed under fatigue.

  9. Optimized surgical space during low-pressure laparoscopy with deep neuromuscular blockade

    DEFF Research Database (Denmark)

    Staehr-Rye, Anne K; Rasmussen, Lars S; Rosenberg, Jacob

    2013-01-01

    Laparoscopic cholecystectomy (LC) can be performed using low intra-abdominal pressure (space conditions using either deep, continuous muscle relaxation or moderate blockade during low-pressure (8 mm......Hg) LC. We hypothesized that a deep neuromuscular block would be associated with a higher proportion of optimal surgical space conditions....

  10. Optimized surgical space during low-pressure laparoscopy with deep neuromuscular blockade

    DEFF Research Database (Denmark)

    Staehr-Rye, Anne K; Rasmussen, Lars S; Rosenberg, Jacob

    2013-01-01

    Laparoscopic cholecystectomy (LC) can be performed using low intra-abdominal pressure (surgical conditions may not be optimal. The present study aimed at comparing surgical space conditions using either deep, continuous muscle relaxation or moderate blockade during low-pressure (8 mm......Hg) LC. We hypothesized that a deep neuromuscular block would be associated with a higher proportion of optimal surgical space conditions....

  11. Myths and facts in neuromuscular pharmacology - New developments in reversing neuromuscular blockade

    NARCIS (Netherlands)

    Fink, H.; Hollmann, M. W.

    2012-01-01

    Pharmacologic reversal of neuromuscular blockade is a topic nor very well acknowledged and controversially discussed. Reasons for this are numerous and include missing perception of the potential complications of residual neuromuscular paralysis including an increased morbidity and mortality, as

  12. Prolonged neuromuscular block in a preeclamptic patient induced ...

    African Journals Online (AJOL)

    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 ...

  13. Asystole Following Profound Vagal Stimulation During Hepatectomy

    Directory of Open Access Journals (Sweden)

    Preeta John

    2008-01-01

    Full Text Available Asystole in a non laparoscopic upper abdominal surgery following intense vagal stimulation is a rare event. This case report highlights the need for awareness of such a complication when a thoracic epidural anaesthetic has been given in addition to a general anaesthetic for an upper abdominal procedure. A combined thoracic epidural and general anaesthetic was given. The anterior abdominal wall was retracted forty minutes after administration of the epidural bolus. This maneuver resulted in a profound vagal response with bradycardia and asystole. The patient was resuscitated successfully with a cardiac massage, atropine and adrenaline and the surgery was resumed. Surgery lasted eleven hours and was uneventful.

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

    National Research Council Canada - National Science Library

    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...

  15. Kinship and interaction in neuromuscular pharmacology

    NARCIS (Netherlands)

    Schiere, Sjouke

    2006-01-01

    The background of this thesis is presented in the introductory chapters and stafts with a brief history of neuromuscular relaxants. It is followed by a short description of the neuromuscular physiology and pharmacology in chapters 2 and 3, respectively. In chapter 4 the aim of the thesis is

  16. Improving Neuromuscular Monitoring and Reducing Residual Neuromuscular Blockade With E-Learning: Protocol for the Multicenter Interrupted Time Series INVERT Study.

    Science.gov (United States)

    Thomsen, Jakob Louis Demant; Mathiesen, Ole; Hägi-Pedersen, Daniel; Skovgaard, Lene Theil; Østergaard, Doris; Engbaek, Jens; Gätke, Mona Ring

    2017-10-06

    Muscle relaxants facilitate endotracheal intubation under general anesthesia and improve surgical conditions. Residual neuromuscular blockade occurs when the patient is still partially paralyzed when awakened after surgery. The condition is associated with subjective discomfort and an increased risk of respiratory complications. Use of an objective neuromuscular monitoring device may prevent residual block. Despite this, many anesthetists refrain from using the device. Efforts to increase the use of objective monitoring are time consuming and require the presence of expert personnel. A neuromuscular monitoring e-learning module might support consistent use of neuromuscular monitoring devices. The aim of the study is to assess the effect of a neuromuscular monitoring e-learning module on anesthesia staff's use of objective neuromuscular monitoring and the incidence of residual neuromuscular blockade in surgical patients at 6 Danish teaching hospitals. In this interrupted time series study, we are collecting data repeatedly, in consecutive 3-week periods, before and after the intervention, and we will analyze the effect using segmented regression analysis. Anesthesia departments in the Zealand Region of Denmark are included, and data from all patients receiving a muscle relaxant are collected from the anesthesia information management system MetaVision. We will assess the effect of the module on all levels of potential effect: staff's knowledge and skills, patient care practice, and patient outcomes. The primary outcome is use of neuromuscular monitoring in patients according to the type of muscle relaxant received. Secondary outcomes include last recorded train-of-four value, administration of reversal agents, and time to discharge from the postanesthesia care unit as well as a multiple-choice test to assess knowledge. The e-learning module was developed based on a needs assessment process, including focus group interviews, surveys, and expert opinions. The e

  17. Autophagy Impairment in Muscle Induces Neuromuscular Junction Degeneration and Precocious Aging

    Directory of Open Access Journals (Sweden)

    Silvia Carnio

    2014-09-01

    Full Text Available The cellular basis of age-related tissue deterioration remains largely obscure. The ability to activate compensatory mechanisms in response to environmental stress is an important factor for survival and maintenance of cellular functions. Autophagy is activated both under short and prolonged stress and is required to clear the cell of dysfunctional organelles and altered proteins. We report that specific autophagy inhibition in muscle has a major impact on neuromuscular synaptic function and, consequently, on muscle strength, ultimately affecting the lifespan of animals. Inhibition of autophagy also exacerbates aging phenotypes in muscle, such as mitochondrial dysfunction, oxidative stress, and profound weakness. Mitochondrial dysfunction and oxidative stress directly affect acto-myosin interaction and force generation but show a limited effect on stability of neuromuscular synapses. These results demonstrate that age-related deterioration of synaptic structure and function is exacerbated by defective autophagy.

  18. Autoantibodies in neuromuscular transmission disorders

    Directory of Open Access Journals (Sweden)

    Vincent Angela

    2008-01-01

    Full Text Available It is a great pleasure to be asked to honour the memory of Dr. Baldev Singh by reviewing the field of autoantibodies in myasthenia gravis and other neurotransmission disorders. The neuromuscular junction (NMJ is the site of a number of different autoimmune and genetic disorders, and it is also the target of many neurotoxins from venomous snakes, spiders, scorpions and other species. The molecular organization of the NMJ is graphically represented in [Figure 1A], where different ion channels, receptors and other proteins are shown. Four of the ion channels or receptors are directly involved in autoimmune diseases. This brief review will not only concentrate on these conditions but also illustrate how their study is helping us to understand the etiology of rare but treatable neurological syndromes of the central nervous system.

  19. FUNCTIONS OF A NEUROMUSCULAR CENTRE

    Directory of Open Access Journals (Sweden)

    Janez Zidar

    2004-12-01

    Full Text Available Main functions of a neuromuscular (NM centre are making diagnosis, treatment and counselling. Some other functions, e. g. forming a register and epidemiological endeavours, could be added. All these activities are expected to be achieved by multidisciplinary approach with the idea that members use the same guidelines and share the same knowledge.NM diseases affect lower levels of the nervous system that is motor units (motor cells in the brainstem and spinal cord, nerve roots, cranial and peripheral nerves, neuromuscular junction, and muscles. There are many such diseases; a few are more common others are rare.Rational approach in making a diagnosis can be divided into several steps. The process begins with a person with clinical symptoms and signs which raise the suspicion of NM disease. The first step is the description of the predominant pattern of muscular wasting and weakness (e. g. limb-girdle, distal, ocular, facio-scapulo-humeral. Each of these syndromes require a differential diagnosis within the motor unit territory what is achieved by means of EMG and muscle biopsy. The latter is even more important to define the nature of the abnormality. Disease nature can also be determined biochemically and, as NM disorders are commonly genetically determined, at the molecular genetic level. Treatment modalities include drugs (causative and symptomatic and other measures such as promoting and maintaining good general health, preventing skeletal deformities, physiotherapy, orthoses, surgery, and prevention of respiratory and cardiac functions. Counselling is mainly by social workers that focus on the practical aspects of coping with illness and disability and by genetic counsellors who gave advise on family planning.

  20. Staffs' documentation of participation for adults with profound intellectual disability or profound intellectual and multiple disabilities.

    Science.gov (United States)

    Talman, Lena; Gustafsson, Christine; Stier, Jonas; Wilder, Jenny

    2017-06-21

    This study investigated what areas of International Classification of Functioning, Disability and Health were documented in implementation plans for adults with profound intellectual disability or profound intellectual and multiple disabilities with focus on participation. A document analysis of 17 implementation plans was performed and International Classification of Functioning, Disability and Health was used as an analytic tool. One hundred and sixty-three different codes were identified, especially in the components Activities and participation and Environmental factors. Participation was most frequently coded in the chapters Community, social and civic life and Self-care. Overall, the results showed that focus in the implementation plans concerned Self-care and Community, social and civic life. The other life areas in Activities and participation were seldom, or not at all, documented. A deeper focus on participation in the implementation plans and all life areas in the component Activities and participation is needed. It is important that the documentation clearly shows what the adult wants, wishes, and likes in everyday life. It is also important to ensure that the job description for staff contains both life areas and individual preferences so that staff have the possibility to work to fulfill social and individual participation for the target group. Implications for rehabilitation There is a need for functioning working models to increase participation significantly for adults with profound intellectual disability or profound intellectual and multiple disabilities. For these adults, participation is achieved through the assistance of others and support and services carried out must be documented in an implementation plan. The International Classification of Functioning, Disability and Health can be used to support staff and ensure that information about the most important factors in an individual's functioning in their environment is not omitted in

  1. Profound sedation with propofol modifies atrial fibrillation dynamics.

    Science.gov (United States)

    Cervigón, Raquel; Moreno, Javier; Pérez-Villacastín, Julián; Castells, Francisco

    2013-09-01

    During atrial fibrillation (AF), multiple wandering propagation wavelets at high rates drift around both atria under controversial hierarchical models. Antiarrhythmic drugs modify the cardiac ionic currents supporting the fibrillation process within the atria, and can alter AF propagation dynamics and even terminate the arrhythmia. However, some other drugs, theoretically nonantiarrhythmic, may slightly block particular cardiac ionic currents through uncertain mechanisms in such a subtle way at regular heart rates that may have been pharmacologically overlooked. These potential effects might be better exposed at much higher activation rates as in AF, where atrial cells depolarize over 400 times per second. In this review, we aimed to compile and discuss results from several studies evaluating the net effect of profound sedation with propofol on atrial cells and atrioventricular (AV) conduction. Propofol is a very commonly used anesthetic agent, and its possible effect on AF dynamics has systematically not been taken into account in the myriad of clinical studies dealing with AF intracardiac recordings. The possible effect of sedation with propofol on AF was evaluated through the analysis of AF propagation patterns before and after its infusion in a series of patients submitted to pulmonary vein ablation. Effect on AV conduction will be discussed as well. ©2013, The Authors. Journal compilation ©2013 Wiley Periodicals, Inc.

  2. REHABILITATION PROGRAMS FOR PEOPLE WITH NEUROMUSCULAR DISORDERS

    Directory of Open Access Journals (Sweden)

    Anton Zupan

    2004-12-01

    Full Text Available Neuromuscular diseases are inherited, chronic, degenerative and progressive. The main characteristics of neuromuscular diseases are: muscular weakness, contractures, scoliosis, respiratory insufficiency, cardiac affection, nutrition disturbances, dependence on the help of others, possible social isolation and physiological problems. Appropriate rehabilitation programs should influence all mentioned characteristics. A special unit for rehabilitation of patients with neuromuscular diseases within the Institute for rehabilitation of the Republic of Slovenia was established in 1993 at the initiative of the Muscular Dystrophy Association of Slovenia. The main aim of this establishment was to try to guide the patient and his family through the course of the disease. The article describes the work of the mentioned unit. Different clinical rehabilitation programs for people with neuromuscular diseases are presented and some research results of the unit are mentioned.

  3. Rapid synthesis of acetylcholine receptors at neuromuscular junctions.

    Science.gov (United States)

    Ramsay, D A; Drachman, D B; Pestronk, A

    1988-10-11

    The rate of acetylcholine receptor (AChR) degradation in mature, innervated mammalian neuromuscular junctions has recently been shown to be biphasic; up to 20% are rapidly turned over (RTOs; half life less than 1 day) whereas the remainder are lost more slowly ('stable' AChRs; half life 10-12 days). In order to maintain normal junctional receptor density, synthesis and insertion of AChRs should presumably be sufficiently rapid to replace both the RTOs and the stable receptors. We have tested this prediction by blocking pre-existing AChRs in the mouse sternomastoid muscle with alpha-bungarotoxin (alpha-BuTx), and monitoring the subsequent appearance of 'new' junctional AChRs at intervals of 3 h to 20 days by labeling them with 125I-alpha-BuTx. The results show that new receptors were initially inserted rapidly (16% at 24 h and 28% at 48 h). The rate of increase of 'new' 125I-alpha-BuTx binding sites gradually slowed down during the remainder of the time period studied. Control observations excluded possible artifacts of the experimental procedure including incomplete blockade of AChRs, dissociation of toxin-receptor complexes, or experimentally induced alteration of receptor synthesis. The present demonstration of rapid synthesis and incorporation of AChRs at innervated neuromuscular junctions provides support for the concept of a subpopulation of rapidly turned over AChRs. The RTOs may serve as precursors for the larger population of stable receptors and have an important role in the metabolism of the neuromuscular synapse.

  4. Eccentric Exercise to Enhance Neuromuscular Control.

    Science.gov (United States)

    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.

  5. Neuromuscular function in healthy occlusion.

    Science.gov (United States)

    Forrester, S E; Allen, S J; Presswood, R G; Toy, A C; Pain, M T G

    2010-09-01

    This study aimed to measure neuromuscular function for the masticatory muscles under a range of occlusal conditions in healthy, dentate adults. Forty-one subjects conducted maximum voluntary clenches under nine different occlusal loading conditions encompassing bilateral posterior teeth contacts with the mandible in different positions, anterior teeth contacts and unilateral posterior teeth contacts. Surface electromyography was recorded bilaterally from the anterior temporalis, superficial masseter, sternocleidomastoid, anterior digastric and trapezius muscles. Clench condition had a significant effect on muscle function (P = 0.0000) with the maximum function obtained for occlusions with bilateral posterior contacts and the mandible in a stable centric position. The remaining contact points and moving the mandible to a protruded position, whilst keeping posterior contacts, resulted in significantly lower muscle activities. Clench condition also had a significant effect on the per cent overlap, anterior-posterior and torque coefficients (P = 0.0000-0.0024), which describe the degree of symmetry in these muscle activities. Bilateral posterior contact conditions had significantly greater symmetry in muscle activities than anterior contact conditions. Activity in the sternocleidomastoid, anterior digastric and trapezius was consistently low for all clench conditions, i.e. centric position, whilst with anterior teeth contacts, both the muscle activity and the degree of symmetry in muscle activity are significantly reduced.

  6. Profound Impacts of AN Arctic Face Lift

    Science.gov (United States)

    Nghiem, Son

    Son Nghiem, son.v.nghiem@jpl.nasa.gov Jet Propulsion Laboratory, California Institute of Technology, Pasadena, California, United States The ice cover on the Arctic Ocean has undergone a face lift that removes much of the older and thicker perennial ice and replaces it with the younger and thinner seasonal ice. Although the sea ice cover is a thin skin compared to the depth of the Arctic Ocean, this face lift exerts profound change in the Arctic environment. Here, we present scatterometer remote sensing of Arctic sea ice change and its implication on chemical processes from the ice surface to the troposphere extending into the internal continental land. In the context of a half century change, the extent of perennial ice declines at rate of 0.5 million km2 per decade in the 1970s-1990s while there is no discernable trend in the 1950s-1960s. Abruptly, the rate of decrease has tripled to 1.5 million km2 per decade in the 2000s. A record was set in the reduction of Arctic perennial ice extent in winter 2008. By 1 March 2008, perennial ice extent was reduced by one million km2 compared to that at the same time in 2007. On 1 May 2009, perennial ice extent was reduced to 2.1 million km2 , which is a virtual tie to 2.2 million km2 of perennial ice extent on 1 May 2008 given the uncertainty of ±0.2 million km2 . Although the extent of perennial ice extent is similar, its distribution is quite different, with a significant perennial ice pack in the Beaufort Sea in 2008, and in contrast a large expanse of perennial ice along the Transpolar Drift Stream in 2009. The continuing drastic reduction of perennial ice significantly decreases the overall surface albedo, resulting in enhanced solar heat absorption in spring and summer, which further decreases the Arctic ice pack through the ice-albedo feedback mechanism and ice melt from the underside due to oceanic thermodynamic interactions. Satellite maps of sea ice class distribution show the closely conformation with patterns of

  7. Electromyographic and neuromuscular variables in unstable postpolio subjects, stable postpolio subjects, and control subjects.

    Science.gov (United States)

    Rodriquez, A A; Agre, J C; Franke, T M

    1997-09-01

    To compare strength and endurance variables obtained in the quadriceps muscles of postpolio and control subjects over a 7-year interval with macro and single fiber electromyography (EMG) variables. A controlled inception cohort study. Neuromuscular research laboratory of a university hospital. A cohort of 23 postpolio and 14 control subjects. All postpolio subjects had a history, physical examination, and EMG consistent with previous poliomyelitis, and had greater than antigravity strength in the quadriceps muscle tested. Unstable postpolio subjects acknowledged new quadriceps weakness over the 7-year period of the study (n = 11), and stable postpolio subjects denied new weakness of the quadriceps over the same period (n = 12). All subjects had tests of neuromuscular function of the quadriceps muscles at the onset of this study and yearly over a 7-year period. EMG variables were determined on a separate day after the seventh year of neuromuscular measurements. Neuromuscular variables measured were isometric knee extension peak torque, isometric endurance (time to inability to maintain knee extensor contraction at 40% of maximal torque), tension time index (TTI) (product of isometric endurance time and 40% of maximal torque), and recovery of torque at 10 minutes after the endurance test. EMG variables were macro EMG and single fiber EMG (jitter, fiber density, and percent blocking). Unstable postpolio subjects did not lose strength more rapidly than stable postpolio subjects or control subjects. Unstable postpolio subjects were significantly weaker, had decreased TTI, larger macro EMG amplitude, greater jitter, blocking, and fiber density in comparison with stable postpolio subjects (all p postpolio group (p .05) with neuromuscular or EMG variables in control, stable, or unstable postpolio subjects.

  8. Deep neuromuscular blockade and low insufflation pressure during laparoscopic hysterectomy

    DEFF Research Database (Denmark)

    Madsen, Matias Vested; Istre, Olav; Springborg, Henrik Halvor

    2017-01-01

    . The gynaecologists registered increasing abdominal tensions in no versus eight procedures (p = 0.006) in the deep and standard NMB group, respectively. CONCLUSION: Deep NMB in combination with 8 mmHg pneumoperitoneum prevented sudden abdominal contractions during laparoscopic hysterectomy. FUNDING: This work......INTRODUCTION: 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. METHODS: 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...

  9. Employment profiles in neuromuscular diseases.

    Science.gov (United States)

    Fowler, W M; Abresch, R T; Koch, T R; Brewer, M L; Bowden, R K; Wanlass, R L

    1997-01-01

    Consumer and rehabilitation provider factors that might limit employment opportunities for 154 individuals with six slowly progressive neuromuscular diseases (NMD) were investigated. The NMDs were spinal muscular atrophy (SMA), hereditary motor sensory neuropathy (HMSN), Becker's muscular dystrophy (BMD), facioscapulohumeral muscular dystrophy (FSHD), myotonic muscular dystrophy (MMD), and limb-girdle syndrome (LGS). Forty percent were employed in the competitive labor market at the time of the study, 50% had been employed in the past, and 10% had never been employed. The major consumer barrier to employment was education. Other important factors were type of occupation, intellectual capacity, psychosocial adjustment, and the belief by most individuals that their physical disability was the only or major barrier to obtaining a job. Psychological characteristics were associated with level of unemployment. However, physical impairment and disability were not associated with level of unemployment. There also were differences among the types of NMDs. Compared with the SMA, HMSN, BMD, and FSHD groups, the MMD and LGS groups had significantly higher levels of unemployment, lower educational levels, and fewer employed professional, management, and technical workers. Nonphysical impairment factors such as a low percentage of college graduates, impaired intellectual function in some individuals, and poor psychological adjustment were correlated with higher unemployment levels in the MMD group. Unemployment in the LGS group was correlated with a failure to complete high school. Major provider barriers to employment were the low level of referrals to Department of Rehabilitation by physicians and the low percentage of acceptance into the State Department of Rehabilitation. The low rate of acceptance was primarily attributable to the low number of referrals compounded by a lack of counselor experience with individuals with NMD. Both consumer and provider barriers may

  10. Protein defects in neuromuscular diseases

    Directory of Open Access Journals (Sweden)

    Vainzof M.

    2003-01-01

    Full Text Available Muscular dystrophies are a heterogeneous group of genetically determined progressive disorders of the muscle with a primary or predominant involvement of the pelvic or shoulder girdle musculature. The clinical course is highly variable, ranging from severe congenital forms with rapid progression to milder forms with later onset and a slower course. In recent years, several proteins from the sarcolemmal muscle membrane (dystrophin, sarcoglycans, dysferlin, caveolin-3, from the extracellular matrix (alpha2-laminin, collagen VI, from the sarcomere (telethonin, myotilin, titin, nebulin, from the muscle cytosol (calpain 3, TRIM32, from the nucleus (emerin, lamin A/C, survival motor neuron protein, and from the glycosylation pathway (fukutin, fukutin-related protein have been identified. Mutations in their respective genes are responsible for different forms of neuromuscular diseases. Protein analysis using Western blotting or immunohistochemistry with specific antibodies is of the utmost importance for the differential diagnosis and elucidation of the physiopathology of each genetic disorder involved. Recent molecular studies have shown clinical inter- and intra-familial variability in several genetic disorders highlighting the importance of other factors in determining phenotypic expression and the role of possible modifying genes and protein interactions. Developmental studies can help elucidate the mechanism of normal muscle formation and thus muscle regeneration. In the last fifteen years, our research has focused on muscle protein expression, localization and possible interactions in patients affected by different forms of muscular dystrophies. The main objective of this review is to summarize the most recent findings in the field and our own contribution.

  11. Optimized surgical space during low-pressure laparoscopy with deep neuromuscular blockade.

    Science.gov (United States)

    Staehr-Rye, Anne K; Rasmussen, Lars S; Rosenberg, Jacob; Juul, Poul; Gätke, Mona R

    2013-02-01

    Laparoscopic cholecystectomy (LC) can be performed using low intra-abdominal pressure (space conditions using either deep, continuous muscle relaxation or moderate blockade during low-pressure (8 mmHg) LC. We hypothesized that a deep neuromuscular block would be associated with a higher proportion of optimal surgical space conditions. This was an investigator-initiated, patient- and assessor-blinded study. Up to 72 patients scheduled for elective LC were randomised to either deep neuromuscular blockade (post-tetanic count 0-1) or moderate neuromuscular blockade, where at least one response to train-of-four nerve stimulation was present. The primary outcome was surgical space conditions at the time during surgery when conditions were worst. The secondary outcomes included the proportion of procedures completed at pneumoperitoneum 8 mmHg, post-operative pain, and incidence of nausea and vomiting. This study was the first randomised study to assess the association between depth of neuromuscular blockade and surgical space conditions during low-pressure LC. The study findings may be applicable to a general surgical population undergoing LC. The University of Copenhagen, Denmark and Sophus Johansens Foundation of 1981, Denmark funded this study, which was also financed by a research grant from the Investigator Initiated Studies Program of Merck Sharp and Dohme Corp. NCT 01523886.

  12. Effects of Aldrin-transdiol on neuromuscular facilitation and depression

    NARCIS (Netherlands)

    Akkermans, L.M.A.; Bercken, J. van den; Zalm, J.M. van der

    The effects of aldrin-transdiol, one of the active metabolites of the insecticide dieldrin, on evoked transmitter release, neuromuscular facilitation and neuromuscular depression have been studied in frog sartorius nerve-muscle preparations. Conventional techniques of intracellular recordings were

  13. Quantitative skeletal muscle ultrasonography in children with suspected neuromuscular disease.

    NARCIS (Netherlands)

    Pillen, S.; Scholten, R.R.; Zwarts, M.J.; Verrips, A.

    2003-01-01

    We determined prospectively the diagnostic value of quantitative ultrasonography in detecting neuromuscular disorders in children. Ultrasonographic scans of four muscles were made in 36 children with symptoms or signs suggestive of neuromuscular disease, such as muscle weakness and hypotonia. The

  14. Caenorhabditis elegans neuromuscular junction: GABA receptors and ivermectin action.

    Directory of Open Access Journals (Sweden)

    Guillermina Hernando

    Full Text Available 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

  15. Neuromuscular Junction Dismantling in Amyotrophic Lateral Sclerosis

    Directory of Open Access Journals (Sweden)

    Valentina Cappello

    2017-10-01

    Full Text Available Neuromuscular junction assembly and plasticity during embryonic, postnatal, and adult life are tightly regulated by the continuous cross-talk among motor nerve endings, muscle fibers, and glial cells. Altered communications among these components is thought to be responsible for the physiological age-related changes at this synapse and possibly for its destruction in pathological states. Neuromuscular junction dismantling plays a crucial role in the onset of Amyotrophic Lateral Sclerosis (ALS. ALS is characterized by the degeneration and death of motor neurons leading to skeletal muscle denervation, atrophy and, most often, death of the patient within five years from diagnosis. ALS is a non-cell autonomous disease as, besides motor neuron degeneration, glial cells, and possibly muscle fibers, play a role in its onset and progression. Here, we will review the recent literature regarding the mechanisms leading to neuromuscular junction disassembly and muscle denervation focusing on the role of the three players of this peripheral tripartite synapse.

  16. Population Blocks.

    Science.gov (United States)

    Smith, Martin H.

    1992-01-01

    Describes an educational game called "Population Blocks" that is designed to illustrate the concept of exponential growth of the human population and some potential effects of overpopulation. The game material consists of wooden blocks; 18 blocks are painted green (representing land), 7 are painted blue (representing water); and the remaining…

  17. [Electrodiagnosis in disorders of neuromuscular transmission].

    Science.gov (United States)

    Chumillas, M J; Cortés, V

    Electrophysiological studies are of recognized use in the confirmation of alterations of neuromuscular transmission in further determining their physiopathological characteristics, helping to differentiate them from other conditions with secondary effects on their function. In our study we review the physiopathology of these disorders which compromise the safety factor of the neuromuscular junction, by presynaptic or postsynaptic alterations, and forms the basis of the results of electrophysiological studies. We describe the techniques currently most used: repetitive stimulation and single fibre electromyography complemented by conventional electromyography. Their application and findings in the commonest syndromes are discussed. Finally, their sensitivity, specificity and difficulties are considered.

  18. Research highlights of partial neuromuscular disorders

    Directory of Open Access Journals (Sweden)

    Cheng ZHANG

    2014-05-01

    Full Text Available In order to understand the latest progression on neuromuscular disorders for clinicians, this review screened and systemized the papers on neuromuscular disorders which were collected by PubMed from January 2013 to February 2014. This review also introduced the clinical diagnosis and treatment hightlights on glycogen storage disease type Ⅱ (GSD Ⅱ, Duchenne muscular dystrophy (DMD, amyotrophic lateral sclerosis (ALS and spinal muscular atrophy (SMA. The important references will be useful for clinicians. doi: 10.3969/j.issn.1672-6731.2014.05.004

  19. 21 CFR 882.5860 - Implanted neuromuscular stimulator.

    Science.gov (United States)

    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...

  20. Effect of ropivacaine combined with pancuronium on neuromuscular transmission and effectiveness of neostigmine and 4-aminopyridine for blockade reversal: experimental study

    Directory of Open Access Journals (Sweden)

    Angélica de Fátima Braga

    2015-04-01

    Full Text Available BACKGROUND AND OBJECTIVES: The local anesthetic effects on neuromuscular junction and its influence on blockade produced by nondepolarizing neuromuscular blockers are still under-investigated; however, this interaction has been described in experimental studies and in humans. The aim of this study was to evaluate in vitro the interaction between ropivacaine and pancuronium, the influence on transmission and neuromuscular blockade, and the effectiveness of neostigmine and 4-aminopyridine to reverse the blockade. METHODS: Rats were divided into groups (n = 5 according to the study drug: ropivacaine (5 µg mL-1; pancuronium (2 µg mL-1; ropivacaine + pancuronium. Neostigmine and 4-aminopyridine were used at concentrations of 2 µg mL-1 and 20 µg mL-1, respectively. The effects of ropivacaine on membrane potential and miniature endplate potential, the amplitude of diaphragm responses before and 60 min after the addition of ropivacaine (degree of neuromuscular blockade with pancuronium and with the association of pancuronium-ropivacaine, and the effectiveness of neostigmine and 4-aminopyridine on neuromuscular block reversal were evaluated. RESULTS: Ropivacaine did not alter the amplitude of muscle response (the membrane potential, but decreased the frequency and amplitude of the miniature endplate potential. Pancuronium blockade was potentiated by ropivacaine, and partially and fully reversed by neostigmine and 4-aminopyridine, respectively. CONCLUSIONS: Ropivacaine increased the neuromuscular block produced by pancuronium. The complete antagonism with 4-aminopyridine suggests presynaptic action of ropivacaine.

  1. Differences between acceleromyography and electromyography during neuromuscular function monitoring in anesthetized Beagle dogs.

    Science.gov (United States)

    Sakai, Daniel M; Martin-Flores, Manuel; Tomak, Emily A; Martin, Matthew J; Campoy, Luis; Gleed, Robin D

    2015-05-01

    Quantitative neuromuscular monitoring is essential for studies of potency and duration of neuromuscular blocking agents, and for detecting residual paralysis in anesthetized patients. This investigation evaluates whether there are systematic differences between acceleromyography (AMG) and electromyography (EMG); two quantitative methods for monitoring neuromuscular block. Prospective. Ten healthy Beagle dogs. Dogs were anesthetized with isoflurane and dexmedetomidine. Both ulnar nerves were stimulated with a train-of-four (TOF) pattern every 15 seconds. The magnitude of the first twitch (T1) and the TOF ratio (magnitude of T4/T1; TOFR) were quantified simultaneously with AMG and EMG, applied randomly to each extremity. The extent of maximal block (T1 depression) and onset time were measured by AMG and EMG during TOF monitoring after the administration of cisatracurium (0.05 mg kg(-1)). In addition, recovery of T1 to 25% and 75%, the recovery index (time between T1 of 25% and 75%), and recovery of the TOFR to 0.9 were used to characterize recovery from cisatracurium and were compared between monitors. Regression and Bland-Altman plots for T1 and TOFR were also created. Maximal block and onset time were not different between monitors. Time to recovery of T1 to 25% and 75%, and time to TOF ratio 0.9 was significantly shorter with AMG. The recovery index was not different between monitors. When the TOFR returned to 0.9 with AMG, EMG still measured considerable residual block (TOFR 0.47). Electromyography consistently detected residual NMB when recovery from NMB was complete as assessed by AMG. © 2014 Association of Veterinary Anaesthetists and the American College of Veterinary Anesthesia and Analgesia.

  2. Improving Neuromuscular Monitoring and Reducing Residual Neuromuscular Blockade With E-Learning

    DEFF Research Database (Denmark)

    Thomsen, Jakob Louis Demant; Mathiesen, Ole; Hägi-Pedersen, Daniel

    2017-01-01

    . A neuromuscular monitoring e-learning module might support consistent use of neuromuscular monitoring devices. OBJECTIVE: The aim of the study is to assess the effect of a neuromuscular monitoring e-learning module on anesthesia staff's use of objective neuromuscular monitoring and the incidence of residual...... as well as a multiple-choice test to assess knowledge. The e-learning module was developed based on a needs assessment process, including focus group interviews, surveys, and expert opinions. RESULTS: The e-learning module was implemented in 6 anesthesia departments on 21 November 2016. Currently, we...... are collecting postintervention data. The final dataset will include data from more than 10,000 anesthesia procedures. We expect to publish the results in late 2017 or early 2018. CONCLUSIONS: With a dataset consisting of thousands of general anesthesia procedures, the INVERT study will assess whether an e-learning...

  3. Association between levobupivacaine and pancuronium. Interference in neuromuscular transmission and blockade in rats.

    Science.gov (United States)

    Carvalho, Vanessa Henriques; Braga, Angélica de Fátima de Assunção; Braga, Franklin Sarmento da Silva; Potério, Gloria Maria Braga; Santos, Filipe Nadir Caparica; Junqueira, Fernando Eduardo Féres

    2016-07-01

    To evaluate the effects of levobupivacaine on neuromuscular transmission and neuromuscular blockade produced by pancuronium in vitro. Thirty rats were distributed into groups (n = 5) according to the drug used alone or in combination: Group I - levobupivacaine (5 µg.mL-1); Group II - pancuronium (2 µg.mL-1); Group III - pancuronium (2 µg.mL-1) + levobupivacaine (5µg.mL-1). The following parameters were evaluated: 1) amplitude of diaphragmatic response to indirect stimulation, before and 60 minutes after the addition of levobupivacaine and pancuronium alone, and after the addition of levobupivacaine combined with pancuronium; 2) membrane potentials (MP) and miniature endplate potentials (MEPP). Levobupivacaine alone did not alter the amplitude of muscle response and MP. In preparations previoulsy exposed to levobupivacaine, the block with pancuronium was significantly denser (90.2 ± 15.2%), showing a significant difference (p=0.031) in comparison to the block produced by pancuronium alone (48.9% ± 9.8%). There was a decrease in the frequency and amplitude of MEPPs. Levobupivacaine potentiated the neuromuscular blockade produced by pancuronium, confirming a presynaptic action by a decrease in miniature endplate potentials.

  4. How do profoundly deaf children learn to read?

    OpenAIRE

    伊藤, 泰子

    2013-01-01

    We know that children who were born profoundly deaf have much difficulty to learn to speak English or Japanese. But is it possible that profoundly deaf children learn to read written English or Japanese? Some researchers mention that early exposure to fingerspelling actually helps deaf children become better readers. Then I tried to find the reason why fingerspelling helps deaf children develop their reading ability and examined how to develop deaf children’s reading ability with fingerspelli...

  5. Neuromuscular Exercise Post Partial Medial Meniscectomy

    DEFF Research Database (Denmark)

    Hall, Michelle; Hinman, Rana S; Wrigley, Tim V

    2015-01-01

    : In patients 3-12 months following a medial arthroscopic partial meniscectomy, a neuromuscular exercise program did not alter the peak knee adduction moment, a key predictor of osteoarthritis structural disease progression. Australia and New Zealand Clinical Trials Registry (#ACTRN12612000542897)....

  6. Experienced and physiological fatigue in neuromuscular disorders.

    NARCIS (Netherlands)

    Schillings, M.L.; Kalkman, J.S.; Janssen, H.M.; Engelen, B.G.M. van; Bleijenberg, G.; Zwarts, M.J.

    2007-01-01

    OBJECTIVE: Fatigue has been described as a typical symptom of neurological diseases. It might be caused both by changes at the peripheral and at the central level. This study measured the level of experienced fatigue and physiological correlates of fatigue in three genetically defined neuromuscular

  7. Postoperative neuromuscular function following non-depolarising ...

    African Journals Online (AJOL)

    2016-07-03

    Jul 3, 2016 ... speaking, difficulty swallowing, unsteady gait and sensations of fatigue and muscle weakness.8,10,24–26 These newer studies have resulted in a recovery to a TOF of 0.9 or greater prior to extubation becoming widely adopted as a standard of anaesthetic practice following non-depolarising neuromuscular ...

  8. Neuromuscular dysfunction associated with delayed weaning from ...

    African Journals Online (AJOL)

    Yehia Khalil

    2012-03-21

    Mar 21, 2012 ... Neuromuscular dysfunction associated with delayed weaning from mechanical ventilation in patients with respiratory failure. Yehia Khalil a. , Emad El Din Mustafa a. , Ahmed Youssef a. ,. Mohamed Hassan Imam b,. *, Amni Fathy El Behiry a a Department of Chest, Faculty of Medicine, Alexandria University, ...

  9. O uso de bloqueadores neuromusculares no Brasil El uso de bloqueadores neuromusculares en Brasil Neuromuscular blockers in Brazil

    Directory of Open Access Journals (Sweden)

    Maria Cristina Simões de Almeida

    2004-12-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: Dados estatísticos referentes ao uso de bloqueadores neuromusculares no Brasil são desconhecidos. Este trabalho se propõe a análise estatística desse tópico. MÉTODO: Foram compiladas 831 respostas de um questionário preenchido em parte por anestesiologistas presentes ao 48º Congresso Brasileiro de Anestesiologia em Recife, 2001 e em parte via Internet, por anestesiologistas cujos endereços eletrônicos constam na página da Sociedade Brasileira de Anestesiologia (www.sba.com.br. Foram analisados os seguintes dados: tempo de contato com a especialidade, região onde atuam os anestesiologistas, uso de bloqueadores neuromusculares (BNM em ordem de preferência, indicações do uso de succinilcolina, uso do monitor da transmissão neuromuscular, critérios para se considerar o paciente descurarizado, uso de neostigmina, forma de administração dos BNM e descrição de complicações observadas. RESULTADOS: A maioria dos anestesiologistas em questão exerce a profissão há mais de 11 anos e o maior número de respostas foi proveniente da região sudeste do Brasil. O BNM mais empregado é o atracúrio, seguido de pancurônio e succinilcolina. A succinilcolina é mais empregada na indução rápida e em crianças (80% e 25% respectivamente. Monitores da transmissão neuromuscular, 53% dos anestesiologistas nunca usam, e como critério de recuperação, 92% consideram o paciente descurarizado mediante sinais clínicos. Em 45% das vezes os profissionais empregam a neostigmina de forma rotineira, e 94% administra os BNM sob forma de bolus. Cerca de 30% registra ter havido complicação decorrente do uso de BNM. As complicações mais apontadas foram o bloqueio prolongado, o broncoespasmo grave e a curarização residual. CONCLUSÕES: O atracúrio é o bloqueador neuromuscular mais empregado no Brasil, há percentual alto de uso da succinilcolina em situações não emergenciais, o uso de monitores da transmiss

  10. INTERACTION OF VERAPAMIL AND LITHIUM AT THE NEUROMUSCULAR JUNCTION ON RAT ISOLATED MUSCLE-HEMIDIAPHRAGM

    Directory of Open Access Journals (Sweden)

    H. R. Sadeghipour

    1998-08-01

    Full Text Available It has been reported that cither lithium or verapamil can potentiate the neuromuscular blocking activity of certain neuromuscular blockers. In the present investigation, possible interaction of verapamil with lithium has been described. The dose ■ response effects of verapamil and lithium on diaphragmatic contractility were assessed in vitro. Mechanical responses of the muscle to indirect (nerve and direct (muscle electrical stimulation were recorded. Verapamil depressed rat diaphragm twitch tensions induced by nerve stimulation in a dose - dependent manner with the 50 percent depression of the original twitch tensions (ICSQ by 5.6 xlO^mmol/l."nThe IC50 of verapamil for direct stimulation of the muscle was LI x W'5 mmol II. Partial replacement of sodium chloride by lithium chloride (0.5, 1.5 and 5 mmol /1 in the medium did not change the depressant effect of verapamil on muscle twitches induced by direct (muscle or indirect (nerve electrical stimulation.

  11. [Genetic defects and disorders at the neuromuscular junction].

    Science.gov (United States)

    Ohno, Kinji

    2011-07-01

    Genetic defects in molecules expressed at the neuromuscular junction (NMJ) cause congenital myasthenic syndromes (CMSs), which are characterized by muscle weakness, abnormal fatigability, amyotrophy, and minor facial anomalies. Muscle weakness mostly develops under 2 years but is also sometimes seen in adults. Mutations identified to date include (i) muscle nicotinic acetylcholine receptor (AChR) subunits, (ii) rapsyn that anchors and clusters AChRs at the neuromuscular junction, (iii) agrin that is released from the nerve terminal and induces AChR clustering by stimulating the downstream LRP4/MuSK/Dok-7/rapsyn/AChR pathway, (iv) muscle-specific kinase (MuSK) that transmits the AChR-clustering signal from agrin/LRP4 to rapsyn/AChR, (v) Dok-7 that transmits the AChR-clustering signal from agrin/LRP4/MuSK to rapsyn/AChR, (vi) skeletal muscle sodium channel type 1.4 (Nav1.4) that spreads the depolarization potential from the endplate throughout muscle fibers, (vii) collagen Q that anchors acetylcholinesterase to the synaptic basal lamina, and (viii) choline acetyltransferase that resynthesizes acetylcholine from recycled choline at the nerve terminal. In addition, mutations in the heparin sulfate proteoglycan perlecan, which binds to many molecules including collagen Q and dystroglycan, causes Schwartz-Jampel syndrome. Interestingly, mutations in LRP4 cause Cenani-Lenz syndactyly syndrome but not CMS. AChR, MuSK, and LRP4 are also targets of auto-antibodies in myasthenia gravis. In addition, molecules at the NMJ are targets of many other disease states AChRs are blocked by the snake toxin alpha-bungarotoxin and the plant poison curare. The presynaptic SNARE complex is attacked by botulinum toxin. Acetylcholinesterase is inhibited by the nerve gas sarin and by organophosphate pesticides. This review focuses on the molecular bases underlying defects of AChR, rapsyn, Nav1.4, collagen Q, and choline acetyltransferase.

  12. Human dignity and the profoundly disabled: a theological perspective.

    Science.gov (United States)

    Matthews, Pia

    2011-01-01

    One challenge to the concept of human dignity is that it is a rootless notion invoked simply to mask inequalities that inevitably exist between human beings. This privileging of humans is speciesist and its weak point is the profoundly disabled human being. This article argues that far from being a weak point, the profoundly disabled person is a source of strength and witness to the intrinsic dignity that all human beings have by virtue of being human. The disabled represent the reality of human existence that is both strong and fragile. Although human dignity can be understood philosophically its depth is rooted in Christian theological insights. The profoundly disabled occupy a privileged position and share in a theology of mission since they testify to the interdependence of every human being and human dependence on God to a myopic world that only values strength, autonomy and independence.

  13. Improvement in Offaxis Neuromuscular Control Under Slippery Conditions Following Six-Week Pivoting Leg Neuromuscular Training.

    Science.gov (United States)

    Lee, Song Joo; Ren, Yupeng; Press, Joel M; Lee, Jungwha; Zhang, Li-Qun

    2017-11-01

    Knee injuries are usually associated with offaxis loadings in the transverse and frontal planes. Thus, improvement of lower limb offaxis neuromuscular control is important in knee injury prevention and post-injury rehabilitation. The goal of this paper was to investigate the effects of six-week pivoting offaxis intensity adjustable neuromuscular control training (POINT) using a custom-made offaxis elliptical trainer on lower limb offaxis neuromuscular control performance in trained and untrained functional tasks under slippery conditions. Twenty-six subjects participated in 18 sessions of POINT (three sessions per week for six weeks) and 25 subjects served as controls who did a regular workout. Offaxis neuromuscular control performance measures in terms of pivoting instability, sliding instability, and time-to-peak offaxis EMG entropy were evaluated on both groups under slippery conditions including a trained free pivoting task and untrained free sliding task and free pivoting and sliding task. Compared with the control group, the training group significantly decreased pivoting instability and the time-to-peak offaxis EMG entropy in lower limb muscles, indicating improvement in offaxis neuromuscular control performance. Furthermore, the training group showed reduced pivoting instability and sliding instability during the untrained free pivoting and sliding task. This paper may help us develop more focused and effective offaxis training programs to reduce knee injuries associated with offaxis loadings.

  14. Neuromuscular blockade during laparoscopic ventral herniotomy

    DEFF Research Database (Denmark)

    Medici, Roar; Madsen, Matias V; Asadzadeh, Sami

    2015-01-01

    's rating of surgical conditions during suturing, duration of surgery and duration of the suturing of the hernia. CONCLUSION: This randomised cross-over study investigated a potential effect on the surgical workspace in laparoscopic ventral herniotomy using deep NMB compared with no NMB. The study may......INTRODUCTION: Laparoscopic herniotomy is the preferred technique for some ventral hernias. Several factors may influence the surgical conditions, one being the depth of neuromuscular blockade (NMB) applied. We hypothesised that deep neuromuscular blockade defined as a post-tetanic count below eight...... would provide a better surgical workspace. METHODS: This was an investigator-initiated, assessor- and patient-blinded randomised cross-over study. A total of 34 patients with planned laparoscopic umbilical, incisional and linea alba herniotomy were studied. Patients would be randomised to receive deep...

