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IEEE Trans Neural Syst Rehabil Eng 2020 Jul;28(7):1642-1650

Spasticity is a major impairment that can occur following a hemispheric stroke and is often treated with injections of botulinum toxin, a neurotoxin that impairs transmission at the neuromuscular junction. Hyperreflexia is a defining feature of spasticity. Our main objective here was to quantify the time course of changes in the deep tendon reflex (DTR) responses and voluntary activation capacity following BT injection as well as to track changes in a clinical assessment of spasticity. Read More

Sci Rep 2019 12 27;9(1):20241. Epub 2019 Dec 27.

Institut de Radioprotection et de Sûreté Nucléaire (IRSN), PSE-ENV/SRTE/LECO, Cadarache, Saint-Paul-lez-Durance, 13115, France.

Contamination of the environment after the Chernobyl and Fukushima Daiichi nuclear power plant (NPP) disasters led to the exposure of a large number of humans and wild animals to radioactive substances. However, the sub-lethal consequences induced by these absorbed radiological doses remain understudied and the long-term biological impacts largely unknown. We assessed the biological effects of chronic exposure to ionizing radiation (IR) on embryonic development by exposing zebrafish embryo from fertilization and up to 120 hours post-fertilization (hpf) at dose rates of 0. Read More

Mar Drugs 2019 Jul 20;17(7). Epub 2019 Jul 20.

Mitochondrial and Cardiovascular Pathophysiology (MITOVASC), Cardiovascular Mechanotransduction, UMR CNRS 6015, INSERM U1083, Angers University, 49045 Angers, France.

Pinnatoxins (PnTXs) are emerging neurotoxins that were discovered about 30 years ago. They are solely produced by the marine dinoflagellate , and may be transferred into the food chain, as they have been found in various marine invertebrates, including bivalves. No human intoxication has been reported to date although acute toxicity was induced by PnTxs in rodents. Read More

Handb Clin Neurol 2019 ;160:303-310

Department of Neurology, Duke University School of Medicine, Durham, NC, United States. Electronic address:

Single fiber electromyography (SFEMG) is a highly selective technique that permits assessment of individual muscle fiber action potentials (MFAPs). This selectivity is achieved with a specialized concentric needle electrode with a 25-μm diameter recording surface located in a side port 3mm from the needle tip. Additional selectivity is achieved with 500-Hz low-frequency filtering. Read More

Elife 2019 06 10;8. Epub 2019 Jun 10.

Department of Anatomy and Cell Biology, University of Iowa Carver College of Medicine, Iowa City, United States.

Synapses and circuits rely on neuroplasticity to adjust output and meet physiological needs. Forms of homeostatic synaptic plasticity impart stability at synapses by countering destabilizing perturbations. The larval neuromuscular junction (NMJ) is a model synapse with robust expression of homeostatic plasticity. Read More

J Allergy Clin Immunol Pract 2019 Jul - Aug;7(6):1715-1723. Epub 2019 Mar 27.

Department of Respiratory Medicine, King's College Hospital NHS Foundation Trust, London, United Kingdom; Department of Respiratory Medicine, Charing Cross Hospital, Imperial College Healthcare NHS Trust, London, United Kingdom. Electronic address:

The accurate and consistent assessment of cough is essential not only for optimum standards of clinical care but also to drive forward advances in our understanding of cough. A range of tools for the subjective and objective measurement of cough exists, and their ongoing development has coincided with growth in clinical and preclinical research in cough, resulting in novel findings and promising new treatments. The most widely used and most fruitful approaches to assessing cough are discussed, along with some newer and preliminary techniques and directions for the future. Read More

Ann Indian Acad Neurol 2019 Jan-Mar;22(1):91-95

Department of Medicine, Jawaharlal Nehru Medical College and KLES Dr. Prabhakar Kore Hospital and MRC, Belagavi, Karnataka, India.

Introduction: Acute organophosphate (OP) poisoning is one of the most common poisoning causing significant morbidity and mortality in developing countries. Acute cholinergic manifestations predominate with many patients requiring intensive care management and ventilator support. Nerve conduction studies including repetitive nerve stimulation can evaluate the altered neuromuscular transmission and peripheral nerve function by OPs. Read More

Adv Exp Med Biol 2018;1088:47-72

National Institute of Health and Nutrition, National Institutes of Biomedical Innovation, Health and Nutrition Tokyo, Tokyo, Japan.

Skeletal muscle mass (SMM) and muscle strengh reach their peak in 20s to 40s of age in human life and then decrease with advancing age. The decrease rate of muscle strength or power was twice to four times as large as that of the SMM. Thus, the normalized muscle force (muscle strength divided by SMM) also decreases in aging. Read More

Front Pharmacol 2018 30;9:969. Epub 2018 Aug 30.

Department of Neurology, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Wenzhou, China.

Myasthenia gravis (MG) is an acquired autoimmune disease with the disorder of the neuromuscular junction transmission caused by autoantibodies. Currently, various Chinese herbal medicines (CHMs) are widely used for MG. This meta-analysis was conducted to assess the effectiveness and safety of CHMs for MG and its possible mechanisms. Read More

Curr Anesthesiol Rep 2018 20;8(2):178-185. Epub 2018 Mar 20.

Anesthesiology and Critical Care Medicine, Onze-Lieve-Vrouw Ziekenhuis, Moorselbaan 164, 9300 Aalst, Belgium.

Purpose Of Review: The purpose of this review is to assess how sugammadex impacts postoperative residual curarization using appropriate doses based on neuromuscular transmission monitoring and whether the advantages of sugammadex versus neostigmine outweigh its higher cost.

Recent Findings: An accurate assessment of neuromuscular blockade with monitoring is necessary before selecting neostigmine versus sugammadex for reversal at the end of surgery to overcome incomplete neuromuscular recovery. The main advantages of sugammadex over neostigmine are its predictability and its ability to extend the range of blockade reversal. Read More

Int J Mol Sci 2018 Feb 22;19(2). Epub 2018 Feb 22.

Department of Clinical Neurophysiology, Great Ormond Street Hospital for Children National Health Service (NHS) Foundation Trust, London WC1N 3JH, UK.

The function of the neuromuscular junction in children is amenable to electrophysiological testing. Of the two tests available, repetitive nerve stimulation is uncomfortable and has a reduced sensitivity compared with single-fibre methodology. The latter is the method of choice, recording the variability in neuromuscular transmission as a value called jitter. Read More

Front Physiol 2017 28;8:874. Epub 2017 Nov 28.

Health, Sport and Human Performance, University of Waikato, Hamilton, New Zealand.

The prevalence of compression garment (CG) use is increasing with athletes striving to take advantage of the purported benefits to recovery and performance. Here, we investigated the effect of CG on muscle force and movement velocity performance in athletes. Ten well-trained male rugby athletes wore a wrestling-style CG suit applying 13-31 mmHg of compressive pressure during a training circuit in a repeated-measures crossover design. Read More

Chin Med J (Engl) 2017 Oct;130(19):2279-2282

Department of Neurology, Chinese People's Liberation Army General Hospital, Beijing 100853, China.

Background: Congenital myasthenic syndromes (CMSs) are a group of clinically and genetically heterogeneous disorders caused by impaired neuromuscular transmission. The defect of AGRN was one of the causes of CMS through influencing the development and maintenance of neuromuscular transmission. However, CMS reports about this gene mutation were rare. Read More

eNeuro 2017 May-Jun;4(3). Epub 2017 Jul 4.

Department of Anatomy and Neuroscience, The University of Melbourne, VIC 3010, Australia.