  15. Neuromuscular blockade during laparoscopic ventral herniotomy

    DEFF Research Database (Denmark)

    Medici, Roar; Madsen, Matias V; Asadzadeh, Sami

    2015-01-01

    INTRODUCTION: Laparoscopic herniotomy is the preferred technique for some ventral hernias. Several factors may influence the surgical conditions, one being the depth of neuromuscular blockade (NMB) applied. We hypothesised that deep neuromuscular blockade defined as a post-tetanic count below eight......'s rating of surgical conditions during suturing, duration of surgery and duration of the suturing of the hernia. CONCLUSION: This randomised cross-over study investigated a potential effect on the surgical workspace in laparoscopic ventral herniotomy using deep NMB compared with no NMB. The study may...... provide knowledge relevant to other laparoscopic techniques. FUNDING: The study is funded by a research grant from the Investigator Initiated Studies Program of Merck Sharp & Dohme Corp. TRIAL REGISTRATION: NCT02247466....

  16. Progressive inhibition of neuromuscular structures (PINS) technique.

    Science.gov (United States)

    Dowling, D J

    2000-05-01

    Progressive inhibition of neuromuscular structures (PINS) is a technique that can be included in the osteopathic manipulative treatment repertoire. It relies on knowledge of anatomy and neuromuscular physiologic features as well as on standard forms of osteopathic palpatory diagnosis and treatment. It is a variant of the inhibition technique that has been taught as an osteopathic manipulative technique for many years, and it bears some resemblance to other manual medicine techniques. The emphasis of the approach is the determination of the alteration of the tissues due to dysfunction, delivering treatment based on palpatory evaluation and patient feedback. Two related points are initially chosen, followed by a progression from one to the other. Relationships to similar techniques are also discussed. Theoretical as well as selected practical applications are presented.

  17. Options for mechanical ventilation in neuromuscular diseases.

    Science.gov (United States)

    Unterborn, J N; Hill, N S

    1994-12-01

    A variety of mechanical devices may be used to provide assistance when ventilation and cough are severely impaired by progressive respiratory weakness caused by neuromuscular disease. Traditionally, positive pressure ventilation via a tracheostomy has been used, but if upper airway function is adequate, a variety of noninvasive devices also may be considered. Although positive pressure ventilation is the preferred noninvasive mode for assisting ventilation, other modes may be selected depending on patient needs, preferences, and physical characteristics.

  18. Neuromuscular adaptation to actual and simulated weightlessness

    Science.gov (United States)

    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 RELAXOMETER - A COMPLETE AND COMPREHENSIVE COMPUTER-CONTROLLED NEUROMUSCULAR-TRANSMISSION MEASUREMENT SYSTEM DEVELOPED FOR CLINICAL RESEARCH ON MUSCLE-RELAXANTS

    NARCIS (Netherlands)

    ROWAAN, CJ; VANDENBROM, RHG; WIERDA, JMKH

    The Relaxometer is a computer-controlled system developed for reliable clinical experimental measurements on neuromuscular block. This system is based on an adapted personal computer (Atari 1040 ST) with a monochrome monitor (Atari SM 124), and a microcomputer-driven slave unit (stimulator). There

  20. Heart block

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/007658.htm Heart block To use the sharing features on this page, ... Date 4/16/2017 Updated by: Michael A. Chen, MD, PhD, Associate Professor of Medicine, Division of ...

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

    OpenAIRE

    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...

  2. [Neurotoxic effects of cobra venom (Naja haje haje) on the neuromuscular junction. Electroclinical study of two cases in Tunisia].

    Science.gov (United States)

    Zouari, N; Choyakh, F

    1995-01-01

    Two patients were bitten by tunisian cobras (Naja haje haje). A few hours after the bite they developed generalized paralysis which progressed to respiratory paralysis. Electrophysiological features are similar to those of myasthenia gravis and exhibit decremental pattern after stimulation at a frequency of three per second. It is probable that cobra neurotoxins act postsynaptically. The neuromuscular blocking activity of snake-venom can be reversed by intravenous neostigmine.

  3. Multisensory Speech Perception by Profoundly Hearing-Impaired Children.

    Science.gov (United States)

    Lynch, Michael P.; And Others

    1989-01-01

    Eight profoundly hearing-impaired children, aged 5-11, received tactual word recognition training with tactual speech perception aids. Following training, subjects were tested on trained words and new words. Performance was significantly better on both sets of words when words were presented with a combined condition of tactual aid and aided…

  4. Standing Ovations and Profound Learning: Cultural Diversity in Theatre.

    Science.gov (United States)

    Ellis, Roger

    2000-01-01

    Describes the profound learning that took place at the International Children's Theatre Festival in Toyama City, Japan in July 2000. Argues that participation by the Japanese-American Drama Ensemble, a youth group from the public schools in Lexington, Massachusetts, and more than 400 children from all over the planet, showcased the cultural…

  5. Teaching Profoundly Retarded Adults to Ascend Stairs Safely.

    Science.gov (United States)

    Cipani, Ennio; And Others

    1982-01-01

    The study was designed to modify the stair climbing behavior of two profoundly retarded residents through backward shaping with graduated guidance, edible rewards, a correction procedure, and a 30 second timeout. Both residents showed an increase in the number of correct steps used while ascending the stairs.

  6. Pre-Language Activities for the Profoundly Mentally Retarded.

    Science.gov (United States)

    Poole, Marilyn R.; And Others

    Provided are sample lesson plans for a program to develop pre-language skills in profoundly retarded children and adults. Characteristic of the suggested activities is the stimulation of all sensory channels through structured infant-like play activities in five general areas: oral stimulation, sensory arousal, motor stimulation, vocal play, and…

  7. Profound Haemaological Changes In Rats Fed On Different Diet ...

    African Journals Online (AJOL)

    At the end of six weeks feeding period, blood samples were obtained and total leukocyte count was done. The results of total court show that animals fed in protein supplemented diet had a profound increase in their leukocyte court when compered with the control. The study shows that specific dietary elements can induce ...

  8. Urgencias en patología neuromuscular Emergencies in neuromuscular pathology

    OpenAIRE

    T. Ayuso; I. Jericó

    2008-01-01

    La debilidad muscular aguda (DMA) es el síntoma predominante de las urgencias neuromusculares, especialmente si afecta a la musculatura respiratoria u orofaríngea. La DMA es un síndrome plurietiológico y con distintos niveles lesionales en la unidad motora. Dentro del amplio grupo de enfermedades neuromusculares, las que con mayor frecuencia provocan DMA e insuficiencia respiratoria son el síndrome de Guillain-Barré (SGB) y la miastenia gravis (MG). El SGB constituye la causa más frecuente de...

  9. Prognostic importance of complete atrioventricular block complicating acute myocardial infarction

    DEFF Research Database (Denmark)

    Aplin, Mark; Engstrøm, Thomas; Vejlstrup, Niels G

    2003-01-01

    Third-degree atrioventricular block after acute myocardial infarction is considered to have prognostic importance. However, its importance in conjunction with thrombolytic therapy and its relation to left ventricular function remains uncertain. This report also outlines an important distinction...... between atrioventricular block in the setting of anterior and inferior wall acute myocardial infarction, with profound clinical and prognostic implications....

  10. RhoE deficiency produces postnatal lethality, profound motor deficits and neurodevelopmental delay in mice.

    Directory of Open Access Journals (Sweden)

    Enric Mocholí

    Full Text Available Rnd proteins are a subfamily of Rho GTPases involved in the control of actin cytoskeleton dynamics and other cell functions such as motility, proliferation and survival. Unlike other members of the Rho family, Rnd proteins lack GTPase activity and therefore remain constitutively active. We have recently described that RhoE/Rnd3 is expressed in the Central Nervous System and that it has a role in promoting neurite formation. Despite their possible relevance during development, the role of Rnd proteins in vivo is not known. To get insight into the in vivo function of RhoE we have generated mice lacking RhoE expression by an exon trapping cassette. RhoE null mice (RhoE gt/gt are smaller at birth, display growth retardation and early postnatal death since only half of RhoE gt/gt mice survive beyond postnatal day (PD 15 and 100% are dead by PD 29. RhoE gt/gt mice show an abnormal body position with profound motor impairment and impaired performance in most neurobehavioral tests. Null mutant mice are hypoactive, show an immature locomotor pattern and display a significant delay in the appearance of the hindlimb mature responses. Moreover, they perform worse than the control littermates in the wire suspension, vertical climbing and clinging, righting reflex and negative geotaxis tests. Also, RhoE ablation results in a delay of neuromuscular maturation and in a reduction in the number of spinal motor neurons. Finally, RhoE gt/gt mice lack the common peroneal nerve and, consequently, show a complete atrophy of the target muscles. This is the first model to study the in vivo functions of a member of the Rnd subfamily of proteins, revealing the important role of Rnd3/RhoE in the normal development and suggesting the possible involvement of this protein in neurological disorders.

  11. Neuromuscular Manifestations of West Nile Virus Infection

    Directory of Open Access Journals (Sweden)

    A. Arturo eLeis

    2012-03-01

    Full Text Available 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, peripheral nerve (Guillain-Barré syndrome, brachial plexopathy. In addition, involvement of spinal sympathetic neurons and ganglia provides a plausible 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 neu¬ropathies, 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

  12. Potential protective effects of l-carnitine against neuromuscular ischemia-reperfusion injury: From experimental data to potential clinical applications.

    Science.gov (United States)

    Moghaddas, Azadeh; Dashti-Khavidaki, Simin

    2016-08-01

    Ischemia-reperfusion (I/R) injury plays important role in morbidity and mortality in several pathologies, including myocardial infarction, ischemic stroke, acute kidney injury, trauma, and circulatory arrest. An imbalance in metabolic supply and tissue's demand during ischemia results in profound tissue hypoxia and microvascular dysfunction. Subsequently, reperfusion further results in activation of immune responses and cell death programs. l-carnitine and its derivatives have been administered to improve tolerance against I/R injury in various tissues. Anti-ischemic properties of l-carnitine and its derivative in neuromuscular organs will be reviewed here at the light of pertinent results from basic and clinical researches. All available in vitro and in vivo studies, patents, clinical trials, and meeting abstracts in English language that examined the protective effects of l-carnitine against I/R induced injury in neuromuscular organs were reviewed. Materials were obtained by searching ELSEVIER, web of knowledge, PubMed, Scopus, clinical trials, and Cochrane database of systematic reviews. Although animal studies on central nervous system and some human studies on muscular system were in favors of effects of l-carnitine against I/R injury, however, more clinical trials are needed to clarify the clinical importance of l-carnitine as a treatment option to manage I/R-induced injury of neuromuscular system. Copyright © 2015 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.

  13. Visual impairment in severe and profound sensorineural deafness.

    OpenAIRE

    Armitage, I M; Burke, J. P.; Buffin, J T

    1995-01-01

    The frequency of reversible and irreversible visual impairment was determined in children with severe and profound sensorineural deafness, as subnormal vision can adversely affect their educational and social development. Eighty three of 87 such children attending an audiology service were examined to assess the incidence and severity of visual impairment. Each child underwent a detailed ophthalmic assessment. The criteria for visual impairment were visual acuity < 6/9 Snellen or equivalent a...

  14. Observations on Working Psychoanalytically with a Profoundly Amnesic Patient.

    Science.gov (United States)

    Moore, Paul A; Salas, Christian E; Dockree, Suvi; Turnbull, Oliver H

    2017-01-01

    Individuals with profound amnesia are markedly impaired in explicitly recalling new episodic events, but appear to preserve the capacity to use information from other sources. Amongst these preserved capacities is the ability to form new memories of an emotional nature - a skill at the heart of developing and sustaining interpersonal relationships. The psychoanalytic study of individuals with profound amnesia might contribute to the understanding the importance of each memory system, including effects on key analytic processes such as transference and countertransference. However, psychoanalytic work in the presence of profound amnesia might also require important technical modifications. In the first report of its kind, we describe observations from a long term psychoanalytic process (72 sessions) with an individual (JL) who has profound amnesia after an anoxic episode. The nature of therapy was shaped by JL's impairment in connecting elements that belong to distant (and even relatively close) moments in the therapeutic process. However, we were also able to document areas of preservation, in what appears to be a functioning therapeutic alliance. As regards transference, the relationship between JL and his analyst can be viewed as the evolution of a narcissistic transference, and case material is provided that maps this into three phases: (i) rejecting; (ii) starting to take in; and (iii) full use of the analytic space - where each phase exhibits differing degrees of permeability between JL and the analyst. This investigation appears to have important theoretical implications for psychoanalytic practice, and for psychotherapy in general - and not only with regard to brain injured populations. We especially note that it raises questions concerning the mechanism of therapeutic action in psychoanalysis and psychotherapy, and the apparent unimportance of episodic memory for many elements of therapeutic change.

  15. Profound hyperlipidaemia due to concomitant diabetes and hypothyroidism

    OpenAIRE

    Samaan, M Constantine; Murphy, Nuala; Costigan, Colm

    2010-01-01

    A previously well 5-year-old girl presented with new onset type 1 diabetes mellitus and diabetic ketoacidosis, and was found to be profoundly hyperlipidaemic. Further investigations showed that she had associated hypothyroidism. She responded to insulin and L-thyroxine treatments and her lipid profile returned to normal 2 months after diagnosis. Despite starting anticoagulant therapy early, she developed deep vein thrombosis of the lower limb. Her family screen did not demonstrate familial hy...

  16. Observations on Working Psychoanalytically with a Profoundly Amnesic Patient

    Science.gov (United States)

    Moore, Paul A.; Salas, Christian E.; Dockree, Suvi; Turnbull, Oliver H.

    2017-01-01

    Individuals with profound amnesia are markedly impaired in explicitly recalling new episodic events, but appear to preserve the capacity to use information from other sources. Amongst these preserved capacities is the ability to form new memories of an emotional nature – a skill at the heart of developing and sustaining interpersonal relationships. The psychoanalytic study of individuals with profound amnesia might contribute to the understanding the importance of each memory system, including effects on key analytic processes such as transference and countertransference. However, psychoanalytic work in the presence of profound amnesia might also require important technical modifications. In the first report of its kind, we describe observations from a long term psychoanalytic process (72 sessions) with an individual (JL) who has profound amnesia after an anoxic episode. The nature of therapy was shaped by JL’s impairment in connecting elements that belong to distant (and even relatively close) moments in the therapeutic process. However, we were also able to document areas of preservation, in what appears to be a functioning therapeutic alliance. As regards transference, the relationship between JL and his analyst can be viewed as the evolution of a narcissistic transference, and case material is provided that maps this into three phases: (i) rejecting; (ii) starting to take in; and (iii) full use of the analytic space – where each phase exhibits differing degrees of permeability between JL and the analyst. This investigation appears to have important theoretical implications for psychoanalytic practice, and for psychotherapy in general – and not only with regard to brain injured populations. We especially note that it raises questions concerning the mechanism of therapeutic action in psychoanalysis and psychotherapy, and the apparent unimportance of episodic memory for many elements of therapeutic change. PMID:28890703

  17. Observations on Working Psychoanalytically with a Profoundly Amnesic Patient

    Directory of Open Access Journals (Sweden)

    Paul A. Moore

    2017-08-01

    Full Text Available Individuals with profound amnesia are markedly impaired in explicitly recalling new episodic events, but appear to preserve the capacity to use information from other sources. Amongst these preserved capacities is the ability to form new memories of an emotional nature – a skill at the heart of developing and sustaining interpersonal relationships. The psychoanalytic study of individuals with profound amnesia might contribute to the understanding the importance of each memory system, including effects on key analytic processes such as transference and countertransference. However, psychoanalytic work in the presence of profound amnesia might also require important technical modifications. In the first report of its kind, we describe observations from a long term psychoanalytic process (72 sessions with an individual (JL who has profound amnesia after an anoxic episode. The nature of therapy was shaped by JL’s impairment in connecting elements that belong to distant (and even relatively close moments in the therapeutic process. However, we were also able to document areas of preservation, in what appears to be a functioning therapeutic alliance. As regards transference, the relationship between JL and his analyst can be viewed as the evolution of a narcissistic transference, and case material is provided that maps this into three phases: (i rejecting; (ii starting to take in; and (iii full use of the analytic space – where each phase exhibits differing degrees of permeability between JL and the analyst. This investigation appears to have important theoretical implications for psychoanalytic practice, and for psychotherapy in general – and not only with regard to brain injured populations. We especially note that it raises questions concerning the mechanism of therapeutic action in psychoanalysis and psychotherapy, and the apparent unimportance of episodic memory for many elements of therapeutic change.

  18. Foveal Processing Under Concurrent Peripheral Load in Profoundly Deaf Adults

    Science.gov (United States)

    2016-01-01

    Development of the visual system typically proceeds in concert with the development of audition. One result is that the visual system of profoundly deaf individuals differs from that of those with typical auditory systems. While past research has suggested deaf people have enhanced attention in the visual periphery, it is still unclear whether or not this enhancement entails deficits in central vision. Profoundly deaf and typically hearing adults were administered a variant of the useful field of view task that independently assessed performance on concurrent central and peripheral tasks. Identification of a foveated target was impaired by a concurrent selective peripheral attention task, more so in profoundly deaf adults than in the typically hearing. Previous findings of enhanced performance on the peripheral task were not replicated. These data are discussed in terms of flexible allocation of spatial attention targeted towards perceived task demands, and support a modified “division of labor” hypothesis whereby attentional resources co-opted to process peripheral space result in reduced resources in the central visual field. PMID:26657078

  19. Medical back belt with integrated neuromuscular electrical stimulation

    NARCIS (Netherlands)

    Bottenberg, E. (Eliza); Brinks, G.J. (Ger); Hesse, J. (Jenny)

    2014-01-01

    The medical back belt with integrated neuromuscular electrical stimulation is anorthopedic device, which has two main functions. The first function is to stimulate the backmuscles by using a neuromuscular electrical stimulation device that releases regular,electrical impulses. The second function of

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

    Science.gov (United States)

    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.

  1. Intubation of Profoundly Agitated Patients Treated with Prehospital Ketamine.

    Science.gov (United States)

    Olives, Travis D; Nystrom, Paul C; Cole, Jon B; Dodd, Kenneth W; Ho, Jeffrey D

    2016-12-01

    Profound agitation in the prehospital setting confers substantial risk to patients and providers. Optimal chemical sedation in this setting remains unclear. The goal of this study was to describe intubation rates among profoundly agitated patients treated with prehospital ketamine and to characterize clinically significant outcomes of a prehospital ketamine protocol. This was a retrospective cohort study of all patients who received prehospital ketamine, per a predefined protocol, for control of profound agitation and who subsequently were transported to an urban Level 1 trauma center from May 1, 2010 through August 31, 2013. Identified records were reviewed for basic ambulance run information, subject characteristics, ketamine dosing, and rate of intubation. Emergency Medical Services (EMS) ambulance run data were matched to hospital-based electronic medical records. Clinically significant outcomes are characterized, including unadjusted and adjusted rates of intubation. Overall, ketamine was administered 227 times in the prehospital setting with 135 cases meeting study criteria of use of ketamine for treatment of agitation. Endotracheal intubation was undertaken for 63% (85/135) of patients, including attempted prehospital intubation in four cases. Male gender and late night arrival were associated with intubation in univariate analyses (χ2=12.02; P=.001 and χ2=5.34; P=.021, respectively). Neither ketamine dose, co-administration of additional sedating medications, nor evidence of ethanol (ETOH) or sympathomimetic ingestion was associated with intubation. The association between intubation and both male gender and late night emergency department (ED) arrival persisted in multivariate analysis. Neither higher dose (>5mg/kg) ketamine nor co-administration of midazolam or haloperidol was associated with intubation in logistic regression modeling of the 120 subjects with weights recorded. Two deaths were observed. Post-hoc analysis of intubation rates suggested a

  2. Electrophysiological study in neuromuscular junction disorders

    Directory of Open Access Journals (Sweden)

    Ajith Cherian

    2013-01-01

    Full Text Available This review is on ultrastructure and subcellular physiology at normal and abnormal neuromuscular junctions. The clinical and electrophysiological findings in myasthenia gravis, Lambert-Eaton myasthenic syndrome (LEMS, congenital myasthenic syndromes, and botulinum intoxication are discussed. Single fiber electromyography (SFEMG helps to explain the basis of testing neuromuscular junction function by repetitive nerve stimulation (RNS. SFEMG requires skill and patience and its availability is limited to a few centers. For RNS supramaximal stimulation is essential and so is display of the whole waveform of each muscle response at maximum amplitude. The amplitudes of the negative phase of the first and fourth responses are measured from baseline to negative peak, and the percent change of the fourth response compared with the first represents the decrement or increment. A decrement greater than 10% is accepted as abnormal and smooth progression of response amplitude train and reproducibility form the crux. In suspected LEMS the effect of fast rates of stimulation should be determined after RNS response to slow rates of stimulation. Caution is required to avoid misinterpretation of potentiation and pseudofacilitation.

  3. Cardiorespiratory and neuromuscular responses to motocross riding.

    Science.gov (United States)

    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 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.

  4. Neuromuscular imaging in inherited muscle diseases

    Energy Technology Data Exchange (ETDEWEB)

    Wattjes, Mike P. [VU University Medical Center, Department of Radiology, De Boelelaan 1117, HV, Amsterdam (Netherlands); Kley, Rudolf A. [Klinken Bergmannsheil, Ruhr-University, Department of Neurology, Neuromuscular Centre Ruhrgebiet, Bochum (Germany); Fischer, Dirk [University Hospital of Basel, Department of Neurology, Basel (Switzerland); University Children' s Hospital Basel, Department of Neuropaediatrics, Basel (Switzerland)

    2010-10-15

    Driven by increasing numbers of newly identified genetic defects and new insights into the field of inherited muscle diseases, neuromuscular imaging in general and magnetic resonance imaging (MRI) in particular are increasingly being used to characterise the severity and pattern of muscle involvement. Although muscle biopsy is still the gold standard for the establishment of the definitive diagnosis, muscular imaging is an important diagnostic tool for the detection and quantification of dystrophic changes during the clinical workup of patients with hereditary muscle diseases. MRI is frequently used to describe muscle involvement patterns, which aids in narrowing of the differential diagnosis and distinguishing between dystrophic and non-dystrophic diseases. Recent work has demonstrated the usefulness of muscle imaging for the detection of specific congenital myopathies, mainly for the identification of the underlying genetic defect in core and centronuclear myopathies. Muscle imaging demonstrates characteristic patterns, which can be helpful for the differentiation of individual limb girdle muscular dystrophies. The aim of this review is to give a comprehensive overview of current methods and applications as well as future perspectives in the field of neuromuscular imaging in inherited muscle diseases. We also provide diagnostic algorithms that might guide us through the differential diagnosis in hereditary myopathies. (orig.)

  5. Neuromuscular dressing effects: a literature review

    Directory of Open Access Journals (Sweden)

    Calero PA

    2012-05-01

    Full Text Available The kinesio taping is a technique that was created in 1979 by Doctor Kenzo Kase I’m looking through it that could generate a new therapeutic option to control pain, improve athletic performance and reduce the impact of musculoskeletal disorders. From the Sydney 2000 Olympic Games, this technique as a therapeutic alternative PTO and is composed of health professionals in the field of sport and physical rehabilitation.Objetive: This article aims to identify theoretical approaches on the bandage neuromuscular. Material and methods: held today, for which conducted a literature search of databases such as como Proquest, Ovid, Cochraine, PEDro, Journal of Orthopedic and Sports Physical, Sciencedirect, Pubmed y Literatura Latinoamericana y del Caribe en Ciencias de la Salud (Lilacs.The paper proposes a scheme of contextualization of the current landscape of the use and effects of kinesio taping in the management of different pathologies of the musculo-skeletal system in sports. Conclusion: it is concluded that currently many health professionals, and take the neuromuscular bandage a good therapeutic option in the management of diseases affecting the human body is investigated and every day more about the subject, which makes these new therapeutic methods to acquire a scientific value and transcends knowledge.

  6. Prevalence of complications in neuromuscular scoliosis surgery

    DEFF Research Database (Denmark)

    Sharma, Shallu; Wu, Chunsen; Andersen, Thomas

    2013-01-01

    PURPOSE: Our objectives were primarily to review the published literature on complications in neuromuscular scoliosis (NMS) surgery and secondarily, by means of a meta-analysis, to determine the overall pooled rates (PR) of various complications associated with NMS surgery. METHODS: PubMed and Em......PURPOSE: Our objectives were primarily to review the published literature on complications in neuromuscular scoliosis (NMS) surgery and secondarily, by means of a meta-analysis, to determine the overall pooled rates (PR) of various complications associated with NMS surgery. METHODS: Pub......Med and Embase databases were searched for studies reporting the outcomes and complications of NMS surgery, published from 1997 to May 2011. We focused on NMS as defined by the Scoliosis Research Society's classification. We measured the pooled estimate of the overall complication rates (PR) using a random....... In regard to surgical complications affiliated with various surgical techniques in NMS, the level of evidence of published literature ranges between 2+ to 2-; the subsequent recommendations are level C. CONCLUSION: NMS patients have diverse and high complication rates after scoliosis surgery. High PRs...

  7. Ensayos en Ambiente Hospitalario de Técnicas de Monitorización de Bloqueo Neuromuscular: Resultados Preliminares

    Directory of Open Access Journals (Sweden)

    Yadel Forneiro Martín Viaña

    2014-07-01

    Introduction: In order to evaluate the magnitude of neuromuscular blocking during anesthesia, the anesthesiologists consider the muscular response to peripheral nerves stimulation. Objective: Study aims to calculate muscle relaxation indicators, through neuromuscular blocking and its algorithms using a Cuban made monitoring device during the medical procedure. Materials and methods: Thumb kinetic response and muscles relaxation indicators were registered and calculated automatically; for this purpose, median and nerves trials using Troin-of – Four (TOF were conducted in shorter surgeries, which allowed to monitor patients’ neuromuscular, intraoperative function. Scientific consel and the Committee of Ethics of the Clinical Surgical Hospital “HermanosAmeijeiras” approved the protocol, based on a research project conducted by the ICID – Medical Digital technology and the Hospital. Results: the monitoring was stable during all medical procedures as indicated on graph data obtained during five sample cases indicating muscle relaxation indicators. Conclusion: muscle relaxation indicators registered during trials showed significant relevance relation to patient’s clinical history, giving support to evidence of the monitoring implemented techniques based on specialists’ criteria.

  8. Interaural comparison of spiral ganglion cell counts in profound deafness.

    Science.gov (United States)

    Seyyedi, Mohammad; Eddington, Donald K; Nadol, Joseph B

    2011-12-01

    This study is designed to measure the degree to which spiral ganglion cell (SGC) survival in the left and right ears is similar in profoundly hearing-impaired human patients with symmetric (right/left) etiology and sensitivity. This is of interest because a small difference between ears would imply that one ear could be used as a control ear in temporal bone studies evaluating the impact on SGC survival of a medical intervention in the other ear. Forty-two temporal bones from 21 individuals with bilaterally symmetric profound hearing impairment were studied. Both ears in each individual were impaired by the same etiology. Rosenthal's canal was reconstructed in two dimensions and segmental and total SGCs were counted. Correlation analysis and t-tests were used to compare segmental and total counts of left and right ears. Statistical power calculations illustrate how the results can be used to estimate the effect size (right/left difference in SGC count) that can be reliably identified as a function of sample size. Left counts (segmental and total) were significantly correlated with those in the right ears (p total count were respectively 0.64, 0.91, 0.93, 0.91 and 0.98. The hypothesis that mean segmental and total counts of right and left are the same could not be rejected by paired t-test. The variance in the between-ear difference across the temporal bones studied indicates that useful effect sizes can be reliably identified using subject numbers that are practical for temporal bone studies. For instance, there is 95% likelihood that an interaural difference in SGC count of approximately 1000 cells associated with a treatment/manipulation of one ear will be reliably detected in a bilaterally-symmetric profound hearing loss population of temporal bones from approximately 10 subjects. Copyright © 2011 Elsevier B.V. All rights reserved.

  9. Profound Muscle Weakness and Pain after One Dose of Actonel

    Directory of Open Access Journals (Sweden)

    Irina Badayan

    2009-01-01

    Full Text Available The World Health Organization (WHO defines osteopenia as a bone density between 1 and 2.5 standard deviation (SD below the bone density of a normal young adult Iqbal 2000. Osteoporosis is defined as 2.5 SD or more below that reference point Iqbal 2000. Bisphosphonates are a group of medications used to treat osteoporosis, Padget's disease of bone, and osteopenia. We report a woman who developed profound muscle weakness and pain after one dose of Risedronate (Actonel.

  10. Deep Neuromuscular Blockade Improves Laparoscopic Surgical Conditions

    DEFF Research Database (Denmark)

    Rosenberg, Jacob; Herring, W Joseph; Blobner, Manfred

    2017-01-01

    Hg; 'standard') insufflation pressure in a 2 × 2 factorial design. Primary endpoint was surgeon's overall satisfaction with surgical conditions, rated at end of surgery using an 11-point numerical scale. Post-operative pain scores were also evaluated. Data were analyzed using analysis of covariance. RESULTS......INTRODUCTION: Sustained deep neuromuscular blockade (NMB) during laparoscopic surgery may facilitate optimal surgical conditions. This exploratory study assessed whether deep NMB improves surgical conditions and, in doing so, allows use of lower insufflation pressures during laparoscopic......: Of 127 randomized patients, 120 had evaluable data for the primary endpoint. Surgeon's score of overall satisfaction with surgical conditions was significantly higher with deep versus moderate NMB indicated by a least-square mean difference of 1.1 points (95% confidence interval 0.1-2.0; P = 0...

  11. Deep Neuromuscular Blockade Improves Laparoscopic Surgical Conditions

    DEFF Research Database (Denmark)

    Rosenberg, Jacob; Herring, W Joseph; Blobner, Manfred

    2017-01-01

    INTRODUCTION: Sustained deep neuromuscular blockade (NMB) during laparoscopic surgery may facilitate optimal surgical conditions. This exploratory study assessed whether deep NMB improves surgical conditions and, in doing so, allows use of lower insufflation pressures during laparoscopic......Hg; 'standard') insufflation pressure in a 2 × 2 factorial design. Primary endpoint was surgeon's overall satisfaction with surgical conditions, rated at end of surgery using an 11-point numerical scale. Post-operative pain scores were also evaluated. Data were analyzed using analysis of covariance. RESULTS......: Of 127 randomized patients, 120 had evaluable data for the primary endpoint. Surgeon's score of overall satisfaction with surgical conditions was significantly higher with deep versus moderate NMB indicated by a least-square mean difference of 1.1 points (95% confidence interval 0.1-2.0; P = 0...

  12. Eptifibatide-induced acute profound thrombocytopenia: a case report.

    Science.gov (United States)

    Graidis, Christos; Golias, Christos; Dimitriadis, Dimokritos; Dimitriadis, Georgios; Bitsis, Theodosis; Dimitrelos, Ilias; Tsiakou, Afroditi; Charalabopoulos, Konstantinos

    2014-02-25

    The interactions among cells or among cells and components of the extracellular matrix, is a crucial pathophysiological process involving some molecules collectively known as adhesion molecules (CAMs). Glycoprotein IIb / IIIa receptors are only restricted to blood platelets and they bind fibrinogen and adhesion proteins such as fibronectin, vitronectin, von Willebrand factor to form cross bridges between adjacent platelets. IIb/IIIa receptor antagonists are an object of intense research activity for target therapy worldwide during the last decades. Three GPIIb/IIIa inhibitors, abciximab, tirofiban, and eptifibatide, have been approved for clinical use. Profound thrombocytopenia is an uncommon but clinically important complication of glycoprotein IIb/IIIa inhibitors. This case report discusses a forty-four-year-old male patient with acute coronary syndrome who underwent percutaneous coronary intervention and developed profound thrombocytopenia within 4 hours of first administration of eptifibatide. This report adds another case of eptifibatide-induced thrombocytopenia to the medical literature and endorses the importance of platelet count monitoring after initiating therapy with this agent.

  13. Monitoring strength training: neuromuscular and hormonal profile.

    Science.gov (United States)

    Bosco, C; Colli, R; Bonomi, R; von Duvillard, S P; Viru, A

    2000-01-01

    This study investigated changes induced by a single heavy resistance training session on neuromuscular and endocrine systems in trained athletes, using the same exercises for training and testing. Five different groups volunteered: track and field male sprinters (MS, N = 6), track and field female sprinters (FS, N = 6), body builders (BB, N = 6), and weight lifters performing low-repetition exercise (WLL, N = 4) and high-repetition exercise (WLH, N = 4). In training, the work performed during half and full squat exercise was monitored for mechanical power output as well as EMG analysis on leg extensor muscles of the subjects belonging to the MS, FS, and BB groups. Just before and immediately after the training session, venous blood samples were obtained for RIA determination of testosterone (T), cortisol (C), lutropin (LH), human prolactin (PRL), and follitropin (FSH) in FS and MS. In the other three groups (BB, WLH, and WLL), the hormonal profile was limited to T and human growth hormone (hGH) only. After training the power developed in full squat demonstrated a statistically significant decrease (P training session. Consequently, the EMG/Power ratio increased in both MS and FS, although only in MS a statistical significance was noted (P < 0.05). In MS immediately after the session the levels of C, T, and LH were significantly lower (P < 0.05). No changes were found in FS. In both groups and in BB significant negative correlation was found between changes in T level and EMG/Power ratio in half squat performance. It is likely that adequate T level may compensate the effect of fatigue in FT fibers by ensuring a better neuromuscular efficiency.

  14. Deep neuromuscular blockade and low insufflation pressure during laparoscopic hysterectomy.

    Science.gov (United States)

    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 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, which permits any non-commercial use, distribution, and reproduction in any

  15. Persistent Thalamic Sound Processing Despite Profound Cochlear Denervation

    Directory of Open Access Journals (Sweden)

    Anna R. Chambers

    2016-08-01

    Full Text Available Neurons at higher stages of sensory processing can partially compensate for a sudden drop in input from the periphery through a homeostatic plasticity process that increases the gain on weak afferent inputs. Even after a profound unilateral auditory neuropathy where > 95% of synapses between auditory nerve fibers and inner hair cells have been eliminated with ouabain, central gain can restore the cortical processing and perceptual detection of basic sounds delivered to the denervated ear. In this model of profound auditory neuropathy, cortical processing and perception recover despite the absence of an auditory brainstem response (ABR or brainstem acoustic reflexes, and only a partial recovery of sound processing at the level of the inferior colliculus (IC, an auditory midbrain nucleus. In this study, we induced a profound cochlear neuropathy with ouabain and asked whether central gain enabled a compensatory plasticity in the auditory thalamus comparable to the full recovery of function previously observed in the auditory cortex (ACtx, the partial recovery observed in the IC, or something different entirely. Unilateral ouabain treatment in adult mice effectively eliminated the ABR, yet robust sound-evoked activity persisted in a minority of units recorded from the contralateral medial geniculate body (MGB of awake mice. Sound-driven MGB units could decode moderate and high-intensity sounds with accuracies comparable to sham-treated control mice, but low-intensity classification was near chance. Pure tone receptive fields and synchronization to broadband pulse trains also persisted, albeit with significantly reduced quality and precision, respectively. MGB decoding of temporally modulated pulse trains and speech tokens were both greatly impaired in ouabain-treated mice. Taken together, the absence of an ABR belied a persistent auditory processing at the level of the MGB that was likely enabled through increased central gain. Compensatory

  16. Seeking a better landscape for therapy development in neuromuscular disorders.

    Science.gov (United States)

    Larkindale, Jane; Porter, John D

    2018-01-01

    Although the neuromuscular field has seen accelerated approval of a drug for Duchenne muscular dystrophy (DMD) and full approval of one for spinal muscular atrophy, these experiences have shown that objective data and an adequate level of effect are essential for drug approval and reimbursement. The appropriateness and validity of biomarkers and clinically meaningful endpoints and an understanding of disease progression rates all played essential roles in the levels of evidence for these drugs. Such tools are best developed through integration of clinical data. The siloing of clinical data for rare neuromuscular diseases represents a considerable barrier to achieving better care and novel therapies for patients living with neuromuscular diseases. We discuss a data-sharing model implemented for DMD and urge cultural changes in the ways natural history and clinical trial data are collected and shared across all neuromuscular diseases in order to benefit the primary stakeholder, the patient. Muscle Nerve 57: 16-19, 2018. © 2017 Wiley Periodicals, Inc.

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

    Science.gov (United States)

    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. © The Author(s) 2015.

  18. Neuromuscular training for sports injury prevention: a systematic review

    National Research Council Canada - National Science Library

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

    2010-01-01

    The aim of this systematic review was to assess the effectiveness of proprioceptive/neuromuscular training in preventing sports injuries by using the best available evidence from methodologically well...

  19. Quantitative skeletal muscle ultrasound: diagnostic value in childhood neuromuscular disease.

    NARCIS (Netherlands)

    Pillen, S.; Verrips, A.; Alfen, N. van; Arts, I.M.P.; Sie, L.T.L.; Zwarts, M.J.

    2007-01-01

    In this study we investigated the diagnostic value of quantitative skeletal muscle ultrasonography in 150 consecutively referred children with symptoms suspect for a neuromuscular disorder. Muscle thickness and quantitatively determined echo intensity of four muscles and the distribution of these

  20. Effects of napping on neuromuscular fatigue in myasthenia gravis.

    Science.gov (United States)

    Kassardjian, Charles D; Murray, Brian J; Kokokyi, Seint; Jewell, Dana; Barnett, Carolina; Bril, Vera; Katzberg, Hans D

    2013-11-01

    The relationship between sleep and neuromuscular fatigue is understood poorly. The goal of this study was to evaluate the effects of napping on quantitative measures of neuromuscular fatigue in patients with myasthenia gravis (MG). Eight patients with mild to moderate MG were recruited. Patients underwent maintenance of wakefulness tests (MWT) and multiple sleep latency tests (MSLT). The Quantitative Myasthenia Gravis Score (QMGS) was measured before nap and after each nap to examine the effects of napping and sleep on neuromuscular weakness. Results showed that QMGS improves only after naps where patients slept more than 5 min but not where patients did not sleep or slept less than 5 min. Daytime napping mitigates neuromuscular fatigue in patients with MG, especially if patients slept for more than 5 min. Copyright © 2013 Wiley Periodicals, Inc.

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

    Science.gov (United States)

    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. [Neuromuscular relaxation and CCMDP. The Zilgrei and Feldenkrais methods 2].

    Science.gov (United States)

    Santoro, F; Maiorana, C; Faccin, C

    1989-10-31

    The Authors show two neuromuscular release methods employed in the treatment of cranio-cervico-mandibular syndrome; these methods work at the place of origin of the pathology resolving the symptoms in different districts of the body.

  3. Exploring employment in consultation reports of patients with neuromuscular diseases

    NARCIS (Netherlands)

    Minis, M.A.H; Cup, E.H.C.; Heerkens, Y.H.; Engels, J.A.; Engelen, B.G.M. van; Oostendorp, R.A.B.

    2012-01-01

    Minis MA, Cup EH, Heerkens YF, Engels JA, van Engelen BG, Oostendorp RA. Exploring employment in consultation reports of patients with neuromuscular diseases. OBJECTIVES: To explore consultation reports for patient and employment characteristics and recommendations on employment regarding patients

  4. Electrostatic control of block copolymer morphology

    Science.gov (United States)

    Sing, Charles E.; Zwanikken, Jos W.; Olvera de La Cruz, Monica

    2014-07-01

    Energy storage is at present one of the foremost issues society faces. However, material challenges now serve as bottlenecks in technological progress. Lithium-ion batteries are the current gold standard to meet energy storage needs; however, they are limited owing to the inherent instability of liquid electrolytes. Block copolymers can self-assemble into nanostructures that simultaneously facilitate ion transport and provide mechanical stability. The ions themselves have a profound, yet previously unpredictable, effect on how these nanostructures assemble and thus the efficiency of ion transport. Here we demonstrate that varying the charge of a block copolymer is a powerful mechanism to predictably tune nanostructures. In particular, we demonstrate that highly asymmetric charge cohesion effects can induce the formation of nanostructures that are inaccessible to conventional uncharged block copolymers, including percolated phases desired for ion transport. This vastly expands the design space for block copolymer materials and is informative for the versatile design of battery electrolyte materials.

  5. EFFECT OF NEUROMUSCULAR TRAINING ON BALANCE AMONG UNIVERSITY ATHLETES

    OpenAIRE

    Mohansundar Sankaravel; Jeffrey Low Fook Lee; Ong Kuan Boon; Sanmuganathan Jeganathan

    2016-01-01

    Background: Proprioceptive deficiency followed by lateral ankle sprain leads to poor balance is not uncommon. It has been linked with increased injury risk among young athletes. Introducing neuromuscular training programs for this have been believed as one of the means of injury prevention. Hence, this study was aimed to determine the effects of six weeks progressive neuromuscular training (PNM Training) on static balance gains among the young athletes with a previous history of ankle sprains...

  6. Children with profound intellectual and multiple disabilities : the effects of functional movement activities

    NARCIS (Netherlands)

    van der Putten, A; Vlaskamp, C; Reynders, K; Nakken, H

    Objective: To determine the effect of functional movement activities within the MOVE ( Mobility Opportunities Via Education) curriculum on the independence of children with profound intellectual and multiple disabilities. Subjects: Forty-four children with profound intellectual and multiple

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

  9. Efficacy and safety of sugammadex versus neostigmine in reversing neuromuscular blockade in adults

    DEFF Research Database (Denmark)

    Hristovska, Ana-Marija; Duch, Patricia; Allingstrup, Mikkel

    2017-01-01

    in adults. SEARCH METHODS: We searched the following databases on 2 May 2016: Cochrane Central Register of Controlled Trials (CENTRAL); MEDLINE (WebSPIRS Ovid SP), Embase (WebSPIRS Ovid SP), and the clinical trials registries www.controlled-trials.com, clinicaltrials.gov, and www.centerwatch.com. We re...... NMB from the second twitch (T2) to TOFR > 0.9 (MD 10.22 minutes, 95% CI 8.48 to 11.96; I2 = 84%; 10 studies, n = 835; GRADE: moderate quality).We compared sugammadex 4 mg/kg and neostigmine 0.07 mg/kg for reversal of rocuronium-induced deep NMB. Sugammadex 4 mg/kg was 45.78 minutes (16.8 times) faster...... of the block. Sugammadex 2 mg/kg is 10.22 minutes (˜ 6.6 times) faster in reversing moderate neuromuscular blockade (T2) than neostigmine 0.05 mg/kg (GRADE: moderate quality), and sugammadex 4 mg/kg is 45.78 minutes (˜ 16.8 times) faster in reversing deep neuromuscular blockade (PTC 1 to 5) than neostigmine 0...

  10. Sleep polygraphic parameters in neuromuscular diseases

    Directory of Open Access Journals (Sweden)

    Márcia Pradella

    1994-12-01

    Full Text Available In a polysomnography study of 32 neuromuscular patients - 22 with a form of muscular dystrophy, 3 with a form of congenital myopathy, 4 with a form of spinal muscular atrophy, 1 with a recurrent form of polymyositis and 1 with osteogenesis imperfecta syndrome - of which 21 were nonambulatory, we observed sleep related respiratory disturbances represented by: drops in oxygen saturation (SaO2, cardiac arrythmia, sleep disruption, apneas, tachypnea, tachycardia and snoring. Nine out of the cohort of 32 patients presented with significant desaturations periods. These patients presented with an associated restrictive syndrome and thoracic deformities, some with tachypnea and/or SaO2 below 90% during wakefulness. In this group, snoring was observed in those patients with a form of muscular dystrophy while tachypnea was observed in patients who presented the highest desaturations levels. Sleep quantification revealed an increase of stage 1 sleep coupled with a decrease or even total absence of REM sleep. This is, we believe, a likely consequence of episodic desaturations that may accompany sleep hypoventilation which is potentialised during REM sleep stage.

  11. Neuromuscular disorders in chronic alcohol intoxication

    Directory of Open Access Journals (Sweden)

    A. Yu. Emelyanova

    2015-01-01

    Full Text Available The paper reviews the present-day Russian and foreign literature on neuromuscular disorders in chronic alcohol intoxication. The most common manifestations of alcohol disease include alcoholic polyneuropathy (PNP and alcohol-induced skeletal muscle injury. The clinical polymorphism of alcoholic PNP is discussed. The paper considers a chronic sensory automatic form due to the direct toxic effects of ethanol and its metabolites during long-term alcohol intoxication, as well as acute/subacute sensorimotor neuropathy, the basis for the pathogenesis of which is B group vitamins, predominantly thiamine, deficiency that develops in the presence of drinking bouts concurrent with malnutrition and/or alcohol-related gastrointestinal tract diseases. In addition to nonuse of alcohol and a properly balanced diet, antioxidant therapy with alphalipoic acid and neurotropic B group vitamins is considered to be pathogenetic therapy for neuropathy. The most common and least studied clinicalform of alcohol-induced musculoskeletal injury is chronic alcoholic myopathy (AM, the diagnostic standard for which is morphometricand immunohistochemical examination of a muscle biopsy specimen. The morphological base for this form of myopathy is predominantly type 2 muscle fiber atrophy caused by impaired protein synthesis and a decreased regenerative potential of muscle fiber. The efficacy of antioxidants and leucine-containing amino acid mixtures in the treatment of chronic AM is discussed.

  12. Neuromuscular Fatigue During 200 M Breaststroke

    Directory of Open Access Journals (Sweden)

    Ana Conceição

    2014-03-01

    Full Text Available 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.