Axonal damage and demyelination are major determinants of disability in patients with peripheral demyelinating neuropathies. The neurotrophin family of growth factors are essential for the normal development and myelination of the peripheral nervous system (PNS), and as such are potential therapeutic candidates for ameliorating axonal and myelin damage. In particular, BDNF promotes peripheral nerve myelination via p75 neurotrophin receptor (p75) receptors. Read More

Medicina (B Aires) 2017 ;77(2):117-120

Unidad de Neuroinmunología Clínica y Electrofisiología, Hospital General de Agudos Dr. J. M. Ramos Mejía, Buenos Aires, Argentina.

Myasthenia gravis is a chronic autoimmune disease that affects the neuromuscular transmission. Controversial findings had been reported about cognitive impairment in this disease. The aim of this study was to investigate the cognitive pattern of patients with myasthenia gravis. Read More

J Morphol 2017 07 26;278(7):987-996. Epub 2017 Apr 26.

Institute of Anatomy and Cell Biology, Julius-Maximilians University Wuerzburg, Koellikerstraße 6, Wuerzburg, Germany.

Drosophila melanogaster has recently emerged as model system for studying synaptic transmission and plasticity during adulthood, aging and neurodegeneration. However, still little is known about the basic neuronal mechanisms of synaptic function in the adult fly. Per se, adult Drosophila neuromuscular junctions should be highly suited for studying these aspects as they allow for genetic manipulations in combination with ultrastructural and electrophysiological analyses. Read More

Exp Neurol 2017 07 27;293:43-52. Epub 2017 Mar 27.

Neurology IV -Neuroimmunology and Neuromuscular Diseases Unit, Fondazione Istituto Neurologico "Carlo Besta", Milan 20133, Italy. Electronic address:

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by selective motor neuron degeneration in the motor cortex, brainstem and spinal cord. It is generally accepted that ALS is caused by death of motor neurons, however the exact temporal cascade of degenerative processes is not yet completely known. To identify the early pathological changes in spinal cord of G93A-SOD1 ALS mice we performed a comprehensive longitudinal analysis employing diffusion-tensor magnetic resonance imaging alongside histology and electron microscopy, in parallel with peripheral nerve histology. Read More

Crit Rev Biomed Eng 2017 ;45(1-6):187-218

Department of Anaesthesiology. Medical School, University of Tampere, and Department of Anaesthesia, Tampere University Hospital, Tampere, Finland.

Assessing adequacy of anesthesia requires evaluation of its components: hypnosis, analgesia, and neuromuscular transmission. In order to do this, many methods have been developed that process signals representing different modalities. Assessment of hypnosis requires cortical measures of the central nervous system (CNS); methods that assess analgesia concentrate on subcortical and spinal levels of the CNS; and neuromuscular transmission is a peripheral phenomenon. Read More

JAMA Neurol 2017 Jan;74(1):60-66

Program in Clinical and Translational Neuromuscular Research, Division of Neuromuscular Medicine, Department of Neurology, Yale University School of Medicine, New Haven, Connecticut.

Importance: Myasthenia gravis (MG), an autoimmune disorder of neuromuscular transmission, is treated by an array of immunotherapeutics, many of which are nonspecific. Even with current therapies, a subset of patients has medically refractory MG. The benefits of B-cell-targeted therapy with rituximab have been observed in MG; however, the duration of these benefits after treatment is unclear. Read More

Chemosphere 2016 Jun 19;152:468-80. Epub 2016 Mar 19.

Department of Biochemistry, University of Madras, Guindy Campus, Chennai, 600 025, India. Electronic address:

Rotenone is well-documented to cause neurodegenerative condition such as Parkinson's, in the exposed systems. However, its detrimental effect on particular sites of neuronal pathway is still under investigation. We aimed at elucidating the impact of rotenone on cerebral ganglions (CG) of Lumbricus terrestris which control movement and behaviour of the worms. Read More

Anesth Analg 2016 09;123(3):587-96

From the *Department of Anesthesia, Critical Care and Pain Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts; †Broad Institute of MIT and Harvard, Cambridge, Massachusetts; ‡Department of Biomedical Informatics, Harvard Medical School, Boston, Massachusetts; §Department of Clinical Pharmacy and Toxicology, Leiden University Medical Center, Leiden University, Leiden, The Netherlands; ‖Department of Psychiatry, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts; ¶Patient Care Services/Special Care Nursery, Massachusetts General Hospital, Boston, Massachusetts; and #Clinic for Anesthesiology and Intensive Care, Essen University Hospital, Essen, Germany.