  13. Effect of hypocarbia and hypercarbia on the antagonism of pancuronium-induced neuromuscular blockade with neostigmine in man.

    Science.gov (United States)

    Wirtavuori, K; Salmenperä, M; Tammisto, T

    1982-01-01

    The effects of variations in carbon dioxide concentration on the antagonism of pancuronium-induced neuromuscular block by neostigmine were studied in 21 patients: normocarbia (PE'CO2 5.4%, PaCO2 4.93 kPa, n = 7), hypocarbia (PE'CO2 3.6%, PaCO2 3.30 kPa, n = 7) and hypercarbia (PE'CO2 7.5%, PaCO2 7.13 kPa, n = 7). Mechanical and electromyographic responses to ulnar nerve stimulation (0.1 Hz and 2 Hz) were recorded. A 90% block during nitrous oxide in oxygen anaesthesia was maintained by incremental single injections of pancuronium and reversed with neostigmine 0.35 mg kg-1 with atropine 0.0175 mg kg-1. The recovery of twitch tension up to 50% was similar in all groups but thereafter slower in the hypercarbia group. The recovery times from 25% to 75% twitch tension correlated with PaCO2 (r = 0.55, P less than 0.05). A residual block of about 10% was seen in hypercarbic patients. However, the recovery of e.m.g. amplitude and train-of-four ratios was similar in all groups. Thus, the impaired recovery of twitch tension seems to be the result of depressed contractility rather than failure of neuromuscular transmission.

  14. Adductor canal block can result in motor block of the quadriceps muscle.

    Science.gov (United States)

    Chen, Junping; Lesser, Jonathan B; Hadzic, Admir; Reiss, Wojciech; Resta-Flarer, Francesco

    2014-01-01

    The block of nerves in the adductor canal is considered to cause a sensory block without a motor component. In this report, we describe a case of significant quadriceps muscle weakness after an adductor canal block (ACB). A 65-year-old female patient for ambulatory knee surgery was given an ACB for postoperative pain management. The block was performed under ultrasound guidance at the midthigh level using the transsartorial approach. Twenty milliliters of 0.5% ropivacaine was deposited adjacent to the anterior and posterior areas of the femoral artery. On discharge from the hospital, the patient realized that her thigh muscles were weak and she was unable to extend her leg at the knee. A neuromuscular examination indicated that the patient had no strength in her quadriceps muscle, along with sensory deficit in the medial-anterior lower leg and area in front of knee up to the midthigh. The weakness lasted 20 hours, and the sensory block lasted 48 hours before complete recovery. The optimal level and amount of local anesthetic for adductor canal block are currently not well defined. Proximal spread of local anesthetic and anatomical variation may explain our observation. Several studies have reported that ACB involves no motor blockade. However, our case report illustrates that the ACB can result in clinically significant quadriceps muscle paralysis. This report suggests that patients should be monitored vigilantly for this occurrence to decrease the risk of falls.

  15. Multisensory speech perception of young children with profound hearing loss.

    Science.gov (United States)

    Kishon-Rabin, L; Haras, N; Bergman, M

    1997-10-01

    The contribution of a two-channel vibrotactile aid (Trill VTA 2/3, AVR Communications LTD) to the audiovisual perception of speech was evaluated in four young children with profound hearing loss using words and speech pattern contrasts. An intensive, hierarchical, and systematic training program was provided. The results show that the addition of the tactile (T) modality to the auditory and visual (A+V) modalities enhanced speech perception performance significantly on all tests. Specifically, at the end of the training sessions, the tactile supplementation increased word recognition scores in a 44-word, closed-set task by 12 percentage points; detection of consonant in final position by 50 percentage points; detection of sibilant in final position by 30 percentage points; and detection of voicing in final position by 25 percentage points. Significant learning over time was evident for all test materials, in all modalities. As expected, fastest learning (i.e., smallest time constants) was found for the AVT condition. The results of this study provide further evidence that sensory information provided by the tactile modality can enhance speech perception in young children.

  16. EFFECT OF JUMPS IN PROFOUNDNESS ON THE FOOTBALLER REFLECTION

    Directory of Open Access Journals (Sweden)

    Nebojša Đošić

    2011-09-01

    Full Text Available The treatment endured one month. For this time 8 trainings and totally 322 jums in profoundness were done with differant altitudes from 42 cm to 105 cm. The complete time of work with time-out between sequences was about 120 minutes. The time-out between the sequences was from 2 ' til 6'. The pause between the training was from 2 to 5 days. The puls after 30 '' from the finishing of the jums in th sequence was from 100 to 140 pulsation in the minute measured by palpation. On the finaly measurement is constated that the two leg jump from place in height was better for 7 cm , and the one leg jump with three footsteps spring was better for 2 cm. This such result indicate on the assumption that the progress must bi greater if the program would be longer , for example two, three months and if this program should be done by footbalers which are active and in the best player years i.e. between 18 -30 year. The author was by this time 40 years old.

  17. Cancer Prevention Knowledge of People with Profound Hearing Loss

    Science.gov (United States)

    Meador, Helen E.; Reed, Barbara D.; Sen, Ananda; Gorenflo, Daniel W.

    2009-01-01

    BACKGROUND Deaf persons, a documented minority population, have low reading levels and difficulty communicating with physicians. The effect of these on their knowledge of cancer prevention recommendations is unknown. METHODS A cross-sectional study of 222 d/Deaf persons in Michigan, age 18 and older, chose one of four ways (voice, video of a certified American Sign Language interpreter, captions, or printed English) to complete a self-administered computer video questionnaire about demographics, hearing loss, language history, health-care utilization, and health-care information sources, as well as family and social variables. Twelve questions tested their knowledge of cancer prevention recommendations. The outcome measures were the percentage of correct answers to the questions and the association of multiple variables with these responses. RESULTS Participants averaged 22.9% correct answers with no gender difference. Univariate analysis revealed that smoking history, types of medical problems, last physician visit, and women having previous cancer preventive tests did not affect scores. Improved scores occurred with computer use (p = 0.05), higher education (p English in multiple situations (p English use (p = 0.01) and believing that smoking was bad (p = 0.05) were associated with improved scores. CONCLUSION Persons with profound hearing loss have poor knowledge of recommended cancer prevention interventions. English use in multiple settings was strongly associated with increased knowledge. PMID:19132325

  18. The Xq22 inversion breakpoint interrupted a novel Ras-like GTPase gene in a patient with Duchenne muscular dystrophy and profound mental retardation.

    Science.gov (United States)

    Saito-Ohara, Fumiko; Fukuda, Yoji; Ito, Masahiro; Agarwala, Kishan Lal; Hayashi, Masaharu; Matsuo, Masafumi; Imoto, Issei; Yamakawa, Kazuhiro; Nakamura, Yusuke; Inazawa, Johji

    2002-09-01

    A male patient with profound mental retardation, athetosis, nystagmus, and severe congenital hypotonia (Duchenne muscular dystrophy [DMD]) was previously shown to carry a pericentric inversion of the X chromosome, 46,Y,inv(X)(p21.2q22.2). His mother carried this inversion on one X allele. The patient's condition was originally misdiagnosed as cerebral palsy, and only later was it diagnosed as DMD. Because the DMD gene is located at Xp21.2, which is one breakpoint of the inv(X), and because its defects are rarely associated with severe mental retardation, the other clinical features of this patient were deemed likely to be associated with the opposite breakpoint at Xq22. Our precise molecular-cytogenetic characterization of both breakpoints revealed three catastrophic genetic events that had probably influenced neuromuscular and cognitive development: deletion of part of the DMD gene at Xp21.2, duplication of the human proteolipid protein gene (PLP) at Xq22.2, and disruption of a novel gene. The latter sequence, showing a high degree of homology to the Sec4 gene of yeast, encoded a putative small guanine-protein, Ras-like GTPase that we have termed "RLGP." Immunocytochemistry located RLGP at mitochondria. We speculate that disruption of RLGP was responsible for the patient's profound mental retardation.

  19. Genome Editing of Monogenic Neuromuscular Diseases: A Systematic Review.

    Science.gov (United States)

    Long, Chengzu; Amoasii, Leonela; Bassel-Duby, Rhonda; Olson, Eric N

    2016-11-01

    Muscle weakness, the most common symptom of neuromuscular disease, may result from muscle dysfunction or may be caused indirectly by neuronal and neuromuscular junction abnormalities. To date, more than 780 monogenic neuromuscular diseases, linked to 417 different genes, have been identified in humans. Genome-editing methods, especially the CRISPR (clustered regularly interspaced short palindromic repeats)-Cas9 (CRISPR-associated protein 9) system, hold clinical potential for curing many monogenic disorders, including neuromuscular diseases such as Duchenne muscular dystrophy, spinal muscular atrophy, amyotrophic lateral sclerosis, and myotonic dystrophy type 1. To provide an overview of genome-editing approaches; to summarize published reports on the feasibility, efficacy, and safety of current genome-editing methods as they relate to the potential correction of monogenic neuromuscular diseases; and to highlight scientific and clinical opportunities and obstacles toward permanent correction of disease-causing mutations responsible for monogenic neuromuscular diseases by genome editing. PubMed and Google Scholar were searched for articles published from June 30, 1989, through June 9, 2016, using the following keywords: genome editing, CRISPR-Cas9, neuromuscular disease, Duchenne muscular dystrophy, spinal muscular atrophy, amyotrophic lateral sclerosis, and myotonic dystrophy type 1. The following sources were reviewed: 341 articles describing different approaches to edit mammalian genomes; 330 articles describing CRISPR-Cas9-mediated genome editing in cell culture lines (in vitro) and animal models (in vivo); 16 websites used to generate single-guide RNA; 4 websites for off-target effects; and 382 articles describing viral and nonviral delivery systems. Articles describing neuromuscular diseases, including Duchenne muscular dystrophy, spinal muscular atrophy, amyotrophic lateral sclerosis, and myotonic dystrophy type 1, were also reviewed. Multiple proof

  20. Quality of Recovery After Low-Pressure Laparoscopic Donor Nephrectomy Facilitated by Deep Neuromuscular Blockade: A Randomized Controlled Study.

    Science.gov (United States)

    Özdemir-van Brunschot, Denise M D; Scheffer, Gert J; van der Jagt, Michel; Langenhuijsen, Hans; Dahan, Albert; Mulder, Janneke E E A; Willems, Simone; Hilbrands, Luuk B; Donders, Rogier; van Laarhoven, Cees J H M; d'Ancona, Frank A; Warlé, Michiel C

    2017-11-01

    The use of low intra-abdominal pressure (<10 mmHg) reduces postoperative pain scores after laparoscopic surgery. To investigate whether low-pressure pneumoperitoneum with deep neuromuscular blockade improves the quality of recovery after laparoscopic donor nephrectomy (LDN). In a single-center randomized controlled trial, 64 live kidney donors were randomly assigned to 6 or 12 mmHg insufflation pressure. A deep neuromuscular block was used in both groups. Surgical conditions were rated by the five-point Leiden-surgical rating scale (L-SRS), ranging from 5 (optimal) to 1 (extremely poor) conditions. If the L-SRS was insufficient, the pressure was increased stepwise. The primary outcome measure was the overall score on the quality of recovery-40 (QOR-40) questionnaire at postoperative day 1. The difference in the QOR-40 scores on day 1 between the low- and standard-pressure group was not significant (p = .06). Also the overall pain scores and analgesic consumption did not differ. Eight procedures (24%), initially started with low pressure, were converted to a standard pressure (≥10 mmHg). A L-SRS score of 5 was significantly more prevalent in the standard pressure as compared to the low-pressure group at 30 min after insufflation (p < .01). Low-pressure pneumoperitoneum facilitated by deep neuromuscular blockade during LDN does not reduce postoperative pain scores nor improve the quality of recovery in the early postoperative phase. The question whether the use of deep neuromuscular blockade during laparoscopic surgery reduces postoperative pain scores independent of the intra-abdominal pressure should be pursued in future studies. The trial was registered at clinicaltrial.gov before the start of the trial (NCT02146417).

  1. Neuromuscular Fatigue during Prolonged Exercise in Hypoxia.

    Science.gov (United States)

    Jubeau, Marc; Rupp, Thomas; Temesi, John; Perrey, Stéphane; Wuyam, Bernard; Millet, Guillaume Y; Verges, Samuel

    2017-03-01

    Prolonged cycling exercise performance in normoxia is limited because of both peripheral and central neuromuscular impairments. It has been reported that cerebral perturbations are greater during short-duration exercise in hypoxia compared with normoxia. The purpose of this study was to test the hypothesis that central deficits are accentuated in hypoxia compared with normoxia during prolonged (three bouts of 80 min separated by 25 min) whole-body exercise at the same relative intensity. Ten subjects performed two sessions consisting of three 80-min cycling bouts at 45% of their relative maximal aerobic power in normoxia and hypoxia (FiO2 = 0.12). Before exercise and after each bout, maximal voluntary force, voluntary activation assessed with nerve stimulation and transcranial magnetic stimulation, corticospinal excitability (motor evoked potential), intracortical inhibition (cortical silent period), and electrical (M-wave) and contractile (twitch and doublet peak forces) properties of the knee extensors were measured. Prefrontal and motor cortical oxygenation was also recorded during each cycling bout in both conditions. A significant but similar force reduction (≈-22%) was observed at the end of exercise in normoxia and hypoxia. The modifications of voluntary activation assessed with transcranial magnetic stimulation and nerve stimulation, motor evoked potential, cortical silent period, and M-wave were also similar in both conditions. However, cerebral oxygenation was reduced in hypoxia compared with normoxia. These findings show that when performed at the same relative low intensity, prolonged exercise does not induce greater supraspinal fatigue in hypoxia compared with normoxia. Despite lower absolute exercise intensities in hypoxia, reduced brain O2 availability might contribute to similar amounts of central fatigue compared with normoxia.

  2. Cochlear implantation in autistic children with profound sensorineural hearing loss.

    Science.gov (United States)

    Lachowska, Magdalena; Pastuszka, Agnieszka; Łukaszewicz-Moszyńska, Zuzanna; Mikołajewska, Lidia; Niemczyk, Kazimierz

    2016-11-19

    Cochlear implants have become the method of choice for the treatment of severe-to-profound hearing loss in both children and adults. Its benefits are well documented in the pediatric and adult population. Also deaf children with additional needs, including autism, have been covered by this treatment. The aim of this study was to assess the benefits from cochlear implantation in deafened children with autism as the only additional disability. This study analyzes data of six children. The follow-up time was at least 43 months. The following data were analyzed: medical history, reaction to music and sound, Ling's six sounds test, onomatopoeic word test, reaction to spoken child's name, response to requests, questionnaire given to parents, sound processor fitting sessions and data. After cochlear implantation each child presented other communication skills. In some children, the symptoms of speech understanding were observed. No increased hyperactivity associated with daily use cochlear implant was observed. The study showed that in autistic children the perception is very important for a child's sense of security and makes contact with parents easier. Our study showed that oral communication is not likely to be a realistic goal in children with cochlear implants and autism. The implantation results showed benefits that varied among those children. The traditional methods of evaluating the results of cochlear implantation in children with autism are usually insufficient to fully assess the functional benefits. These benefits should be assessed in a more comprehensive manner taking into account the limitations of communication resulting from the essence of autism. It is important that we share knowledge about these complex children with cochlear implants. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  3. Metabolic Surgery Profoundly Influences Gut Microbial-Host Metabolic Crosstalk

    Science.gov (United States)

    Li, Jia V.; Ashrafian, Hutan; Bueter, Marco; Kinross, James; Sands, Caroline; le Roux, Carel W; Bloom, Stephen R.; Darzi, Ara; Athanasiou, Thanos; Marchesi, Julian R.; Nicholson, Jeremy K.; Holmes, Elaine

    2013-01-01

    Background and Aims Bariatric surgery is increasingly performed worldwide to treat morbid obesity and is also known as metabolic surgery to reflect its beneficial metabolic effects especially with respect to improvement in type 2 diabetes. Understanding surgical weight loss mechanisms and metabolic modulation is required to enhance patient benefits and operative outcomes. Methods We apply a parallel and statistically integrated metagenomic and metabonomic approach to characterize Roux-en-Y gastric bypass (RYGB) effects in a rat model. Results We show substantial shifts of the main gut phyla towards higher levels of Proteobacteria (52-fold) specifically Enterobacter hormaechei. We also find low levels of Firmicutes (4.5-fold) and Bacteroidetes (2-fold) in comparison to sham-operated rats. Faecal extraction studies reveal a decrease in faecal bile acids and a shift from protein degradation to putrefaction through decreased faecal tyrosine with concomitant increases in faecal putrescine and diamnoethane. We find decreased urinary amines and cresols and demonstrate indices of modulated energy metabolism post-RYGB including decreased urinary succinate, 2-oxoglutarate, citrate and fumarate. These changes could also indicate renal tubular acidosis, which associates with increased flux of mitochondrial tricarboxylic acid cycle intermediates. A surgically-induced effect on the gut-brain-liver metabolic axis is inferred by increased neurotropic compounds; faecal γ-aminobutyric acid (GABA) and glutamate. Conclusion This profound co-dependence of mammalian and microbial metabolism, which is systematically altered following RYGB surgery, suggests that RYGB exerts local and global metabolic activities. The effect of RYGB surgery on the host metabolic-microbial crosstalk augments our understanding of the metabolic phenotype of bariatric procedures and can facilitate enhanced treatments for obesity-related diseases. PMID:21572120

  4. Vertebral column resection in children with neuromuscular spine deformity.

    Science.gov (United States)

    Sponseller, Paul D; Jain, Amit; Lenke, Lawrence G; Shah, Suken A; Sucato, Daniel J; Emans, John B; Newton, Peter O

    2012-05-15

    Retrospective analysis. To determine, in pediatric patients with neuromuscular deformity undergoing vertebral column resection (VCR), the (1) characteristics of the surgery performed; (2) amount of pelvic obliquity restoration, and coronal and sagittal correction achieved; (3) associated blood loss and complications; and (4) extent to which curve type and VCR approach influenced correction, blood loss, and complications. VCR allows for correction of severe, rigid spinal deformity. This technique has not been previously reported in children with neuromuscular disorders. We retrospectively reviewed the records of 23 children with neuromuscular disorders (mean age, 15 years) and spinal deformities (severe scoliosis, 9; global kyphosis or angular kyphosis, 4; kyphoscoliosis, 10) who underwent VCR. The Student t test was used to compare correction differences (statistical significance, P deformity. However, this challenging procedure involves the potential for major complications.

  5. Effects of sugammadex on incidence of postoperative residual neuromuscular blockade

    DEFF Research Database (Denmark)

    Brueckmann, B; Sasaki, N; Grobara, P

    2015-01-01

    by randomized allocation to sugammadex (2 or 4 mg kg(-1)) or usual care (neostigmine/glycopyrrolate, dosing per usual care practice) for reversal of neuromuscular blockade. Timing of reversal agent administration was based on the providers' clinical judgement. Primary endpoint was the presence of residual...... measured at PACU entry. Zero out of 74 sugammadex patients and 33 out of 76 (43.4%) usual care patients had TOF-Watch® SX-assessed residual neuromuscular blockade at PACU admission (odds ratio 0.0, 95% CI [0-0.06], Pcare patients, 2 also had clinical evidence of partial...... paralysis. Time between reversal agent administration and operating room discharge-readiness was shorter for sugammadex vs usual care (14.7 vs 18.6 min respectively; P=0.02). CONCLUSIONS: After abdominal surgery, sugammadex reversal eliminated residual neuromuscular blockade in the PACU, and shortened...

  6. A neuromuscular test battery for osteoporotic women: a pilot study.

    Science.gov (United States)

    Kerschan-Schindl, K; Uher, E; Grampp, S; Kaider, A; Ghanem, A H; Fialka-Moser, V; Preisinger, E

    2001-05-01

    To examine the efficacy of a short neuromuscular test battery in elderly women suffering from osteoporosis in accordance with the World Health Organization criteria, with and without a history of fractures. Reduced bone mass and a high likelihood of falling increase the risk of osteoporotic fractures. There is a need for neuromuscular tests to identify individuals at risk for falls and fractures. The women were assessed twice. Forty-two women, with a mean age of 70.0 +/- 5.1 (SD) yr, completed the first assessment. The number of postmenopausal fractures and the women's history with regard to agility and falls were assessed. The women performed neuromuscular tests (one-leg stance, tandem walk, and body sway); bone mineral density of the spine and femoral neck were measured. For the follow-up assessment, 13.2 +/- 1.3 mo later, 39 women were studied. The same outcome measurements were obtained at both evaluations. During the observation period, five women fell once and one woman fell twice; there were only two vertebral fractures and no nonvertebral fracture. Neuromuscular performance did not change during this observation period. The median changes in bone mineral density between the two assessments were clinically not relevant. A comparison between patients suffering from established osteoporosis and osteoporotic patients without a history of postmenopausal fractures showed that both groups of patients did not differ with respect to age, neuromuscular performance, bone mineral density, and fear of falling. This neuromuscular test battery is a feasible and practical tool because it is brief and economical to perform. However, its efficacy as a predictor of fractures must be tested in additional studies with a long-term follow-up and a larger group of subjects.

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

    Science.gov (United States)

    1997-07-15

    reaction in any persons with a known egg allergy caused by the use of egg lecithin to prepare the emulsion (Geniton, 1992). Pain may be experienced...mcotinic receptor, the postjunctional membrane is depolarized causing muscle activity (Stoelting, 1991). Succinylcholine exhibits acetylcholine...inhibition of muscle activity because the depolarized membrane can not respond to acetylcholine released subsequent to succinylcholine

  8. REVERSAL BY SURAMIN OF NEUROMUSCULAR BLOCK PRODUCED BY PANCURONIUM IN THE ANESTHETIZED RAT

    NARCIS (Netherlands)

    HENNING, RH; NELEMANS, A; HOUWERTJES, M; AGOSTON, S

    1 Rats were anaesthetized with sodium pentobarbitone and maximal twitches of a tibialis anterior muscle were evoked by stimulation of the motor nerve. 2 Suramin, injected intravenously in a series of cumulative bolus doses, each 15 mg kg-1, completely reversed a 90% depression of twitches maintained

  9. Ultrasound guided supraclavicular block.

    LENUS (Irish Health Repository)

    Hanumanthaiah, Deepak

    2013-09-01

    Ultrasound guided regional anaesthesia is becoming increasingly popular. The supraclavicular block has been transformed by ultrasound guidance into a potentially safe superficial block. We reviewed the techniques of performing supraclavicular block with special focus on ultrasound guidance.

  10. Blocking the interleukin 2 (IL2)-induced systemic autophagic syndrome promotes profound antitumor effects and limits toxicity.

    Science.gov (United States)

    Lotze, Michael T; Buchser, William J; Liang, Xiaoyan

    2012-08-01

    Cancer is the leading cause of death in the United States in those dying under the age of 85. Although cancer is increasingly controlled as a chronic disease, true cures of patients with metastatic epithelial malignancies have rarely been obtained with currently available systemic therapies. For example, administration of high-dose recombinant interleukin 2 (IL2), enhancing cytolytic immune cell proliferation and delivery, promotes complete antitumor responses in IL2 promotes both T-cell and NK cell induction of immune cell-mediated autophagy (iC-MA) in tumor targets. We have demonstrated that HMGB1 is detected at high levels in the serum of IL2-treated mice with translocation to the cytoplasm from the nucleus in the liver, consistent with HMGB1's release in response to stress, and ability to sustain autophagy. Limiting autophagy in mice with coadministration of chloroquine (CQ) diminishes serum levels of HMGB1, cytokines (IFNG and IL6 but not IL18), and autophagic flux, attenuating weight gain, enhancing DC, T-cell and NK cell numbers, and promoting long-term tumor control in a murine hepatic metastases model. Autophagy (programmed cell survival) is a metabolic process associated with promotion of late cancer growth. In tumor cell lines, CQ treatment limits ATP production through inhibition of oxidative phosphorylation and promotion of apoptosis. CQ increases autophagic vacuoles and LC3-II levels in tumor cells, associated with increased annexin V(+)/PI(-) cells, cleaved-PARP, cleaved-CASP3, and cytochrome c release from mitochondria. These observations, limiting toxicity and prolonging antitumor effects, with a combination of IL2 and autophagy inhibition in murine models are now being tested by the Cytokine Working Group in patients with advanced renal cell carcinoma.

  11. [Speech perception test in Italian language for profoundly deaf children].

    Science.gov (United States)

    Genovese, E; Orzan, E; Turrini, M; Babighian, G; Arslan, E

    1995-10-01

    Speech perception tests are an important part of procedures for diagnosing pre-verbal hearing loss. Merely establishing a child's hearing threshold with and without a hearing aid is not sufficient to ensure an adequate evaluation with a view to selecting cases suitable for cochlear implants because it fails to indicate the real benefit obtained from using a conventional hearing aid reliably. Speech perception tests have proved useful not only for patient selection, but also for subsequent evaluation of the efficacy of new hearing aids, such as tactile devices and cochlear implants. In clinical practice, the tests most commonly adopted with small children are: The Auditory Comprehension Test (ACT), Discrimination after Training (DAT), Monosyllable, Trochee, Spondee tests (MTS), Glendonald Auditory Screening Priocedure (GASP), Early Speech Perception Test (ESP), Rather than considering specific results achieved in individual cases, reference is generally made to the four speech perception classes proposed by Moog and Geers of the CID of St. Louis. The purpose of this classification, made on the results obtained with suitably differentiated tests according to the child's age and language ability, is to detect differences in perception of a spoken message in ideal listening conditions. To date, no italian language speech perception test has been designed to establish the assessment of speech perception level in children with profound hearing impairment. We attempted, therefore, to adapt the existing English tests to the Italian language taking into consideration the differences between the two languages. Our attention focused on the ESP test since it can be applied to even very small children (2 years old). The ESP is proposed in a standard version for hearing-impaired children over the age of 6 years and in a simplified version for younger children. The rationale we used for selecting Italian words reflect the rationale established for the original version, but the

  12. Nanotechnological Inventions and Nanomaterials Produce A Profound Effect

    Directory of Open Access Journals (Sweden)

    VLASOV Vladimir Alexeevich

    2015-02-01

    Full Text Available The inventions in the area of nanotechnologies and nanomaterials produce a profound effect in construction, housing and communal services and adjacent economic fields as they allow us: to increase mechanical strength, coefficient of elasticity, alkali resistance and temperature of products vitrification; to obtain nanostructured coatings with the property of shape memory on the steel; to raise the dynamics of coal burning and its full burnout in the boilers of thermoelectric power station; to produce metal nanopowders with increased stored energy 10–15% etc. For example, the invention «Epoxy composition for high strength, alkali resistant structures» refers to epoxy composition used as a binder for production of high strength, thermal- and alkali-resistant glass-fiber material which can be applied in the manufacture process of construction reinforcement to strengthen concrete structures. The invention «The method to produce nanostructured reaction foil» can be used to join different materials including metal alloys, ceramics, amorphous materials and elements of microelectronic devices that are sensible to the heating. This process provides decreased labour-output ratio and energy consumption as well as the condition to manufacture foil with specified stored energy and high mechanical properties. The invention «The method of intensification of burning lowreactionary coal in the boilers of thermoelectric power station» refers to the thermal energy and can be implemented at the thermal plants. The increased dynamics of inflaming and burning leads to full burnout of powdered-coal low-reactionary fuel and decreased mechanical underfiring. The specialists may be also interested in the following inventions: fine dispersed organic suspension of carbon metal-containing nanostructures and the method to produce it; the dispersion of carbon nanotubes; the composition for reinforcement of building structures; the reinforced plate element made of

  13. Efectos del vendaje neuromuscular sobre la flexibilidad del raquis lumbar

    OpenAIRE

    A.M. Labrador-Cerrato; P. Ortega Sánchez-Diezma; G. Lanzas Melendo; C. Gutiérrez-Ortega

    2015-01-01

    Introducción: El vendaje neuromuscular es una técnica que produce una estimulación muy selectiva sobre la piel a través de la aplicación de unas vendas elásticas especiales con el fin de lograr cambios propioceptivos, aumento o inhibición del tono muscular y mitigación de algias, entre otros. Objetivos: Comprobar si la aplicación del vendaje neuromuscular permite aumentar la flexión del raquis lumbar comparándola con otras técnicas de vendaje placebo (esparadrapo rígido convencional; Omniplas...

  14. Miller Fisher syndrome with presynaptic neuromuscular transmission disorder.

    Science.gov (United States)

    Ehler, Edvard; Latta, Jan

    2014-11-01

    Miller Fisher syndrome is defined by a triad of symptoms, namely areflexia, ataxia, and ophthalmoparesis. The ophthalmoparesis is mostly severe, undulating weakness of eye movements with ptosis and increased fatigability resembling a neuromuscular transmission disorder. We present a 52-year-old man with severe Miller Fisher syndrome with a high level of anti-GQ1b antibodies and a presynaptic type of neuromuscular transmission disorder. The diagnosis was confirmed by stimulated single-fiber electromyography with the use of a concentric needle electrode and various stimulation rates. Copyright © 2014 Elsevier Ltd. All rights reserved.

  15. Exercise Therapy in Spinobulbar Muscular Atrophy and Other Neuromuscular Disorders

    DEFF Research Database (Denmark)

    Dahlqvist, Julia Rebecka; Vissing, John

    2016-01-01

    There is no curative treatment for most neuromuscular disorders. Exercise, as a treatment for these diseases, has therefore received growing attention. When executed properly, exercise can maintain and improve health and reduce the risk of cardiovascular disease, obesity, and diabetes. In persons...... in patients with neuromuscular diseases associated with weakness and wasting. We review studies that have investigated different types of exercise in both myopathies and motor neuron diseases, with particular emphasis on training of persons affected by spinobulbar muscular atrophy (SBMA). Finally, we provide...

  16. Neuromuscular Activity and Knee Kinematics in Adolescents with Patellofemoral Pain

    DEFF Research Database (Denmark)

    Rathleff, Michael Skovdal; Samani, Afshin; Olesen, Jens L

    2013-01-01

    This study aimed to investigate the neuromuscular control of the knee during stair descent among female adolescents with patellofemoral pain (PFP) and to report its association with self-reported clinical status assessed by the Knee Injury and Osteoarthritis Outcome Score (KOOS).......This study aimed to investigate the neuromuscular control of the knee during stair descent among female adolescents with patellofemoral pain (PFP) and to report its association with self-reported clinical status assessed by the Knee Injury and Osteoarthritis Outcome Score (KOOS)....

  17. Myotubular myopathy and the neuromuscular junction: a novel therapeutic approach from mouse models

    Directory of Open Access Journals (Sweden)

    James J. Dowling

    2012-11-01

    Myotubular myopathy (MTM is a severe congenital muscle disease characterized by profound weakness, early respiratory failure and premature lethality. MTM is defined by muscle biopsy findings that include centralized nuclei and disorganization of perinuclear organelles. No treatments currently exist for MTM. We hypothesized that aberrant neuromuscular junction (NMJ transmission is an important and potentially treatable aspect of the disease pathogenesis. We tested this hypothesis in two murine models of MTM. In both models we uncovered evidence of a disorder of NMJ transmission: fatigable weakness, improved strength with neostigmine, and electrodecrement with repetitive nerve stimulation. Histopathological analysis revealed abnormalities in the organization, appearance and size of individual NMJs, abnormalities that correlated with changes in acetylcholine receptor gene expression and subcellular localization. We additionally determined the ability of pyridostigmine, an acetylcholinesterase inhibitor, to ameliorate aspects of the behavioral phenotype related to NMJ dysfunction. Pyridostigmine treatment resulted in significant improvement in fatigable weakness and treadmill endurance. In all, these results describe a newly identified pathological abnormality in MTM, and uncover a potential disease-modifying therapy for this devastating disorder.

  18. Muscle Mitochondrial Uncoupling Dismantles Neuromuscular Junction and Triggers Distal Degeneration of Motor Neurons

    Science.gov (United States)

    Dupuis, Luc; Gonzalez de Aguilar, Jose-Luis; Echaniz-Laguna, Andoni; Eschbach, Judith; Rene, Frédérique; Oudart, Hugues; Halter, Benoit; Huze, Caroline; Schaeffer, Laurent; Bouillaud, Frédéric; Loeffler, Jean-Philippe

    2009-01-01

    Background Amyotrophic lateral sclerosis (ALS), the most frequent adult onset motor neuron disease, is associated with hypermetabolism linked to defects in muscle mitochondrial energy metabolism such as ATP depletion and increased oxygen consumption. It remains unknown whether muscle abnormalities in energy metabolism are causally involved in the destruction of neuromuscular junction (NMJ) and subsequent motor neuron degeneration during ALS. Methodology/Principal Findings We studied transgenic mice with muscular overexpression of uncoupling protein 1 (UCP1), a potent mitochondrial uncoupler, as a model of muscle restricted hypermetabolism. These animals displayed age-dependent deterioration of the NMJ that correlated with progressive signs of denervation and a mild late-onset motor neuron pathology. NMJ regeneration and functional recovery were profoundly delayed following injury of the sciatic nerve and muscle mitochondrial uncoupling exacerbated the pathology of an ALS animal model. Conclusions/Significance These findings provide the proof of principle that a muscle restricted mitochondrial defect is sufficient to generate motor neuron degeneration and suggest that therapeutic strategies targeted at muscle metabolism might prove useful for motor neuron diseases. PMID:19404401

  19. The respiratory neuromuscular system in Pompe disease☆

    Science.gov (United States)

    Fuller, David D.; ElMallah, Mai K.; Smith, Barbara K.; Corti, Manuela; Lawson, Lee Ann; Falk, Darin J.; Byrne, Barry J.

    2014-01-01

    Pompe disease is due to mutations in the gene encoding the lysosomal enzyme acid α-glucosidase (GAA). Absence of functional GAA typically results in cardiorespiratory failure in the first year; reduced GAA activity is associated with progressive respiratory failure later in life. While skeletal muscle pathology contributes to respiratory insufficiency in Pompe disease, emerging evidence indicates that respiratory neuron dysfunction is also a significant part of dysfunction in motor units. Animal models show profound glycogen accumulation in spinal and medullary respiratory neurons and altered neural activity. Tissues from Pompe patients show central nervous system glycogen accumulation and motoneuron pathology. A neural mechanism raises considerations about the current clinical approach of enzyme replacement since the recombinant protein does not cross the blood-brain-barrier. Indeed, clinical data suggest that enzyme replacement therapy delays symptom progression, but many patients eventually require ventilatory assistance, especially during sleep. We propose that treatments which restore GAA activity to respiratory muscles, neurons and networks will be required to fully correct ventilatory insufficiency in Pompe disease. PMID:23797185

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

    Science.gov (United States)

    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.

  1. Roles of neuro-exocytotic proteins at the neuromuscular junction

    NARCIS (Netherlands)

    Sons-Michel, Michèle S.

    2011-01-01

    The aim of the studies described in the thesis was to elucidate the roles of several neuro-exocytotic proteins at the motor nerve terminal in neuromuscular synaptic transmission, making use of genetic knockout (KO) mice, each missing one (or more) neuro-exocytotic proteins. In addition, it was

  2. [Neuro-muscular apparatus state in lumbosacral radiculopathy in miners].

    Science.gov (United States)

    Battakova, Sh B; Amanbekov, U A; Otarbaeva, M B; Fazylova, M D; Sraĭmanov, K S; Miianova, G A; Kozhakhmetova, K M

    2008-01-01

    Based on clinical and electrophysiologic studies, the authors analysed neuro-muscular apparatus of "spinal center--periphery" axis for miners with radicular pain caused by occupational lumbosacral radiculopathy. Findings are that constantly irritated receptors in lumbar motor segment during occupational activities alter habitual motor stereotype and cause specific compensatory muscular reactions, rearrangement of motor activity in segmental apparatus.

  3. Imaging of respiratory muscles in neuromuscular disease: A review.

    Science.gov (United States)

    Harlaar, L; Ciet, P; van der Ploeg, A T; Brusse, E; van der Beek, N A M E; Wielopolski, P A; de Bruijne, M; Tiddens, H A W M; van Doorn, P A

    2017-11-24

    Respiratory muscle weakness frequently occurs in patients with neuromuscular disease. Measuring respiratory function with standard pulmonary function tests provides information about the contribution of all respiratory muscles, the lungs and airways. Imaging potentially enables the study of different respiratory muscles, including the diaphragm, separately. In this review, we provide an overview of imaging techniques used to study respiratory muscles in neuromuscular disease. We identified 26 studies which included a total of 573 patients with neuromuscular disease. Imaging of respiratory muscles was divided into static and dynamic techniques. Static techniques comprise chest radiography, B-mode (brightness mode) ultrasound, CT and MRI, and are used to assess the position and thickness of the diaphragm and the other respiratory muscles. Dynamic techniques include fluoroscopy, M-mode (motion mode) ultrasound and MRI, used to assess diaphragm motion in one or more directions. We discuss how these imaging techniques relate with spirometric values and whether these can be used to study the contribution of the different respiratory muscles in patients with neuromuscular disease. Copyright © 2017. Published by Elsevier B.V.

  4. Neuromuscular stimulation after stroke: from technology to clinical deployment

    NARCIS (Netherlands)

    IJzerman, Maarten Joost; Renzenbrink, Gerbert J.; Geurts, Alexander C.H.

    2009-01-01

    Since the early 1960s, electrical or neuromuscular electrical stimulation (NMES) has been used to support the rehabilitation of stroke patients. One of the earliest applications of NMES included the use of external muscle stimulation to correct drop-foot after stroke. During the last few decades

  5. Neuromuscular stimulation after stroke: from technology to clinical deployment.

    NARCIS (Netherlands)

    IJzerman, M.J.; Renzenbrink, G.J.; Geurts, A.C.H.

    2009-01-01

    Since the early 1960s, electrical or neuromuscular electrical stimulation (NMES) has been used to support the rehabilitation of stroke patients. One of the earliest applications of NMES included the use of external muscle stimulation to correct drop-foot after stroke. During the last few decades

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

    Science.gov (United States)

    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.

  7. [New surgical treatment of late-stage neuromuscular ureteral dysplasia].

    Science.gov (United States)

    Lopatkin, N A; Zhitnikova, L H; Berestennikov, K A

    1999-01-01

    A surgical treatment of neuromuscular ureteral, dysplasia (NUD) is proposed which provides development of restenosis in vesicoureteral anastomosis and vesicoureteral reflux in maintenance of normal urodynamics of the upper urinary tracts. This organ-saving method can be applied at late disease. Multichannel impedance ureterography proved useful in definition of the operation's scope.

  8. Radiation fibrosis syndrome: neuromuscular and musculoskeletal complications in cancer survivors.

    Science.gov (United States)

    Stubblefield, Michael D

    2011-11-01

    Radiation-induced toxicity is a major cause of long-term disability after cancer treatment. Radiation fibrosis describes the insidious pathologic fibrotic tissue sclerosis that can occur in response to radiation exposure. Radiation fibrosis syndrome describes the myriad clinical manifestations of progressive fibrotic tissue sclerosis resulting from radiation treatment. Radiation-induced damage can include "myelo-radiculo-plexo-neuro-myopathy," causing muscle weakness and dysfunction and contributing to neuromuscular injury. Similarly, radiation damage to neuromuscular structures contributes to radiation-induced trismus and cervical dystonia in head and neck cancer survivors. This narrative review discusses the pathophysiology, anatomy, evaluation, and treatment of neuromuscular, musculoskeletal, and functional disorders that can result as late effects of radiation treatment. Rehabilitation medicine physicians with extensive training in neuromuscular and musculoskeletal medicine as well as in the principles of functional restoration are uniquely positioned to help lead efforts to improve the quality of life for cancer survivors with radiation fibrosis syndrome. Copyright © 2011 American Academy of Physical Medicine and Rehabilitation. Published by Elsevier Inc. All rights reserved.

  9. Intraorbital neuromuscular choristoma adjacent to the optic nerve

    Directory of Open Access Journals (Sweden)

    Arie Perry, M.D.

    2017-03-01

    Full Text Available Neuromuscular choristoma is a rare tumor that incorporates mature skeletal muscle within fascicles of peripheral nerve. The etiology is poorly understood, yet most present in large nerves of children, with a tight link to post-operative fibromatosis recently appreciated. Herein, we report an exceptional intra-orbital example in a 53-year-old man with optic nerve compression.

  10. Comparison of the Effect of Neuromuscular Electrical Stimulation ...

    African Journals Online (AJOL)

    Children with cerebral palsy (CP) often demonstrate poor hand function due to spasticity. Thus spasticity in the wrist and finger flexors poses a great deal of functional limitations. This study was therefore designed to compare the effectiveness of Cryotherapy and Neuromuscular Electrical Stimulation (NMES) on spasticity ...

  11. Neuromuscular and mitochondrial disorders: what is relevant to the anaesthesiologist?

    NARCIS (Netherlands)

    Driessen, J.J.

    2008-01-01

    PURPOSE OF REVIEW: The review provides an up-to-date information to the anaesthesiologist about the more frequent and important neuromuscular disorders for which new basic insights or clinical implications have been reported. RECENT FINDINGS: The findings include the mechanisms of the hyperkalemia

  12. Is deep neuromuscular blockade beneficial in laparoscopic surgery?

    DEFF Research Database (Denmark)

    Madsen, M. V.; Staehr-Rye, A K; Claudius, C

    2016-01-01

    this in narrative form. We have shared our analysis and text with the authors of the 'Con-' side of these paired position papers during the preparation of the manuscripts. RESULTS: There are a few low risk of bias studies indicating that use of deep neuromuscular blockade improve surgical conditions and improve...

  13. Neuromuscular function during stair descent in meniscectomized patients and controls

    DEFF Research Database (Denmark)

    Thorlund, Jonas Bloch; Roos, Ewa M; Aagaard, Per

    2011-01-01

    The aim of this study was to identify differences in knee range of motion (ROM), movement speed, ground reaction forces (GRF) profile, neuromuscular activity, and muscle coactivation during the transition between stair descent and level walking in meniscectomized patients at high risk of knee...

  14. Types of Heart Block

    Science.gov (United States)

    ... defects. Acquired heart block is more common than congenital heart block. Damage to the heart muscle or its electrical system causes acquired heart block. Diseases, surgery, or medicines can cause this damage. The three types of heart block are first degree, second degree, ...

  15. What Causes Heart Block?

    Science.gov (United States)

    ... defects. Acquired heart block is more common than congenital heart block. Damage to the heart muscle or its electrical system causes acquired heart block. Diseases, surgery, or medicines can cause this damage. The three types of heart block are first degree, second degree, ...

  16. Living with Heart Block

    Science.gov (United States)

    ... defects. Acquired heart block is more common than congenital heart block. Damage to the heart muscle or its electrical system causes acquired heart block. Diseases, surgery, or medicines can cause this damage. The three types of heart block are first degree, second degree, ...

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

    Directory of Open Access Journals (Sweden)

    Alejandro Carbajal-Saucedo

    2014-01-01

    Full Text Available 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.

  18. Endomicroscopy and electromyography of neuromuscular junctions in situ

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

  20. Neuromuscular and perceptual fatigue responses to consecutive tag football matches.

    Science.gov (United States)

    Hogarth, Luke W; Burkett, Brendan J; McKean, Mark R

    2015-07-01

    To examine the neuromuscular and perceptual fatigue responses to consecutive tag football matches played on the same day and determine the relationship between fatigue and match running performance. Neuromuscular and perceptual fatigue responses of 15 national tag football players were assessed before and during the 2014 State of Origin tournament. Global positioning systems (GPS) provided data on players' match running performance, and a vertical-jump test and subjective questionnaire were used to assess players' neuromuscular and perceptual fatigue, respectively. There were small to moderate reductions in the majority of match-running-performance variables over consecutive matches, including distance (ES=-0.81), high-speed-running (HSR) distance (ES=-0.51), HSR efforts (ES=-0.64), and maximal accelerations (ES=-0.76). Prematch vertical jump was initially below baseline values before the first match (ES=0.68-0.88). There were no substantial reductions in vertical-jump performance from baseline values over consecutive matches, although there was a small decline from after match 2 to after match 3 (3.3%; ES=-0.45±0.62). There were progressive reductions in perceived well-being scores after matches 1 (ES=-0.38), 2 (ES=-0.70), and 3 (ES=-1.14). There were small to moderate associations between changes in fatigue measures and match running performance. Perceptual fatigue accumulates over consecutive tag football matches, although there were only marginal increases in neuromuscular fatigue. However, both neuromuscular and perceptual fatigue measures were found to contribute to reduced match running performance in the final match.

  1. Healhy Ageing in People with Profound Intellectual and Multiple Disabilities : Promoting Physical Activity

    NARCIS (Netherlands)

    van Alphen, Helena; Bossink, Leontien; Schalen, Gertruud Henrike; van der Putten, Annette

    2016-01-01

    Physical activity is beneficial, also for people who are characterized by profound intellectual and severe motor disabilities. However, these people are totally dependent on others to participate in physical activities. To date, promoting physical activity in people with these profound disabilities

  2. Teaching Individuals with Profound Multiple Disabilities to Access Preferred Stimuli with Multiple Microswitches

    Science.gov (United States)

    Tam, Gee May; Phillips, Katrina J.; Mudford, Oliver C.

    2011-01-01

    We replicated and extended previous research on microswitch facilitated choice making by individuals with profound multiple disabilities. Following an assessment of stimulus preferences, we taught 6 adults with profound multiple disabilities to emit 2 different responses to activate highly preferred stimuli. All participants learnt to activate…

  3. Joint Attention Behaviours in People with Profound Intellectual and Multiple Disabilities: The Influence of the Context

    Science.gov (United States)

    Neerinckx, Heleen; Maes, Bea

    2016-01-01

    Background: In spite of the profound cognitive and physical problems, people with profound intellectual and multiple disabilities (PIMD) are able to develop joint attention behaviours (JAB) and benefit from positive interactions. Aims: To investigate which context factors influence the JAB of people with PIMD. Method: Based on video recordings of…

  4. Fungal contamination of paraffin wax blocks in a pathology archive.

    Science.gov (United States)

    Müller, K; Ellenberger, C; Aupperle, H; Schmäschke, R; Scheller, R; Wittenbrink, M M; Schoon, H-A

    2011-01-01

    While searching for paraffin wax blocks for research purposes in our archive we detected numerous larval and some dead adult moths. Some wax blocks were riddled with a white-brown crumbling substance. The entire archive was checked and profoundly-infested blocks were separated from unaffected blocks. Mycological and parasitological investigations were performed. Fungi were identified by culture and polymerase chain reaction, which revealed high sequence homology to six different fungal species. The moths were determined to be Nemapogon personellus. A total of 8,484 wax blocks had to be removed from the archive and destroyed. Pathologists should be alerted to the importance of checking the humidity of the air where archival material is stored. Copyright © 2011 Elsevier Ltd. All rights reserved.

  5. Histochemical and immunohistological approach to comparative neuromuscular diseases.

    Directory of Open Access Journals (Sweden)

    Serenella Papparella

    2009-12-01

    Full Text Available The broad category of neuromuscular diseases covers conditions that involve the weakness or wasting of the body muscles. These problems may occur in the spinal cord, the peripheral nerves or the muscle fibers. Some may be hereditary, while others are acquired. Commonly recognized conditions fall into the categories of myopathies, which are diseases of the muscle like muscular dystrophy, disorders of the junction where the nerve impulses are transmitted to the muscle like myasthenia gravis, and neuropathies, which are diseases of the peripheral nervous system. The diagnosis of most neuromuscular diseases rest on careful clinical evaluation of the patient, electromyography, the muscle biopsy, and in some instances, molecular genetic studies. Muscle biopsy, associated to histochemical and immunohistological techniques, plays a key role in diagnosis of many neuromuscular disorders. A number of morphological abnormalities of muscle can be recognized on histological stains such as haematoxylin and eosin and Engel trichrome. Histochemical techniques are essential for the study of muscle biopsies for four main reasons. First, they demonstrate the non-uniform nature of the muscle highlighting the different biochemical properties of specific fibre type and their selective involvement in certain disease processes. Second, they may show an absences of a particular enzyme. Third, an excess of a particular substrate can be demonstrated. Fourth, they may show structural changes in the muscle which would not be apparent with routine histological stains, such as the enzyme-deficient cores in central core disease "mouth-eaten" fibers, and abnormalities in the distribution of mitochondria. In some neuromuscular disorders there could be only non-specific myopathological features. However, a number of proteins, including sarcolemmal, sarcomeric, and nuclear proteins as well as enzymes with defects responsible for neuromuscular disorders, have been identified during

  6. Síndrome de Tako-Tsubo em decorrência de bloqueio neuromuscular residual: relato de caso Síndrome de Tako-Tsubo como consecuencia de bloqueo neuromuscular residual: relato de caso Tako-Tsubo syndrome secondary to residual neuromuscular blockade: case report

    Directory of Open Access Journals (Sweden)

    Marcos Guilherme Cunha Cruvinel

    2008-12-01

    neuromuscular residual. RELATO DEL CASO: Paciente del sexo femenino, 61 años, estado físico ASA I, sometida a la anestesia general asociada al bloqueo paravertebral cervical para la reparación artroscópica de lesión de manguito de los rotadores. Después de la extubación, quedó demostrado el bloqueo neuromuscular residual a través del examen clínico. En la sala de recuperación postanestésica, evolucionó con somnolencia, taquicardia, hipertensión arterial y acidosis respiratoria grave. Después de la reintubación evolucionó con parada cardíaca en actividad eléctrica sin pulso, revertida con adrenalina y masaje cardíaco externo. En el postoperatorio presentó una elevación de segmento ST, aumento de troponina y acinesia de segmento medio-apical del ventrículo izquierdo, con fracción de eyección estimada en un 30%. La cineangiocoronariografía mostró coronarias exentas de ateromatosis significativa y un grave comprometimiento de la función sistólica con acinesia inferior y ántero-septo-apical, con hipercontratilidad compensatoria de sus porciones basales. Con el tratamiento iniciado, hubo una recuperación funcional completa. CONCLUSIONES: El bloqueo neuromuscular residual asociado a la parálisis diafragmática y la posible atelectasia pulmonar, que conlleva a la insuficiencia respiratoria, hipercapnia y descarga adrenérgica, fueron los factores desencadenantes del síndrome de Tako-Tsubo con su grave repercusión clínica.BACKGROUND AND OBJECTIVES: Tako-Tsubo syndrome is a rare postoperative complication with a 5% mortality rate. The objective of this report was to present residual neuromuscular blockade as a trigger for this syndrome, discuss this disorder, and call attention to the risks of residual neuromuscular blockade. CASE REPORT: A 61-year old female, physical status ASA I, who underwent general anesthesia associated with paravertebral cervical block for arthroscopic repair of a rotator cuff lesion. Physical exam after extubation detected

  7. Misperceiving Bullshit as Profound Is Associated with Favorable Views of Cruz, Rubio, Trump and Conservatism.

    Science.gov (United States)

    Pfattheicher, Stefan; Schindler, Simon

    2016-01-01

    The present research investigates the associations between holding favorable views of potential Democratic or Republican candidates for the US presidency 2016 and seeing profoundness in bullshit statements. In this contribution, bullshit is used as a technical term which is defined as communicative expression that lacks content, logic, or truth from the perspective of natural science. We used the Bullshit Receptivity scale (BSR) to measure seeing profoundness in bullshit statements. The BSR scale contains statements that have a correct syntactic structure and seem to be sound and meaningful on first reading but are actually vacuous. Participants (N = 196; obtained via Amazon Mechanical Turk) rated the profoundness of bullshit statements (using the BSR) and provided favorability ratings of three Democratic (Hillary Clinton, Martin O'Malley, and Bernie Sanders) and three Republican candidates for US president (Ted Cruz, Marco Rubio, and Donald Trump). Participants also completed a measure of political liberalism/conservatism. Results revealed that favorable views of all three Republican candidates were positively related to judging bullshit statements as profound. The smallest correlation was found for Donald Trump. Although we observe a positive association between bullshit and support for the three Democrat candidates, this relationship is both substantively small and statistically insignificant. The general measure of political liberalism/conservatism was also related to judging bullshit statements as profound in that individuals who were more politically conservative had a higher tendency to see profoundness in bullshit statements. Of note, these results were not due to a general tendency among conservatives to see profoundness in everything: Favorable views of Republican candidates and conservatism were not significantly related to profoundness ratings of mundane statements. In contrast, this was the case for Hillary Clinton and Martin O'Malley. Overall, small

  8. Misperceiving Bullshit as Profound Is Associated with Favorable Views of Cruz, Rubio, Trump and Conservatism.

    Directory of Open Access Journals (Sweden)

    Stefan Pfattheicher

    Full Text Available The present research investigates the associations between holding favorable views of potential Democratic or Republican candidates for the US presidency 2016 and seeing profoundness in bullshit statements. In this contribution, bullshit is used as a technical term which is defined as communicative expression that lacks content, logic, or truth from the perspective of natural science. We used the Bullshit Receptivity scale (BSR to measure seeing profoundness in bullshit statements. The BSR scale contains statements that have a correct syntactic structure and seem to be sound and meaningful on first reading but are actually vacuous. Participants (N = 196; obtained via Amazon Mechanical Turk rated the profoundness of bullshit statements (using the BSR and provided favorability ratings of three Democratic (Hillary Clinton, Martin O'Malley, and Bernie Sanders and three Republican candidates for US president (Ted Cruz, Marco Rubio, and Donald Trump. Participants also completed a measure of political liberalism/conservatism. Results revealed that favorable views of all three Republican candidates were positively related to judging bullshit statements as profound. The smallest correlation was found for Donald Trump. Although we observe a positive association between bullshit and support for the three Democrat candidates, this relationship is both substantively small and statistically insignificant. The general measure of political liberalism/conservatism was also related to judging bullshit statements as profound in that individuals who were more politically conservative had a higher tendency to see profoundness in bullshit statements. Of note, these results were not due to a general tendency among conservatives to see profoundness in everything: Favorable views of Republican candidates and conservatism were not significantly related to profoundness ratings of mundane statements. In contrast, this was the case for Hillary Clinton and Martin O

  9. Anormalidades neuromuscular no desuso, senilidade e caquexia Neuromuscular abnormalities in disuse, cachexia and ageing

    Directory of Open Access Journals (Sweden)

    João Aris Kouyoumdjian

    1993-09-01

    Full Text Available É feita revisão de literatura sobre as principais alterações do sistema neuromuscular no desuso, senilidade e caquexia no ser humano e em modelos animais. A diminuição do diâmetro das fibras musculares após período de inatividade/imobilidade (desuso deve-se à perda de miofibrilas periféricas não ocorrendo formação de core-targetóides ou diminuição da atividade da miofosforilase, próprias da desnervação; mantêm-se a liberação espontânea de acetilcolina e fatores tróficos na junção mio-neural; em geral são afetadas preferencialmente fibras II, que podem assumir forma angular. Existe um processo contínuo intrínseco de envelhecimento de nervos e músculos, com desnervação e reinervação lenta e progressiva; o número de unidades motoras se reduz após 60 anos, sem ocorrência de atividade elétrica desnervatória; a quantidade de acetilcolina liberada nos neurônios terminais e a capacidade máxima de utilização de oxigênio estão diminuídas; a redução da capacidade oxidativa mitocondrial pode explicar o aumento de fibras I, mantendo-se o equilíbrio energético. Após poucas semanas de caquexia as fibras musculares podem ter o diâmetro reduzido em 30%, essa redução ocorre em ordem decrescente nos músculos dos membros inferiores, superiores e tronco; existe atrofia II preferencial com fibras angulares ocasionais, redução de RNA/síntese proteica, mantendo-se DNA normal.Cachexia, ageing and disuse and their effects on the human and animals neuromuscular system are reviewed. Disuse induces reduction of muscle fibers (mainly II diameter with peripheral myofibrils lost; there is no core-targetoid or even reduction on myophosphorilase activity, both typical of denervation; the acetylcholine spontaneous release and trophic factors on myoneural junction are maintained; muscle fibers could change to angular shape. Ageing affects nerve and muscle by a continuous and progressive process of denervation and reinner

  10. [Electromyography (EMG) and neurography in patients with severe neuromuscular diseases].

    Science.gov (United States)

    Løseth, Sissel; Torbergsen, Torberg

    2013-01-22

    Many neuromuscular diseases are potentially severe, and EMG and neurography are methods used in the assessment of these conditions. The article is based on the authors' knowledge and experience, with special emphasis on the use of these methods in the assessment of severe diseases affecting striated muscle and peripheral nerves. A PubMed search was performed with the cut-off fifteen years back in time, and in addition a discretionary selection was made of articles known to the authors. EMG is the most valuable method for assessing myopathy, and neurography provides most information about neuropathy, but the methods are complementary. These examinations are the most sensitive for diagnosing some conditions (for example myasthaenia) A high level of expertise is necessary for diagnosing these conditions. EMG and neurography are important and often necessary means of assessing patients with severe neuromuscular disease.

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

    Science.gov (United States)

    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 (Pmuscle activation after training and detraining was significantly lower compared to baseline values (Ptraining-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.

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

    Science.gov (United States)

    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.

  13. Early appearance and possible roles of non-neuromuscular cholinesterases.

    Directory of Open Access Journals (Sweden)

    Carla eFalugi

    2012-04-01

    Full Text Available The biological function of the cholinesterase (ChE enzymes is well known and has been studied since the beginning of the XXth century; in particular, acetylcholinesterase (AChE, E.C. 3.1.1.7 is an enzyme playing a key role in the modulation of neuromuscular impulse transmission. However, in the past decades, there has been increasing interest concerning its role in regulating non-neuromuscular cell-to-cell interactions mediated by intracellular ion concentration changes, like the ones occurring during gamete interaction and embryonic development. An understanding of the mechanisms of the cholinergic regulation of these events can help us foresee the possible impact on environmental and human health, including gamete efficiency and possible teratogenic effects on different models, and help elucidate the extent to which exposure to ChE inhibitors may affect human health.

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

    Science.gov (United States)

    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.

  15. Monitoring of intense neuromuscular blockade in a pig model

    DEFF Research Database (Denmark)

    Madsen, Matias V; Donatsky, Anders M; Jensen, Bente Rona

    2014-01-01

    Intense neuromuscular blockade (NMB) measured by post tetanic count (PTC) was monitored, reversed and verified in this pig model. In a cross-over assessor blinded design six pigs were randomized to either no NMB followed by intense NMB, or intense NMB followed by no NMB. Neuromuscular measurements...... were performed with acceleromyography [train-of-four (TOF) Watch SX]. In all pigs, the response to TOF nerve stimulation was stable and intense NMB (PTC 0-1) was established with rocuronium 3 mg/kg. For reversal, the pigs received sugammadex 20-35 mg/kg and returned to TOF-ratio above 0.90 within 2.......15 min after injection. We established a pig model for monitoring intense NMB with surface stimulation electrodes and acceleromyography. We verified total relaxation of the diaphragm and the abdominal muscles at the PTC 0-1 by suction test and with surface electromyography. This pig model is suitable...

  16. Neuromuscular fatigue induced by whole-body vibration exercise.

    Science.gov (United States)

    Maffiuletti, Nicola A; Saugy, Jonas; Cardinale, Marco; Micallef, Jean-Paul; Place, Nicolas

    2013-06-01

    The aim of this study was to examine the magnitude and the origin of neuromuscular fatigue induced by half-squat static whole-body vibration (WBV) exercise, and to compare it to a non-WBV condition. Nine healthy volunteers completed two fatiguing protocols (WBV and non-WBV, randomly presented) consisting of five 1-min bouts of static half-squat exercise with a load corresponding to 50 % of their individual body mass. Neuromuscular fatigue of knee and ankle muscles was investigated before and immediately after each fatiguing protocol. The main outcomes were maximal voluntary contraction (MVC) torque, voluntary activation, and doublet peak torque. Knee extensor MVC torque decreased significantly (P fatiguing protocols. Doublet peak torque decreased significantly and to a similar extent following WBV and non-WBV exercise, for both knee extensors (-25 %; P fatigue and did not change its causative factors compared to non-WBV half-squat resistive exercise in recreationally active subjects.

  17. [From joint to neuromuscular, from mechanics to electronics: the concept of neuromuscular compatibility].

    Science.gov (United States)

    Toubol, J P; Duret, F

    1989-06-01

    The development of devices to record the functional mandibular kinetics, has permitted the physiological analysis of the masticatory cycles. Therefore, it is possible to consider a functional integration of the corrections, by addition or subtraction to the occlusal surfaces of the teeth. For this purpose, we have established the conditions of a physiological concept of occlusion taking into account the efficiency of the masticatory apparatus through a velocity analysis. This is the N.M.C. concept or concept of Neuro-Muscular Compatibility. This concept has led us to define the ISCA (Intra-Cuspid Spatial Angle), represented by the angle defined in the three spatial planes, by the trajectory of the occlusal contact point of a cusp liding over its opposite, in any excursive opportunity. We have shortly reported the studies by Levin on the SIROGNATOGRAPH and the prospects resulting from these studies. Then, we have studied in greater details the Saphon VISI TRAINER III developed by HOBO and described the various potentials of its use in the GBM Laboratory in Vienna (France), with François DURET. We have also analyzed the specific points which seem to represent the weaknesses of this device and have outlined some solutions to this problem. This approach, within a clinical context, may at term give rise to a complex analyser-expert-system intends to facilitate the establishment of a reliable diagnosis and to control the physiological integration of our procedures. The establishment, at term, of parameters and constants of this function, will guide this progression towards success.

  18. Neuromuscular Electrical Stimulation for Motor Restoration in Hemiplegia.

    Science.gov (United States)

    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. Copyright © 2015 Elsevier Inc. All rights reserved.

  19. Thermal, circulatory, and neuromuscular responses to whole-body cryotherapy

    OpenAIRE

    Westerlund, T. (Tarja)

    2009-01-01

    Abstract The purpose of this study was to examine thermal (body temperature, thermal sensation and comfort ratings), circulatory (blood pressure, heart rate variability) and neuromuscular performance responses to whole-body cryotherapy (WBC, -110 °C). Altogether 66 healthy subjects were exposed to WBC for two minutes. The acute and long-term changes were examined, when the subjects were exposed to WBC three times a week during three months. Skin temperatures decreased very rapidl...

  20. Neuromuscular control and rehabilitation of the unstable ankle

    OpenAIRE

    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. Howeve...

  1. Effect of newly proposed CK reference limits on neuromuscular diagnosis.

    Science.gov (United States)

    Nardin, Rachel A; Zarrin, Amy R; Horowitz, Gary L; Tarulli, Andrew W

    2009-04-01

    The objective was to determine the effect of a proposed increase in the upper reference limits of serum creatine kinase (CK) on neuromuscular disease diagnosis. This was a retrospective study of 94 Caucasian subjects (49 women and 45 men) in whom a neuromuscular physician ordered a CK as part of their evaluation. The patients were divided into two groups: those with diagnoses that either should or could elevate serum CK, and those with diagnoses that should not elevate serum CK. Sensitivities and specificities of the manufacturer's and the newly proposed upper reference limits were determined. For women, raising the upper reference limit of CK from 140 IU/L to 201 IU/L reduced the sensitivity of the test from 50% to 29%, while increasing the specificity from 67% to 80%. For men, raising the upper reference limit of CK from 174 IU/L to 322 IU/L reduced the sensitivity from 80% to 60%, while increasing the specificity from 63% to 80%. The newly proposed upper reference limits resulted in a false-negative CK of clinical significance in 7 of 94 subjects. Increasing the upper reference limit for CK reduced the sensitivity and increased the specificity of serum CK for neuromuscular disease diagnosis. Such a change will reduce unnecessary referrals and invasive diagnostic testing in patients with asymptomatic CK elevations. The clinical impact of the loss in sensitivity is small. If these new upper reference limits are adopted, neuromuscular physicians should be aware that a normal CK level does not exclude a diagnosis of myopathy.

  2. Ultrastructural muscle and neuro-muscular junction alterations in polymyositis

    Directory of Open Access Journals (Sweden)

    L. L. Babakova

    2012-01-01

    Full Text Available Ultrastructural analysis of 7 biopsies from m.palmaris longus and m.deltoideus in patients with confirmed polymyositis revealed alterationand degeneration of muscle fibers and anomalies of neuro-muscular junction (NMJ. The NMJ abnormalities and following denervation ofmuscle fibers in polymyositis start with subsynaptic damages. The occurance of regeneration features in muscle fibers at any stage is characteristic for PM.

  3. Computed tomography of skeletal muscles in neuromuscular disease

    Energy Technology Data Exchange (ETDEWEB)

    Rodiek, S.O.; Kuether, G.

    1985-06-01

    CT-documentation of skeletal muscular lesions caused by neuromuscular diseases implies an essential contribution to conventional techniques in the macroscopic field. Size, distribution and degree of lesions as well as compensatory mechanisms are proved thereby. We report about the different effects on muscle appearance referring to 106 patients of our own experience in amyotrophic lateral sclerosis, spinal muscular atrophy, poliomyelitis, polyradiculitis, polyneuropathy as well as peripheral traumatic nerve lesions.

  4. Generalized Block Failure

    DEFF Research Database (Denmark)

    Jönsson, Jeppe

    2015-01-01

    Block tearing is considered in several codes as a pure block tension or a pure block shear failure mechanism. However in many situations the load acts eccentrically and involves the transfer of a substantial moment in combination with the shear force and perhaps a normal force. A literature study...

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

    Science.gov (United States)

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

    2014-09-18

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  7. Altered neuromuscular control mechanisms of the trapezius muscle in fibromyalgia

    Directory of Open Access Journals (Sweden)

    Karlsson Stefan J

    2010-03-01

    Full Text Available Abstract Background fibromyalgia is a relatively common condition with widespread pain and pressure allodynia, but unknown aetiology. For decades, the association between motor control strategies and chronic pain has been a topic for debate. One long held functional neuromuscular control mechanism is differential activation between regions within a single muscle. The aim of this study was to investigate differences in neuromuscular control, i.e. differential activation, between myalgic trapezius in fibromyalgia patients and healthy controls. Methods 27 fibromyalgia patients and 30 healthy controls performed 3 minutes bilateral shoulder elevations with different loads (0-4 Kg with a high-density surface electromyographical (EMG grid placed above the upper trapezius. Differential activation was quantified by the power spectral median frequency of the difference in EMG amplitude between the cranial and caudal parts of the upper trapezius. The average duration of the differential activation was described by the inverse of the median frequency of the differential activations. Results the median frequency of the differential activations was significantly lower, and the average duration of the differential activations significantly longer in fibromyalgia compared with controls at the two lowest load levels (0-1 Kg (p Conclusion these findings illustrate a different neuromuscular control between fibromyalgia patients and healthy controls during a low load functional task, either sustaining or resulting from the chronic painful condition. The findings may have clinical relevance for rehabilitation strategies for fibromyalgia.

  8. Factors influencing attentiveness of people with profound intellectual and multiple disabilities in multisensory storytelling

    NARCIS (Netherlands)

    Ten Brug, Annet; Van der Putten, Annette A.J.; Penne, Anneleen; Maes, Bea; Vlaskamp, Carla

    Multisensory storytelling (MSST) is a storytelling method developed for people with profound intellectual and multiple disabilities (PIMD). The developers of MSST have established specific guidelines aimed at increasing the listener's attention. Whether, and to what extent, these guidelines indeed

  9. Acute profound thrombocytopenia with second exposure to eptifibatide associated with a strong antibody reaction

    Science.gov (United States)

    ATTAYA, SHARIFF; KANTHI, YOGENDRA; ASTER, RICHARD; MCCRAE, KEITH

    2015-01-01

    We present a case of eptifibatide-induced acute profound thrombocytopenia in a 64-year-old male receiving eptifibatide for the second time during percutaneous coronary intervention. Although rare, short and self-limited episodes of acute and profound thrombocytopenia have been associated with eptifibatide exposure. The thrombocytopenia is thought to be immune mediated, and assays are available to test for eptifibatide-induced platelet antibodies. PMID:19172524

  10. Acute profound thrombocytopenia with second exposure to eptifibatide associated with a strong antibody reaction

    OpenAIRE

    ATTAYA, SHARIFF; KANTHI, YOGENDRA; ASTER, RICHARD; MCCRAE, KEITH

    2009-01-01

    We present a case of eptifibatide-induced acute profound thrombocytopenia in a 64-year-old male receiving eptifibatide for the second time during percutaneous coronary intervention. Although rare, short and self-limited episodes of acute and profound thrombocytopenia have been associated with eptifibatide exposure. The thrombocytopenia is thought to be immune mediated, and assays are available to test for eptifibatide-induced platelet antibodies.

  11. Allan-Herndon-Dudley syndrome with unusual profound sensorineural hearing loss.

    Science.gov (United States)

    Gagliardi, Lucia; Nataren, Nathalie; Feng, Jinghua; Schreiber, Andreas W; Hahn, Christopher N; Conwell, Louise S; Coman, David; Scott, Hamish S

    2015-08-01

    The Allan-Herndon-Dudley syndrome is caused by mutations in the thyroid hormone transporter, Monocarboxylate transporter 8 (MCT8). It is characterized by profound intellectual disability and abnormal thyroid function. We report on a patient with Allan-Herndon-Dudley syndrome (AHDS) with profound sensorineural hearing loss which is not usually a feature of AHDS and which may have been due to a coexisting nonsense mutation in Microphthalmia-associated transcription factor (MITF). © 2015 Wiley Periodicals, Inc.

  12. The neuromuscular blockade produced by pure alkaloid, mitragynine and methanol extract of kratom leaves (Mitragyna speciosa Korth.).

    Science.gov (United States)

    Chittrakarn, Somsmorn; Keawpradub, Niwat; Sawangjaroen, Kitja; Kansenalak, Supaporn; Janchawee, Benjamas

    2010-06-16

    The effects of pure alkaloid, mitragynine and a methanolic extract of kratom leaves were investigated on neuromuscular junction and compound nerve action potential. Wistar rats were killed by cervical dislocation and decapitated. The phrenic nerve-hemidiaphragms, hemidiaphragms and sciatic nerve were isolated. Kratom methanolic extract present at 0.1-1 mg/mL and mitragynine (0.0156 mg/mL) decreased the muscle twitch on the isolated phrenic nerve-hemidiaphragm and hemidiaphragm preparation. Muscle relaxation caused by kratom extract (1 mg/mL) was greater than the effect of mitragynine. Pancuronium and succinylcholine potentiated the effect of kratom extract. It also had a direct relaxation effect on the hemidiaphragm muscle. The muscle relaxation caused by kratom extract was not antagonized by neostigmine, tetraethylammonium and calcium chloride. High concentrations of kratom extract (10-40 mg/mL) and mitragynine (2 mg/mL) blocked the nerve conduction, amplitude and duration of compound nerve action potential. The mechanism of action of kratom extract might not act as a competitive antagonist of acetylcholine yet its dominant effect was at the neuromuscular junction and not at the skeletal muscle or somatic nerve. Copyright 2010 Elsevier Ireland Ltd. All rights reserved.

  13. Kinematics and mechanics of tectonic block rotations

    Science.gov (United States)

    Nur, Amos; Scotti, Oona; Ron, Hagai

    1989-01-01

    Paleomagnetic, structural geology, and rock mechanics data are combined to explore the validity of the block rotation concept and its significance. The analysis is based on data from (1) Northern Israel, where fault slip and spacing are used to predict block rotation; (2) the Mojave Desert, with well-documented strike-slip fault sets, organized in at least three major domains; (3) the Lake Mead, Nevada, fault system with well-defined sets of strike-slip faults, which, in contrast to the Mojave region, are surrounded with domains of normal faults; and (4) the San Gabriel Mountains domain with a multiple set of strike-slip faults. It is found that block rotations can have a profound influence on the interpretation of geodetic measurements and the inversion of geodetic data, especially the type collected in GPS surveys. Furthermore, block rotations and domain boundaries may be involved in creating the heterogeneities along active fault systems which are responsible for the initiation and termination of earthquake rupture.

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

    Science.gov (United States)

    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.

  15. Non-invasive neuromuscular electrical stimulation in patients with central nervous system lesions: an educational review

    National Research Council Canada - National Science Library

    Schuhfried, Othmar; Crevenna, Richard; Fialka-Moser, Veronika; Paternostro-Sluga, Tatjana

    2012-01-01

    ...) 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...

  16. Aspectos cinemáticos e neuromusculares relacionados com o desempenho da saída do bloco na natação Kinematical and neuromuscular aspects related to performance during the swimming start

    Directory of Open Access Journals (Sweden)

    Daniele Detanico

    2011-12-01

    Full Text Available O objetivo deste estudo foi relacionar o desempenho (distância e tempo de voo na saída de bloco com variáveis cinemáticas e neuromusculares. Participaram 10 nadadores do sexo masculino (26,4 ± 6,6 anos, 76,4 ± 6,6 kg, 1,81 ± 0,07 m de estatura. Utilizou-se uma câmera filmadora (30 Hz para a aquisição das variáveis cinemáticas, sendo analisadas: distância de voo, tempo de voo, altura máxima do quadril, ângulos de saída de bloco e entrada na água. Para a obtenção da potência muscular foi utilizado o "Squat Jump" realizado sobre uma plataforma de força. Foram obtidas as seguintes variáveis: altura do salto, potência e pico de velocidade. Utilizou-se estatística descritiva e correlação de Pearson para relacionar as variáveis neuromusculares e cinemáticas com o desempenho na saída de bloco, utilizando nível de significância de 5%. Encontrou-se correlação significativa do ângulo de saída com a distância (r = -0,59 e com o tempo de voo (r = 0,88, além da altura máxima com o tempo de voo (r = 0,59. Não foi observada correlação significativa da distância e do tempo de voo com as variáveis neuromusculares (p > 0,05. Com base nos resultados pode-se concluir que o desempenho na saída de bloco da natação foi relacionado com o ângulo de saída de bloco e com a altura máxima do quadril na fase aérea. A potência muscular não interferiu no desempenho nos nadadores investigados.The aim of this study was to investigate the association between the performance of the start off the swimming block with kinematical and neuromuscular parameters (flight distance and flight time. Ten male swimmers (26.4 ± 6.6 years, 76.4 ± 6.6 kg, 1.81 ± 0.07 m participated in the study. A video camera (30 Hz was used to obtain the kinematic variables: flight distance, flight time, maximum height, start angle and entry angle. Muscle power was assessed via the performance of a Squat Jump on a force plate. Jump height, power and peak of

  17. Blocked Randomization with Randomly Selected Block Sizes

    Directory of Open Access Journals (Sweden)

    Jimmy Efird

    2010-12-01

    Full Text Available When planning a randomized clinical trial, careful consideration must be given to how participants are selected for various arms of a study. Selection and accidental bias may occur when participants are not assigned to study groups with equal probability. A simple random allocation scheme is a process by which each participant has equal likelihood of being assigned to treatment versus referent groups. However, by chance an unequal number of individuals may be assigned to each arm of the study and thus decrease the power to detect statistically significant differences between groups. Block randomization is a commonly used technique in clinical trial design to reduce bias and achieve balance in the allocation of participants to treatment arms, especially when the sample size is small. This method increases the probability that each arm will contain an equal number of individuals by sequencing participant assignments by block. Yet still, the allocation process may be predictable, for example, when the investigator is not blind and the block size is fixed. This paper provides an overview of blocked randomization and illustrates how to avoid selection bias by using random block sizes.

  18. Blocked randomization with randomly selected block sizes.

    Science.gov (United States)

    Efird, Jimmy

    2011-01-01

    When planning a randomized clinical trial, careful consideration must be given to how participants are selected for various arms of a study. Selection and accidental bias may occur when participants are not assigned to study groups with equal probability. A simple random allocation scheme is a process by which each participant has equal likelihood of being assigned to treatment versus referent groups. However, by chance an unequal number of individuals may be assigned to each arm of the study and thus decrease the power to detect statistically significant differences between groups. Block randomization is a commonly used technique in clinical trial design to reduce bias and achieve balance in the allocation of participants to treatment arms, especially when the sample size is small. This method increases the probability that each arm will contain an equal number of individuals by sequencing participant assignments by block. Yet still, the allocation process may be predictable, for example, when the investigator is not blind and the block size is fixed. This paper provides an overview of blocked randomization and illustrates how to avoid selection bias by using random block sizes.

  19. Static balance and function in children with cerebral palsy submitted to neuromuscular block and neuromuscular electrical stimulation: study protocol for prospective, randomized, controlled trial

    National Research Council Canada - National Science Library

    Kazon, Soráia; Grecco, Luanda A C; Pasini, Hugo; Corrêa, João C F; Christovão, Thaluanna C L; de Carvalho, Paulo de Tarsocamillo; Giannasi, Lilian Chrystiane; Lucareli, Paulo R G; de Oliveira, Luis Vicente Franco; Salgado, Afonso Shiguemi Inoue; Sampaio, Luciana M M; Oliveira, Claudia S

    2012-01-01

    .... The aim of the present study is to describe a protocol for a study aimed at analyzing the effects of NMES on dorsiflexors combined with physical therapy on static and functional balance in children...

  20. DMPD: Gram-negative endotoxin: an extraordinary lipid with profound effects oneukaryotic signal transduction. [Dynamic Macrophage Pathway CSML Database

    Lifescience Database Archive (English)

    Full Text Available 1916089 Gram-negative endotoxin: an extraordinary lipid with profound effects oneuk...ep;5(12):2652-60. (.png) (.svg) (.html) (.csml) Show Gram-negative endotoxin: an extraordinary lipid with profound effect...tive endotoxin: an extraordinary lipid with profound effects oneukaryotic signal transduction. Authors Raetz

  1. Glial Processes at the Drosophila Larval Neuromuscular Junction Match Synaptic Growth

    Science.gov (United States)

    Brink, Deidre L.; Gilbert, Mary; Xie, Xiaojun; Petley-Ragan, Lindsay; Auld, Vanessa J.

    2012-01-01

    Glia are integral participants in synaptic physiology, remodeling and maturation from blowflies to humans, yet how glial structure is coordinated with synaptic growth is unknown. To investigate the dynamics of glial development at the Drosophila larval neuromuscular junction (NMJ), we developed a live imaging system to establish the relationship between glia, neuronal boutons, and the muscle subsynaptic reticulum. Using this system we observed processes from two classes of peripheral glia present at the NMJ. Processes from the subperineurial glia formed a blood-nerve barrier around the axon proximal to the first bouton. Processes from the perineurial glial extended beyond the end of the blood-nerve barrier into the NMJ where they contacted synapses and extended across non-synaptic muscle. Growth of the glial processes was coordinated with NMJ growth and synaptic activity. Increasing synaptic size through elevated temperature or the highwire mutation increased the extent of glial processes at the NMJ and conversely blocking synaptic activity and size decreased the presence and size of glial processes. We found that elevated temperature was required during embryogenesis in order to increase glial expansion at the nmj. Therefore, in our live imaging system, glial processes at the NMJ are likely indirectly regulated by synaptic changes to ensure the coordinated growth of all components of the tripartite larval NMJ. PMID:22666403

  2. Glial processes at the Drosophila larval neuromuscular junction match synaptic growth.

    Directory of Open Access Journals (Sweden)

    Deidre L Brink

    Full Text Available Glia are integral participants in synaptic physiology, remodeling and maturation from blowflies to humans, yet how glial structure is coordinated with synaptic growth is unknown. To investigate the dynamics of glial development at the Drosophila larval neuromuscular junction (NMJ, we developed a live imaging system to establish the relationship between glia, neuronal boutons, and the muscle subsynaptic reticulum. Using this system we observed processes from two classes of peripheral glia present at the NMJ. Processes from the subperineurial glia formed a blood-nerve barrier around the axon proximal to the first bouton. Processes from the perineurial glial extended beyond the end of the blood-nerve barrier into the NMJ where they contacted synapses and extended across non-synaptic muscle. Growth of the glial processes was coordinated with NMJ growth and synaptic activity. Increasing synaptic size through elevated temperature or the highwire mutation increased the extent of glial processes at the NMJ and conversely blocking synaptic activity and size decreased the presence and size of glial processes. We found that elevated temperature was required during embryogenesis in order to increase glial expansion at the nmj. Therefore, in our live imaging system, glial processes at the NMJ are likely indirectly regulated by synaptic changes to ensure the coordinated growth of all components of the tripartite larval NMJ.

  3. Molecular Mechanisms of Treadmill Therapy on Neuromuscular Atrophy Induced via Botulinum Toxin A

    Science.gov (United States)

    Tsai, Sen-Wei; Chen, Hsiao-Ling

    2013-01-01

    Botulinum toxin A (BoNT-A) is a bacterial zinc-dependent endopeptidase that acts specifically on neuromuscular junctions. BoNT-A blocks the release of acetylcholine, thereby decreasing the ability of a spastic muscle to generate forceful contraction, which results in a temporal local weakness and the atrophy of targeted muscles. BoNT-A-induced temporal muscle weakness has been used to manage skeletal muscle spasticity, such as poststroke spasticity, cerebral palsy, and cervical dystonia. However, the combined effect of treadmill exercise and BoNT-A treatment is not well understood. We previously demonstrated that for rats, following BoNT-A injection in the gastrocnemius muscle, treadmill running improved the recovery of the sciatic functional index (SFI), muscle contraction strength, and compound muscle action potential (CMAP) amplitude and area. Treadmill training had no influence on gastrocnemius mass that received BoNT-A injection, but it improved the maximal contraction force of the gastrocnemius, and upregulation of GAP-43, IGF-1, Myo-D, Myf-5, myogenin, and acetylcholine receptor (AChR) subunits α and β was found following treadmill training. Taken together, these results suggest that the upregulation of genes associated with neurite and AChR regeneration following treadmill training may contribute to enhanced gastrocnemius strength recovery following BoNT-A injection. PMID:24327926

  4. Molecular Mechanisms of Treadmill Therapy on Neuromuscular Atrophy Induced via Botulinum Toxin A

    Directory of Open Access Journals (Sweden)

    Sen-Wei Tsai

    2013-01-01

    Full Text Available Botulinum toxin A (BoNT-A is a bacterial zinc-dependent endopeptidase that acts specifically on neuromuscular junctions. BoNT-A blocks the release of acetylcholine, thereby decreasing the ability of a spastic muscle to generate forceful contraction, which results in a temporal local weakness and the atrophy of targeted muscles. BoNT-A-induced temporal muscle weakness has been used to manage skeletal muscle spasticity, such as poststroke spasticity, cerebral palsy, and cervical dystonia. However, the combined effect of treadmill exercise and BoNT-A treatment is not well understood. We previously demonstrated that for rats, following BoNT-A injection in the gastrocnemius muscle, treadmill running improved the recovery of the sciatic functional index (SFI, muscle contraction strength, and compound muscle action potential (CMAP amplitude and area. Treadmill training had no influence on gastrocnemius mass that received BoNT-A injection, but it improved the maximal contraction force of the gastrocnemius, and upregulation of GAP-43, IGF-1, Myo-D, Myf-5, myogenin, and acetylcholine receptor (AChR subunits α and β was found following treadmill training. Taken together, these results suggest that the upregulation of genes associated with neurite and AChR regeneration following treadmill training may contribute to enhanced gastrocnemius strength recovery following BoNT-A injection.

  5. A Prognostic Model for Development of Profound Shock among Children Presenting with Dengue Shock Syndrome.

    Directory of Open Access Journals (Sweden)

    Phung Khanh Lam

    Full Text Available To identify risk factors and develop a prediction model for the development of profound and recurrent shock amongst children presenting with dengue shock syndrome (DSS.We analyzed data from a prospective cohort of children with DSS recruited at the Paediatric Intensive Care Unit of the Hospital for Tropical Disease in Ho Chi Minh City, Vietnam. The primary endpoint was "profound DSS", defined as ≥2 recurrent shock episodes (for subjects presenting in compensated shock, or ≥1 recurrent shock episodes (for subjects presenting initially with decompensated/hypotensive shock, and/or requirement for inotropic support. Recurrent shock was evaluated as a secondary endpoint. Risk factors were pre-defined clinical and laboratory variables collected at the time of presentation with shock. Prognostic model development was based on logistic regression and compared to several alternative approaches.The analysis population included 1207 children of whom 222 (18% progressed to "profound DSS" and 433 (36% had recurrent shock. Independent risk factors for both endpoints included younger age, earlier presentation, higher pulse rate, higher temperature, higher haematocrit and, for females, worse hemodynamic status at presentation. The final prognostic model for "profound DSS" showed acceptable discrimination (AUC=0.69 for internal validation and calibration and is presented as a simple score-chart.Several risk factors for development of profound or recurrent shock among children presenting with DSS were identified. The score-chart derived from the prognostic models should improve triage and management of children presenting with DSS in dengue-endemic areas.

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

    Science.gov (United States)

    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. © Georg Thieme Verlag KG Stuttgart · New York.

  7. Temperature dependent modulation of lobster neuromuscular properties by serotonin.

    Science.gov (United States)

    Hamilton, Jonna L; Edwards, Claire R; Holt, Stephen R; Worden, Mary Kate

    2007-03-01

    In cold-blooded species the efficacy of neuromuscular function depends both on the thermal environmental of the animal's habitat and on the concentrations of modulatory hormones circulating within the animal's body. The goal of this study is to examine how temperature variation within an ecologically relevant range affects neuromuscular function and its modulation by the neurohormone serotonin (5-HT) in Homarus americanus, a lobster species that inhabits a broad thermal range in the wild. The synaptic strength of the excitatory and inhibitory motoneurons innervating the lobster dactyl opener muscle depends on temperature, with the strongest neurally evoked muscle movements being elicited at cold (temperatures. However, whereas neurally evoked contractions can be elicited over the entire temperature range from 2 to >20 degrees C, neurally evoked relaxations of resting muscle tension are effective only at colder temperatures at which the inhibitory junction potentials are hyperpolarizing in polarity. 5-HT has two effects on inhibitory synaptic signals: it potentiates their amplitude and also shifts the temperature at which they reverse polarity by approximately +7 degrees C. Thus 5-HT both potentiates neurally evoked relaxations of the muscle and increases the temperature range over which neurally evoked muscle relaxations can be elicited. Neurally evoked contractions are maximally potentiated by 5-HT at warm (18 degrees C) temperatures; however, 5-HT enhances excitatory junction potentials in a temperature-independent manner. Finally, 5-HT strongly increases resting muscle tension at the coldest extent of the temperature range tested (2 degrees C) but is ineffective at 22 degrees C. These data demonstrate that 5-HT elicits several temperature-dependent physiological changes in the passive and active responses of muscle to neural input. The overall effect of 5-HT is to increase the temperature range over which neurally evoked motor movements can be elicited in this

  8. Neuromuscular Responses to Simulated Brazilian Jiu-Jitsu Fights

    Directory of Open Access Journals (Sweden)

    Corrêa da Silva Bruno Victor

    2014-12-01

    Full Text Available 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 and the 15th min 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.

  9. Quality of life after surgery for neuromuscular scoliosis

    Directory of Open Access Journals (Sweden)

    Peter Obid

    2013-02-01

    Full Text Available Surgery in patients with neuromuscular scoliosis is associated with a higher rate of complications. It is still controversially discussed whether the patients truly benefit from deformity correction. The purpose of this study is to investigate if the quality of life has been improved and if the patients and their caregivers are satisfied with the results of surgery. This is a retrospective clinical outcome study of 46 patients with neuromuscular scoliosis which were treated with primary stable posterior pedicle screw instrumentation and correction. To achieve fusion only autologous bone was used. Follow up was minimum 2 years and maximum 5 years with an average of 36 months. The patients and/or their caregivers received a questionnaire based on the PEDI (pediatric disability inventory and the GMFS (gross motor function score. The patients (and their caregivers were also asked if the quality of life has improved after surgery. Only 32 of 46 patients answered the questionnaire. The answers showed a high approval-rate regarding the patients satisfaction with the surgery and the improvement of quality of life. The questionnaire could be answered from 1 (I do not agree to 4 (I completely agree. The average agreement to the following statements was: i the quality of life has improved: 3.35; ii I am satisfied with surgery: 3.95; iii the operation has fulfilled my expectations: 3.76. The average age at surgery was 12.7 years. The mean pre-operative cobb-angle of the main curve was 83.1° with a correction post-operatively to a mean of 36.9° and 42.6° at final follow-up. That is an average correction of 56.9%. Although spinal fusion in neuromuscular scoliosis is associated with a higher rate of complications our results show that the patients and their caregivers are satisfied with the operation and the quality of life has improved after surgery.

  10. To Block or Not to Block?

    NARCIS (Netherlands)

    A.S. (Arona) Cristina; N.M.G. (Nicole) Tweeboom; E.M. (Ellen) Wesselingh

    2013-01-01

    An investigation into whether or not young people studying in higher education in the Netherlands have modified their download behaviour, in the light of a legal obligation to block The Pirate Bay (TPB) by Dutch Internet Service Providers (ISPs). In the lawsuit, it is argued that a blockade by the

  11. Acute Neuromuscular Adaptations in the Postural Control of Patients with Parkinson’s Disease after Perturbed Walking

    Directory of Open Access Journals (Sweden)

    Cristian F. Pasluosta

    2017-09-01

    Full Text Available Patients suffering from Parkinson’s disease (PD present motor impairments reflected in the dynamics of the center of pressure (CoP adjustments during quiet standing. One method to study the dynamics of CoP adjustments is the entropic half-life (EnHL, which measures the short-term correlations of a time series at different time scales. Changes in the EnHL of CoP time series suggest neuromuscular adaptations in the control of posture. In this study, we sought to investigate the immediate changes in the EnHL of CoP adjustments of patients with PD during one session of perturbed (experimental group and unperturbed treadmill walking (control group. A total of 39 patients with PD participated in this study. The experimental group (n = 19 walked on a treadmill providing small tilting of the treadmill platform. The control group (n = 20 walked without perturbations. Each participant performed 5-min practice followed by three 5-min training blocks of walking with or without perturbation (with 3-min resting in between. Quiet standing CoP data was collected for 30 s at pre-training, after each training block, immediately post-training, and after 10 min retention. The EnHL was computed on the original and surrogates (phase-randomized CoP signals in the medio-lateral (ML and anterior–posterior (AP directions. Data was analyzed using four-way mixed ANOVA. Increased EnHL values were observed for both groups (Time effect, p < 0.001 as the intervention progressed, suggesting neuromuscular adaptations in the control of posture. The EnHL of surrogate signals were significantly lower than for original signals (p < 0.001, confirming that these adaptations come from non-random control processes. There was no Group effect (p = 0.622, however by analyzing the significant Group by Direction by Time interaction (p < 0.05, a more pronounced effect in the ML direction of the perturbed group was observed. Altogether, our findings show that treadmill walking decreases

  12. [The silent yet successful revolution of mechanical ventilation in patients with neuromuscular disorders, in particular Duchenne muscular dystrophy

    NARCIS (Netherlands)

    Engelen, B.G.M. van

    2007-01-01

    While developments in molecular biology have been giving rise to breakthroughs in diagnosis, management and promising treatment approaches for neuromuscular disorders, there has been a silent revolution in ventilatory support of patients with a range of neuromuscular disorders with respiratory

  13. Preventing Ischial Pressure Ulcers: I. Review of Neuromuscular Electrical Stimulation

    Directory of Open Access Journals (Sweden)

    Hilton M. Kaplan

    2011-01-01

    Full Text Available Objective: Pressure ulcers (PUs are common and debilitating wounds that arise when immobilized patients cannot shift their weight. Treatment is expensive and recurrence rates are high. Pathophysiological mechanisms include reduced bulk and perfusion of chronically atrophic muscles as well as prolonged occlusion of blood flow to soft tissues from lack of voluntary postural shifting of body weight. This has suggested that PUs might be prevented by reanimating the paralyzed muscles using neuromuscular electrical stimulation (NMES. A review of the published literature over the past 2 decades is detailed.