Background: Neuromuscular blockade is required to control excessive muscle contractions during electroconvulsive therapy (ECT). In a crossover, assessor-blinded, prospective randomized study, we studied the minimum effective dose (MED) of succinylcholine and rocuronium for ECT. The MED was the lowest dose to provide a predefined qualitative measure of acceptable control of muscle strength during induced convulsions. Read More

Brain 2016 Feb;139(Pt 2):380-91

3 Institute of Neurology, University College London and MRC Centre for Neuromuscular Disease, Queen Square, UK 5 Neurogenetics Unit, National Hospital for Neurology, Queen Square, UK.

Ion channel dysfunction causes a range of neurological disorders by altering transmembrane ion fluxes, neuronal or muscle excitability, and neurotransmitter release. Genetic neuronal channelopathies affecting peripheral axons provide a unique opportunity to examine the impact of dysfunction of a single channel subtype in detail in vivo. Episodic ataxia type 2 is caused by mutations in CACNA1A, which encodes the pore-forming subunit of the neuronal voltage-gated calcium channel Cav2. Read More

Can J Anaesth 2016 Jun 10;63(6):709-17. Epub 2016 Feb 10.

Department of Anesthesia and Intensive Care Medicine, University of Rostock, Rostock, Germany.

Purpose: Positioning for surgery can restrict access to the patient's hand, thereby limiting assessment of the response at the adductor pollicis muscle to ulnar nerve stimulation. We evaluated a novel site to assess neuromuscular block by stimulating the accessory nerve and measuring the acceleromyographic response at the trapezius muscle.

Methods: In this prospective non-blinded observational study, we assessed neuromuscular transmission in anesthetized adult female patients undergoing elective laparoscopic gynecological surgery. Read More

J Hand Ther 2016 Apr-Jun;29(2):154-65. Epub 2015 Dec 12.

Department of Rehabilitation and Regenerative Medicine, Columbia University, New York, NY, USA.

Unlabelled: This clinical review discusses the organization, neuroanatomy, assessment, clinical relevance, and rehabilitation of sensorimotor (SM) control impairment after wrist trauma. The wrist SM control system encompasses complex SM pathways that control normal wrist active range of motion and mediate wrist joint neuromuscular stability for maintaining joint function. Among various known assessment methods of wrist SM control impairment, the active wrist joint position sense test is determined to be a clinically meaningful and responsive measure for wrist SM control impairment after wrist fracture. Read More

Neurology 2015 Dec 30;85(22):1964-71. Epub 2015 Oct 30.

From the Institute of Neuroscience (R.G.W., B.A.S.H.) and John Walton Muscular Dystrophy Research Centre, Institute of Genetic Medicine (B.B., R.M.L., R.H., H.L.), Newcastle University, Newcastle, UK; Department of Neurology (D.N.H., E.L.L., J.E.S.), University of Rochester Medical Center, NY; Department of Neurology (J.L.A.), Dartmouth Hitchcock Clinic, Geisel School of Medicine, Hanover, NH; The Picower Institute for Learning and Memory (J.T.L.), Department of Biology and Department of Brain and Cognitive Sciences, Massachusetts Institute of Technology, Cambridge, MA; and Dr. John T. Macdonald Department of Human Genetics and Hussman Institute for Human Genomics (S.Z.), University of Miami, Miller School of Medicine, Miami, FL.

Objectives: To describe the clinical and electrophysiologic features of synaptotagmin II (SYT2) mutations, a novel neuromuscular syndrome characterized by foot deformities and fatigable ocular and lower limb weakness, and the response to modulators of acetylcholine release.

Methods: We performed detailed clinical and neurophysiologic assessment in 2 multigenerational families with dominant SYT2 mutations (c.920T>G [p. Read More

J Vis Exp 2015 Sep 13(103). Epub 2015 Sep 13.

INSERM U1093, Faculty of Sport Sciences, Univ. Bourgogne Franche-Comté

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. Read More

Comp Biochem Physiol C Toxicol Pharmacol 2015 Oct-Nov;176-177:52-61. Epub 2015 Jul 29.

Department of Biology, University of Kentucky, USA; Lexington, KY, USA. Electronic address:

The commonly used mood altering drug fluoxetine (Prozac) in humans has a low occurrence in reports of harmful effects from overdose; however, individuals with altered metabolism of the drug and accidental overdose have led to critical conditions and even death. We addressed direct actions of high concentrations on synaptic transmission at neuromuscular junctions (NMJs), neural properties, and cardiac function unrelated to fluoxetine's action as a selective 5-HT reuptake inhibitor. There appears to be action in blocking action potentials in crayfish axons, enhanced occurrences of spontaneous synaptic vesicle fusion events in the presynaptic terminals at NMJs of both Drosophila and crayfish. Read More

Int J Clin Exp Med 2015 15;8(2):2392-8. Epub 2015 Feb 15.

Key Laboratory of Hand Reconstruction, Ministry of Health Shanghai 200032, China ; Shanghai Key Laboratory of Peripheral Nerve and Microsurgery Shanghai 200032, China ; Department of Hand Surgery, Huashan Hospital, Fudan University Shanghai 200040, China.

There are 2 critical steps in neural regeneration: nerve fibres successfully crossing the suture and restoration of neuromuscular transmission. For the second step, the compound muscle action potential (CMAP) is the standard electrophysiological technique used to assess regeneration, but it is difficult to detect changes in the CMAP during early regeneration after nerve repair. There is a need for better, noninvasive quantitative electrophysiological techniques to assess regeneration in an earlier stage after nerve repair. Read More

Ann Clin Transl Neurol 2015 Apr 16;2(4):362-72. Epub 2015 Feb 16.

Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Univ Paris 06, UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM F-75013, Paris, France ; APHP, Hôpital Pitié-Salpêtrière, Centre de référence de pathologie neuromusculaire Paris-Est, Institut de Myologie Paris, France.

Objective: Data from mouse models of amyotrophic lateral sclerosis (ALS) suggest early morphological changes in neuromuscular junctions (NMJs), with loss of nerve-muscle contact. Overexpression of the neurite outgrowth inhibitor Nogo-A in muscle may play a role in this loss of endplate innervation.

Methods: We used confocal and electron microscopy to study the structure of the NMJs in muscle samples collected from nine ALS patients (five early-stage patients and four long-term survivors). Read More

Clin Neurophysiol 2015 Dec 12;126(12):2381-9. Epub 2015 Feb 12.

School of Kinesiology, Faculty of Health Sciences, Western University, London, Ontario, Canada; Department of Physical Medicine and Rehabilitation, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada; Department of Clinical Neurological Sciences, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada. Electronic address:

Objective: To study the potential utility of using near fiber (NF) jiggle as an assessment of neuromuscular transmission stability in healthy older subjects using decomposition-based quantitative electromyography (DQEMG).

Methods: The tibialis anterior (TA) and vastus medialis (VM) muscles were tested in 9 older men (77 ± 5 years) and 9 young male control subjects (23 ± 0.3 years). Read More

J Neurosci 2015 Jan;35(1):325-38

Department of Cell Biology, Emory University, Atlanta, Georgia 30322, Center for Social Translational Neuroscience, Emory University, Atlanta, Georgia 30322

Neurodevelopmental disorders arise from single or multiple gene defects. However, the way multiple loci interact to modify phenotypic outcomes remains poorly understood. Here, we studied phenotypes associated with mutations in the schizophrenia susceptibility gene dysbindin (dysb), in isolation or in combination with null alleles in the dysb network component Blos1. Read More

PLoS One 2014 22;9(12):e115754. Epub 2014 Dec 22.