  14. El vendaje neuromuscular en podología

    OpenAIRE

    Vázquez Amela, F. Xavier (Francesc Xavier); Verdaguer Sanmartí, Josefina; Lluch Fruns, Joan; Genís Barniol, Silvia

    2008-01-01

    Desde hace unos cinco años la introducción en España de los vendajes neuromusculares ha ido cobrando mayor relevancia, llegando el momento más álgido las imágenes de las olimpiadas de Beijing, donde se pudo observar el uso de este tipo de vendajes en muchas de las disciplinas deportivas. En este artículo pretendemos hacer una introducción general a este tipo de vendajes y la aplicación en patologías podales de gran incidencia en nuestras consultas.

  15. Segmental spinal instrumentation in the management of neuromuscular spinal deformity.

    Science.gov (United States)

    Taddonio, R F

    1982-01-01

    Seventeen patients with progressive neuromuscular spinal deformity were critically analyzed. All patients were surgically managed by employing segmental spinal instrumentation with Luque rods accompanied by posterior spinal fusion to sacrum. Satisfactory correction of scoliosis, kyphosis, and lordosis was achieved. Furthermore, maintainence and production of physiologic postural curves was possible with this method of instrumentation. Head and trunk decompensation and pelvic obliquity were not well controlled in this series. Respiratory complications in this high-risk group were minimal. Partial postoperative immobilization with bivalved thoraco-lumbosacral orthoses (TLSO) was employed in the majority of patients. Segmental spinal instrumentation provides significant benefits to justify its continued use and development.

  16. MRI in neuromuscular disorders; MRT bei neuromuskulaeren Erkrankungen

    Energy Technology Data Exchange (ETDEWEB)

    Fischmann, Arne [Klinik St. Anna, Luzern (Switzerland). Inst. fuer Radiologie und Nuklearmedizin; Fischer, Dirk [Kantonsspital Bruderholz (Switzerland)

    2014-03-15

    Neuromuscular disorders are caused by damage of the skeletal muscles or supplying nerves, in many cases due to a genetic defect, resulting in progressive disability, loss of ambulation and often a reduced life expectancy. Previously only supportive care and steroids were available as treatments, but several novel therapies are under development or in clinical trial phase. Muscle imaging can detect specific patterns of involvement and facilitate diagnosis and guide genetic testing. Quantitative MRT can be used to monitor disease progression either to monitor treatment or as a surrogate parameter for clinical trails. Novel imaging sequences can provide insights into disease pathology and muscle metabolism. (orig.)

  17. Resúmenes de los trabajos sobre las Enfermedades Neuromusculares

    OpenAIRE

    Congreso Nacional de Neurología

    2010-01-01

    Las enfermedades neuromusculares constituyen un conjunto de afectaciones que afectan las neuronas motoras periférica, las vías motoras eferentes o los efectores (músculos esqueléticos). Sus manifestaciones clínicas son muy variadas y dependen de la causa y de los niveles de afectación. En este acápite se pueden encontrar los resúmenes de trabajos relacionados con el síndrome de Guillain Barre, polineuropatía diabética, Atrofia Muscular Espinal, Distrofia miotónica y otros todos presentados en...

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

    NARCIS (Netherlands)

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

    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

  19. Lumbar hyperlordosis of neuromuscular origin: pathophysiology and surgical strategy for correction

    OpenAIRE

    Vialle, Raphaël; Khouri, Nejib; Glorion, Christophe; Lechevallier, Joël; Morin, Christian

    2006-01-01

    Lumbar hyperlordosis of neuromuscular origin is rare and requires surgical treatment in order to preserve a good sitting posture. We report twenty-seven cases of a preponderantly sagittal hyperlordosis deformity of the lumbar spine in patients with neuromuscular disorders and identify the indications and results of treatment.

  20. Plantar flexor neuromuscular adjustments following match-play football in hot and cool conditions

    DEFF Research Database (Denmark)

    Girard, O; Nybo, Lars; Mohr, Magni

    2015-01-01

    We assessed neuromuscular fatigue and recovery of the plantar flexors after playing football with or without severe heat stress. Neuromuscular characteristics of the plantar flexors were assessed in 17 male players at baseline and ∼30 min, 24, and 48 h after two 90-min football matches in temperate...

  1. INFORMATION AND COMMUNICATIONS TECHNOLOGIES – THE WAY OF SOCIALIZATION OF PEOPLE WITH PROFOUND VISUAL IMPAIRMENTS

    Directory of Open Access Journals (Sweden)

    Serhii I. Netosov

    2015-03-01

    Full Text Available The article analyzes the software and hardware, that give people with profound visual impairments the opportunity to work on the computer. Attention is drawn to the Braille printers, relief-dot displays, voice synthesizers, scanners that can read, adaptation and correction programs and so on. It is emphasized that ICT for the blind is a factor of their inclusion in the life as the subjects of action. For solving this problem people with profound visual impairments need systemic help of the state and civil society in getting programs and equipment, because they are high-tech, and therefore expensive. It is important to spread the information about the activity of the centers of tiflo-computerization, to organize the laboratories of correction and socialization of people with profound visual impairments, to provide the training of the specialists.

  2. Related Drupal Nodes Block

    NARCIS (Netherlands)

    Van der Vegt, Wim

    2010-01-01

    Related Drupal Nodes Block This module exposes a block that uses Latent Semantic Analysis (Lsa) internally to suggest three nodes that are relevant to the node a user is viewing. This module performs three tasks. 1) It periodically indexes a Drupal site and generates a Lsa Term Document Matrix.

  3. Effects of a short proprioceptive neuromuscular facilitation stretching bout on quadriceps neuromuscular function, flexibility, and vertical jump performance.

    Science.gov (United States)

    Place, Nicolas; Blum, Yannick; Armand, Stéphane; Maffiuletti, Nicola A; Behm, David G

    2013-02-01

    The inclusion of relatively long bouts of stretching (repeated static stretches of ∼30 seconds) in the warm-up is usually associated with a drop in muscle performance. The purpose of this study was to assess the effect of a novel self-administered proprioceptive neuromuscular facilitation (PNF) paradigm with short periods of stretching and contraction on quadriceps neuromuscular function, vertical jump performance, and articular range of motion (ROM). Twelve healthy men (age: 27.7 ± 7.3 years, height: 178.4 ± 10.4 cm, weight: 73.8 ± 16.9 kg) volunteered to participate in a PNF session and a control session separated by 2-7 days. The PNF stretching lasted 2 minutes and consisted of 4 sets of 5-second isometric hamstring contraction immediately followed by 5 seconds of passive static stretch of the quadriceps immediately followed by 5 seconds isometric quadriceps contraction for each leg. For the control session, the participants were asked to walk at a comfortable speed for 2 minutes. Active ROM of knee flexion, vertical jump performance, and quadriceps neuromuscular function were tested before, immediately after, and 15 minutes after the intervention. The PNF stretching procedure did not affect ROM, squat jump, and countermovement jump performances. Accordingly, we did not observe any change in maximal voluntary contraction force, voluntary activation level, M-wave and twitch contractile properties that could be attributed to PNF stretching. The present self-administered PNF stretching of the quadriceps with short (5-second) stretches is not recommended before sports where flexibility is mandatory for performance.

  4. Variation in Dube3a expression affects neurotransmission at the Drosophila neuromuscular junction

    Directory of Open Access Journals (Sweden)

    Colleen Valdez

    2015-07-01

    Full Text Available Changes in UBE3A expression levels in neurons can cause neurogenetic disorders ranging from Angelman syndrome (AS (decreased levels to autism (increased levels. Here we investigated the effects on neuronal function of varying UBE3A levels using the Drosophila neuromuscular junction as a model for both of these neurogenetic disorders. Stimulations that evoked excitatory junction potentials (EJPs at 1 Hz intermittently failed to evoke EJPs at 15 Hz in a significantly higher proportion of Dube3a over-expressors using the pan neuronal GAL4 driver C155-GAL4 (C155-GAL4>UAS-Dube3a relative to controls (C155>+ alone. However, in the Dube3a over-expressing larval neurons with no failures, there was no difference in EJP amplitude at the beginning of the train, or the rate of decrease in EJP amplitude over the course of the train compared to controls. In the absence of tetrodotoxin (TTX, spontaneous EJPs were observed in significantly more C155-GAL4>UAS-Dube3a larva compared to controls. In the presence of TTX, spontaneous and evoked EJPs were completely blocked and mEJP amplitude and frequency did not differ among genotypes. These data suggest that over-expression of wild type Dube3a, but not a ubiquitination defective Dube3a-C/A protein, compromises the ability of motor neuron axons to support closely spaced trains of action potentials, while at the same time increasing excitability. EJPs evoked at 15 Hz in the absence of Dube3a (Dube3a15b homozygous mutant larvae decayed more rapidly over the course of 30 stimulations compared to w1118 controls, and Dube3a15b larval muscles had significantly more negative resting membrane potentials (RMP. However, these results could not be recapitulated using RNAi knockdown of Dube3a in muscle or neurons alone, suggesting more global developmental defects contribute to this phenotype. These data suggest that reduced UBE3A expression levels may cause global changes that affect RMP and neurotransmitter release from

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

    Science.gov (United States)

    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.

  6. [Basic principles of non-respiratory physiotherapy for neuromuscular diseases].

    Science.gov (United States)

    Watzek, I; Winterholler, M

    2008-03-01

    Loss of function, muscle pain and secondary muscoloskeletal complaints are common symptoms of patients with neuromuscular disease. Many patients develop a progressive handicap. Physiotherapeutic treatment is often used in the management of neuromuscular diseases. Different therapeutic strategies are useful depending on the stage and pathophysiology of the disease and with regard to the extent of the patient's handicap. The aims of the physiotherapy and realistic targets should be discussed critically with the patient at the beginning of the treatment. We propose different physiotherapeutic strategies depending on the stage of the underlying disease: 1) Patient is able to walk--active phase: education in self-training with regard to the risks of exhaustion. Manual and physical treatment of mycofascial complaints. 2) Progressive functional loss--assistive phase: support of compensation and daily functioning. 3) Patient in wheelchair or bedbound, loss of most voluntary functions--passive phase. The knowledge of the pathopysiology of the underlying disease is essential for the development of therapeutic strategies. Loss of upper neurons leads to the development of spasticity and muscle hypertonia whereas muscular atrophy and weakness is a prominent feature of lower motor neuron loss. Overtreatment and exhaustive training may lead to secondary muscle damage in primary myopathies. Training in short sessions with intervals between may have protective effects.

  7. Innervation and neuromuscular control in ageing skeletal muscle.

    Science.gov (United States)

    Hepple, Russell T; Rice, Charles L

    2016-04-15

    Changes in the neuromuscular system affecting the ageing motor unit manifest structurally as a reduction in motor unit number secondary to motor neuron loss; fibre type grouping due to repeating cycles of denervation-reinnervation; and instability of the neuromuscular junction that may be due to either or both of a gradual perturbation in postsynaptic signalling mechanisms necessary for maintenance of the endplate acetylcholine receptor clusters or a sudden process involving motor neuron death or traumatic injury to the muscle fibre. Functionally, these changes manifest as a reduction in strength and coordination that precedes a loss in muscle mass and contributes to impairments in fatigue. Regular muscle activation in postural muscles or through habitual physical activity can attenuate some of these structural and functional changes up to a point along the ageing continuum. On the other hand, regular muscle activation in advanced age (>75 years) loses its efficacy, and at least in rodents may exacerbate age-related motor neuron death. Transgenic mouse studies aimed at identifying potential mechanisms of motor unit disruptions in ageing muscle are not conclusive due to many different mechanisms converging on similar motor unit alterations, many of which phenocopy ageing muscle. Longitudinal studies of ageing models and humans will help clarify the cause and effect relationships and thus, identify relevant therapeutic targets to better preserve muscle function across the lifespan. © 2015 The Authors. The Journal of Physiology © 2015 The Physiological Society.

  8. A neuromuscular monitoring system based on a personal computer.

    Science.gov (United States)

    White, D A; Hull, M

    1992-07-01

    We have developed a computerized neuromuscular monitoring system (NMMS) using commercially available subsystems, i.e., computer equipment, clinical nerve stimulator, force transducer, and strip-chart recorder. This NMMS was developed for acquisition and analysis of data for research and teaching purposes. Computer analysis of the muscle response to stimulation allows graphic and numeric presentation of the twitch response and calculated ratios. Since the system can store and recall data, research data can be accessed for analysis and graphic presentation. An IBM PC/AT computer is used as the central controller and data processor. The computer controls timing of the nerve stimulator output, initiates data acquisition, and adjusts the paper speed of the strip chart recorder. The data processing functions include establishing control response values (when no neuromuscular blockade is present), displaying force versus time and calculated data graphically and numerically, and storing these data for further analysis. The general purpose nature of the computer and strip chart recording equipment allow modification of the system primarily by changes in software. For example, new patterns of nerve stimulation, such as the posttetanic count, can be programmed into the computer system along with appropriate data display and analysis routines. The NMMS has functioned well in the operating room environment. We have had no episodes of electrocautery interference with the computer functions. The automated features have enhanced the utility of the NMMS.(ABSTRACT TRUNCATED AT 250 WORDS)

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

    Science.gov (United States)

    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.

  10. Synaptic Homeostasis and Its Immunological Disturbance in Neuromuscular Junction Disorders

    Directory of Open Access Journals (Sweden)

    Masaharu Takamori

    2017-04-01

    Full Text Available In the neuromuscular junction, postsynaptic nicotinic acetylcholine receptor (nAChR clustering, trans-synaptic communication and synaptic stabilization are modulated by the molecular mechanisms underlying synaptic plasticity. The synaptic functions are based presynaptically on the active zone architecture, synaptic vesicle proteins, Ca2+ channels and synaptic vesicle recycling. Postsynaptically, they are based on rapsyn-anchored nAChR clusters, localized sensitivity to ACh, and synaptic stabilization via linkage to the extracellular matrix so as to be precisely opposed to the nerve terminal. Focusing on neural agrin, Wnts, muscle-specific tyrosine kinase (a mediator of agrin and Wnts signalings and regulator of trans-synaptic communication, low-density lipoprotein receptor-related protein 4 (the receptor of agrin and Wnts and participant in retrograde signaling, laminin-network (including muscle-derived agrin, extracellular matrix proteins (participating in the synaptic stabilization and presynaptic receptors (including muscarinic and adenosine receptors, we review the functional structures of the synapse by making reference to immunological pathogenecities in postsynaptic disease, myasthenia gravis. The synapse-related proteins including cortactin, coronin-6, caveolin-3, doublecortin, R-spondin 2, amyloid precursor family proteins, glia cell-derived neurotrophic factor and neurexins are also discussed in terms of their possible contribution to efficient synaptic transmission at the neuromuscular junction.

  11. Fundamental Molecules and Mechanisms for Forming and Maintaining Neuromuscular Synapses

    Directory of Open Access Journals (Sweden)

    Steven J. Burden

    2018-02-01

    Full Text Available The neuromuscular synapse is a relatively large synapse with hundreds of active zones in presynaptic motor nerve terminals and more than ten million acetylcholine receptors (AChRs in the postsynaptic membrane. The enrichment of proteins in presynaptic and postsynaptic membranes ensures a rapid, robust, and reliable synaptic transmission. Over fifty years ago, classic studies of the neuromuscular synapse led to a comprehensive understanding of how a synapse looks and works, but these landmark studies did not reveal the molecular mechanisms responsible for building and maintaining a synapse. During the past two-dozen years, the critical molecular players, responsible for assembling the specialized postsynaptic membrane and regulating nerve terminal differentiation, have begun to be identified and their mechanism of action better understood. Here, we describe and discuss five of these key molecular players, paying heed to their discovery as well as describing their currently understood mechanisms of action. In addition, we discuss the important gaps that remain to better understand how these proteins act to control synaptic differentiation and maintenance.

  12. Neuromuscular compensation mechanisms in vocal fold paralysis and paresis.

    Science.gov (United States)

    Dewan, Karuna; Vahabzadeh-Hagh, Andrew; Soofer, Donna; Chhetri, Dinesh K

    2017-07-01

    Vocal fold paresis and paralysis are common conditions. Treatment options include augmentation laryngoplasty and voice therapy. The optimal management for this condition is unclear. The objective of this study was to assess possible neuromuscular compensation mechanisms that could potentially be used in the treatment of vocal fold paresis and paralysis. In vivo canine model. In an in vivo canine model, we examined three conditions: 1) unilateral right recurrent laryngeal nerve (RLN) paresis and paralysis, 2) unilateral superior laryngeal nerve (SLN) paralysis, and 3) unilateral vagal nerve paresis and paralysis. Phonatory acoustics and aerodynamics were measured in each of these conditions. Effective compensation was defined as improved acoustic and aerodynamic profile. The most effective compensation for all conditions was increasing RLN activation and decreasing glottal gap. Increasing RLN activation increased the percentage of possible phonatory conditions that achieved phonation onset. SLN activation generally led to decreased number of total phonation onset conditions within each category. Differential effects of SLN (cricothyroid [CT] muscle) activation were seen. Ipsilateral SLN activation could compensate for RLN paralysis; normal CT compensated well in unilateral SLN paralysis; and in vagal paresis/paralysis, contralateral SLN and RLN displayed antagonistic relationships. Methods to improve glottal closure should be the primary treatment for large glottal gaps. Neuromuscular compensation is possible for paresis. This study provides insights into possible compensatory mechanisms in vocal fold paresis and paralysis. NA Laryngoscope, 127:1633-1638, 2017. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

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

    Science.gov (United States)

    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.

  14. The Structure of Human Neuromuscular Junctions: Some Unanswered Molecular Questions

    Directory of Open Access Journals (Sweden)

    Clarke R. Slater

    2017-10-01

    Full Text Available The commands that control animal movement are transmitted from motor neurons to their target muscle cells at the neuromuscular junctions (NMJs. The NMJs contain many protein species whose role in transmission depends not only on their inherent properties, but also on how they are distributed within the complex structure of the motor nerve terminal and the postsynaptic muscle membrane. These molecules mediate evoked chemical transmitter release from the nerve and the action of that transmitter on the muscle. Human NMJs are among the smallest known and release the smallest number of transmitter “quanta”. By contrast, they have the most deeply infolded postsynaptic membranes, which help to amplify transmitter action. The same structural features that distinguish human NMJs make them particularly susceptible to pathological processes. While much has been learned about the molecules which mediate transmitter release and action, little is known about the molecular processes that control the growth of the cellular and subcellular components of the NMJ so as to give rise to its mature form. A major challenge for molecular biologists is to understand the molecular basis for the development and maintenance of functionally important aspects of NMJ structure, and thereby to point to new directions for treatment of diseases in which neuromuscular transmission is impaired.

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

    Science.gov (United States)

    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.

  16. Antisense Oligonucleotide-Based Therapy for Neuromuscular Disease

    Directory of Open Access Journals (Sweden)

    Valentina Sardone

    2017-04-01

    Full Text Available Neuromuscular disorders such as Duchenne Muscular Dystrophy and Spinal Muscular Atrophy are neurodegenerative genetic diseases characterized primarily by muscle weakness and wasting. Until recently there were no effective therapies for these conditions, but antisense oligonucleotides, a new class of synthetic single stranded molecules of nucleic acids, have demonstrated promising experimental results and are at different stages of regulatory approval. The antisense oligonucleotides can modulate the protein expression via targeting hnRNAs or mRNAs and inducing interference with splicing, mRNA degradation, or arrest of translation, finally, resulting in rescue or reduction of the target protein expression. Different classes of antisense oligonucleotides are being tested in several clinical trials, and limitations of their clinical efficacy and toxicity have been reported for some of these compounds, while more encouraging results have supported the development of others. New generation antisense oligonucleotides are also being tested in preclinical models together with specific delivery systems that could allow some of the limitations of current antisense oligonucleotides to be overcome, to improve the cell penetration, to achieve more robust target engagement, and hopefully also be associated with acceptable toxicity. This review article describes the chemical properties and molecular mechanisms of action of the antisense oligonucleotides and the therapeutic implications these compounds have in neuromuscular diseases. Current strategies and carrier systems available for the oligonucleotides delivery will be also described to provide an overview on the past, present and future of these appealing molecules.

  17. Acute and chronic neuromuscular adaptations to local vibration training.

    Science.gov (United States)

    Souron, Robin; Besson, Thibault; Millet, Guillaume Y; Lapole, Thomas

    2017-10-01

    Vibratory stimuli are thought to have the potential to promote neural and/or muscular (re)conditioning. This has been well described for whole-body vibration (WBV), which is commonly used as a training method to improve strength and/or functional abilities. Yet, this technique may present some limitations, especially in clinical settings where patients are unable to maintain an active position during the vibration exposure. Thus, a local vibration (LV) technique, which consists of applying portable vibrators directly over the tendon or muscle belly without active contribution from the participant, may present an alternative to WBV. The purpose of this narrative review is (1) to provide a comprehensive overview of the literature related to the acute and chronic neuromuscular changes associated with LV, and (2) to show that LV training may be an innovative and efficient alternative method to the 'classic' training programs, including in the context of muscle deconditioning prevention or rehabilitation. An acute LV application (one bout of 20-60 min) may be considered as a significant neuromuscular workload, as demonstrated by an impairment of force generating capacity and LV-induced neural changes. Accordingly, it has been reported that a training period of LV is efficient in improving muscular performance over a wide range of training (duration, number of session) and vibration (frequency, amplitude, site of application) parameters. The functional improvements are principally triggered by adaptations within the central nervous system. A model illustrating the current research on LV-induced adaptations is provided.

  18. Magnesium sulfate in femoral nerve block, does postoperative analgesia differ? A comparative study

    Directory of Open Access Journals (Sweden)

    Hossam A. ELShamaa

    2014-04-01

    Conclusion: The current study concluded that the admixture of magnesium to bupivacaine provides a profound prolongation of the femoral nerve block, in addition to a significant decrease in postoperative pain scores and total dose of rescue analgesia, with a longer bearable pain periods in the first postoperative day.

  19. Profound Endothelial Damage Predicts Impending Organ Failure and Death in Sepsis

    DEFF Research Database (Denmark)

    Johansen, Maria E; Johansson, Pär I.; Ostrowski, Sisse R

    2015-01-01

    Endothelial damage contributes to organ failure and mortality in sepsis, but the extent of the contribution remains poorly quantified. Here, we examine the association between biomarkers of superficial and profound endothelial damage (syndecan-1 and soluble thrombomodulin [sTM], respectively), or...

  20. Visual impairments in people with severe and profound multiple disabilities: An inventory of visual functioning

    NARCIS (Netherlands)

    van den Broek, E.G.C.; Janssen, C.G.C.; van Ramshorst, T.; Deen, L.

    2006-01-01

    Background: The prevalence of visual impairments in people with severe and profound multiple disabilities (SPMD) is the subject of considerable debate and is difficult to assess. Methods: In a typical Dutch care organization, all clients with SPMD (n = 76) participated in the study and specific

  1. The Relationship between Communication Problems and Psychological Difficulties in Persons with Profound Acquired Hearing Loss.

    Science.gov (United States)

    Knutson, John F.; Lansing, Charissa R.

    1990-01-01

    Twenty-seven adults with postlingually acquired profound deafness were administered the Communication Profile for the Hearing Impaired and several tests of psychological functioning and adjustment. Inadequate communication strategies and poor accommodations to deafness were associated with depression, social introversion, loneliness, and social…

  2. The Role of Sound in Residential Facilities for People With Profound Intellectual and Multiple Disabilities

    NARCIS (Netherlands)

    van den Bosch, Kirsten A.; Andringa, Tjeerd C.; Baskent, Deniz; Vlaskamp, Carla

    2016-01-01

    Attention to the auditory environment of people with profound intellectual and multiple disabilities (PIMD) is limited, both in research and practice. As there is a dynamic interplay between the quality of the auditory environment and well-being, a study was undertaken to test the validity of the

  3. Staff interactive style during multisensory storytelling with persons with profound intellectual and multiple disabilities

    NARCIS (Netherlands)

    Penne, A.; ten Brug, A.; Munde, V.; van der Putten, A.; Vlaskamp, C.; Maes, B.

    Background Multisensory storytelling (MSST) is an individualised activity for people with profound intellectual and multiple disabilities (PIMD) in which a story is being told with an emphasis on sensory experiences and social interaction. MSST is a promising approach, but needs more empirical

  4. Staff Interactive Style during Multisensory Storytelling with Persons with Profound Intellectual and Multiple Disabilities

    Science.gov (United States)

    Penne, A.; ten Brug, A.; Munde, V.; van der Putten, A.; Vlaskamp, C.; Maes, B.

    2012-01-01

    Background: Multisensory storytelling (MSST) is an individualised activity for people with profound intellectual and multiple disabilities (PIMD) in which a story is being told with an emphasis on sensory experiences and social interaction. MSST is a promising approach, but needs more empirical research evidence. In general, there is a lack of…

  5. The Curriculum for Children with Severe and Profound Learning Difficulties at Stephen Hawking School

    Science.gov (United States)

    Rayner, Matthew

    2011-01-01

    The increasing number of children with profound and multiple learning difficulties means that many schools for children with severe learning difficulties are having to review the curriculum that they offer. In addition, these schools are continuing to question whether a subject-based approach, in line with the National Curriculum, is the most…

  6. The Influence of Phonological Mechanisms in Written Spelling of Profoundly Deaf Children

    Science.gov (United States)

    Colombo, Lucia; Arfe, Barbara; Bronte, Tiziana

    2012-01-01

    In the present study, the effect of phonological and working memory mechanisms involved in spelling Italian single words was explored in two groups of children matched for grade level: a group of normally hearing children and a group of pre-verbally deaf children, with severe-to-profound hearing loss. Three-syllable and four-syllable familiar…

  7. The role of attention in the affective life of people with severe or profound intellectual disabilities

    NARCIS (Netherlands)

    Vos, Pieter; De Cock, Paul; Munde, Vera; Neerinckx, Heleen; Petry, Katja; Van den Noortgate, Wim; Maes, Bea

    Although it is shown that attention plays an important role both in the onset and in the regulation of emotions in people without disabilities there is no information about how attention is related to emotions in people with severe or profound intellectual disability (ID). Therefore, in our study,

  8. The impact of medical conditions on the support of children with profound intellectual and multiple disabilities

    NARCIS (Netherlands)

    Zijlstra, HP; Vlaskamp, C

    Background The aim of this study was to analyse the impact of medical conditions of children with profound intellectual and multiple disabilities on the professional support they receive in centres for special education. Method The medical files, the daily records and daily communication records

  9. Assessing the Efficacy of an Academic Hearing Peer Tutor for a Profoundly Deaf Student.

    Science.gov (United States)

    Burley, Suzanne; And Others

    1994-01-01

    Evaluation of using a hearing peer tutor to provide daily 20-minute math instruction for a profoundly deaf sixth-grade girl indicated that the peer tutoring intervention was highly successful, with the tutee meeting accuracy criteria for each of 4 curriculum objectives after only a brief period of intervention. (Author/DB)

  10. Profoundly Gifted Girls and Autism Spectrum Disorder: A Psychometric Case Study Comparison

    Science.gov (United States)

    Assouline, Susan G.; Nicpon, Megan Foley; Doobay, Alissa

    2009-01-01

    A case study of the psychometric characteristics of two profoundly gifted girls, one with autism spectrum disorder (ASD) and the other without ASD, is used to describe the nuances and subtleties most relevant in understanding the relationship between extreme giftedness and social difficulties. Through the presentation of the results from…

  11. Identification Audiometry in an Institutionalized Severely and Profoundly Mentally Retarded Population.

    Science.gov (United States)

    Moore, Ernest J.; And Others

    An audiometric screening survey was conducted on a severely and profoundly mentally retarded population using noise-makers and pure tone audiometry. Of those tested with noise-makers, 83% gave an identifiable response to sound, 7% did not respond, and 10% were considered difficult-to-test. By contrast, 4% passed, 2% failed, and 94% were…

  12. Incidence of Short-Sleep Patterns in Institutionalized Individuals with Profound Mental Retardation.

    Science.gov (United States)

    Poindexter, Ann R.; Bihm, Elson M.

    1994-01-01

    Sleep patterns of 103 institutionalized individuals with profound mental retardation were explored. Almost 40% were found to have short-sleep patterns. Short-sleep was predicted by blindness; nonshort-sleep was predicted by diagnosis of cerebral palsy and sodium valproate usage. Techniques for minimizing possible negative consequences of…

  13. What parents find important in the support of a child with profound intellectual and multiple disabilities

    NARCIS (Netherlands)

    Jansen, S. L. G.; van der Putten, A. A. J.; Vlaskamp, C.

    Background The importance of a partnership between parents and professionals in the support of children with disabilities is widely acknowledged and is one of the key elements of family-centred care'. To what extent family-centred principles are also applied to the support of persons with profound

  14. Motor interventions in children with severe or profound intellectual disabilities: motor, cognitive and social effects

    NARCIS (Netherlands)

    Houwen, Suzanne; van der Putten, Annette; Vlaskamp, Carla

    2014-01-01

    Background: It is generally agreed that motor activity promotes motor, cognitive, and social development, but the specific benefits in children with severe or profound intellectual disabilities (S-PID) are as yet unknown. The aim of this study was to systematically review the evidence related to

  15. Multisensory Narrative Tracking by a Profoundly Deaf Subject Using an Electrocutaneous Vocoder and a Vibrotactile Aid.

    Science.gov (United States)

    Lynch, Michael P.; And Others

    1989-01-01

    The study assessed the ability to track connected discourse by a congenitally profoundly deaf adult using an electrocutaneous vocoder and/or a vibrotactile aid in conjunction with or without lipreading and aided hearing. Overall, improvement in tracking performance occurred within and across phases of the study. (Author/DB)

  16. Investigating the Relationship between Observed Mood and Emotions in People with Severe and Profound Intellectual Disabilities

    Science.gov (United States)

    Vos, P.; De Cock, P.; Petry, K.; Van Den Noortgate, W.; Maes, B.

    2013-01-01

    Background: The measurement of subjective well-being in people with severe and profound intellectual disabilities (ID) is a difficult challenge. As they cannot self-report about their life satisfaction, because of severe communicative and cognitive limitations, behavioural observations of their emotions and moods are important in the measurement…

  17. Eptifibatide induced profound thrombocytopenia in a patient with pelvic malignancy: A case report

    OpenAIRE

    Squires, Kathryn C.; Guntupalli, Saketh R.; Thaker, Premal H.

    2012-01-01

    ► Eptifibatide is associated with profound thrombocytopenia and thrombosis secondary to a HITT-like mechanism associated with drug-dependant antibodies. ► Caution with eptifibatide use is needed in those pre-disposed to hypercoaguability, particularly those with an underlying malignancy.

  18. The Development of Plato Computer-Based Instruction for the Severely and Profoundly Developmentally Disabled.

    Science.gov (United States)

    Siegel, Martin A.; Clapp, Elizabeth Jane

    The 2 year project (July 1, 1978 through June 30, 1980) sought to determine the viability, attractiveness, and effectiveness of computer based instruction with approximately 225 severely and profoundly mentally handicapped and developmentally disabled institutionalized children and adults. Over 100 instructional formats were developed by staff…

  19. Peer Interactions among children with profound intellectual and multiple disabilities during group activities

    NARCIS (Netherlands)

    Nijs, Sara; Penne, Anneleen; Vlaskamp, Carla; Maes, Bea

    Background Children with profound intellectual and multiple disabilities (PIMD) meet other children with PIMD in day care centres or schools. This study explores the peer-directed behaviours of children with PIMD, the peer interaction-influencing behaviour of the direct support workers and the

  20. Profound hypotension after an intradermal injection of indigo carmine for sentinel node mapping.

    Science.gov (United States)

    Jo, Youn Yi; Lee, Mi Geum; Yun, Soon Young; Lee, Kyung Cheon

    2013-03-01

    Intradermal injections of indigo carmine for sentinel node mapping are considered safe and no report of an adverse reaction has been published. The authors described two cases of profound hypotension in women that underwent breast-conserving surgery after an intradermal injection of indigo carmine into the periareolar area for sentinel node mapping.

  1. Profound Hypotension after an Intradermal Injection of Indigo Carmine for Sentinel Node Mapping

    OpenAIRE

    Jo, Youn Yi; Lee, Mi Geum; Yun, Soon Young; Lee, Kyung Cheon

    2013-01-01

    Intradermal injections of indigo carmine for sentinel node mapping are considered safe and no report of an adverse reaction has been published. The authors described two cases of profound hypotension in women that underwent breast-conserving surgery after an intradermal injection of indigo carmine into the periareolar area for sentinel node mapping.

  2. Extent, Duration, and Content of Day Services' Activities for Persons with Profound Intellectual and Multiple Disabilities

    Science.gov (United States)

    Vlaskamp, Carla; Hiemstra, Saskia J.; Wiersma, Linda A.; Zijlstra, Bonne J. H.

    2007-01-01

    In the Netherlands, the Dutch government instituted policies that enable persons with profound intellectual and multiple disabilities (PIMD) to attend day services. Over the past 15 years, surveys have indicated a progressive increase in the number of hours that such adults spend at day activities centers. However, information about how these…

  3. Individual Focus in an Activity Centre: An Observational Study among Persons with Profound and Multiple Disabilities

    Science.gov (United States)

    Hiemstra, S. J.; Vlaskamp, C.; Wiersma, L. A.

    2007-01-01

    Increasing numbers of adults with profound intellectual and multiple disabilities (PIMD) are being offered more--and more frequent--day services at activity centres. Little is known about the way direct support persons (DSP) in activity centres divide their time over the various tasks they have to perform and to what extent they are focused on…

  4. Interaction between Persons with Profound Intellectual and Multiple Disabilities and Their Partners: A Literature Review

    Science.gov (United States)

    Hostyn, Ine; Maes, Bea

    2009-01-01

    Background: High quality interactions are of crucial importance for quality of life of persons with profound intellectual and multiple disabilities (PIMD). This literature review describes and synthesises studies addressing the interaction between persons with PIMD and their partners. Method: A computerised literature search using defined…

  5. A functionally focused curriculum for children with profound multiple disabilities : A goal analysis

    NARCIS (Netherlands)

    van der Putten, A; Reynders, K; Vlaskamp, C; Nakken, H

    Background This study analysed goals formulated in a functionally focused curriculum called Mobility Opportunities Via Education(TM) (MOVE). Method The subjects were 49 children with profound multiple disabilities (PMD) who attended a centre for special education where the MOVE curriculum was

  6. Heart rate and physical activity patterns in persons with profound intellectual and multiple disabilities

    NARCIS (Netherlands)

    Waninge, A.; Putten, A.A.J. van der; Stewart, R.E.; Steenbergen, B.; Wijck, R. van; Schans, C.P. van der

    2013-01-01

    Because physical fitness and health are related to physical activity, it is important to gain an insight into the physical activity levels of persons with profound intellectual and multiple disabilities (PIMD). The purpose of this study was to examine heart rate patterns to measure the activity

  7. Heart rate and physical activity patterns in persons with profound intellectual and multiple disabilities.

    NARCIS (Netherlands)

    Waninge, A.; Putten, A.A. van der; Stewart, R.E.; Steenbergen, B.; Wijck, R. van; Schans, C.P. van der

    2013-01-01

    Because physical fitness and health are related to physical activity, it is important to gain an insight into the physical activity levels of persons with profound intellectual and multiple disabilities (PIMD). The purpose of this study was to examine heart rate patterns to measure the activity

  8. The Effects of Governing Board Configuration on Profound Organizational Change in Hospitals

    Science.gov (United States)

    Alexander, Jeffrey A.; Ye, Yining; Lee, Shoou-Yih D.; Weiner, Bryan J.

    2006-01-01

    This study extends the literature on governing boards and organizational change by examining how governing board configurations have influenced profound organizational change in U.S. hospitals, and the conditions under which such change occurs. Hospitals governed by boards that more closely resembled a corporate governance model were more likely…

  9. HEART RATE AND PHYSICAL ACTIVITY PATTERNS IN PERSONS WITH PROFOUND INTELLECTUAL AND MULTIPLE DISABILITIES

    NARCIS (Netherlands)

    Waninge, Aly; van der Putten, Annette A. J.; Stewart, Roy E.; Steenbergen, Bert; van Wijck, Ruud; van der Schans, Cees P.

    2013-01-01

    Because physical fitness and health are related to physical activity, it is important to gain an insight into the physical activity levels of persons with profound intellectual and multiple disabilities (PIMD). The purpose of this study was to examine heart rate patterns to measure the activity

  10. Connections that Count: Brain-Computer Interface Enables the Profoundly Paralyzed to Communicate

    Science.gov (United States)

    ... a team of researchers in developing a brain-computer interface (BCI) system to help the profoundly paralyzed communicate. Dr. Wolpaw has received support from two NIH Institutes—NIBIB and the National Institute of Child Health and Human Development—and the James S. McDonnell Foundation. "For the ...

  11. Making Sense of Bereavement in People with Profound Intellectual and Multiple Disabilities: Carer Perspectives

    Science.gov (United States)

    Young, Hannah; Hogg, James; Garrard, Brenda

    2017-01-01

    Background: People with intellectual disabilities are thought to have a reduced capacity for understanding death. Drawing on cognitive theory, researchers have suggested that those with profound intellectual and multiple disabilities mainly perceive loss as a mismatch between past and present experiences. However, very little research has…

  12. Assessment of Computer-Based Preferences of Students with Profound Multiple Disabilities

    Science.gov (United States)

    Mechling, Linda C.; Bishop, Vanessa A.

    2011-01-01

    This article reports on two studies investigating the use of computer-based stimuli that may then be used to develop activities and programming for students with profound multiple disabilities (PMD). Both studies used an alternating treatments design and systematic assessment strategy to present stimuli sequentially and to measure student…

  13. Peer Interactions among Children with Profound Intellectual and Multiple Disabilities during Group Activities

    Science.gov (United States)

    Nijs, Sara; Penne, Anneleen; Vlaskamp, Carla; Maes, Bea

    2016-01-01

    Background: Children with profound intellectual and multiple disabilities (PIMD) meet other children with PIMD in day care centres or schools. This study explores the peer-directed behaviours of children with PIMD, the peer interaction-influencing behaviour of the direct support workers and the children's positioning. Method: Group activities for…

  14. The Structure of Informal Social Networks of Persons with Profound Intellectual and Multiple Disabilities

    Science.gov (United States)

    Kamstra, A.; van der Putten, A. A. J.; Vlaskamp, C.

    2015-01-01

    Background: Persons with less severe disabilities are able to express their needs and show initiatives in social contacts, persons with profound intellectual and multiple disabilities (PIMD), however, depend on others for this. This study analysed the structure of informal networks of persons with PIMD. Materials and Methods: Data concerning the…

  15. Social Peer Interactions in Persons with Profound Intellectual and Multiple Disabilities: A Literature Review

    Science.gov (United States)

    Nijs, Sara; Maes, Bea

    2014-01-01

    Social interactions may positively influence developmental and quality of life outcomes. Research in persons with profound intellectual and multiple disabilities (PIMD) mostly investigated interactions with caregivers. This literature review focuses on peer interactions of persons with PIMD. A computerized literature search of three databases was…

  16. An Analysis of Snoezelen Equipment to Reinforce Persons with Severe or Profound Mental Retardation

    Science.gov (United States)

    Matson, Johnny L.; Bamburg, Jay W.; Smalls, Yemonja

    2004-01-01

    Systematically developing methods of reinforcement for persons with severe and profound mental retardation has only recently received a good deal of attention. This topic is important since professionals in the field often have difficulty identifying sufficient numbers of positive stimuli. Snoezelen equipment as reinforcement for individuals with…

  17. Ropivacaine 7.5 mg/ml versus bupivacaine 5 mg/ml for interscalene brachial plexus block--a comparative study.

    Science.gov (United States)

    Hofmann-Kiefer, K; Herbrich, C; Seebauer, A; Schwender, D; Peter, K

    2002-06-01

    We investigated ropivacaine 75 mg/ml in comparison with bupivacaine 5 mg/ml in patients receiving interscalene brachial plexus block (ISB) and general anaesthesia. In this randomized, double-blind, prospective clinical trial, each patient received an ISB block according to the technique originally described by Winnie and a catheter technique as per Meier. The rapidity of onset and the quality of sensory and motor block were determined. After general anaesthesia had been induced further parameters evaluated were consumption of local anaesthetic, opioid and neuromuscular blocking drug. After arrival in the recovery room, the patients were assessed for intensity of pain using a visual analog scale (VAS). One hundred and twenty patients were included in the study. The onset and development of sensory block was similar in both groups. Development and quality of motor block was also nearly identical for both local anaesthetics. Consumption of neuromuscular blocking drug and opioid did not differ between ropivacaine and bupivacaine. In the recovery room the mean pain score was less than 25 in both groups. There were no significant differences in terms of onset and quality of sensory or motor block during the intraoperative and early postoperative period. In addition we did not identify any side-effects related to the administration of the local anaesthetics. Ropivacaine 7.5 mg/ml and bupivacaine 5mg/ml proved to be nearly indistinguishable when administered for interscalene brachial plexus block.

  18. Ultrasound-guided paravertebral block for pyloromyotomy in 3 neonates with congenital hypertrophic pyloric stenosis

    Directory of Open Access Journals (Sweden)

    Javier Mata-Gómez

    2015-08-01

    Full Text Available BACKGROUND AND OBJECTIVES: Hypertrophic pyloric stenosis is a relatively common affection of gastrointestinal tract in childhood that results in symptoms, such as projectile vomiting and metabolic disorders that imply a high risk of aspiration during anesthetic induction. In this way, the carrying out of a technique with general anesthesia and intravenous rapid sequence induction, preoxygenation and cricoid pressure are recommended. After the correction of systemic metabolic alkalosis and pH normalization, cerebrospinal fluid can keep a state of metabolic alkalosis. This circumstance, in addition to the residual effect of neuromuscular blocking agents, inhalant anesthetics and opioids could increase the risk of postoperative apnea after a general anesthesia.CASE REPORT: We present the successful management in 3 neonates in those a pyloromyotomy was carried out because they had presented congenital hypertrophic pyloric stenosis. This procedure was done under general anesthesia with orotracheal intubation and rapid sequence induction. Then, ultrasound-guided paravertebral block was performed as analgesic method without the need for administrating opioids within intraoperative period and keeping an appropriate analgesic level.CONCLUSIONS: Local anesthesia has demonstrated to be safe and effective in pediatric practice. We consider the ultrasound-guided paravertebral block with one dose as a possible alternative for other local techniques described, avoiding the use of opioids and neuromuscular blocking agents during general anesthesia, and reducing the risk of central apnea within postoperative period.

  19. Bundle Branch Block

    Science.gov (United States)

    ... 2015. Bundle branch block Symptoms & causes Diagnosis & treatment Advertisement Mayo Clinic does not endorse companies or products. ... a Job Site Map About This Site Twitter Facebook Google YouTube Pinterest Mayo Clinic is a not- ...

  20. Concrete Block Pavements

    Science.gov (United States)

    1983-03-01

    intersections, bus loading areas, and pedestrian crosswalks. The change in surface texture between conven- tional and block pavements has been successful...blocks polished under traffic within a few weeks, providing a pavement surface with unsatisfactory skid resistance. U. K. Cement and Concrete Association...further problems of this type. Kellersman (1980) reports that although many brick pavements have become polished and slippery under traffic, no concrete

  1. Defining block character

    OpenAIRE

    A E Stamps

    1999-01-01

    In this paper I propose a clear, efficient, and accurate method for determining if a block of contiguous buildings has an overall character. The work is needed because most contemporary design reviews presuppose the existence of visual character, but existing design principles are often too vague to make the required determination. Clarity is achieved by shifting from vague notions to a definite concept for block character: a design feature will be perceived as part of the overall character o...