Klinik für Anaesthesiologie, Technische Universität München, Klinikum rechts der Isar, Ismaninger Str. 22, 81675, München, Germany.

Spontaneous reinnervation after diaphragmatic paralysis due to trauma, surgery, tumors and spinal cord injuries is frequently observed. A possible explanation could be collateral reinnervation, since the diaphragm is commonly double-innervated by the (accessory) phrenic nerve. Permutation entropy (PeEn), a complexity measure for time series, may reflect a functional state of neuromuscular transmission by quantifying the complexity of interactions across neural and muscular networks. Read More

Curr Pharm Des 2015 ;21(4):484-90

Department of Medicine, Division of Cardiology, McMaster University, 50 Charlton Ave East, Hamilton, Ontario, Canada, L8N 4A6.

We have gained considerable insight and understanding about the etiology, embryogenesis of the myocardium, genetic background, diagnosis and outcome of left ventricular non-compaction (LVNC) over the last 2 decades. LVNC has a distinct morphological appearance with a thickened, two-layered myocardium consisting of an epicardial compacted and a thicker endocardial non-compacted layer. These features make the recognition with non-invasive imaging modalities highly feasible. Read More

Neurol Neurochir Pol 2014 10;48(5):337-41. Epub 2014 Sep 10.

Department of Neurology, Medical University of Warsaw, Warsaw, Poland.

Background: The main hypotheses regarding mechanisms of transient global amnesia (TGA) are ischemia in hippocampal structures, epileptic genesis, and migraine. In accordance with the hypothesis of a shared, common pathophysiological mechanism in both TGA and migraine, neuromuscular transmission (NMT) abnormalities previously found in migraine were also suspected in TGA.

Objective: The aim of our study was to analyze NMT in TGA patients to reveal a subclinical impairment of neuromuscular transmission as a possible indicator of underlying channelopathy, which would point to a shared etiology with migraine. Read More

Clin Neurophysiol 2015 Apr 18;126(4):794-802. Epub 2014 Aug 18.

School of Kinesiology, Faculty of Health Sciences, University of Western Ontario, London, Ontario, Canada; Department of Anatomy and Cell Biology, Schulich School of Medicine and Dentistry, University of Western Ontario, London, Ontario, Canada.

Objective: To assess the degree of neuromuscular transmission variability and motor unit (MU) remodelling in patients with diabetic polyneuropathy (DPN) using decomposition-based quantitative electromyography (DQEMG) and near fibre (NF) motor unit potential (MUP) parameters.

Methods: The tibialis anterior (TA) muscle was tested in 12 patients with DPN (65 ± 15 years) and 12 controls (63 ± 15 years). DQEMG was used to analyze electromyographic (EMG) signals collected during voluntary contractions. Read More

J Biomech 2014 Sep 2;47(12):2858-62. Epub 2014 Aug 2.

Human Performance Laboratory, Faculty of Kinesiology, University of Calgary, Calgary, AB, Canada.

In order to evaluate potential risks of whole-body vibration (WBV) training, it is important to understand the transfer of vibrations from the WBV platform to the muscles. Therefore, the purpose of this study was to quantify the transmissibility of vibrations from the WBV platform to the triceps surae and quadriceps soft tissue compartments. Sixteen healthy, male participants were exposed to side-altering WBV at 2. Read More

IEEE Trans Neural Syst Rehabil Eng 2014 Jul 12;22(4):810-9. Epub 2014 Feb 12.