  2. Block ciphers in UMTS

    OpenAIRE

    Mathisen, Tor-Erik

    2004-01-01

    As we are entering the third generation of mobile technology (3G) the number of services needing security grows larger. To assess if the security provided by 3G is sufficient, we take a closer look at the security mechanisms and their building blocks. Within the 3G security environment the Kasumi block cipher plays an important role in the integrity and confidentiality provided. Thus the security of Kasumi, the integrity mode ($9) and confidentiality mode (f8) is vital. ...

  3. Block Cipher Analysis

    DEFF Research Database (Denmark)

    Miolane, Charlotte Vikkelsø

    (AES).Wedescribe the mostgeneraltypes ofblock cipher cryptanalysis but concentrate on the algebraic attacks. While the algebraic techniques have been successful oncertainstreamcipherstheirapplicationtoblock ciphershasnot shown any significant results so far. This thesis contributes to the field of algebraic attacks on block ciphers...... on small scale variants of AES. In the final part of the thesis we present a new block cipher proposal Present and examine its security against algebraic and differential cryptanalysis in particular....

  4. The Preparation Period in Basketball: Training Load and Neuromuscular Adaptations.

    Science.gov (United States)

    Ferioli, Davide; Bosio, Andrea; Bilsborough, Johann C; Torre, Antonio La; Tornaghi, Michele; Rampinini, Ermanno

    2018-01-18

    To investigate the 1) effect of the preparation period on the neuromuscular characteristics of 12 professional (PRO) and 16 semi-professional (SEMI-PRO) basketball players; 2) relationships between training load indices and changes in neuromuscular physical performance. Prior to and following the preparation period, players underwent a counter-movement jump (CMJ) test, followed by a repeated change of direction (COD) test consisting of 4 levels with increasing intensities. The peripheral neuromuscular functions of the knee extensors (peak torque, PT) were measured using electrical stimulations after each level (PT1, PT2, PT3 and PT4). Furthermore, PT Max (the highest value of PT) and PT Dec (PT decrement from PT Max to PT4) were calculated. Trivial-to-small (effect size, ES: -0.17 to 0.46) improvements were found in CMJ variables, regardless of the competitive levels. After the preparation period, peripheral fatigue induced by a COD test was similarly reduced in both PRO (PT Dec: from 27.8±21.3% to 11.4±13.7%, ES±90%CI= -0.71±0.30) and SEMI-PRO (PT Dec: from 26.1±21.9% to 10.2±8.2%, ES±90%CI= -0.69±0.32). Moderate-to-large relationships were found between session rating of perceived exertion training load and changes in PPO measured during the CMJs (r s ±90%CI: PPOabs, -0.46±0.26; PPOrel, -0.53±0.23) and in some PTs measured during the COD test (PT1, -0.45±0.26; PT2, -0.44±0.26; PT3, -0.40±0.27 and PT Max, -0.38±0.28). Preparation period induced minimal changes in the CMJ, while the ability to sustain repeated COD efforts was improved. Reaching high session rating of perceived exertion training loads might partially and negatively affect the ability to produce strength and power.

  5. Alterations in neuromuscular function in girls with generalized joint hypermobility

    DEFF Research Database (Denmark)

    Jensen, Bente Rona; Melcher, Jesper Sandfeld; Melcher, Pia Grethe Sandfeld

    2016-01-01

    (90°) at 20 % Maximum Voluntary Contraction, and explosive isometric knee flexions while sitting. EMG was recorded from knee flexor and extensor muscles. RESULTS: Early rate of torque development was 53 % faster for GJH. Reduced hamstring muscle activation in girls with GJH was found while knee......BACKGROUND: Generalized Joint Hypermobility (GJH) is associated with increased risk of musculoskeletal joint pain. We investigated neuromuscular performance and muscle activation strategy. METHODS: Girls with GJH and non-GJH (NGJH) performed isometric knee flexions (90°,110°,130°), and extensions...... extensor and calf muscle activation did not differ between groups. Flexion-extension and medial-lateral co-activation ratio during flexions were higher for girls with GJH than NGJH girls. CONCLUSIONS: Girls with GJH had higher capacity to rapidly generate force than NGJH girls which may reflect motor...

  6. A novel neuromuscular syndrome associated with clenbuterol-tainted heroin.

    Science.gov (United States)

    Manini, Alex; Labinson, Robert M; Kirrane, Barbara; Hoffman, Robert S; Rao, Rama; Stajic, Marina; Nelson, Lewis S

    2008-12-01

    Clenbuterol is a potent, long-acting beta-adrenergic agonist that has been reported as an adulterant of heroin. We describe an atypical syndrome in five users of clenbuterol-tainted heroin. All cases were referred to a regional Poison Control Center. Urine and blood were analyzed using gas and liquid chromatography as well as mass spectrometry. Five heroin users presented with a syndrome characterized by muscular spasm, tremor, hyperreflexia, and elevated serum creatine phosphokinase concentrations. All patients lacked findings of acute clenbuterol toxicity but tested positive for clenbuterol and negative for strychnine and a battery of common potential adulterants. We report five cases of a novel neuromuscular syndrome in users of clenbuterol-adulterated heroin. It is unclear whether these reactions represent an atypical response to clenbuterol or another unidentified contaminant.

  7. Correlation between specific histological and electromyographic findings in neuromuscular disorders

    Directory of Open Access Journals (Sweden)

    Lineu Cesar Werneck

    1988-09-01

    Full Text Available An attempt was made to find a correlation between specific electromyography (EMG abnormalities with histological findings in muscle biopsies (MB in 100 patients with neuromuscular disorders. Quantified EMG and MB with histochemistry was made in the same muscle, but on the opposite side, within a period of 3 weeks. The isolated findings of EMG and MB were analysed with a computer through a chi-square test. A statistical relation (p<0.01 was found between the isolated findings of MB and EMG in only 6.99% (39 in 558 attempts of the abnormalities expected to occur in myopathy and denervation. Also was found 2.51% (14 in 558 attempts of inconsistences with the current literature.

  8. Isozyme patterns and protein profiles in neuromuscular disorders.

    Science.gov (United States)

    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.

  9. Agrin to YAP in Cancer and Neuromuscular Junctions.

    Science.gov (United States)

    Xiong, Wen-Cheng; Mei, Lin

    2017-04-01

    Agrin is utilized by motor neurons to stimulate the LRP4-MuSK receptor in muscles for neuromuscular junction (NMJ) formation. Recent studies of cancer have identified novel functions of the low-density lipoprotein receptor-related protein 4-muscle-specific kinase (LRP4-MuSK) pathway. Agrin may act as a mechanotransduction signal in the extracellular matrix (ECM) to coordinate the cross-talk between the LRP4-MuSK pathway and integrin-focal adhesion pathway. Ensuing Yes-associated protein (YAP) activation promotes hepatocellular carcinoma (HCC). Here, we discuss the implications of the converged pathways in NMJ formation and liver cancer. Copyright © 2017 Elsevier Inc. All rights reserved.

  10. Cellular and Molecular Anatomy of the Human Neuromuscular Junction

    Directory of Open Access Journals (Sweden)

    Ross A. Jones

    2017-11-01

    Full Text Available The neuromuscular junction (NMJ plays a fundamental role in transferring information from lower motor neuron to skeletal muscle to generate movement. It is also an experimentally accessible model synapse routinely studied in animal models to explore fundamental aspects of synaptic form and function. Here, we combined morphological techniques, super-resolution imaging, and proteomic profiling to reveal the detailed cellular and molecular architecture of the human NMJ. Human NMJs were significantly smaller, less complex, and more fragmented than mouse NMJs. In contrast to mice, human NMJs were also remarkably stable across the entire adult lifespan, showing no signs of age-related degeneration or remodeling. Super-resolution imaging and proteomic profiling revealed distinctive distribution of active zone proteins and differential expression of core synaptic proteins and molecular pathways at the human NMJ. Taken together, these findings reveal human-specific cellular and molecular features of the NMJ that distinguish them from comparable synapses in other mammalian species.

  11. Neuromuscular blockade for improvement of surgical conditions during laparotomy

    DEFF Research Database (Denmark)

    Madsen, Matias Vested; Scheppan, Susanne; Kissmeyer, Peter

    2015-01-01

    scale every 30 min. Primary outcome is the average score for a patient's surgical condition. Secondary outcomes are, among others, surgical rating score during fascial closure, wound dehiscence, wound infection requiring surgical drainage and incisional hernia at the six-month follow-up. CONCLUSIONS......INTRODUCTION: During laparotomy, surgeons frequently experience difficult surgical conditions if the patient's abdominal wall or diaphragm is tense. This issue is particularly pertinent while closing the fascia and placing the intestines into the abdominal cavity. Establishment of a deep...... neuromuscular blockade (NMB), defined as a post-tetanic-count (PTC) of 0-1, paralyses the abdominal wall muscles and the diaphragm. We hypothesised that deep NMB (PTC 0-1) would improve surgical conditions during upper laparotomy as compared to standard NMB with bolus administration. METHODS...

  12. Supervised neuromuscular exercise prior to hip and knee replacement

    DEFF Research Database (Denmark)

    Fernandes, Linda; Roos, Ewa M; Overgaard, Søren

    2017-01-01

    BACKGROUND: There are indications of beneficial short-term effect of pre-operative exercise in reducing pain and improving activity of daily living after total hip replacement (THR) and total knee replacement (TKR) surgery. Though, information from studies conducting longer follow-ups and economic...... for standard THR or TKR at a hospital located in a rural area of Denmark. The patients were randomised to replacement surgery with or without an 8-week preoperative supervised neuromuscular exercise program (Clinical Trials registration no.: NCT01003756). Clinical effect was measured with Hip disability...... and Osteoarthritis Outcome Score (HOOS) and Knee injury and Osteoarthritis Outcome Score (KOOS). Quality adjusted life years (QALYs) were based on EQ-5D-3L and Danish preference weights. Resource use was extracted from national registries and valued using standard tariffs (2012-EUR). Incremental net benefit...

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

    Science.gov (United States)

    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

  14. Neuromuscular training for sports injury prevention: a systematic review.

    Science.gov (United States)

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

    2010-03-01

    The aim of this systematic review was to assess the effectiveness of proprioceptive/neuromuscular training in preventing sports injuries by using the best available evidence from methodologically well-conducted randomized controlled trials and controlled clinical trials without randomization. Two independent researchers performed a literature search in various electronic databases and reference lists. The reviewers independently assessed trials for inclusion criteria and methodological quality and extracted the data. Focusing on studies of high methodological quality, relative risks (RR) and 95% confidence intervals (CI) were used to estimate treatment effects. From a total of 32 relevant studies, 7 methodologically well-conducted studies were considered for this review. Pooled analysis revealed that multi-intervention training was effective in reducing the risk of lower limb injuries (RR = 0.61, 95% CI = 0.49-0.77, P injuries (RR = 0.46, 95% CI = 0.28-0.76, P injuries (RR = 0.50, 95% CI = 0.31-0.79, P training alone resulted in a significant risk reduction of ankle sprain injuries (RR = 0.64, 95% CI = 0.46-0.9, P injuries overall (RR = 0.49, 95% CI = 0.13-1.8, P = 0.28). Exercise interventions were more effective in athletes with a history of sports injury than in those without. On the basis of the results of seven high-quality studies, this review showed evidence for the effectiveness of proprioceptive/neuromuscular training in reducing the incidence of certain types of sports injuries among adolescent and young adult athletes during pivoting sports. Future research should focus on the conduct of comparative trials to identify the most appropriate and effective training components for preventing injuries in specific sports and populations.

  15. LRP4 is critical for neuromuscular junction maintenance.

    Science.gov (United States)

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

    2014-10-15

    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. Copyright © 2014 the authors 0270-6474/14/3413892-14$15.00/0.

  16. Neuromuscular Fatigue After Repeated Jumping With Concomitant Electrical Stimulation.

    Science.gov (United States)

    Neyroud, Daria; Samararatne, Jimmy; Kayser, Bengt; Place, Nicolas

    2017-12-18

    To evaluate the etiology and extent of neuromuscular fatigue induced by 50 squat jumps performed with and without neuromuscular electrical stimulation (NMES) of the knee extensors. Nine healthy, recreationally active men (24 ± 2 y) took part in 2 experiments. These consisted of 50 squat jumps performed with stimulation (NMES) or without (CON). Maximal voluntary contraction (MVC) force, maximal voluntary activation level (VAL), and forces evoked by single and double (10 and 100 Hz) stimulations were recorded before and after the 50 jumps. NMES was delivered at the maximal tolerated intensity. Despite average jump height being ∼16% lower in the NMES than in the CON session, a reduction over time in jump height was only found in the NMES condition (-6%). After the 50 jumps, MVC force was reduced to a greater extent in NMES than in CON (-25% ± 11% vs -11% ± 12%). Similarly, forces evoked by single stimulations, as well as by 10-Hz and 100-Hz paired stimulations, were reduced to a greater extent in NMES (-33% ± 12%, -42% ± 15%, and -25% ± 13%) than in CON (-21% ± 6%, -30% ± 9%, and -14% ± 11%). VAL was not significantly altered by either condition. Performing repeated squat jumps with concomitant NMES induced a greater fatigue than squat jumps performed alone and might potentially represent a stronger training stimulus.

  17. EFFECT OF NEUROMUSCULAR TRAINING ON BALANCE AMONG UNIVERSITY ATHLETES

    Directory of Open Access Journals (Sweden)

    Mohansundar Sankaravel

    2016-06-01

    Full Text Available Background: Proprioceptive deficiency followed by lateral ankle sprain leads to poor balance is not uncommon. It has been linked with increased injury risk among young athletes. Introducing neuromuscular training programs for this have been believed as one of the means of injury prevention. Hence, this study was aimed to determine the effects of six weeks progressive neuromuscular training (PNM Training on static balance gains among the young athletes with a previous history of ankle sprains. Methods: This study was an experimental study design, with pre and post test method to determine the effects of PNM Training on static balance gains. All data were collected at university’s sports rehabilitation lab before and after six weeks of intervention period. There were 20 male and female volunteer young athletes (20.9 ± 0.85 years of age with a previous history of ankle sprain involving various sports were recruited from the University community. All the subjects were participated in a six week PNM Training that included stability, strength and power training. Outcome measures were collected by calculating the errors on balance error scoring system made by the athletes on static balance before and after the six weeks of intervention period. Static balance was tested in firm and foam surfaces and recorded accordingly. Results: The researchers found a significant decrease (2.40 ± 0.82 in total errors among the samples at the post test compared with their pre test (P >0.05. Conclusions: The study demonstrates that a PNM Training can improve the static balance on both the firm and foam surfaces among the young athletes with a previous history of ankle sprains.

  18. Correcting Neuromuscular Deficits With Gene Therapy in Pompe Disease

    Science.gov (United States)

    Todd, Adrian G.; McElroy, Jessica A.; Grange, Robert W.; Fuller, David D.; Walter, Glenn A.; Byrne, Barry J.; Falk, Darin J.

    2015-01-01

    Objective We have recently reported on the pathology of the neuromuscular junction (NMJ) in Pompe disease, reflecting disruption of neuronal and muscle homeostasis as a result of glycogen accumulation. The aim of this study was to examine how the alteration of NMJ physiology contributes to Pompe disease pathology; we performed molecular, physiological, and histochemical analyses of NMJ-related measures of the tibialis anterior muscles of young-, mid-, and late-stage alpha-glucosidase (GAA)-deficient mice. Methods We performed intramuscular injection of an adeno-associated virus (AAV)9 vector expressing GAA (AAV9-hGAA) into the tibialis anterior muscle of Gaa–/– mice at early, mid, and severe pathological time points. We analyzed expression of NMJ-related genes, in situ muscle force production, and clearance of glycogen in conjunction with histological assessment of the NMJ. Results Our data demonstrate that AAV9-hGAA is able to replace GAA to the affected tissue and modify AChR mRNA expression, muscle force production, motor endplate area, and innervation status. Importantly, the degree of restoration for these outcomes is limited by severity of disease. Early restoration of GAA activity was most effective, whereas late correction of GAA expression was not effective in modifying parameters reflecting NMJ structure and function nor in force restoration despite resolution of glycogen storage in muscle. Interpretation Our data provide new mechanistic insight into the pathology of Pompe disease and suggest that early systemic correction to both neural and muscle tissues may be essential for successful correction of neuromuscular function in Pompe disease. PMID:25925726

  19. Oral Communication Development in Severe to Profound Hearing Impaired Children After Receiving Aural Habilitation

    Directory of Open Access Journals (Sweden)

    Soleimani Farin

    2009-10-01

    Full Text Available Communication, cognition, language, and speech are interrelated and develop together. It should come as no surprise to us that the key to intervention with deaf children is to establish, as early as possible, a functional communication system for the child and the parents. Early intervention programs need to be multidisciplinary, technologically sound and most important, it should take cognizance of the specific context (community, country in which the child and family function. The main aim of this study was to obtain oral communication development regarding current status of the intervention (aural habilitation and speech therapyfor children with severe to profound hearing impairment in Iran. A prospective longitudinal study was undertaken on a consecutive group of children with severe to profound deafness. Nine severe to profound hearing-impaired children out of the primer 42 cases, who were detected below two years old, had been selected in the previous study to receive aural habilitation. The average of their speech intelligibility scores was near 70% at age 6, which was accounted as poor oral communication and only two of them were able to communicate by spoken language. An integrated intervention services continued again for one year and their oral communication skill was assessed by their speech intelligibility. The intelligibility test of children was recorded on audio-tape, when they read 10 questions such as where is your home. This can be answered only in one word. Each tape was presented to10 normal hearing listeners, and their task was to write down, the answers in Persian orthography. At the beginning (at age 6 the average speech intelligibility score of these children was 72% and only two of them had score of 90% and 100%. At age 7, all of the severe groups were over 90%, and only two profound ones achieved the score of 48% and 62%. All of severe groups develop oral communication, but profound ones had a semi-intelligible speech

  20. Motor neuron apoptosis and neuromuscular junction perturbation are prominent features in a Drosophila model of Fus-mediated ALS

    Directory of Open Access Journals (Sweden)

    Xia Ruohan

    2012-03-01

    Full Text Available Abstract Backgound Amyotrophic lateral sclerosis (ALS is progressive neurodegenerative disease characterized by the loss of motor function. Several ALS genes have been identified as their mutations can lead to familial ALS, including the recently reported RNA-binding protein fused in sarcoma (Fus. However, it is not clear how mutations of Fus lead to motor neuron degeneration in ALS. In this study, we present a Drosophila model to examine the toxicity of Fus, its Drosophila orthologue Cabeza (Caz, and the ALS-related Fus mutants. Results Our results show that the expression of wild-type Fus/Caz or FusR521G induced progressive toxicity in multiple tissues of the transgenic flies in a dose- and age-dependent manner. The expression of Fus, Caz, or FusR521G in motor neurons significantly impaired the locomotive ability of fly larvae and adults. The presynaptic structures in neuromuscular junctions were disrupted and motor neurons in the ventral nerve cord (VNC were disorganized and underwent apoptosis. Surprisingly, the interruption of Fus nuclear localization by either deleting its nuclear localization sequence (NLS or adding a nuclear export signal (NES blocked Fus toxicity. Moreover, we discovered that the loss of caz in Drosophila led to severe growth defects in the eyes and VNCs, caused locomotive disability and NMJ disruption, but did not induce apoptotic cell death. Conclusions These data demonstrate that the overexpression of Fus/Caz causes in vivo toxicity by disrupting neuromuscular junctions (NMJs and inducing apoptosis in motor neurons. In addition, the nuclear localization of Fus is essential for Fus to induce toxicity. Our findings also suggest that Fus overexpression and gene deletion can cause similar degenerative phenotypes but the underlying mechanisms are likely different.

  1. Oseltamivir produces hypothermic and neuromuscular effects by inhibition of nicotinic acetylcholine receptor functions: comparison to procaine and bupropion.

    Science.gov (United States)

    Fukushima, Akihiro; Chazono, Kaori; Hashimoto, Yuichi; Iwajima, Yui; Yamamoto, Shohei; Maeda, Yasuhiro; Ohsawa, Masahiro; Ono, Hideki

    2015-09-05

    Oseltamivir, an anti-influenza virus drug, induces marked hypothermia in normal mice. We have proposed that the hypothermic effect arises from inhibition of the nicotinic acetylcholine receptor function of sympathetic ganglion neurons which innervate the brown adipose tissue (a heat generator). It has been reported that local anesthetics inhibit nicotinic acetylcholine receptor function by acting on its ionic channels, and that bupropion, a nicotinic antagonist, induces hypothermia. In this study, we compared the effects of oseltamivir, procaine and bupropion on body temperature, cardiovascular function and neuromuscular transmission. Intraperitoneal administration of oseltamivir (100mg/kg), procaine (86.6mg/kg) and bupropion (86.7mg/kg) lowered the core body temperature of normal mice. At lower doses (10-30mg/kg oseltamivir, 8.7-26mg/kg procaine and bupropion), when administered subcutaneously, the three drugs antagonized the hypothermia induced by intraperitoneal injection of nicotine (1mg/kg). In anesthetized rats, intravenous oseltamivir (30-100mg/kg), procaine (10mg/kg) and bupropion (10mg/kg) induced hypotension and bradycardia. Oseltamivir alone (100mg/kg) did not inhibit neuromuscular twitch contraction of rats, but at 3-30mg/kg it augmented the muscle-relaxing effect of d-tubocurarine. Similar effects were observed when lower doses of procaine (10-30mg/kg) and bupropion (3-10mg/kg) were administered, suggesting that systemic administration of oseltamivir inhibits muscular nicotinic acetylcholine receptors. These results support the idea that the hypothermic effect of oseltamivir is due to its effects on sympathetic ganglia which innervate the brown adipose tissue, and suggest that oseltamivir may exert non-selective ion channel blocking effects like those of ester-type local anesthetics. Copyright © 2015 Elsevier B.V. All rights reserved.

  2. Turning on the central contribution to contractions evoked by neuromuscular electrical stimulation.

    Science.gov (United States)

    Dean, J C; Yates, L M; Collins, D F

    2007-07-01

    Neuromuscular electrical stimulation can generate contractions through peripheral and central mechanisms. Direct activation of motor axons (peripheral mechanism) recruits motor units in an unnatural order, with fatigable muscle fibers often activated early in contractions. The activation of sensory axons can produce contractions through a central mechanism, providing excitatory synaptic input to spinal neurons that recruit motor units in the natural order. Presently, we quantified the effect of stimulation frequency (10-100 Hz), duration (0.25-2 s of high-frequency bursts, or 20 s of constant-frequency stimulation), and intensity [1-5% maximal voluntary contraction (MVC) torque generated by a brief 100-Hz train] on the torque generated centrally. Electrical stimulation (1-ms pulses) was delivered over the triceps surae in eight subjects, and plantar flexion torque was recorded. Stimulation frequency, duration, and intensity all influenced the magnitude of the central contribution to torque. Central torque did not develop at frequencies or = 80 Hz. Increasing the duration of high-frequency stimulation increased the central contribution to torque, as central torque developed over 11 s. Central torque was greatest at a relatively low contraction intensity. The largest amount of central torque was produced by a 20-s, 100-Hz train (10.7 +/- 5.5 %MVC) and by repeated 2-s bursts of 80- or 100-Hz stimulation (9.2 +/- 4.8 and 10.2 +/- 8.1% MVC, respectively). Therefore, central torque was maximized by applying high-frequency, long-duration stimulation while avoiding antidromic block by stimulating at a relatively low intensity. If, as hypothesized, the central mechanism primarily activates fatigue-resistant muscle fibers, generating muscle contractions through this pathway may improve rehabilitation applications.

  3. Affect attunement in communicative interactions between adults with profound intellectual and multiple disabilities and support workers

    OpenAIRE

    Forster, Sheridan Lee

    2017-01-01

    The quality of life of people with profound intellectual and multiple disabilities (PIMD) is affected by many factors, including health status, involvement in activities, and social networks; but most critical is the quality of interaction experienced by the person on a daily basis. For many people with PIMD, most of whom reside in residential services where they receive 24-hour support, the primary people for interaction are paid disability support workers (DSWs). Quality interaction is ...

  4. Epidemiology of fractures in people with severe and profound developmental disabilities

    Science.gov (United States)

    Glick, N.R.; Fischer, M.H.; Heisey, D.M.; Leverson, G.E.; Mann, D.C.

    2005-01-01

    Fractures are more prevalent among people with severe and profound developmental disabilities than in the general population. In order to characterize the tendency of these people to fracture, and to identify features that may guide the development of preventive strategies, we analyzed fracture epidemiology in people with severe and profound developmental disabilities who lived in a stable environment. Data from a 23-year longitudinal cohort registry of 1434 people with severe and profound developmental disabilities were analyzed to determine the effects of age, gender, mobility, bone fractured, month of fracture, and fracture history upon fracture rates. Eighty-five percent of all fractures involved the extremities. The overall fracture rate increased as mobility increased. In contrast, femoral shaft fracture risk was substantially higher in the least mobile [relative risk (RR), 10.36; 95% confidence interval (CI), 3.29-32.66] compared with the most mobile group. Although the overall fracture rate was not associated with age, the femoral shaft fractures decreased but hand/foot fractures increased with age. Overall fracture risk declined in August and September (RR, 0.70; 95% CI, 0.55-0.89), being especially prominent for tibial/fibular fractures (RR, 0.31; 95% CI, 0.13-0.70). Gender was not a factor in fracture risk. Two primary fracture mechanisms are apparent: one, largely associated with lack of weight-bearing in people with the least mobility, is exemplified by femoral fractures during non-traumatic events as simple as diapering or transfers; the other, probably due to movement- or fall-related trauma, is exemplified by hand/foot fractures in people who ambulate. The fracture experience of people with severe and profound developmental disabilities is unique and, because it differs qualitatively from postmenopausal osteoporosis, may require population-specific methods for assessing risk, for improving bone integrity, and for reduction of falls and accidents

  5. Not all SCN1A epileptic encephalopathies are Dravet syndrome: Early profound Thr226Met phenotype.

    Science.gov (United States)

    Sadleir, Lynette G; Mountier, Emily I; Gill, Deepak; Davis, Suzanne; Joshi, Charuta; DeVile, Catherine; Kurian, Manju A; Mandelstam, Simone; Wirrell, Elaine; Nickels, Katherine C; Murali, Hema R; Carvill, Gemma; Myers, Candace T; Mefford, Heather C; Scheffer, Ingrid E

    2017-09-05

    To define a distinct SCN1A developmental and epileptic encephalopathy with early onset, profound impairment, and movement disorder. A case series of 9 children were identified with a profound developmental and epileptic encephalopathy and SCN1A mutation. We identified 9 children 3 to 12 years of age; 7 were male. Seizure onset was at 6 to 12 weeks with hemiclonic seizures, bilateral tonic-clonic seizures, or spasms. All children had profound developmental impairment and were nonverbal and nonambulatory, and 7 of 9 required a gastrostomy. A hyperkinetic movement disorder occurred in all and was characterized by dystonia and choreoathetosis with prominent oral dyskinesia and onset from 2 to 20 months of age. Eight had a recurrent missense SCN1A mutation, p.Thr226Met. The remaining child had the missense mutation p.Pro1345Ser. The mutation arose de novo in 8 of 9; for the remaining case, the mother was negative and the father was unavailable. Here, we present a phenotype-genotype correlation for SCN1A. We describe a distinct SCN1A phenotype, early infantile SCN1A encephalopathy, which is readily distinguishable from the well-recognized entities of Dravet syndrome and genetic epilepsy with febrile seizures plus. This disorder has an earlier age at onset, profound developmental impairment, and a distinctive hyperkinetic movement disorder, setting it apart from Dravet syndrome. Remarkably, 8 of 9 children had the recurrent missense mutation p.Thr226Met. Copyright © 2017 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

  6. Profound Effects of Population Density on Fitness-Related Traits in an Invasive Freshwater Snail

    OpenAIRE

    Nicholas Zachar; Maurine Neiman

    2013-01-01

    Population density can profoundly influence fitness-related traits and population dynamics, and density dependence plays a key role in many prominent ecological and evolutionary hypotheses. Here, we evaluated how individual-level changes in population density affect growth rate and embryo production early in reproductive maturity in two different asexual lineages of Potamopyrgus antipodarum, a New Zealand freshwater snail that is an important model system for ecotoxicology and the evolution o...

  7. The effects of an environmental "enrichment" program on the behavior of institutionalized profoundly retarded children.

    OpenAIRE

    Horner, R D

    1980-01-01

    This study determined the effects of procedures designed to "enrich" the physical and social environment of an institutional ward on the "adaptive" and "maladaptive" child, adult, self, and object-directed behaviors of five profoundly retarded ambulatory females. Behavior observed in two treatment conditions, an environment "enriched" with toys and objects and an "enriched" environment coupled with differential reinforcement of adaptive behavior, was compared to behavior occurring in correspo...

  8. Speech timing and working memory in profoundly deaf children after cochlear implantation

    OpenAIRE

    Burkholder, Rose A.; Pisoni, David B.

    2003-01-01

    Thirty-seven profoundly deaf children between 8- and 9-years-old with cochlear implants and a comparison group of normal-hearing children were studied to measure speaking rates, digit spans, and speech timing during digit span recall. The deaf children displayed longer sentence durations and pauses during recall and shorter digit spans compared to the normal-hearing children. Articulation rates, measured from sentence durations, were strongly correlated with immediate memory span in both norm...

  9. An epidemiological and genetic study of congenital profound deafness in Tunisia (governorate of Nabeul).

    OpenAIRE

    Ben Arab, S; Bonaïti-Pellié, C.; Belkahia, A

    1990-01-01

    An epidemiological and genetic study of profound deafness has been undertaken in the governorate of Nabeul in Tunisia. This paper deals with sensorineural deafness with no associated abnormalities. The prevalence was estimated to be 0.0007 and four clusters could be identified, two of which represent 51% and 34% respectively of the total number of cases. Segregation analysis performed in 29 pedigrees containing 415 subjects with 129 affected cases provided evidence for simple recessive inheri...

  10. A Vibrotactile Interface to Motivate Movement for Children with Severe to Profound Disabilities

    DEFF Research Database (Denmark)

    Manresa-Yee, Cristina; Morrison, Ann; Larsen, Jeppe Veirum

    2014-01-01

    V-Sense is a vibrotactile interface that encourages children with severe or profound cognitive, sensory and physical impairments to move. The interface makes use of touch, in particular vibrations, as a supportive function to motivate users' actions. Specifically, we propose a vibrotactile...... interface on the arm and around the shoulder using the saltation perceptual illusion to induce movement of the corresponding joint. In this paper we describe the design principles of the interface and the proposed experimental design to evaluate it....

  11. Right bundle branch block

    DEFF Research Database (Denmark)

    Bussink, Barbara E; Holst, Anders Gaarsdal; Jespersen, Lasse

    2013-01-01

    AimsTo determine the prevalence, predictors of newly acquired, and the prognostic value of right bundle branch block (RBBB) and incomplete RBBB (IRBBB) on a resting 12-lead electrocardiogram in men and women from the general population.Methods and resultsWe followed 18 441 participants included.......5%/2.3% in women, P Right bundle branch block was associated with significantly...... increased all-cause and cardiovascular mortality in both genders with age-adjusted hazard ratios (HR) of 1.31 [95% confidence interval (CI), 1.11-1.54] and 1.87 (95% CI, 1.48-2.36) in the gender pooled analysis with little attenuation after multiple adjustment. Right bundle branch block was associated...

  12. Localization training results in individuals with unilateral severe to profound hearing loss.

    Science.gov (United States)

    Firszt, Jill B; Reeder, Ruth M; Dwyer, Noël Y; Burton, Harold; Holden, Laura K

    2015-01-01

    Adults with unilateral hearing loss often demonstrate decreased sound localization ability and report that situations requiring spatial hearing are especially challenging. Few studies have evaluated localization abilities combined with training in this population. The present pilot study examined whether localization of two sound types would improve after training, and explored the relation between localization ability or training benefit and demographic factors. Eleven participants with unilateral severe to profound hearing loss attended five training sessions; localization cues gradually decreased across sessions. Localization ability was assessed pre- and post-training. Assessment stimuli were monosyllabic words and spectral and temporal random spectrogram sounds. Root mean square errors for each participant and stimulus type were used in group and correlation analyses; individual data were examined with ordinary least squares regression. Mean pre-to post-training test results were significantly different for all stimulus types. Among the participants, eight significantly improved following training on at least one localization measure, whereas three did not. Participants with the poorest localization ability improved the most and likewise, those with the best pre-training ability showed the least training benefit. Correlation results suggested that test age, age at onset of severe to profound hearing loss and better ear high frequency audibility may contribute to localization ability. Results support the need for continued investigation of localization training efficacy and consideration of localization training within rehabilitation protocols for individuals with unilateral severe to profound hearing loss. Copyright © 2014 Elsevier B.V. All rights reserved.

  13. Static and Dynamic Balance in Congenital Severe to Profound Hearing-Impaired Children

    Directory of Open Access Journals (Sweden)

    Farideh HajiHeydari

    2011-09-01

    Full Text Available Background and Aim: Research conducted since the early 1900s has consistently identified differences between deaf and hearing children on performance of a wide variety of motor tasks, most notably balance. Our study was performed to test static and dynamic balance skills in congenital severe to profound hearing impaired children in comparison with normal age-matched children.Methods: This cross-sectional study was conducted on 30 severe to profound hearing impaired and 40 normal children with age 6 to 10 years old. Bruininks-Oseretsky test of motor proficiency 2, balance subset with 9 parts was used for evaluation of balance skills.Results: Hearing-impaired children showed 16.7 to 100% fail results in 7 parts of the balance subset. In normal children fail result was revealed just in 3 parts of the balance subset from 2.5 to 57.5%, and differences between two groups were significant (p<0.0001. There was a significant difference between two groups in two static balance skills of standing on one leg on a line and standing on one leg on a balance beam with eyes closed (p<0.0001.conclusion: It seems that development of static balance skills are longer than dynamic ones. Because severe to profound hearing-impaired children showed more weakness than normal children in both static and dynamic balance abilities, functional tests of balance proficiency can help to identify balance disorders in these children.

  14. Frequency compression hearing aid for severe-to-profound hearing impairments.

    Science.gov (United States)

    Sakamoto, S; Goto, K; Tateno, M; Kaga, K

    2000-10-01

    Objective of this study is to know how a frequency compression hearing aid with new concepts is beneficial for severe-to-profound hearing impairments. (2) Clinical trials of this hearing aid were conducted for 11 severe-to-profound hearing impaired listeners. These 11 wore the frequency compression hearing aid in their daily life and reported subjectively on its performance. Speech recognition tests with five listeners and audio-visual short sentence recognition tests with three listeners were also conducted. This hearing aid can separately adjust the fundamental frequency from the spectral envelope of input speech and can adjust frequency response by use of a post-processing digital filter. (3) Five listeners out of these 11 came to prefer this hearing aid in their daily life and are still wearing it. The results of the speech recognition tests show that the speech recognition scores were not improved for all listeners and the results of the audio-visual short sentence recognition tests do that the audio-visual recognition scores were improved for two listeners. (4) There were some severe-to-profound hearing impaired listeners who preferred the frequency compression hearing aid finally. It is also suggested that the benefits of this hearing aid may be evaluated correctly using not only speech but also visual materials.

  15. Transcriptional response of the neuromuscular system to exercise training and potential implications for ALS.

    Science.gov (United States)

    Ferraiuolo, Laura; De Bono, Joseph P; Heath, Paul R; Holden, Hazel; Kasher, Paul; Channon, Keith M; Kirby, Janine; Shaw, Pamela J

    2009-06-01

    The transcriptional adaptive response of motoneurons and muscles to voluntary exercise has been investigated by using laser capture microdissection and microarray analysis. Our results show that motoneurons respond to physical activity by activating a complex transcriptional plan, with changes involved in neurotrophic factor signalling, electrophysiological changes and synaptic reorganization. Gastrocnemius muscle shows increases in transcripts responsible for neovascularization and new myogenesis. Both tissues show transcriptional changes involved in the growth and reinforcement of the neuromuscular junction. This study indicates that the neuromuscular system undergoes significant structural and functional alterations, aiming to optimize the transmission of both chemical and electrical stimuli, thus prompting axonal outgrowth and mechanisms similar to long-term potentiation in hippocampal neurons. Understanding the response of these cells during exercise has potentially important implications for human neuromuscular disease, including amyotrophic lateral sclerosis, by highlighting candidate genes pivotal for the balance between the physiology and the pathology of the neuromuscular system in terms of the stress response to physical exercise.

  16. Incidence and risk factors of prolonged mechanical ventilation in neuromuscular scoliosis surgery

    NARCIS (Netherlands)

    Udink Ten Cate, F.E.A.; van Royen, B.J.; van Heerde, M.; Roerdink, D.; Plotz, F.B.

    2008-01-01

    Patients with neuromuscular scoliosis (NMS) are frequently considered at high risk for postoperative complications based on their underlying disease and comorbidities. Postoperative complications include prolonged mechanical ventilation (MV), defined longer than 72h, at the paediatric intensive care

  17. DNA restriction fragment length polymorphisms in differential diagnosis of genetic disease: application in neuromuscular diseases

    NARCIS (Netherlands)

    Defesche, J. C.; de Vissar, M.; Bakker, E.; Bouwsma, G.; de Vijlder, J. J.; Bolhuis, P. A.

    1989-01-01

    Three families, in which several male individuals suffer from a hereditary neuromuscular disease, were examined by analysis of naturally occurring restriction fragment length polymorphisms (RFLPs) and by screening for deletions. Originally, differential diagnosis included spinal muscular atrophy

  18. Exercise therapy and other types of physical therapy for patients with neuromuscular diseases: a systematic review.

    NARCIS (Netherlands)

    Cup, E.H.C.; Pieterse, A.J.; Broek-Pastoor, J.M. Ten; Munneke, M.; Engelen, B.G.M. van; Hendricks, H.T.; Wilt, G.J. van der; Oostendorp, R.A.B.

    2007-01-01

    OBJECTIVE: To summarize and critically appraise the available evidence on exercise therapy and other types of physical therapies for patients with neuromuscular diseases (NMD). DATA SOURCES: Cochrane Central Register of Controlled Trials and Cochrane Database of Systematic Reviews, Medline, CINAHL,

  19. Neuromuscular electrical stimulation for preventing skeletal-muscle weakness and wasting in critically ill patients

    DEFF Research Database (Denmark)

    Maffiuletti, Nicola A.; Roig, Marc; Karatzanos, Eleftherios

    2013-01-01

    Background: Neuromuscular electrical stimulation (NMES) therapy may be useful in early musculoskeletal rehabilitation during acute critical illness. The objective of this systematic review was to evaluate the effectiveness of NMES for preventing skeletal-muscle weakness and wasting in critically...

  20. Rate of complications due to neuromuscular scoliosis spine surgery in a 30-years consecutive series

    National Research Council Canada - National Science Library

    Turturro, Francesco; Montanaro, Antonello; Calderaro, Cosma; Labianca, Luca; Di Sanzo, Vincenzo; Ferretti, Andrea

    2017-01-01

    ... procedure, or corrupting the final result of the treatment.Of the 358 patients affected by neuromuscular scoliosis treated from January 1985 to December 2010, 185 that met the inclusion criteria were included in the study...

  1. Noninvasive measurement of the tension-time index in children with neuromuscular disease

    National Research Council Canada - National Science Library

    Laura T. Mulreany; Daniel J. Weiner; Joseph M. McDonough; Howard B. Panitch; Julian L. Allen

    2003-01-01

    Respiratory muscle weakness is common in children with neuromuscular disease (NMD). We hypothesized that weakness puts them at risk for respiratory muscle fatigue, a harbinger of chronic respiratory failure...

  2. A Dutch guideline for the treatment of scoliosis in neuromuscular disorders

    NARCIS (Netherlands)

    Mullender, M. G.; Blom, N. A.; de Kleuver, M.; Fock, J. M.; Hitters, W. M. G. C.; Horemans, A. M. C.; Kalkman, C. J.; Pruijs, J. E. H.; Timmer, R. R.; Titarsolej, P. J.; van Haasteren, N. C.; van Tol-de Jager, M. J.; van Vught, A. J.; van Royen, B. J.

    2008-01-01

    ABSTRACT: BACKGROUND: Children with neuromuscular disorders with a progressive muscle weakness such as Duchenne Muscular Dystrophy and Spinal Muscular Atrophy frequently develop a progressive scoliosis. A severe scoliosis compromises respiratory function and makes sitting more difficult. Spinal

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

    OpenAIRE

    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 t...

  4. Relationship between neuromuscular and perceived fatigue and locomotor performance in patients with multiple sclerosis.

    Science.gov (United States)

    Hameau, Sophie; Zory, Raphael; Latrille, Christophe; Roche, Nicolas; Bensmail, Djamel

    2017-12-01

    Fatigue is a common problem in patients with multiple sclerosis; however, the impact of neuromuscular and perceived fatigue on locomotor performance is currently unknown in these patients. The aim of this study was: 1) to determine the relationship between perceived and neuromuscular fatigue and locomotor performance in patients with multiple sclerosis; 2) to determine if neuromuscular and perceived fatigue depend on the severity of the pathology defined by the Expanded disability status scale score (EDSS). Observational study. Outpatients from a hospital rehabilitation department. Thirty patients with multiple sclerosis underwent clinical (spasticity, strength, perceived fatigue and locomotor performance) and isokinetic evaluations (peak torque of knee flexor and extensor muscles and neuromuscular fatigue of knee extensor muscles). The main outcome measures were perceived and neuromuscular fatigue and locomotor performance. Secondary outcomes were strength of the weakest limb and peak isometric torque of knee flexor and extensor muscles, spasticity, EDSS. There were no significant correlations between perceived or neuromuscular fatigue and locomotor performance. Locomotor performance was correlated with EDSS, spasticity, strength of the weakest limb and peak isometric torque of knee flexor and extensor muscles. Eighty‑three percent of the variance of the 6MWT could be explained by EDSS score, strength of the weakest limb (MRCsum), torque produced during the last five contractions of the fatigue protocol and peak isometric torque of knee flexor muscles. Patients with moderate disability (EDSS Score 4-6) had greater levels of neuromuscular fatigue than patients with mild disability (<4). Although fatigue is a disabling symptom of multiple sclerosis, and neuromuscular fatigue was found to be higher in more disabled patients, locomotor performance was correlated with strength and spasticity but not with fatigue. Nevertheless, fatigue was related to 6MWT performance

  5. Physical complications in anorexia nervosa. Haematological and neuromuscular changes in 12 patients.

    Science.gov (United States)

    Alloway, R; Shur, E; Obrecht, R; Russell, G F

    1988-07-01

    Of twelve patients consecutively admitted to the Maudsley Hospital Eating Disorders Unit, four had neuromuscular abnormality, eight haematological abnormality, and four no abnormality. All those having neuromuscular signs had concomitant haematological dysfunction. Vomiting, and food restriction with vegetarianism, appeared more likely to lead to complications than either food restriction alone or laxative abuse. The physical status of severely underweight patients admitted for refeeding needs to be carefully monitored.

  6. Update on the approach of respiratory therapy in patients with neuromuscular diseases

    OpenAIRE

    Priscilla Barreto Paula; Laura Maria de Lima Belizário Facury Lasmar; Maria Teresa Mohallem Fonseca; Marina Belisário Carvalhais; Maria da Glória Rodrigues Machado

    2010-01-01

    Objective: To assess the role of physiotherapy in approaching neuromuscular disease (NMD), with emphasis on preventive and therapeutic aspects of respiratory therapy. Methods: A nonsystematic literature review covering the past twenty years, using the databases MEDLINE and LILACS through the following descriptors: neuromuscular diseases, physical therapy, vital capacity and respiratory failure. Results: The studies reviewed show the need to establish a routine periodic evaluation of respirato...

  7. Research Ethics Committees and the Benefits of Involving People with Profound and Multiple Learning Disabilities in Research

    Science.gov (United States)

    Boxall, Kathy; Ralph, Sue

    2011-01-01

    Although there is increasing interest in service user involvement in research, such involvement rarely extends to people with profound and multiple learning disabilities. New developments in visual methodologies offer the potential for people with profound and multiple learning disabilities to be included in research. At the same time, however,…

  8. How Is Heart Block Treated?

    Science.gov (United States)

    ... defects. Acquired heart block is more common than congenital heart block. Damage to the heart muscle or its electrical system causes acquired heart block. Diseases, surgery, or medicines can cause this damage. The three types of heart block are first degree, second degree, ...

  9. Evaluation of neuromuscular activity in patients with obstructive sleep apnea using chin surface electromyography of polysomnography.