Targeted muscle reinnervation (TMR) redirects nerves that have lost their target, due to amputation, to remaining muscles in the region of the stump with the intent of establishing intuitive myosignals to control a complex prosthetic device. In order to directly recover the neural code underlying an attempted limb movement, in this paper, we present the decomposition of high-density surface electromyographic (EMG) signals detected from three TMR patients into the individual motor unit spike trains. The aim was to prove, for the first time, the feasibility of decoding the neural drive that would reach muscles of the missing limb in TMR patients, to show the accuracy of the decoding, and to demonstrate the representativeness of the pool of extracted motor units. Read More

Hosp Pract (1995) 2013 Oct-Nov;41(4):40-50

Division of the Neuromuscular Center, Department of Neurology, Cleveland Clinic, Cleveland, OH.

Myasthenia gravis is an antibody-mediated disorder of neuromuscular transmission that is characterized by weakness and fatigue of voluntary muscles. Weakness may be ocular, bulbar, or generalized. Diagnostic evaluation of patients consists of bedside assessment, antibody testing, and electrophysiologic studies. Read More

Clin Neurophysiol 2014 Jan 8;125(1):166-9. Epub 2013 Jul 8.

Translational Clinical Neurophysiology Unit, Instituto de Medicina Molecular and Institute of Physiology, Faculty of Medicine, University of Lisbon, Portugal; Departments of Neurology and Neuroscience, Royal London Hospital, Queen Mary University of London, London, UK.

Objectives: We aimed to identify the most appropriate MUP parameter to evaluate reinnervation in very early ALS.

Methods: We studied tibialis anterior (TA), initially of normal strength with normal MUP analysis parameters, in 15 patients with ALS of recent onset. They were studied at the initial diagnostic assessment, and then 3 and 6 months later. Read More

J Child Neurol 2014 Mar 4;29(3):389-93. Epub 2013 Jan 4.

1Unit of Clinical Neurophysiology, Department of Neurological Sciences, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Congenital myasthenic syndromes are inherited disorders caused by various defects in neuromuscular transmission. Although the typical presentation is fatigable weakness with prominent cranial involvement, neonates can lack these hallmark manifestations, and in those with choline acetyltransferase gene mutations, basal electrophysiological testing can yield negative findings. The authors report the case of a male infant presenting at birth with oculomotor and bulbofacial weakness, hypotonia, clubfoot, and severe respiratory insufficiency. Read More

J Sports Sci 2012 31;30(14):1561-9. Epub 2012 Aug 31.

Rostock University, Sport Science, Ulmenstr, 69, House 2, Rostock, 18057, Germany.

Evidence suggests that during interaction with different environmental dynamics the necessity to stabilise the involved joints leads to altered efficiency in force transmission to the surroundings and a specific orchestration of motor control strategies. However, little is known about the modalities of the changes associated with altered environmental dynamics. In 29 healthy participants, electromyographic (EMG) signals from four muscles of the right leg (M. Read More

J Inflamm (Lond) 2012 May 29;9(1):20. Epub 2012 May 29.

Department of Neurology & Psychiatry, 1438 South Grand Boulevard, Saint Louis University School of Medicine, Saint Louis, MO, 63104, USA.

Unlabelled:

Background: Myasthenia gravis (MG) and animal model of experimental autoimmune myasthenia gravis (EAMG) is the most common autoimmune disorder of neuromuscular transmission. The disease is caused by the breakdown of the acetylcholine receptor (AChR) which is largely due to complement activation at the neuromuscular junction (NMJ). Limited knowledge exists to the extent that complement receptor 1-related gene/protein y deficiency (Crry -/-) modulates the adaptive immune response and EAMG outcome. Read More

Brain Nerve 2011 Jul;63(7):713-7

Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.