    Science.gov (United States)

    Yin, Guo-ping; Ye, Jing-ying; Han, De-min; Wang, Xiao-yi; Zhang, Yu-huan; Li, Yan-ru

    2013-01-01

    It is believed that defects in upper airway neuromuscular control play a role in sleep apnea pathogenesis. Currently, there is no simple and non-invasive method for evaluating neuromuscular activity for the purpose of screening in patients with obstructive sleep apnea. This study was designed to assess the validity of chin surface electromyography of routine polysomnography in evaluating the neuromuscular activity of obstructive sleep apnea subjects and probe the neuromuscular contribution in the pathogenesis of the condition. The chin surface electromyography of routine polysomnography during normal breathing and obstructive apnea were quantified in 36 male patients with obstructive sleep apnea. The change of chin surface electromyography from normal breathing to obstructive apnea was expressed as the percent compensated electromyography value, where the percent compensated electromyography value = (normal breath surface electromyography - apnea surface electromyography)/normal breath surface electromyography, and the percent compensated electromyography values among subjects were compared. The relationship between sleep apnea related parameters and the percent compensated electromyography value was examined. The percent compensated electromyography value of the subjects varied from 1% to 90% and had a significant positive correlation with apnea hypopnea index (R(2) = 0.382, P electromyography by routine polysomnography is a valid way of screening the neuromuscular activity in patients with obstructive sleep apnea. The neuromuscular contribution is different among subjects with obstructive sleep apnea.

  10. Sleep monitoring in children during neuromuscular blockade in the pediatric intensive care unit: a pilot study.

    Science.gov (United States)

    Carno, Margaret-Ann; Hoffman, Leslie A; Henker, Richard; Carcillo, Joseph; Sanders, Mark H

    2004-05-01

    Sleep is an important physiologic process that is known to be disrupted in the intensive care unit. Nevertheless, there is little information on how intensive care unit admission affects sleep in children. Because laryngotracheoplasty is elective but entails 5-7 days of neuromuscular blockade following surgery, children undergoing this procedure present a unique opportunity to analyze sleep during neuromuscular blockade apart from confounding variables resulting from critical illness. To determine the feasibility of using polysomnography to assess sleep patterns in children during neuromuscular blockade. Polysomnography recordings were obtained continuously for 4 days (96 hrs) in two children following laryngotracheoplasty. Medication administration (neuromuscular blockades, sedatives) and time of suctioning were also recorded. Both subjects had documented sleep. However, the proportion of time in each stage was markedly different from developmental norms, and a greater proportion of sleep occurred during the day. Furthermore, there was substantial day-night and day-to-day variability. Some rebound of consolidated sleep appeared by day 4. Sedative use varied considerably. However, neither bolus sedation administration nor endotracheal suctioning appeared to affect sleep. Few monitoring difficulties were encountered. Sleep can be monitored with minimal difficulty in children undergoing neuromuscular blockade in the pediatric intensive care unit. Sleep occurred throughout the day, and there was considerable fragmentation. To fully assess sleep in the intensive care unit, monitoring needs to be continuous over several days, rather than only at night or for < or =24 hrs. Further research is needed in the area to determine typical sleep patterns in children undergoing neuromuscular blockade.

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

    Science.gov (United States)

    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.

  12. Effects of Block Scheduling

    Directory of Open Access Journals (Sweden)

    William R. Veal

    1999-09-01

    Full Text Available This study examined the effects of a tri-schedule on the academic achievement of students in a high school. The tri-schedule consists of traditional, 4x4 block, and hybrid schedules running at the same time in the same high school. Effectiveness of the schedules was determined from the state mandated test of basic skills in reading, language, and mathematics. Students who were in a particular schedule their freshman year were tested at the beginning of their sophomore year. A statistical ANCOVA test was performed using the schedule types as independent variables and cognitive skill index and GPA as covariates. For reading and language, there was no statistically significant difference in test results. There was a statistical difference mathematics-computation. Block mathematics is an ideal format for obtaining more credits in mathematics, but the block format does little for mathematics achievement and conceptual understanding. The results have content specific implications for schools, administrations, and school boards who are considering block scheduling adoption.

  13. Ischemic Nerve Block.

    Science.gov (United States)

    Williams, Ian D.

    This experiment investigated the capability for movement and muscle spindle function at successive stages during the development of ischemic nerve block (INB) by pressure cuff. Two male subjects were observed under six randomly ordered conditions. The duration of index finger oscillation to exhaustion, paced at 1.2Hz., was observed on separate…

  14. Rehabilitation interventions for foot drop in neuromuscular disease.

    Science.gov (United States)

    Sackley, Catherine; Disler, Peter B; Turner-Stokes, Lynne; Wade, Derick T; Brittle, Nicola; Hoppitt, Thomas

    2009-07-08

    "Foot drop" or "Floppy foot drop" is the term commonly used to describe weakness or contracture of the muscles around the ankle joint. It may arise from many neuromuscular diseases. To conduct a systematic review of randomised trials for the treatment of foot drop resulting from neuromuscular disease. In this update, we searched the Cochrane Neuromuscular Disease Group Trials Register (April 2009), MEDLINE (January 1966 to April 24 2009), EMBASE January 1980 to April 24 2009), CINAHL (January 1982 to May 6 2009), AMED (January 1985 to April 24 2009), the British Nursing Index (January 1985 to January 2008) and Royal College of Nursing Journal of Databases (January 1985 to January 2008). Randomised and quasi-randomised trials of physical, orthotic and surgical treatments for foot drop resulting from lower motor neuron or muscle disease and related contractures were included. People with primary joint disease were excluded. Interventions included a 'wait and see' approach, physiotherapy, orthoses, surgery and pharmacological therapy. The primary outcome measure was quantified ability to walk whilst secondary outcome measures included range of movement, dorsiflexor torque and strength, measures of activity and participation, quality of life and adverse effects. Methodological quality was evaluated by two authors using the van Tulder criteria. Four studies with a total of n = 152 participants were included in the update to the original review. Heterogeneity of the studies precluded pooling the data. Early surgery did not significantly affect walking speed in a trial including 20 children with Duchenne muscular dystrophy. Both groups deteriorated during the 12 months follow-up. After one year, the mean difference (MD) of the 28 feet walking time was 0.00 seconds (95% confidence interval (CI) -0.83 to 0.83) and the MD of the 150 feet walking time was -2.88 seconds, favouring the control group (95% CI -8.18 to 2.42). Night splinting of the ankle did not significantly

  15. Lumbopelvic neuromuscular training and injury rehabilitation: a systematic review.

    Science.gov (United States)

    Briggs, Matthew S; Givens, Deborah L; Best, Thomas M; Chaudhari, Ajit M

    2013-05-01

    The aim of this systematic review was to assess the evidence for lumbopelvic neuromuscular training (LNMT) in individuals after musculoskeletal (MSK) injury. A literature search of PubMed and EMBASE databases was performed for English studies from January 1990 to March 2012. Search terms including and related to trunk, core, stability, injury, and LNMT were used. All studies directly involving LNMT for MSK injuries were reviewed by 2 authors. These articles were assessed based on the inclusion criteria and if appropriate selected for further analysis. Expert opinion, review articles, and articles involving non-MSK injuries were excluded. Four authors then scored the selected articles for methodological quality. A total of 2312 articles were initially identified. Twenty-nine articles met the inclusion criteria for review and were divided into categories of lower extremity (LE), lumbar, and upper extremity (UE). No trials involving the UE met the inclusion criteria. Data including subject demographics (age, height, weight, gender, etc), injury type, intervention type, and outcome measurements were extracted from the relevant articles. A variety of baseline and follow-up scores were extracted including pain levels, patient satisfaction, disability questionnaires, and other functional outcomes. Two out of 3 LE randomized controlled trials (RCTs) and 9/26 lumbar RCTs were rated with high methodological quality based on the scoring system described by van Tulder et al. The average quality score for the LE RCTs was 6.3 (range = 4-9) and for the lumbar RCTs was 5.1 (range = 2-9). The evidence for the effectiveness of the 3 LE studies was rated as conflicting, whereas 24 lumbar studies demonstrated moderate-to-strong evidence. Unfortunately, heterogeneity of populations, interventions, and outcomes precluded a quantitative meta-analysis and specific clinical recommendations. High-quality evidence is lacking to make specific clinical recommendations for or against the use of

  16. Deficits in Endogenous Adenosine Formation by Ecto-5′-Nucleotidase/CD73 Impair Neuromuscular Transmission and Immune Competence in Experimental Autoimmune Myasthenia Gravis

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    Laura Oliveira

    2015-01-01

    Full Text Available AMP dephosphorylation via ecto-5′-nucleotidase/CD73 is the rate limiting step to generate extracellular adenosine (ADO from released adenine nucleotides. ADO, via A2A receptors (A2ARs, is a potent modulator of neuromuscular and immunological responses. The pivotal role of ecto-5′-nucleotidase/CD73, in controlling extracellular ADO formation, prompted us to investigate its role in a rat model of experimental autoimmune myasthenia gravis (EAMG. Results show that CD4+CD25+FoxP3+ regulatory T cells express lower amounts of ecto-5′-nucleotidase/CD73 as compared to controls. Reduction of endogenous ADO formation might explain why proliferation of CD4+ T cells failed upon blocking A2A receptors activation with ZM241385 or adenosine deaminase in EAMG animals. Deficits in ADO also contribute to neuromuscular transmission failure in EAMG rats. Rehabilitation of A2AR-mediated immune suppression and facilitation of transmitter release were observed by incubating the cells with the nucleoside precursor, AMP. These findings, together with the characteristic increase in serum adenosine deaminase activity of MG patients, strengthen our hypothesis that the adenosinergic pathway may be dysfunctional in EAMG. Given that endogenous ADO formation is balanced by ecto-5′-nucleotidase/CD73 activity and that A2ARs exert a dual role to restore use-dependent neurocompetence and immune suppression in myasthenics, we hypothesize that stimulation of the two mechanisms may have therapeutic potential in MG.

  17. Maturation of the mitochondrial redox response to profound asphyxia in fetal sheep.

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    Paul P Drury

    Full Text Available Fetal susceptibility to hypoxic brain injury increases over the last third of gestation. This study examined the hypothesis that this is associated with impaired mitochondrial adaptation, as measured by more rapid oxidation of cytochrome oxidase (CytOx during profound asphyxia.Chronically instrumented fetal sheep at 0.6, 0.7, and 0.85 gestation were subjected to either 30 min (0.6 gestational age (ga, n = 6, 25 min (0.7 ga, n = 27 or 15 min (0.85 ga, n = 17 of complete umbilical cord occlusion. Fetal EEG, cerebral impedance (to measure brain swelling and near-infrared spectroscopy-derived intra-cerebral oxygenation (ΔHb = HbO(2 - Hb, total hemoglobin (THb and CytOx redox state were monitored continuously. Occlusion was associated with profound, rapid fall in ΔHb in all groups to a plateau from 6 min, greatest at 0.85 ga compared to 0.6 and 0.7 ga (p<0.05. THb initially increased at all ages, with the greatest rise at 0.85 ga (p<0.05, followed by a progressive fall from 7 min in all groups. CytOx initially increased in all groups with the greatest rise at 0.85 ga (p<0.05, followed by a further, delayed increase in preterm fetuses, but a striking fall in the 0.85 group after 6 min of occlusion. Cerebral impedance (a measure of cytotoxic edema increased earlier and more rapidly with greater gestation. In conclusion, the more rapid rise in CytOx and cortical impedance during profound asphyxia with greater maturation is consistent with increasing dependence on oxidative metabolism leading to earlier onset of neural energy failure before the onset of systemic hypotension.

  18. Effect of profound normovolemic hypotension and moderate hypothermia on circulating cytokines and adhesion molecules.

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    Hansen, D; Bogatzki, S; Syben, R; Bechrakis, N E; Dopjans, D; Spies, C; Welte, M; Schaffartzik, W

    1999-11-01

    Hypotension caused by hypovolemic, hemorrhagic shock induces disturbances in the immune system that may contribute to an increased susceptibility to sepsis. The effect of chemically induced hypotension on circulating cytokines and adhesion molecules has not been investigated yet. In 21 patients scheduled for resection of malignant choroidal melanoma of the eye the perioperative serum levels of the cytokines IL-1beta, IL-6, IL-10, TNF-alpha, and the adhesion molecules sE-Selectin and sICAM-1 were investigated. Moderate hypothermia of 32 degrees C was induced in all patients. In 14 patients profound hypotension (mean arterial blood pressure 35-40 mmHg, hypotension group) was induced by enalapril and nitroglycerin for a mean duration of 71 min. In 7 patients the tumor was not resectable, and hypotension was not induced (controls). We did not detect significant differences in serum levels of cytokines or sE-Selectin perioperatively in patients with profound hypotension compared with controls. In both groups IL-6 serum levels increased significantly and reached a maximum after rewarming (17 +/- 6 and 16 +/- 5 pg/dL, respectively, P < 0.001). IL-1beta, IL-10, and TNF-alpha did not change perioperatively in both groups. On the first postoperative day sICAM-1 serum levels were significantly increased in both groups (mean increase of 96 and 54 ng/mL, respectively, P < 0.01 and P < 0.05). We conclude from this study that profound normovolemic arterial hypotension does not seem to have effects on serum levels of circulating IL-1beta, IL-6, IL-10, TNF-alpha, and sE-Selectin. Perioperative moderate hypothermia may be the reason for the postoperative increase in sICAM-1 levels independent of the blood pressure.

  19. Acute profound thrombocytopenia associated with readministration of eptifibatide: case report and review of the literature.

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    Russell, Kimberly N; Schnabel, Joseph G; Rochetto, Richard P; Tanner, Matthew C

    2009-07-01

    The glycoprotein IIb-IIIa inhibitor eptifibatide has been shown to be beneficial in the treatment of acute coronary syndromes and during percutaneous coronary intervention (PCI). Case reports of acute profound thrombocytopenia have been reported with eptifibatide, yet the true incidence of this reaction is unknown. We describe a 50-year-old woman with severe coronary artery disease who developed acute profound thrombocytopenia after readministration of eptifibatide. Eptifibatide was administered through hospital day 3, when it was discontinued in preparation for coronary angiography and PCI; the drug was restarted on day 5. On hospital day 6, she was noted to have a platelet count below 5 x 10(3)/mm,(3) indicating a profound decrease from a baseline of 456 x 10(3)/mm(3) on admission. Eptifibatide, heparin, vancomycin, and clopidogrel were potential causative agents. Anticoagulation and vancomycin were stopped, and her platelet count increased to 30 x 10(3)/mm(3) on day 7. Subsequent reexposure to heparin and vancomycin yielded no adverse effects. The patient's platelet count increased over the remainder of her hospitalization, and she was discharged home on day 19. Based on clinical presentation and negative heparin platelet factor 4 antibody test, eptifibatide was the most likely cause of thrombocytopenia. Use of the Naranjo adverse drug reaction probability scale indicated that eptifibatide was the probable cause of thrombocytopenia (score of 5); scores of 1 (possible) or 0 (doubtful) were derived with heparin, vancomycin, and clopidogrel. We conducted a literature search and compiled information from published case reports to describe the pattern of onset and recovery of eptifibatide-induced thrombocytopenia. In all patients receiving eptifibatide, routine platelet counts should be monitored at baseline and within 2-6 hours after starting the drug.

  20. The Differential Effects of Attentional Focus in Children with Moderate and Profound Visual Impairments

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    Scott W. T. McNamara

    2017-10-01

    Full Text Available It has been consistently reported that an external focus of attention leads to better motor performance than an internal focus, but no research to date has explored this effect in a population with visual impairments (VI. External focus statements typically reference something in the environment (e.g., target that may be difficult to conceptualize for people with VI since they cannot generate a visual representation of the object of focus. Internal focus statements could be more closely identifiable with proprioception that is not impaired in this population. Recent studies have reported that sighted adults with temporarily obstructed vision are able to receive an external focus benefit when performing discrete tasks (i.e., golf putt and vertical jump, however, it is unclear if those with VI would experience the same benefit. The purpose of this investigation was to compare how an internal focus and external focus impact the balance of children with VI. Eighteen children with VI were grouped into a moderate (n = 11 and a profound VI group (n = 7. Participants completed a familiarization trial, an internal focus trial (i.e., focusing on feet and an external focus trial (i.e., focusing on markers in a counterbalanced order. The moderate VI group had a lower root mean square error while using an external focus (p = 0.04, while the profound VI group did not differ between conditions (p > 0.05. These results suggest that while performing a task reliant on sensory feedback, an external focus benefit may be dependent on the severity of VI. Further research is needed to examine whether external focus statements can be presented in a way that may be more intuitive to those with profound VI. These findings may help to influence how professionals in health-related fields (e.g., physical therapist and physical educators give instructions on motor performance to populations with VI.

  1. Neuromuscular strategies contributing to faster multidirectional agility performance.

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    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.

  2. Lola regulates glutamate receptor expression at the Drosophila neuromuscular junction

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    Ai Fukui

    2012-02-01

    Communication between pre- and post-synaptic cells is a key process in the development and modulation of synapses. Reciprocal induction between pre- and postsynaptic cells involves regulation of gene transcription, yet the underlying genetic program remains largely unknown. To investigate how innervation-dependent gene expression in postsynaptic cells supports synaptic differentiation, we performed comparative microarray analysis of Drosophila muscles before and after innervation, and of prospero mutants, which show a delay in motor axon outgrowth. We identified 84 candidate genes that are potentially up- or downregulated in response to innervation. By systematic functional analysis, we found that one of the downregulated genes, longitudinals lacking (lola, which encodes a BTB-Zn-finger transcription factor, is required for proper expression of glutamate receptors. When the function of lola was knocked down in muscles by RNAi, the abundance of glutamate receptors (GluRs, GluRIIA, GluRIIB and GluRIII, as well as that of p-21 activated kinase (PAK, was greatly reduced at the neuromuscular junctions (NMJs. Recordings of the synaptic response revealed a decrease in postsynaptic quantal size, consistent with the reduction in GluR levels. Lola appears to regulate the expression of GluRs and PAK at the level of transcription, because the amount of mRNAs encoding these molecules was also reduced in the mutants. The transcriptional level of lola, in turn, is downregulated by increased neural activity. We propose that Lola coordinates expression of multiple postsynaptic components by transcriptional regulation.

  3. Lower Limb Neuromuscular Asymmetry in Volleyball and Basketball Players.

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    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 (pplayers. When comparing between sexes, significant differences (pjump 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.

  4. Homeostatic synaptic plasticity at the neuromuscular junction in myasthenia gravis.

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    Wang, Xueyong; Rich, Mark M

    2018-01-01

    A number of studies in the past 20 years have shown that perturbation of activity of the nervous system leads to compensatory changes in synaptic strength that serve to return network activity to its original level. This response has been termed homeostatic synaptic plasticity. Despite the intense interest in homeostatic synaptic plasticity, little attention has been paid to its role in the prototypic synaptic disease, myasthenia gravis. In this review, we discuss mechanisms that have been shown to mediate homeostatic synaptic plasticity at the mammalian neuromuscular junction. A subset of these mechanisms have been shown to occur in myasthenia gravis. The homeostatic changes occurring in myasthenia gravis appear to involve the presynaptic nerve terminal and may even involve changes in the excitability of motor neurons within the spinal cord. The finding of presynaptic homeostatic synaptic plasticity in myasthenia gravis leads us to propose that changes in the motor unit in myasthenia gravis may be more widespread than previously appreciated. © 2017 New York Academy of Sciences.

  5. Neuromuscular quadriceps dysfunction prior to osteoarthritis of the knee.

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    Becker, Roland; Berth, Alexander; Nehring, Markus; Awiszus, Friedemann

    2004-07-01

    Decreased maximal quadriceps strength and voluntary activation has been observed in patients with osteoarthritis in previous studies, but those results do not allow any conclusions to be drawn as to whether quadriceps dysfunction precedes or follows osteoarthritis. Thirty-two patients (group a) who underwent partial meniscectomy 48+/-9 month prior to the study were matched according to their weight and body mass index with a control group (group b). The twitch interpolation technique was used to determine maximal voluntary contraction (MVC) and voluntary activation (VA) of the quadriceps muscle of both legs. Subjective assessment of the knee was performed using the Lysholm-Score. AP and lateral X-rays of the operated knee were obtained. None of the participants showed any evidence of characteristic radiological or clinical signs for osteoarthritis. A significantly lower MVC was noticed in both the affected and the contralateral knee of group a in comparison to group b (p weakness as already described in patients with manifest osteoarthritis. The authors hypothesise that muscle dysfunction may be an etiologic factor underlying the pathologic changes of osteoarthritis. Whether muscle dysfunction occurs also at other sites, e.g. in the upper extremity, remains unclear but would be of interest in order to detect a generalized neuromuscular dysfunction.

  6. Extracardiac medical and neuromuscular implications in restrictive cardiomyopathy.

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    Stöllberger, Claudia; Finsterer, Josef

    2007-08-01

    Restrictive cardiomyopathy (RCMP) is characterized by restrictive filling and reduced diastolic volume of either or both ventricles with normal or near-normal systolic function and wall thickness. It may occur idiopathically or as a cardiac manifestation of systemic diseases such as scleroderma, amyloidosis, Churg-Strauss syndrome, cystinosis, sarcoidosis, lymphoma, Gaucher's disease, hemochromatosis, Fabry's disease, pseudoxanthoma elasticum, hypereosinophilic syndrome, carcinoid, Noonan's syndrome, reactive arthritis, or Werner's syndrome and various neuromuscular disorders. Whereas in idiopathic RCMP the therapeutic options are only treatment of cardiac congestion, in cases with an underlying disorder, a causal therapy may be available. Patients with RCMP should be investigated as soon as the cardiac diagnosis is established for extracardiac diseases to detect a possibly treatable cause of RCMP before the disease becomes intractable. These investigations include a diligent clinical history and examination, blood tests, and ophthalmologic, otologic, dermatologic, gastroenterologic, nephrologic, hematologic, and neurologic examinations. If extracardiac examinations do not reveal a plausible cause for RCMP, endomyocardial biopsy is indicated. (c) 2007 Wiley Periodicals, Inc.

  7. HEMODYNAMIC AND LACTIC ACID RESPONSES TO PROPRIOCEPTIVE NEUROMUSCULAR FACILITATION EXERCISE

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    Zuhal Gültekin

    2006-09-01

    Full Text Available The hemodynamic and metabolic responses to proprioceptive neuromuscular facilitation (PNF exercise were examined in 32 male university students (aged 19-28 years. Ten repetitions of PNF exercises were applied to the subjects' dominant upper extremities in the following order: as an agonist pattern flexion, adduction and external rotation; and as an antagonist pattern extension, abduction and internal rotation. Heart rate (HR, systolic blood pressure (SBP, diastolic blood pressure (DBP, double product (DP, and blood lactate concentration (La were determined before, immediately after, and at 1st, 3rd, and 5th minutes after PNF exercise. A one-way ANOVA with repeated measures indicated significant differences in HR, SBP, DBP, DP and La immediately after PNF exercise. HR increased from 81 (±10 to 108 (±15 b·min-1 (p < 0.01, SBP increased from 117 (±10 to 125 (±11 mmHg (p < 0.01, DBP increased from 71 (±10 to 75 (±8 mmHg (p < 0.01, DP increased from 96 (±16 to 135 (±24 (p < 0.01, and La increased from 0.69 (±0.31 to 3.99 (±14.63 mmol·L-1 (p < 0.01. Thus PNF exercise resulted in increased hemodynamic responses and blood lactate concentration that indicate a high strain on the cardiovascular system and anaerobic metabolism in healthy subjects

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

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    Mayana Zatz

    Full Text Available 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.

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

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    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.

  10. Neuromuscular transmission and muscle fatigue changes by nanostructured oxygen.

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    Ivannikov, Maxim V; Sugimori, Mutsuyuki; Llinás, Rodolfo R

    2017-04-01

    Oxygen (O 2 ) nanobubbles offer a new method for tissue oxygenation. The effects of O 2 nanobubbles on transmission at neuromuscular junctions (NMJs) and muscle function were explored in murine diaphragm. Electrophysiological parameters, NMJ ultrastructure, muscle force, and muscle fatigue were studied during superfusion with solutions with different oxygen levels or oxygen nanobubbles. High frequency nerve stimulation of muscles superfused with O 2 nanobubble solution slowed neurotransmission decline over those with either control or hyperoxic solution. O 2 nanobubble solution increased the amplitude of evoked end plate potentials and quantal content but did not affect spontaneous activity. Electron microscopy of stimulated O 2 nanobubble treated NMJs showed accumulation of large synaptic vesicles and endosome-like structures. O 2 nanobubble solution had no effects on isometric muscle force, but it significantly decreased fatigability and maximum force recovery time in nerve stimulated muscles. O 2 nanobubbles increase neurotransmission and reduce the probability of neurotransmission failure in muscle fatigue. Muscle Nerve 55: 555-563, 2017. © 2016 Wiley Periodicals, Inc.

  11. Neuromuscular adjustments of the quadriceps muscle after repeated cycling sprints.

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    Olivier Girard

    Full Text Available PURPOSE: This study investigated the supraspinal processes of fatigue of the quadriceps muscle in response to repeated cycling sprints. METHODS: Twelve active individuals performed 10 × 6-s "all-out" sprints on a cycle ergometer (recovery = 30 s, followed 6 min later by 5 × 6-s sprints (recovery = 30 s. Transcranial magnetic and electrical femoral nerve stimulations during brief (5-s and sustained (30-s isometric contractions of the knee extensors were performed before and 3 min post-exercise. RESULTS: Maximal strength of the knee extensors decreased during brief and sustained contractions (~11% and 9%, respectively; P0.05. While cortical voluntary activation declined (P 40% reduced (P<0.001 following exercise. CONCLUSION: The capacity of the motor cortex to optimally drive the knee extensors following a repeated-sprint test was shown in sustained, but not brief, maximal isometric contractions. Additionally, peripheral factors were largely involved in the exercise-induced impairment in neuromuscular function, while corticospinal excitability was well-preserved.

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

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    Simone Pittaccio

    2015-05-01

    Full Text Available 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.

  13. Neuromuscular factors related to success in Olympic wrestling

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    Alfonso Martínez-Moreno

    2011-03-01

    Full Text Available AbstractThis study was undertaken to determine the relationship between maximum dynamic and isometric strength and success in male and female Olympic wrestling. Thirty-five female and thirty-seven male wrestlers were assigned into 4 groups according to their gender and competitive level: men elite (♂ ET, n = 18 and amateur (AT ♂, n = 19 and female elite (♀ ET n = 13 and amateur (AT ♀, n = 22. All subjects underwent assessments of body composition, countermovement jump, maximum dynamic strength test in full squat and bench press exercises and maximum isometric strength test of grip and hip extension. All the neuromuscular markers studied showed significantly higher values in the two elite groups compared to their respective amateur groups results, except the jump height between ♀ET y ♀AT, where no significant differences were detected. The present results suggest that the higher maximum isometric and dynamic strength values, explained in part by the differences in lean mass, will give elite wrestlers a clear advantage during the most frequently used techniques in Olympic wrestling. Key  Words: bench press; squat; maximum dynamic strength; maximum isometric strength; combat.

  14. Neuromuscular factors related to success in Olympic wrestling

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    Alfonso Martínez-Moreno

    2011-03-01

    Full Text Available Abstract This study was undertaken to determine the relationship between maximum dynamic and isometric strength and success in male and female Olympic wrestling. Thirty-five female and thirty-seven male wrestlers were assigned into 4 groups according to their gender and competitive level: men elite (♂ ET, n = 18 and amateur (AT ♂, n = 19 and female elite (♀ ET n = 13 and amateur (AT ♀, n = 22. All subjects underwent assessments of body composition, countermovement jump, maximum dynamic strength test in full squat and bench press exercises and maximum isometric strength test of grip and hip extension. All the neuromuscular markers studied showed significantly higher values in the two elite groups compared to their respective amateur groups results, except the jump height between ♀ET y ♀AT, where no significant differences were detected. The present results suggest that the higher maximum isometric and dynamic strength values, explained in part by the differences in lean mass, will give elite wrestlers a clear advantage during the most frequently used techniques in Olympic wrestling.  Key  Words: bench press; squat; maximum dynamic strength; maximum isometric strength; combat.

  15. Oxidative stress induces overgrowth of the Drosophila neuromuscular junction.

    Science.gov (United States)

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

    2011-10-18

    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.

  16. An Innovative Ergometer to Measure Neuromuscular Fatigue Immediately after Cycling.

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    Doyle-Baker, Douglas; Temesi, John; Medysky, Mary E; Holash, Robert J; Millet, Guillaume Y

    2018-02-01

    When assessing neuromuscular fatigue (NMF) from dynamic exercise using large muscle mass (e.g., cycling), most studies have delayed measurement for 1 to 3 min after task failure. This study aimed to determine the reliability of an innovative cycling ergometer permitting the start of fatigue measurement within 1 s after cycling. Twelve subjects participated in two experimental sessions. Knee-extensor NMF was assessed by electrical nerve and transcranial magnetic stimulation with both a traditional chair setup (PRE- and POST-Chair, 2 min postexercise) and the new cycling ergometer (PRE, every 3 min during incremental exercise and POST-Bike, at task failure). The reduction in maximal voluntary contraction force POST-Bike (63% ± 12% PRE; P cycling protocol (P > 0.05). Vastus lateralis and rectus femoris M-wave and motor-evoked potential areas showed fair to excellent reliability (ICC, 0.45-0.88). The reduction in maximal voluntary contraction and Db100 was greater on the cycling ergometer than the isometric chair. The innovative cycling ergometer is a reliable tool to assess NMF during and immediately postexercise. This will allow fatigue etiology during dynamic exercise with large muscle mass to be revisited in various populations and environmental conditions.

  17. Peripheral nerve and neuromuscular junction pathology in Pompe disease

    Science.gov (United States)

    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

  18. Spider Silk Violin Strings with a Unique Packing Structure Generate a Soft and Profound Timbre

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    Osaki, Shigeyoshi

    2012-04-01

    We overcome the difficulties in pulling long draglines from spiders, twist bundles of dragline filaments, and succeed in preparing violin strings. The twisting is found to change the cross section shapes of filaments from circular to polygonal and to optimize the packing structure with no openings among filaments providing mechanically strong and elastic strings. The spider string signal peaks of overtones for the violin are relatively large at high frequencies, generating a soft and profound timbre. Such a preferable timbre is considered to be due to the unique polygonal packing structure which provides valuable knowledge for developing new types of materials.

  19. A profound case of neurally mediated syncope with asystole after septoplasty.

    Science.gov (United States)

    Ruhl, Douglas S; Ramsey, Mitchell J; Ruffin, David M

    2012-06-01

    Vasovagal syncope (VVS) is an alarming yet benign condition that may present postoperatively for the first time in otherwise healthy patients. Although VVS is associated anecdotally with nasal manipulation, no data have been found to quantify this incidence with otolaryngology surgeries. We present a case of profound, recurrent syncope and documented asystole with an initial diagnosis of glossopharyngeal neuralgia. We conclude with a discussion of neurally mediated syncope particular to the perioperative setting. It is essential to recognize neurocardiogenic etiology to differentiate it from other more concerning causes of syncope and asystole. Published by Elsevier Inc.

  20. Developmental dysgraphia with profound hearing impairment: intervention by auditory methods enabled by cochlear implant.

    Science.gov (United States)

    Fukushima, Kunihiro; Kawasaki, Akihiro; Nagayasu, Rie; Kunisue, Kazuya; Maeda, Yukihide; Kariya, Shin; Kataoka, Yuko; Nishizaki, Kazunori

    2008-06-01

    Learning disability combined with hearing impairment (LDHI) is a poor prognostic factor for the language development of hearing impaired children after educational intervention. A typical example of a child with LDHI and effective interventions provided by cochlear implants are presented in this report. A case of congenital cytomegaloviral infection that showed dysgraphia as well as profound deafness was reported and an underlying visual processing problem diagnosed in the present case caused the patient's dysgraphia. The dysgraphia could be circumvented by the use of auditory memory fairly established by a cochlear implant.

  1. Lemierre’s Syndrome Associated with Mechanical Ventilation and Profound Deafness

    Directory of Open Access Journals (Sweden)

    Lukas Birkner

    2017-01-01

    Full Text Available Lemierre’s syndrome is a rare disorder that is characterized by anaerobic organisms inducing a thrombophlebitis of the internal jugular vein (IJV following a course of oropharyngeal infection. It often occurs in young and healthy patients. Clinicians continuously misinterpret early symptoms until infection disseminates systematically and life-threatening sepsis transpires. We report the case of a 58-year-old female developing Lemierre’s syndrome accompanied by invasive ventilation support and a profound deafness requiring the implementation of a cochlear implant. This is one of two reported cases of Lemierre’s syndrome associated with mechanical ventilation support and the only case associated with a cochlear implant.

  2. SUPERFICIAL CERVICAL PLEXUS BLOCK

    Directory of Open Access Journals (Sweden)

    Komang Mega Puspadisari

    2014-01-01

    Full Text Available Superficial cervical plexus block is one of the regional anesthesia in  neck were limited to thesuperficial fascia. Anesthesia is used to relieve pain caused either during or after the surgery iscompleted. This technique can be done by landmark or with ultrasound guiding. The midpointof posterior border of the Sternocleidomastoid was identified and the prosedure done on thatplace or on the level of cartilage cricoid.

  3. Acute effect of scapular proprioceptive neuromuscular facilitation (PNF) techniques and classic exercises in adhesive capsulitis: a randomized controlled trial

    National Research Council Canada - National Science Library

    Balcı, Nilay Comuk; Yuruk, Zeliha Ozlem; Zeybek, Aslican; Gulsen, Mustafa; Tekindal, Mustafa Agah

    2016-01-01

    [Purpose] The aim of our study was to compare the initial effects of scapular proprioceptive neuromuscular facilitation techniques and classic exercise interventions with physiotherapy modalities on pain...

  4. Hybrid Neuroprosthesis for the Upper Limb: Combining Brain-Controlled Neuromuscular Stimulation with a Multi-Joint Arm Exoskeleton

    National Research Council Canada - National Science Library

    Grimm, Florian; Walter, Armin; Spüler, Martin; Naros, Georgios; Rosenstiel, Wolfgang; Gharabaghi, Alireza

    2016-01-01

    ...) and the intensity of training. Furthermore, neuromuscular electrical stimulation (NMES) is applied in neurologically impaired patients to restore muscle strength by closing the sensorimotor loop...

  5. E-Block: A Tangible Programming Tool with Graphical Blocks

    Directory of Open Access Journals (Sweden)

    Danli Wang

    2013-01-01

    Full Text Available This paper designs a tangible programming tool, E-Block, for children aged 5 to 9 to experience the preliminary understanding of programming by building blocks. With embedded artificial intelligence, the tool defines the programming blocks with the sensors as the input and enables children to write programs to complete the tasks in the computer. The symbol on the programming block's surface is used to help children understanding the function of each block. The sequence information is transferred to computer by microcomputers and then translated into semantic information. The system applies wireless and infrared technologies and provides user with feedbacks on both screen and programming blocks. Preliminary user studies using observation and user interview methods are shown for E-Block's prototype. The test results prove that E-Block is attractive to children and easy to learn and use. The project also highlights potential advantages of using single chip microcomputer (SCM technology to develop tangible programming tools for children.

  6. Influence of local anesthetics on the neuromuscular blockade produced by rocuronium: effects of lidocaine and 50% enantiomeric excess bupivacaine on the neuromuscular junction.

    Science.gov (United States)

    Braga, Angélica de Fátima de Assunção; Carvalho, Vanessa Henriques; Braga, Franklin Sarmento da Silva; Rodrigues-Simioni, Léa; Loyola, Yolanda Christina S; Potério, Glória Braga

    2009-01-01

    The effects of local anesthetics (LA) on neuromuscular transmission and their influence on the neuromuscular blockade produced by competitive neuromuscular blockers have not been fully investigated. The objective of this study was to evaluate, in vitro, the effects of lidocaine and 50% enantiomeric excess bupivacaine (S75-R25) on the neuromuscular blockade produced by rocuronium. The rats were divided in five groups (n = 5) according to the drug used: isolated lidocaine, bupivacaine (S75-R25), or rocuronium (groups I, II, and II); and rocuronium in preparations previously exposed to LAs (groups IV and V). The concentrations used were as follows: 20 microg x mL(-1), 5 microg x mL(-1), and 4 microg x mL(-1) of lidocaine, bupivacaine (S75-R25), and rocuronium, respectively. The following parameters were evaluated: 1) the strength of muscular contraction of the diaphragm to indirect electrical stimulations, before and 60 minutes after the isolated addition of the LAs and rocuronium, and the association AL-rocuronium; and 2) the effects of LAs on membrane potential (MP) and miniature end-plate potentials (MEPP). The effect of LAs on muscle contraction in response to acetylcholine was evaluated in chick biventer cervicis preparations. Isolated lidocaine and bupivacaine (S75-R25) did not change the muscular response and the levels of MPs. In preparations exposed to LAs, rocuroniuminduced blockade was significantly greater than that produced by rocuronium alone. In chick biventer cervicis preparations, lidocaine and bupivacaine (S75R25) decreased contraction in response to acetylcholine. Lidocaine increased the frequency of MEPPs, which was followed by the blockade; bupivacaine (S75R25) caused a reduction in MEPPs followed by blockade. Local anesthetics caused a potentiation of the neuromuscular blockade produced by rocuronium. The results showed pre- and post-synaptic effects.

  7. Profound loss of neprilysin accompanied by decreased levels of neuropeptides and increased CRP in ulcerative colitis.

    Directory of Open Access Journals (Sweden)

    Zeynep Gök Sargın

    Full Text Available Neprilysin (NEP, CD10 acts to limit excessive inflammation partly by hydrolyzing neuropeptides. Although deletion of NEP exacerbates intestinal inflammation in animal models, its role in ulcerative colitis (UC is not well explored. Herein, we aimed to demonstrate changes in NEP and associated neuropeptides at the same time in colonic tissue. 72 patients with UC and 27 control patients were included. Patients' demographic data and laboratory findings, five biopsy samples from active colitis sites and five samples from uninvolved mucosa were collected. Substance P (SP, calcitonin gene related peptide (CGRP and vasoactive intestinal peptide (VIP were extracted from freshly frozen tissues and measured using ELISA. Levels of NEP expression were determined using immunohistochemistry and immunoreactivity scores were calculated. GEBOES grading system was also used. We demonstrated a profound loss (69.4% of NEP expression in UC, whereas all healthy controls had NEP expression. Patients with UC had lower neuronal SP; however non-neuronal SP remained similar. UC patients had also lower neuronal and non-neuronal VIP levels. CGRP were low in general and no significant changes were observed. Additionally, CRP positive patients with UC had higher rates of NEP loss (80% vs 51.9% and lower SP levels when compared with CRP negative patients with UC. Concurrent decreases in SP and VIP with profound loss of NEP expression observed in UC is likely to be one of the factors in pathogenesis. Further studies are required to define the role of neuropeptides and NEP in UC.

  8. Quantification of structural changes in the corpus callosumin children with profound hypoxic-ischaemic brain injury

    Energy Technology Data Exchange (ETDEWEB)

    Stivaros, Stavros M. [Manchester Academic Health Science Centre, Academic Unit of Paediatric Radiology, Royal Manchester Children' s Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester (United Kingdom); University of Manchester, Centre for Imaging Sciences, Institute of Population Health, Manchester (United Kingdom); Radon, Mark R. [The Walton Centre NHS Foundation Trust, Department of Neuroradiology, Liverpool (United Kingdom); Mileva, Reneta; Gledson, Ann; Keane, John A. [University of Manchester, School of Computer Science, Manchester (United Kingdom); Connolly, Daniel J.A.; Batty, Ruth [Sheffield Children' s Hospital NHS Foundation Trust, Department of Neuroradiology, Sheffield (United Kingdom); Cowell, Patricia E. [University of Sheffield, Department of Human Communication Sciences, Sheffield (United Kingdom); Hoggard, Nigel; Griffiths, Paul D. [University of Sheffield, Academic Unit of Radiology, Sheffield (United Kingdom); Wright, Neville B.; Tang, Vivian [Manchester Academic Health Science Centre, Academic Unit of Paediatric Radiology, Royal Manchester Children' s Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester (United Kingdom)

    2016-01-15

    Birth-related acute profound hypoxic-ischaemic brain injury has specific patterns of damage including the paracentral lobules. To test the hypothesis that there is anatomically coherent regional volume loss of the corpus callosum as a result of this hemispheric abnormality. Study subjects included 13 children with proven acute profound hypoxic-ischaemic brain injury and 13 children with developmental delay but no brain abnormalities. A computerised system divided the corpus callosum into 100 segments, measuring each width. Principal component analysis grouped the widths into contiguous anatomical regions. We conducted analysis of variance of corpus callosum widths as well as support vector machine stratification into patient groups. There was statistically significant narrowing of the mid-posterior body and genu of the corpus callosum in children with hypoxic-ischaemic brain injury. Support vector machine analysis yielded over 95% accuracy in patient group stratification using the corpus callosum centile widths. Focal volume loss is seen in the corpus callosum of children with hypoxic-ischaemic brain injury secondary to loss of commissural fibres arising in the paracentral lobules. Support vector machine stratification into the hypoxic-ischaemic brain injury group or the control group on the basis of corpus callosum width is highly accurate and points towards rapid clinical translation of this technique as a potential biomarker of hypoxic-ischaemic brain injury. (orig.)

  9. Whole exome sequencing identifies mutations in Usher syndrome genes in profoundly deaf Tunisian patients.

    Directory of Open Access Journals (Sweden)

    Zied Riahi

    Full Text Available Usher syndrome (USH is an autosomal recessive disorder characterized by combined deafness-blindness. It accounts for about 50% of all hereditary deafness blindness cases. Three clinical subtypes (USH1, USH2, and USH3 are described, of which USH1 is the most severe form, characterized by congenital profound deafness, constant vestibular dysfunction, and a prepubertal onset of retinitis pigmentosa. We performed whole exome sequencing in four unrelated Tunisian patients affected by apparently isolated, congenital profound deafness, with reportedly normal ocular fundus examination. Four biallelic mutations were identified in two USH1 genes: a splice acceptor site mutation, c.2283-1G>T, and a novel missense mutation, c.5434G>A (p.Glu1812Lys, in MYO7A, and two previously unreported mutations in USH1G, i.e. a frameshift mutation, c.1195_1196delAG (p.Leu399Alafs*24, and a nonsense mutation, c.52A>T (p.Lys18*. Another ophthalmological examination including optical coherence tomography actually showed the presence of retinitis pigmentosa in all the patients. Our findings provide evidence that USH is under-diagnosed in Tunisian deaf patients. Yet, early diagnosis of USH is of utmost importance because these patients should undergo cochlear implant surgery in early childhood, in anticipation of the visual loss.

  10. Loss of CIB2 Causes Profound Hearing Loss and Abolishes Mechanoelectrical Transduction in Mice

    Directory of Open Access Journals (Sweden)

    Yanfei Wang

    2017-12-01

    Full Text Available Calcium and integrin-binding protein 2 (CIB2 belongs to a protein family with four known members, CIB1 through CIB4, which are characterized by multiple calcium-binding EF-hand domains. Among the family members, the Cib1 and Cib2 genes are expressed in mouse cochlear hair cells, and mutations in the human CIB2 gene have been associated with nonsyndromic deafness DFNB48 and syndromic deafness USH1J. To further explore the function of CIB1 and CIB2 in hearing, we established Cib1 and Cib2 knockout mice using the clustered regularly interspaced short palindromic repeat (CRISPR-associated Cas9 nuclease (CRISPR/Cas9 genome editing technique. We found that loss of CIB1 protein does not affect auditory function, whereas loss of CIB2 protein causes profound hearing loss in mice. Further investigation revealed that hair cell stereocilia development is affected in Cib2 knockout mice. Noticeably, loss of CIB2 abolishes mechanoelectrical transduction (MET currents in auditory hair cells. In conclusion, we show here that although both CIB1 and CIB2 are readily detected in the cochlea, only loss of CIB2 results in profound hearing loss, and that CIB2 is essential for auditory hair cell MET.

  11. A quantitative review of self-help research with the severely and profoundly mentally retarded.

    Science.gov (United States)

    Konarski, E A; Diorio, M S

    1985-01-01

    Eighty-seven studies published since 1964 through 1982 on training self-help skills to severely and profoundly mentally retarded persons were analyzed according to 19 parameters reflecting their methodological details. The results showed a steady interest in this research area over time, but 63% of the studies focused on toileting and feeding with fewer studies looking at other self-help skills. Package treatments composed primarily of accelerative techniques were most frequently used to train these skills. Methodologically, it was found that these studies typically involved profoundly mentally retarded people (33% of studies) who were trained by residential staff (69% of studies) in institutional settings (63% of studies). The results also indicated an increase over time in the number of studies rated acceptable on the reliability and design parameters. Finally, very few studies reported assessments of generalization, maintenance, or social validity. It was concluded that, (a) researchers need to broaden their interests in terms of settings, trainers, and behaviors studied to best meet the needs of this population, (b) the experimental quality of this literature is improving, and (c) the social impact of observed behavior changes has yet to be fully explored.

  12. Loss of CIB2 Causes Profound Hearing Loss and Abolishes Mechanoelectrical Transduction in Mice.

    Science.gov (United States)

    Wang, Yanfei; Li, Jie; Yao, Xuerui; Li, Wei; Du, Haibo; Tang, Mingliang; Xiong, Wei; Chai, Renjie; Xu, Zhigang

    2017-01-01

    Calcium and integrin-binding protein 2 (CIB2) belongs to a protein family with four known members, CIB1 through CIB4, which are characterized by multiple calcium-binding EF-hand domains. Among the family members, the Cib1 and Cib2 genes are expressed in mouse cochlear hair cells, and mutations in the human CIB2 gene have been associated with nonsyndromic deafness DFNB48 and syndromic deafness USH1J. To further explore the function of CIB1 and CIB2 in hearing, we established Cib1 and Cib2 knockout mice using the clustered regularly interspaced short palindromic repeat (CRISPR)-associated Cas9 nuclease (CRISPR/Cas9) genome editing technique. We found that loss of CIB1 protein does not affect auditory function, whereas loss of CIB2 protein causes profound hearing loss in mice. Further investigation revealed that hair cell stereocilia development is affected in Cib2 knockout mice. Noticeably, loss of CIB2 abolishes mechanoelectrical transduction (MET) currents in auditory hair cells. In conclusion, we show here that although both CIB1 and CIB2 are readily detected in the cochlea, only loss of CIB2 results in profound hearing loss, and that CIB2 is essential for auditory hair cell MET.

  13. Profound effects of population density on fitness-related traits in an invasive freshwater snail.

    Directory of Open Access Journals (Sweden)

    Nicholas Zachar

    Full Text Available Population density can profoundly influence fitness-related traits and population dynamics, and density dependence plays a key role in many prominent ecological and evolutionary hypotheses. Here, we evaluated how individual-level changes in population density affect growth rate and embryo production early in reproductive maturity in two different asexual lineages of Potamopyrgus antipodarum, a New Zealand freshwater snail that is an important model system for ecotoxicology and the evolution of sexual reproduction as well as a potentially destructive worldwide invader. We showed that population density had a major influence on individual growth rate and early-maturity embryo production, effects that were often apparent even when comparing treatments that differed in population density by only one individual. While individual growth rate generally decreased as population density increased, we detected a hump-shaped relationship between embryo production and density, with females from intermediate-density treatments producing the most embryos and females from low- and high-density treatments producing the fewest embryos. The two lineages responded similarly to the treatments, indicating that these effects of population density might apply more broadly across P. antipodarum. These results indicate that there are profound and complex relationships between population density, growth rate, and early-maturity embryo production in at least two lineages of this important model system, with potential implications for the study of invasive populations, research on the maintenance of sex, and approaches used in ecotoxicology.

  14. Demographic Data - MDC_Block

    Data.gov (United States)

    NSGIC Local Govt | GIS Inventory — A polygon feature class of Miami-Dade Census 2000 Blocks. Census blocks are areas bounded on all sides by visible and/or invisible features shown on a map prepared...

  15. Habitat Blocks and Wildlife Corridors

    Data.gov (United States)

    Vermont Center for Geographic Information — Habitat blocks are areas of contiguous forest and other natural habitats that are unfragmented by roads, development, or agriculture. Vermonts habitat blocks are...

  16. Porous block nanofiber composite filters

    Energy Technology Data Exchange (ETDEWEB)

    Ginley, David S.; Curtis, Calvin J.; Miedaner, Alexander; Weiss, Alan J.; Paddock, Arnold

    2016-08-09

    Porous block nano-fiber composite (110), a filtration system (10) and methods of using the same are disclosed. An exemplary porous block nano-fiber composite (110) includes a porous block (100) having one or more pores (200). The porous block nano-fiber composite (110) also includes a plurality of inorganic nano-fibers (211) formed within at least one of the pores (200).

  17. Learnable Programming: Blocks and Beyond

    OpenAIRE

    Bau, David; Gray, Jeff; Kelleher, Caitlin; Sheldon, Josh; Turbak, Franklyn

    2017-01-01

    Blocks-based programming has become the lingua franca for introductory coding. Studies have found that experience with blocks-based programming can help beginners learn more traditional text-based languages. We explore how blocks environments improve learnability for novices by 1) favoring recognition over recall, 2) reducing cognitive load, and 3) preventing errors. Increased usability of blocks programming has led to widespread adoption within introductory programming contexts across a rang...

  18. Mechanical insufflation-exsufflation for people with neuromuscular disorders.

    Science.gov (United States)

    Morrow, Brenda; Zampoli, Marco; van Aswegen, Helena; Argent, Andrew

    2013-12-30

    People with neuromuscular disorders (NMDs) may have weak respiratory (breathing) muscles which makes it difficult for them to effectively cough and clear mucus from the lungs. This places them at risk of recurrent chest infections and chronic lung disease. Mechanical insufflation-exsufflation (MI-E) is one of a number of techniques available to improve cough efficacy and mucus clearance. To determine the efficacy and safety of MI-E in people with NMDs. On 7 October 2013, we searched the following databases from inception: the Cochrane Neuromuscular Disease Group Specialized Register, CENTRAL (The Cochrane Library), MEDLINE, and EMBASE. We also searched ClinicalTrials.gov and the WHO International Clinical Trials Registry Platform for ongoing trials. We conducted handsearches of reference lists and conference proceedings. We considered randomised or quasi-randomised clinical trials, and randomised cross-over trials of MI-E used to assist airway clearance in people with a NMD and respiratory insufficiency. We considered comparisons of MI-E with no treatment, or alternative cough augmentation techniques. Two authors independently assessed trial eligibility, extracted data, and assessed risk of bias in included studies according to standard Cochrane methodology. The primary outcome was mortality throughout follow-up or at six months follow-up. Five studies with a total of 105 participants were found to be eligible for inclusion in this review. All included trials were short-term studies (two days or less), measuring immediate effects of the interventions. There was insufficient detail in the reports to assess methods of randomisation and allocation concealment. All five studies were at a high risk of bias from lack of blinding. The studies did not report on mortality, morbidity, quality of life, serious adverse events or any of the other prespecified outcomes. One study was a randomised cross-over trial conducted over two days, in which investigators applied two

  19. Effects of involuntary eccentric contraction training by neuromuscular electrical stimulation on the enhancement of muscle strength.

    Science.gov (United States)

    Son, Jongsang; Lee, Dongyeop; Kim, Youngho

    2014-08-01

    Neuromuscular electrical stimulation is well-known as a modality to improve the performance of neuromuscular system, but its clinical value on muscle strengthening remains equivocal. In this study, we designed a system for an involuntary eccentric contraction of biceps brachii muscles using continuous passive movement and commercial neuromuscular electrical stimulation devices. To investigate the effects of involuntary eccentric contraction training by neuromuscular electrical stimulation on the enhancement of muscle strength, seven healthy men between the ages of 24 and 29 years participated in this study. Participants were trained two times per week for 12 weeks. Each exercise session was performed for 30 min with no rest intervals. Isometric elbow flexion torque and biceps brachii muscle thickness were chosen as evaluation indices, and were measured at pre-/post-training. After the 12-week training, the isometric elbow flexion torque of the trained side significantly increased by approximately 23% compared to the initial performance (P<0.01). Meanwhile, the torque of the untrained side showed no significant change (P=0.862). During the 12-week training period, the biceps brachii muscle thickness of the trained side significantly increased by around 8% at rest and 16% at maximum voluntary contraction (P<0.01). The developed system and the technique show promising results, suggesting that it has the potential to be used to increase the muscle strength in patients with neuromuscular disease and to be implemented in design rehabilitative protocols. Copyright © 2014 Elsevier Ltd. All rights reserved.

  20. Time course and dimensions of postural control changes following neuromuscular training in youth field hockey athletes.

    Science.gov (United States)

    Zech, Astrid; Klahn, Philipp; Hoeft, Jon; zu Eulenburg, Christine; Steib, Simon

    2014-02-01

    Injury prevention effects of neuromuscular training have been partly attributed to postural control adaptations. Uncertainty exists regarding the magnitude of these adaptations and on how they can be adequately monitored. The objective was to determine the time course of neuromuscular training effects on functional, dynamic and static balance measures. Thirty youth (14.9 ± 3 years) field hockey athletes were randomised to an intervention or control group. The intervention included a 20-min neuromuscular warm-up program performed twice weekly for 10 weeks. Balance assessments were performed at baseline, week three, week six and post-intervention. They included the star excursion balance test (SEBT), balance error scoring system (BESS), jump-landing time to stabilization (TTS) and center of pressure (COP) sway velocity during single-leg standing. No baseline differences were found between groups in demographic data and balance measures. Adherence was at 86%. All balance measures except the medial-lateral TTS improved significantly over time (p controls (31.8 ± 22.1%). There were no significant group by time interactions in the SEBT, TTS and COP sway velocity. Neuromuscular training was effective in improving postural control in youth team athletes. However, this effect was not reflected in all balance measures suggesting that the neuromuscular training did not influence all dimensions of postural control. Further studies are needed to confirm the potential of specific warm-up programs to improve postural control.

  1. Regulation and restoration of motoneuronal synaptic transmission during neuromuscular regeneration in the pulmonate snail Helisoma trivolvis.

    Science.gov (United States)

    Turner, M B; Szabo-Maas, T M; Poyer, J C; Zoran, M J

    2011-08-01

    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.

  2. A Dutch guideline for the treatment of scoliosis in neuromuscular disorders

    Directory of Open Access Journals (Sweden)

    Titarsolej PJ

    2008-09-01

    Full Text Available Abstract Background Children with neuromuscular disorders with a progressive muscle weakness such as Duchenne Muscular Dystrophy and Spinal Muscular Atrophy frequently develop a progressive scoliosis. A severe scoliosis compromises respiratory function and makes sitting more difficult. Spinal surgery is considered the primary treatment option for correcting severe scoliosis in neuromuscular disorders. Surgery in this population requires a multidisciplinary approach, careful planning, dedicated surgical procedures, and specialized after care. Methods The guideline is based on scientific evidence and expert opinions. A multidisciplinary working group representing experts from all relevant specialties performed the research. A literature search was conducted to collect scientific evidence in answer to specific questions posed by the working group. Literature was classified according to the level of evidence. Results For most aspects of the treatment scientific evidence is scarce and only low level cohort studies were found. Nevertheless, a high degree of consensus was reached about the management of patients with scoliosis in neuromuscular disorders. This was translated into a set of recommendations, which are now officially accepted as a general guideline in the Netherlands. Conclusion In order to optimize the treatment for scoliosis in neuromuscular disorders a Dutch guideline has been composed. This evidence-based, multidisciplinary guideline addresses conservative treatment, the preoperative, perioperative, and postoperative care of scoliosis in neuromuscular disorders.

  3. Neuromuscular fatigue following isometric contractions with similar torque time integral.

    Science.gov (United States)

    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. © Georg Thieme Verlag KG Stuttgart · New York.

  4. Neuromuscular fatigue after maximal exercise in patients with cystic fibrosis.

    Science.gov (United States)

    Vallier, J M; Gruet, M; Mely, L; Pensini, M; Brisswalter, J

    2011-04-01

    The aim of this study was to determine whether patients with cystic fibrosis (CF), despite their ventilatory limitation, would develop neuromuscular fatigue of quadriceps muscles following a maximal cycling exercise. Eleven adults with CF (age=26.8±6.9years; forced expiratory volume in 1s=54.1±12.8% predicted) and 11 age-matched healthy subjects performed a maximal incremental cycle test with respiratory gas exchange measurements. Maximal voluntary contraction (MVC) and electromyographic (EMG) activity of the vastus medialis muscle were recorded before and after exercise. Neural and contractile properties of the quadriceps were also investigated using femoral nerve electrical stimulation. Patients had lower exercise capacity, peak oxygen uptake and MVC than controls. MVC fell significantly postexercise in both groups (CF: -20±10%, controls: -19±6%; pdevelopment (-50.3±13.8%) and relaxation (-35.2±19.5%) were all reduced after exercise in CF patients. The control group followed the same pattern (-38.4±14.4%, -42.1±14.7% and -15±20.4%) but the statistical significance was not reached for the maximal rate of twitch torque relaxation. In conclusion, CF patients demonstrated lower limb fatigue following symptom-limited cycle exercise, which was comparable to that exhibited by healthy controls. This fatigue may be due to contractile impairments and not to transmission failure. Further studies should be conducted in a larger sample to confirm these preliminary results. Copyright © 2010 Elsevier Ltd. All rights reserved.

  5. Recovery of central and peripheral neuromuscular fatigue after exercise.

    Science.gov (United States)

    Carroll, T J; Taylor, J L; Gandevia, S C

    2017-05-01

    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 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 2 min) and aspects of peripheral fatigue associated with excitation-contraction coupling and reperfusion 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, the ability to voluntarily activate muscles may not recover completely within 30 min after exercise. Recovery of peripheral fatigue contributes comparatively little to the fast initial force restitution and is typically incomplete for at least 20-30 min. 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. Copyright © 2017 the American Physiological Society.

  6. Lower Limb Neuromuscular Asymmetry in Volleyball and Basketball Players

    Directory of Open Access Journals (Sweden)

    Fort-Vanmeerhaeghe Azahara

    2016-04-01

    Full Text Available 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.

  7. Effects of hindlimb unloading on neuromuscular development of neonatal rats

    Science.gov (United States)

    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.

  8. Proprioceptive neuromuscular facilitation stretching : mechanisms and clinical implications.

    Science.gov (United States)

    Sharman, Melanie J; Cresswell, Andrew G; Riek, Stephan

    2006-01-01

    Proprioceptive neuromuscular facilitation (PNF) stretching techniques are commonly used in the athletic and clinical environments to enhance both active and passive range of motion (ROM) with a view to optimising motor performance and rehabilitation. PNF stretching is positioned in the literature as the most effective stretching technique when the aim is to increase ROM, particularly in respect to short-term changes in ROM. With due consideration of the heterogeneity across the applied PNF stretching research, a summary of the findings suggests that an 'active' PNF stretching technique achieves the greatest gains in ROM, e.g. utilising a shortening contraction of the opposing muscle to place the target muscle on stretch, followed by a static contraction of the target muscle. The inclusion of a shortening contraction of the opposing muscle appears to have the greatest impact on enhancing ROM. When including a static contraction of the target muscle, this needs to be held for approximately 3 seconds at no more than 20% of a maximum voluntary contraction. The greatest changes in ROM generally occur after the first repetition and in order to achieve more lasting changes in ROM, PNF stretching needs to be performed once or twice per week. The superior changes in ROM that PNF stretching often produces compared with other stretching techniques has traditionally been attributed to autogenic and/or reciprocal inhibition, although the literature does not support this hypothesis. Instead, and in the absence of a biomechanical explanation, the contemporary view proposes that PNF stretching influences the point at which stretch is perceived or tolerated. The mechanism(s) underpinning the change in stretch perception or tolerance are not known, although pain modulation has been suggested.

  9. Neuromuscular consequences of an extreme mountain ultra-marathon.

    Directory of Open Access Journals (Sweden)

    Guillaume Y Millet

    Full Text Available 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.

  10. Electromyographic and neuromuscular fatigue thresholds as concepts of fatigue.

    Science.gov (United States)

    Mäestu, Jarek; Cicchella, Antonio; Purge, Priit; Ruosi, Sergio; Jürimäe, Jaak; Jürimäe, Toivo

    2006-11-01

    The aim of this study was to investigate the concepts of electromyographic (EMG) threshold (EMGT) by integrated EMG (iEMG) signals and neuromuscular fatigue threshold (NMFT) concepts in trained male athletes. Nine competitive national-level male rowers (21.8 +/- 4.4 years; 186.2 +/- 4.6 cm; 79.6 +/- 8.4 kg) took part in this investigation. Subjects were asked to participate in the graded exercise test to volitional exhaustion and 500-, 1,000-, and 2,000-m all-out rowing ergometer tests on a rowing ergometer. During all tests, oxygen consumption parameters, average power, and iEMG of the musculus vastus lateralis were recorded. The second ventilatory threshold (248.9 +/- 26.67 W) and EMGT (258.89 +/- 27.13 W) were not significantly different but were significantly lower than the NMFT (302.25 +/- 45.10 W). During 1,000- and 2,000-m all-out distances, VO(2) increased during the first minute and then leveled on a plateau with a slight decrease at the end of the exercise. Vastus lateralis activity showed a slight increase during all distances that was accompanied by a remarkable increase towards the end of the distance. All measured threshold values were significantly correlated (r > 0.70; p < 0.05) to the rowing ergometer performance characteristics. It was concluded that EMGT is closely related to the aerobic-anaerobic transition phase, because NMFT represents the local fatigue accumulation in the muscle. NMFT indicates the performance capacity of the muscles; therefore, it helps coaches to better predict top athletes' performance.

  11. Use of a Treatment Package in the Management of a Profoundly Mentally Retarded Girl's Pica and Self-Stimulation.

    Science.gov (United States)

    Paniagua, Freddy A.; And Others

    1986-01-01

    Treatment involving verbal reprimands, physical restraint, response interruption, and positive practice overcorrection resulted in rapid and dramatic decreases in the levels of pica and self-stimulation of a 4-year-old profoundly retarded girl. (CL)

  12. An atypical case of successful resuscitation of an accidental profound hypothermia patient, occurring in a temperate climate.

    LENUS (Irish Health Repository)

    Coleman, E

    2010-03-01

    Cases of accidental profound hypothermia occur most frequently in cold, northern climates. We describe an atypical case, occurring in a temperate climate, where a hypothermic cardiac-arrested patient was successfully resuscitated using extracorporeal circulation (ECC).

  13. Brainstem Anesthesia after Retrobulbar Block

    African Journals Online (AJOL)

    GB

    2016-11-06

    Nov 6, 2016 ... BACKGROUND: Retro-bulbar anesthesia is one of the most common regional blocks used for intraocular surgeries. Complications associated with regional blocks may be limited to the eye or may be systemic. CASE REPORT: After a retro-bulbar block for glaucoma surgery, a 60-year-old man developed ...

  14. Abdominal wall blocks in adults

    DEFF Research Database (Denmark)

    Neimann, Jens Dupont Børglum; Gögenür, Ismail; Bendtsen, Thomas F.

    2016-01-01

    Purpose of review Abdominal wall blocks in adults have evolved much during the last decade; that is, particularly with the introduction of ultrasound-guided (USG) blocks. This review highlights recent advances of block techniques within this field and proposes directions for future research...

  15. Blocking the Hawking radiation

    DEFF Research Database (Denmark)

    Autzen, M.; Kouvaris, C.

    2014-01-01

    grows after its formation (and eventually destroys the star) instead of evaporating. The fate of the black hole is dictated by the two opposite mechanics, i.e., accretion of nuclear matter from the center of the star and Hawking radiation that tends to decrease the mass of the black hole. We study how...... the assumptions for the accretion rate can in fact affect the critical mass beyond which a black hole always grows. We also study to what extent degenerate nuclear matter can impede Hawking radiation due to the fact that emitted particles can be Pauli blocked at the core of the star....

  16. [Effects of a neurotoxin isolated from the sea anemone, Anemonia sulcata, at frog neuromuscular junction (author's transl)].

    Science.gov (United States)

    Métézeau, P; Bourneau, R; Mambrini, J; Tazieff-Depierre, F

    1979-01-01

    ATX II is a toxin extracted from tentacles of Anemonia sulcata. It was known that this protein displays neurotoxic effects on frog isolated neuromuscular preparation (Fig. 1, 2) and that muscular contractures observed with ATX II are blocked by d-tubocurarine (Fig. 3) or on a 40-days-denervated gastrocnemius (Fig. 4). Part of these experiments has already appeared. 1. These effects of ATX II depend on calcium concentration in the bathing medium, as is the case for transmitter release. The same results were observed when we substituted strontium to calcium. 2. On an intact sciatic sartorius preparation, ATX II does not act on the amplitude of the miniature endplate potentials (mepps, Fig. 6). The muscular action potential is not modified by this toxin. 3. ATX II increases the frequency of the mepps (Fig. 5). The evoked transmitter release (quantal content) after ATX II is also largely increased (Fig. 7). 4. In conclusion, it is suggested that ATX II acts indirectly on the muscle through an increase in acetylcholine release from the motor nerve terminals.

  17. Long-term effect of Helicobacter pylori eradication on the reversibility of acid secretion in profound hypochlorhydria.

    Science.gov (United States)

    Iijima, K; Sekine, H; Koike, T; Imatani, A; Ohara, S; Shimosegawa, T

    2004-06-01

    Although profound hypochlorhydria is considered to be an important risk factor for development of gastric cancer, long-term effect of Helicobacter pylori eradication on its reversibility remains uncertain. To clarify the change in acid secretion after eradication in a long-term follow-up over 5 years in patients with profound hypochlorhydria. Twenty-three H. pylori-positive patients with hypochlorhydria (hypochlorhydria.

  18. Program structure-based blocking

    Science.gov (United States)

    Bertolli, Carlo; Eichenberger, Alexandre E.; O'Brien, John K.; Sura, Zehra N.

    2017-09-26

    Embodiments relate to program structure-based blocking. An aspect includes receiving source code corresponding to a computer program by a compiler of a computer system. Another aspect includes determining a prefetching section in the source code by a marking module of the compiler. Yet another aspect includes performing, by a blocking module of the compiler, blocking of instructions located in the prefetching section into instruction blocks, such that the instruction blocks of the prefetching section only contain instructions that are located in the prefetching section.

  19. Host-associated and free-living phage communities differ profoundly in phylogenetic composition.

    Directory of Open Access Journals (Sweden)

    J Gregory Caporaso

    2011-02-01

    Full Text Available Phylogenetic profiling has been widely used for comparing bacterial communities, but has so far been impossible to apply to viruses because of the lack of a single marker gene analogous to 16S rRNA. Here we developed a reference tree approach for matching viral sequences and applied it to the largest viral datasets available. The resulting technique, Shotgun UniFrac, was used to compare host-associated and non-host-associated phage communities (130 total metagenomes, and revealed a profound split similar to that found with bacterial communities. This new informatics approach complements analysis of bacterial communities and promises to provide new insights into viral community dynamics, such as top-down versus bottom-up control of bacterial communities by viruses in a range of systems.

  20. Making Sense of Bereavement in People with Profound Intellectual and Multiple Disabilities: Carer Perspectives.

    Science.gov (United States)

    Young, Hannah; Hogg, James; Garrard, Brenda

    2017-11-01

    People with intellectual disabilities are thought to have a reduced capacity for understanding death. Drawing on cognitive theory, researchers have suggested that those with profound intellectual and multiple disabilities mainly perceive loss as a mismatch between past and present experiences. However, very little research has considered how carers conceptualize bereavement in relation to this group. Semi-structured interviews obtained responses from seven carers. Transcripts were examined using interpretative phenomenological analysis. Two superordinate themes emerged: 'difficulty articulating the experience of loss' and 'making sense of bereavement through familiar patterns'. Carers conceptualize bereavement primarily in cognitive terms, but also take account of relational factors mediating loss. Implications for training and further research are outlined. © 2016 John Wiley & Sons Ltd.

  1. Care versus treatment at the end of life for profoundly disabled persons.

    Science.gov (United States)

    Spike, Jeffrey P

    2012-01-01

    Individuals who are profoundly mentally handicapped do not have the capacity to make their own decisions and also do not have a past record of decisions, from when they had capacity, to guide us in making decisions for them. They represent a difficult group, ethically, for surrogate decision making. Here I propose some guidelines, distinguishing between these patients and patients in a persistent vegetative state (PVS). As the life span of patients becomes shorter, or their level of consciousness becomes permanently impaired, the presumption for comfort care should become an imperative, and the standard of evidence to justify any invasive intervention should become higher. For members of this population, who have no more ability to refuse treatment than to consent to it, protection of the vulnerable must mean allowing a peaceful death as well as a comfortable life. Reasonable legal safeguards are also proposed to allow improved end-of-life decisions to be made for this population.

  2. Experience with cochlear implants in Greenlanders with profound hearing loss living in Greenland

    DEFF Research Database (Denmark)

    Homøe, Preben; Andersen, Ture; Grøntved, Aksel

    2013-01-01

    OBJECTIVE: Cochlear implant (CI) treatment was introduced to the world in the 1980s and has become a routine treatment for congenital or acquired severe-to-profound hearing loss. CI treatment requires access to a highly skilled team of ear, nose and throat specialists, audiologists and speech...... years are in need of a CI every second year in Greenland often due to sequelae from meningitis, which may cause postinfectious deafness. Screening of new-borns for hearing has been started in Greenland establishing the basis for early diagnosis of congenital hearing impairment and subsequent...... intervention. The logistics and lack of availability of speech therapists in Greenland hampers possibilities for optimal language and speech therapy of CI patients in Greenland. This study aims at describing the results of CI treatment in Greenlanders and the outcome of the CI operations along...

  3. A longitudinal study of active treatment of adaptive skills of individuals with profound mental retardation.

    Science.gov (United States)

    Bat-haee, M A

    2001-10-01

    A longitudinal study indicated that a single group of adults living in a large state institution (O'Berry Center in Goldsboro, North Carolina) benefited from receiving active treatment, although no control group was permitted. After five years, 59 individuals with profound mental retardation improved their skills in six general areas of dressing, grooming, eating, toileting, communication, and social interaction. After five more years of active treatment, 51 persons who had remained in the program maintained their skills in dressing, grooming, eating, and communication. Significant improvement in the use of toilet and a loss in social interaction were observed. Although the participants were 10 years older at the end of this study, their adaptive behaviors, in comparison to the date of entry, either improved or were maintained. Therefore, aging was not associated with decline in skills.

  4. Acquisition and generalization of instruction following behavior in profoundly retarded individuals.

    Science.gov (United States)

    Ivy, R; Dubin, W

    1979-08-01

    Four profoundly retarded male residents of a state institution learned to comply with the instructions "come here," "stay," and "sit." Acquisition occurred in a special therapy room and instructions were presented in a fixed sequence. All subjects learned to comply with the instructions in the training environment. Testing occurred in the subject's ward dayroom where instructions were presented in a random sequence. Despite dramatic changes in the stimulus conditions associated with the transfer to the testing situation the subjects performed close to their asymptotic level. Three experimentally naive subjects participated in a second experiment which was similar to the first, except that the number of acquisition sessions and the number of trials per session were reduced. The reduction in number of acquisition trials did not appear to affect performance in the testing environment. The possibility of terminating off-task behavior of institutionalized individuals by eliciting previously acquired instruction following behavior was discussed.

  5. Profound hypoglycemia-ınduced by vaccinium corymbosum juice and laurocerasus fruit.

    Science.gov (United States)

    Aktan, Ahmet Hamdi; Ozcelik, Abdullah; Cure, Erkan; Cure, Medine Cumhur; Yuce, Suleyman

    2014-01-01

    An emergency intervention was performed in a 75-year-old male patient with hypoglycemic attack and blackout. Although he was diagnosed with prediabetes before 2 years, he did not take any anti-diabetic drug or follow dietary advice. He drank Vaccinium corymbosum L (VC) juice daily with a belief that it increases sexual potency. Before the development of hypoglycemia, the patient had consumed about 500 ml VC juice in addition to eating 200-300 gram of Laurocerasus officinalis (LO) fruit. The measured plasma glucose (PG) level during loss of consciousness was 30 mg/dl. The profound hypoglycemia may be an unexpected side effect of an interaction between the chemical compositions of the two plants, occurred as a result of LO fruit intake that may have a strong PG-lowering effect or related to excessive intake of VC juice. Both plants may be considered in the alternative treatment of diabetes.

  6. Peer Interactions among Children with Profound Intellectual and Multiple Disabilities during Group Activities.

    Science.gov (United States)

    Nijs, Sara; Penne, Anneleen; Vlaskamp, Carla; Maes, Bea

    2016-07-01

    Children with profound intellectual and multiple disabilities (PIMD) meet other children with PIMD in day care centres or schools. This study explores the peer-directed behaviours of children with PIMD, the peer interaction-influencing behaviour of the direct support workers and the children's positioning. Group activities for children with PIMD initiated by a direct support worker were video-recorded. The behaviour and positioning of the children and the behaviour of the direct support workers were coded. Limited peer-directed behaviour of the children with PIMD and peer interaction-influencing behaviour of the direct support workers are observed. Weak associations were found between the positioning or peer interaction-influencing behaviours and the behaviour of children with PIMD. Children with PIMD show social interest in each other during group activities. More knowledge is needed to create an environment which facilitates peer-directed behaviours of persons with PIMD. © 2015 John Wiley & Sons Ltd.

  7. Sensorimotor functioning and prelinguistic communication of severely and profoundly retarded individuals.

    Science.gov (United States)

    Lobato, D; Barrera, R D; Feldman, R S

    1981-03-01

    The prelinguistic, nonverbal communicative behavior of 40 institutionalized severely and profoundly retarded children and adolescents functioning at various stages of Piaget's sensorimotor period was examined. Five scales of the Uzgiris and Hunt (1975) sensorimotor assessment were used to determine general level of sensorimotor functioning. A standard set of communication elicitation tasks was employed to examine the gestures used to communicate in both imperative and declarative contexts. More competent sensorimotor performance was associated with higher frequency of more sophisticated and symbolic forms of gestural communication, and subjects generally used more complex gestures to communicate in the imperative than in the declarative tasks. Applicability of the results to the design of future language-intervention programs and their implications for general theory concerning the relations between language and cognition and between normal and atypical development were discussed.

  8. Profound vision loss impairs psychological well-being in young and middle-aged individuals

    Directory of Open Access Journals (Sweden)

    Garcia GA

    2017-02-01

    Full Text Available Giancarlo A Garcia,1,2 Matin Khoshnevis,1,3 Jesse Gale,1,4 Starleen E Frousiakis,1,5 Tiffany J Hwang,1,6 Lissa Poincenot,1 Rustum Karanjia,1,7–9 David Baron,6 Alfredo A Sadun1,7 1Doheny Eye Institute, Los Angeles, CA, USA; 2University of California, Irvine School of Medicine, Irvine, CA, USA; 3Department of Ophthalmology, Temple University, Philadelphia, PA, USA; 4Wellington School of Medicine and Health Sciences, University of Otago, Wellington, NZ; 5Department of Ophthalmology, New York Medical College, Valhalla, NY, USA; 6Department of Psychiatry & The Behavioral Sciences, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA; 7Doheny Eye Centers, Department of Ophthalmology, David Geffen School of Medicine at UCLA, Los Angeles California, CA, USA; 8Department of Ophthalmology, University of Ottawa, Ottawa, ON, Canada; 9Ottawa Hospital Health Research Institute, Ottawa, ON, Canada Purpose: The aim of this study was to evaluate the effects of profound vision loss on psychological well-being in adolescents, young adults, and middle-aged adults with regard to mood, interpersonal interactions, and career-related goals. In addition, we assessed the significance of the resources that may be used to enhance psychological well-being in cases of profound vision loss, and in particular, examined the utility of low vision aids and the role of the ophthalmologist as a provider of emotional support.Methods: A questionnaire was issued to individuals aged 13–65 years with profound vision loss resulting from Leber’s hereditary optic neuropathy (LHON. Depression prevalence was evaluated with questions regarding major depressive disorder symptomatology. Participants appraised the effects of vision loss on their interpersonal interactions and career goals by providing an impact rating (IR on a 21-point psychometric scale from −10 to +10. Social well-being index was defined as the average of interpersonal IR and career IR

  9. Identifying perception behaviours in people with profound intellectual and multiple disabilities.

    Science.gov (United States)

    Roemer, Miriam; Verheul, Ellen; Velthausz, Frank

    2017-12-21

    To support people with profound intellectual and multiple disabilities (PIMD), it is essential to understand how they experience their environment. Insight into perception behaviour may provide an entry point for improved understanding. A random sample of a 30-min video registration of five participants with PIMD was used to code behaviours per second based on an ethogram containing 157 different perception behaviours in nine categories. Eighty-nine different perception behaviours were observed, of which movements with eyes, head and arms were most common. The senses used most were seeing, hearing and touching. Finally, the function of five perception patterns was established in relation to their function:awareness, focusing attention and tension regulation. Close observation using an observation ethogram provides insight into how people with PIMD perceive their environment. © 2017 John Wiley & Sons Ltd.

  10. Effect of exercise therapy on neuromuscular activity and knee strength in female adolescents with patellofemoral pain

    DEFF Research Database (Denmark)

    Rathleff, Michael S.; Samani, Afshin; Olesen, Jens L.

    2016-01-01

    BACKGROUND: Female adolescents with patellofemoral pain are characterized by altered neuromuscular knee control and reduced maximal quadriceps torque. The purpose of this study is to investigate whether exercise therapy and patient education are associated with larger improvements in neuromuscular...... knee control and maximal quadriceps torque compared with patient education alone. METHODS: This is an ancillary analysis of a cluster randomized controlled trial investigating the effect of patient education and exercise therapy on self-reported recovery in 121 adolescents with patellofemoral pain...... flexion/extension kinematics and maximal quadriceps torque. FINDINGS: There was an 8-15% greater decrease in the complexity of surface electromyography suggesting an improvement in neuromuscular knee control among those randomized to exercise therapy (0.08

  11. Update on the approach of respiratory therapy in patients with neuromuscular diseases

    Directory of Open Access Journals (Sweden)

    Priscilla Barreto Paula

    2010-03-01

    Full Text Available Objective: To assess the role of physiotherapy in approaching neuromuscular disease (NMD, with emphasis on preventive and therapeutic aspects of respiratory therapy. Methods: A nonsystematic literature review covering the past twenty years, using the databases MEDLINE and LILACS through the following descriptors: neuromuscular diseases, physical therapy, vital capacity and respiratory failure. Results: The studies reviewed show the need to establish a routine periodic evaluation of respiratory function in order to introduce physical therapy measures relevant to each stage of the disease. The monitoring should include pulmonary function tests and specific techniques of chest physiotherapy, in order to avoid complications such as respiratory failure. Conclusion: The introduction of regular monitoring and preventive physiotherapy measures have helped to increase survival and improve quality of life of patients with neuromuscular diseases.

  12. Congenital myopathies with secondary neuromuscular transmission defects; a case report and review of the literature.

    Science.gov (United States)

    Rodríguez Cruz, Pedro M; Sewry, Caroline; Beeson, David; Jayawant, Sandeep; Squier, Waney; McWilliam, Robert; Palace, Jacqueline

    2014-12-01

    Congenital myopathies are a clinically and genetically heterogeneous group of disorders characterized by early onset hypotonia, weakness and characteristic, but not pathognomonic, structural abnormalities in muscle fibres. The clinical features overlap with muscular dystrophies, myofibrillar myopathies, neurogenic conditions and congenital myasthenic syndromes. We describe a case of cap myopathy with myasthenic features due to a mutation in the TPM2 gene that responded to anticholinesterase therapy. We also review other published cases of congenital myopathies with neuromuscular transmission abnormalities. This report expands the spectrum of congenital myopathies with secondary neuromuscular transmission defects. The recognition of these cases is important since these conditions can benefit from treatment with drugs enhancing neuromuscular transmission. Copyright © 2014 Elsevier B.V. All rights reserved.

  13. The use of in-flight foot pressure as a countermeasure to neuromuscular degradation

    Science.gov (United States)

    Layne, C. S.; Mulavara, A. P.; Pruett, C. J.; McDonald, P. V.; Kozlovskaya, I. B.; Bloomberg, J. J.

    The purpose of this study was to determine whether applying foot pressure to unrestrained subjects during space flight could enhance the neuromuscular activation associated with rapid arm movements. Four men performed unilateral arm raises while wearing —or not wearing—specially designed boots during a 81- or 115-day space flight. Arm acceleration and surface EMG were obtained from selected lower limb and trunk muscles. Pearson r coefficients were used to evaluate similarity in phasic patterns between the two in-flight conditions. In-flight data also were magnitude normalized to the mean voltage value of the muscle activation waveforms obtained during the no-foot-pressure condition to facilitate comparison of activation amplitude between the two in-flight conditions. Foot pressure enhanced neuromuscular activation and somewhat modified the phasic features of the neuromuscular activation during the arm raises.

  14. Reliability of the 2- and 6-minute walk tests in neuromuscular diseases

    DEFF Research Database (Denmark)

    Knak, Kirsten Lykke; Andersen, Linda Kahr; Witting, Nanna

    2017-01-01

    OBJECTIVE: The 2- and 6-minute walk tests are used to evaluate walking capacity, but reliability has been sparsely investigated in patients with neuromuscular diseases. The aim of this study was to investigate the relative and absolute reliability of the 2- and 6-minute walk tests in patients...... with neuromuscular diseases. DESIGN: Each patient performed a 2- and a 6-minute walk test on 2 test days separated by 1-2 weeks. SUBJECTS: A total of 93 adult patients (mean age 53 years, age range 22-83 years) with 12 different neuromuscular diseases were included. RESULTS: The mean walking distance increased by 4.......3 and 11.2 m (p walk tests, respectively. Intraclass correlation coefficient in the 2- and 6-minute walk tests was 0.99 (p walk test and 14.0 m in the 6-minute walk test. Minimal detectable difference...

  15. Assessment of bone density in patients with scoliosis neuromuscular secondary to cerebral palsy

    Directory of Open Access Journals (Sweden)

    Charbel Jacob Júnior

    2014-09-01

    Full Text Available OBJECTIVE: To evaluate bone mineral density in patients with neuromuscular scoliosis secondary to spastic quadriplegic cerebral palsy. METHODS: A prospective descriptive study in which, in addition to bone densitometry, the anthropometric data of the patients were assessed. As inclusion criterion we adopted patients with spastic quadriplegic cerebral palsy, wheelchair users, aged between 10 and 20 years and with neuromuscular scoliosis. RESULTS: We evaluated 31 patients, 20 female, whose average age was 14.2 years. The mean bone density was -3.2 standard deviation (Z-score, with mean biceps circumference of 19.4 cm, calf circumference 18.6 cm and BMI of 13.6 kg/m². CONCLUSION: There is a high incidence of osteoporosis in patients with neuromuscular scoliosis secondary to spastic quadriplegic cerebral palsy.

  16. Arogenate Dehydratase Isoenzymes Profoundly and Differentially Modulate Carbon Flux into Lignins*

    Science.gov (United States)

    Corea, Oliver R. A.; Ki, Chanyoung; Cardenas, Claudia L.; Kim, Sung-Jin; Brewer, Sarah E.; Patten, Ann M.; Davin, Laurence B.; Lewis, Norman G.

    2012-01-01

    How carbon flux differentially occurs in vascular plants following photosynthesis for protein formation, phenylpropanoid metabolism (i.e. lignins), and other metabolic processes is not well understood. Our previous discovery/deduction that a six-membered arogenate dehydratase (ADT1–6) gene family encodes the final step in Phe biosynthesis in Arabidopsis thaliana raised the fascinating question whether individual ADT isoenzymes (or combinations thereof) differentially modulated carbon flux to lignins, proteins, etc. If so, unlike all other lignin pathway manipulations that target cell wall/cytosolic processes, this would be the first example of a plastid (chloroplast)-associated metabolic process influencing cell wall formation. Homozygous T-DNA insertion lines were thus obtained for five of the six ADTs and used to generate double, triple, and quadruple knockouts (KOs) in different combinations. The various mutants so obtained gave phenotypes with profound but distinct reductions in lignin amounts, encompassing a range spanning from near wild type levels to reductions of up to ∼68%. In the various KOs, there were also marked changes in guaiacyl:syringyl ratios ranging from ∼3:1 to 1:1, respectively; these changes were attributed to differential carbon flux into vascular bundles versus that into fiber cells. Laser microscope dissection/pyrolysis GC/MS, histochemical staining/lignin analyses, and pADT::GUS localization indicated that ADT5 preferentially affects carbon flux into the vascular bundles, whereas the adt3456 knock-out additionally greatly reduced carbon flux into fiber cells. This plastid-localized metabolic step can thus profoundly differentially affect carbon flux into lignins in distinct anatomical regions and provides incisive new insight into different factors affecting guaiacyl:syringyl ratios and lignin primary structure. PMID:22311980

  17. Arogenate dehydratase isoenzymes profoundly and differentially modulate carbon flux into lignins.

    Science.gov (United States)

    Corea, Oliver R A; Ki, Chanyoung; Cardenas, Claudia L; Kim, Sung-Jin; Brewer, Sarah E; Patten, Ann M; Davin, Laurence B; Lewis, Norman G

    2012-03-30

    How carbon flux differentially occurs in vascular plants following photosynthesis for protein formation, phenylpropanoid metabolism (i.e. lignins), and other metabolic processes is not well understood. Our previous discovery/deduction that a six-membered arogenate dehydratase (ADT1-6) gene family encodes the final step in Phe biosynthesis in Arabidopsis thaliana raised the fascinating question whether individual ADT isoenzymes (or combinations thereof) differentially modulated carbon flux to lignins, proteins, etc. If so, unlike all other lignin pathway manipulations that target cell wall/cytosolic processes, this would be the first example of a plastid (chloroplast)-associated metabolic process influencing cell wall formation. Homozygous T-DNA insertion lines were thus obtained for five of the six ADTs and used to generate double, triple, and quadruple knockouts (KOs) in different combinations. The various mutants so obtained gave phenotypes with profound but distinct reductions in lignin amounts, encompassing a range spanning from near wild type levels to reductions of up to ∼68%. In the various KOs, there were also marked changes in guaiacyl:syringyl ratios ranging from ∼3:1 to 1:1, respectively; these changes were attributed to differential carbon flux into vascular bundles versus that into fiber cells. Laser microscope dissection/pyrolysis GC/MS, histochemical staining/lignin analyses, and pADT::GUS localization indicated that ADT5 preferentially affects carbon flux into the vascular bundles, whereas the adt3456 knock-out additionally greatly reduced carbon flux into fiber cells. This plastid-localized metabolic step can thus profoundly differentially affect carbon flux into lignins in distinct anatomical regions and provides incisive new insight into different factors affecting guaiacyl:syringyl ratios and lignin primary structure.

  18. Plasma antidiuretic hormone in cases with the early onset of profound unilateral deafness.

    Science.gov (United States)

    Takeda, Taizo; Kakigi, Akinobu; Nishioka, Rie; Taguchi, Daizo; Nishimura, Masahiko

    2008-12-01

    The p-ADH level in cases of juvenile unilateral profound deafness (JUPD) and the timecourse of the level were examined to investigate whether or not an increase of p-ADH is involved in the development of delayed endolymphatic hydrops (DEH) in JUPD. In 90 consecutive patients with unilateral profound or total sensorineural deafness with the onset in early childhood, pure-tone audiometric examination and the measurement of p-ADH and plasma osmolality (p-OSM) were followed up once or twice a year as far as possible. At every testing, we performed careful history-taking about episodic vertigo/dizziness, fluctuant hearing loss, and tinnitus in order to find out whether patients had experienced these clinical signs of the development of DEH. Means and standard deviation (S.D.) of p-ADH level and osmolality in all samples tested (n=368) were 7.3+/-7.0 pg/mL (0.7-52.0 pg/mL), and 288.6+/-4.4 mOsm/L (273-306 mOsm/L), respectively. The mean of p-ADH level was much higher than those previously reported in children and adolescents. High levels of p-ADH (over 5.0 pg/mL) were often observed in subjects between 6 and 19 years of age, but not so frequently in subjects of 20 years of age or older. Long-term follow-up of p-ADH levels revealed that DEH frequently developed in cases with persistent elevation of p-ADH. The elevation of p-ADH is likely to promote the development of DEH in cases of JUPD, although the underlying mechanism remains to be elucidated.

  19. Expression and function of striatal enriched protein tyrosine phosphatase is profoundly altered in cerebral ischemia

    Science.gov (United States)

    Braithwaite, Steven P.; Xu, Jian; Leung, John; Urfer, Roman; Nikolich, Karoly; Oksenberg, Donna; Lombroso, Paul J.; Shamloo, Mehrdad

    2009-01-01

    Striatal enriched protein tyrosine phosphatase (STEP) acts in the central nervous system to dephosphorylate a number of important proteins involved in synaptic function including ERK and NMDA receptor subunits. These proteins are also linked to stroke, in which cerebral ischemia triggers a complex cascade of events. Here we demonstrate that STEP is regulated at both the transcriptional and the post-transcriptional levels in rat models of cerebral ischemia and that its regulation may play a role in the outcome of ischemic insults. After transient middle cerebral artery occlusion, there are profound decreases in the levels of STEP mRNA, whilst in global ischemia STEP mRNA is selectively down-regulated in areas susceptible to ischemic damage. In a neuroprotective preconditioning paradigm, and in regions of the brain that are relatively resistant to ischemic damage, STEP mRNA levels are increased. Furthermore, there is a significant processing of STEP after ischemia to generate a novel species, STEP33, resulting in a redistribution of STEP from membrane-bound to soluble compartments. Concomitant with the cleavage of mature forms of STEP, there are changes in the phosphorylation state of ERK. We show that the cleavage of STEP leads to a catalytically active form, but this cleaved form no longer binds to and dephosphorylates its substrate pERK. Therefore, in response to ischemic insults, there are profound reductions in both the amount and the activity of STEP, its localization, as well as the activity of one of its key substrates, pERK. These changes in STEP may reflect a critical role in the outcomes of ischemic brain injury. PMID:18445231

  20. The effects of neuromuscular training on knee joint motor control during sidecutting in female elite soccer and handball players

    DEFF Research Database (Denmark)

    Zebis, Mette K; Bencke, Jesper; Andersen, Lars

    2008-01-01

    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. RESULTS: Neuromuscular training markedly increased before...