The neuromuscular junction (NMJ) is a specialized synapse with a complex structural organization. Muscle contraction involves several steps: (1) nerve conduction to depolarize the motor nerve terminals, (2) opening of voltage-gated calcium channels (VGCCs) in the presynaptic membrane, (3) generation of endplate potential in the postsynaptic membrane via acetylcholine receptors, (4) depolarization of muscle sodium channels, and (5) excitation-contraction (E-C) coupling. Each step can be affected by various diseases. Read More

Cell Mol Neurobiol 2011 Aug 3;31(6):909-20. Epub 2011 May 3.

Neurobehavioral Toxicology Branch, Analytical Toxicology Division, US Army Medical Research Institute of Chemical Defense, Aberdeen Proving Ground, MD 21010-5400, USA.

Electrophysiological and ultrastructural studies were performed on phrenic nerve-hemidiaphragm preparations isolated from wild-type and acetylcholinesterase (AChE) knockout (KO) mice to determine the compensatory mechanisms manifested by the neuromuscular junction to excess acetylcholine (ACh). The diaphragm was selected since it is the primary muscle of respiration, and it must adapt to allow for survival of the organism in the absence of AChE. Nerve-elicited muscle contractions, miniature endplate potentials (MEPPs) and evoked endplate potentials (EPPs) were recorded by conventional electrophysiological techniques from phrenic nerve-hemidiaphragm preparations isolated from 1. Read More

Gastrointest Endosc 2011 May;73(5):1029-34

Institut National de la Santé et de la Recherche Médicale U913, Université de Nantes Faculté des Sciences et Techniques, Institut des Maladies de l'Appareil Digestif, CHU de Nantes, Nantes, France.

Background: Better understanding of the pathophysiological mechanisms involved in severe dysmotility disorders is crucial to improve patient management and identify novel therapeutic targets. Recent studies suggested that endoscopic full-thickness biopsies (eFTBs) could be developed as an alternative to surgical biopsies. However, currently it remains unknown whether eFTBs would allow myenteric plexus analysis on whole mounts and the evaluation of neuromuscular transmission. Read More

Adv Physiol Educ 2011 Mar;35(1):82-91

Department of Zoology, University of Cambridge, Cambridge, United Kingdom.

Here we incorporate recent advances in Drosophila neurogenetics and "optogenetics" into neuroscience laboratory exercises. We used the light-activated ion channel channelrhodopsin-2 (ChR2) and tissue-specific genetic expression techniques to study the neural basis of behavior in Drosophila larvae. We designed and implemented exercises using inexpensive, easy-to-use systems for delivering blue light pulses with fine temporal control. Read More

GMS Health Technol Assess 2010 Jun 14;6:Doc08. Epub 2010 Jun 14.

Centre of pneumology and thoracic surgery, Asklepios Hospital Gauting, Department of intensive-care medicine and long-term ventilation, Gauting, Germany.

Introduction And Background: Invasive home mechanical ventilation is used for patients with chronic respiratory insufficiency. This elaborate and technology-dependent ventilation is carried out via an artificial airway (tracheal cannula) to the trachea. Exact numbers about the incidence of home mechanical ventilation are not available. Read More

Synapse 2011 Apr 17;65(4):287-99. Epub 2010 Sep 17.

Department of Biology, University of Kentucky, Lexington, Kentucky 40506-0225, USA.

The release of transmitter at neuromuscular junctions (NMJ) of the opener muscle in crayfish is quantal in nature. This NMJ offers the advantage of being able to record quantal events at specific visually identified release sites, thus allowing measurement of the physiological parameters of vesicle release and its response to be directly correlated with synaptic structure. These experiments take advantage of areas between the varicosities on the nerve terminal that we define as "stems. Read More

Clin Neurophysiol 2011 Mar 31;122(3):440-455. Epub 2010 Jul 31.

Department of Clinical Neurophysiology, Aarhus University Hospital, Aarhus, Denmark.

The aim of this review is to present the status of electrodiagnostic standards and guidelines in neuromuscular disorders. Electrodiagnostic guidelines are developed on the background of medical technology assessment, wherefore a short presentation of medical technology assessment is given covering: (1) Evidence-based medicine, i.e. Read More