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Neurol Res Pract 2021 May 4;3(1):23. Epub 2021 May 4.

Klinik und Poliklinik für Hör-, Stimm- und Sprachheilkunde, Universitäres Dysphagiezentrum Hamburg, Universitätsklinikum Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany.

Introduction: Neurogenic dysphagia defines swallowing disorders caused by diseases of the central and peripheral nervous system, neuromuscular transmission, or muscles. Neurogenic dysphagia is one of the most common and at the same time most dangerous symptoms of many neurological diseases. Its most important sequelae include aspiration pneumonia, malnutrition and dehydration, and affected patients more often require long-term care and are exposed to an increased mortality. Read More

Biomedicines 2021 Apr 24;9(5). Epub 2021 Apr 24.

Department of Pharmaceutical and Pharmacological Sciences, University of Padova, 35131 Padova, Italy.

Antidopaminergic gastrointestinal prokinetics are indeed commonly used to treat gastrointestinal motility disorders, although the precise role of dopaminergic transmission in the gut is still unclear. Since dopamine transporter (DAT) is involved in several brain disorders by modulating extracellular dopamine in the central nervous system, this study evaluated the impact of DAT genetic reduction on the morpho-functional integrity of mouse small intestine enteric nervous system (ENS). In DAT heterozygous (DAT) and wild-type (DAT) mice (14 ± 2 weeks) alterations in small intestinal contractility were evaluated by isometrical assessment of neuromuscular responses to receptor and non-receptor-mediated stimuli. Read More

Neurobiol Aging 2021 May 5;101:285-296. Epub 2021 Feb 5.

Department of Neurology, Neuromuscular Division, The Ohio State University Wexner Medical Center, Columbus, OH, USA; Department of Physical Medicine and Rehabilitation, The Ohio State University Wexner Medical Center, Columbus, OH, USA; Department of Neuroscience, The Ohio State University Wexner Medical Center, Columbus, OH, USA; Department of Physiology and Cell Biology, The Ohio State University Wexner Medical Center, Columbus, OH, USA. Electronic address:

Sarcopenia, or pathological loss of muscle mass and strength during aging, is an important contributor to loss of physical function in older adults. Sarcopenia is a multifactorial syndrome associated with intrinsic muscle and upstream neurological dysfunction. Exercise is well-established as an effective intervention for sarcopenia, but less is known about the long-term neurobiological impact of exercise. Read More

Ideggyogy Sz 2021 Jan;74(1-2):33-40

Istanbul University Cerrahpasa Medical Faculty, Neurology Department, Istanbul, Turkey.

Background And Purpose: Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular transmission. Autonomic dysfunction is not a commonly known association with MG. We conducted this study to evaluate autonomic functions in MG & subgroups and to investigate the effects of acetylcholinesterase inhibitors. Read More

Front Hum Neurosci 2020 7;14:560860. Epub 2020 Dec 7.

Department of Neuropediatrics, Developmental Neurology and Social Pediatrics, University Children's Hospital Essen, University Duisburg-Essen, Essen, Germany.

: Congenital myasthenic syndromes (CMS) refer to a heterogenic group of neuromuscular transmission disorders. CMS-subtypes are diverse regarding exercise intolerance and muscular weakness, varying from mild symptoms to life-limiting forms with neonatal onset. Long-term follow-up studies on disease progression and treatment-response in pediatric patients are rare. Read More

Open Med (Wars) 2020 13;15(1):556-562. Epub 2020 Jun 13.

Department of Plastic, Endocrine and General Surgery, Pomeranian Medical University, Szczecin, Poland.

Background: Many factors contribute to successful nerve reconstruction. The correct technique of anastomosis is one of the key elements that determine the final result of a surgery. The aim of this study is to examine how useful an electromyography (EMG) can be as an objective intraoperative anastomosis assessment method. Read More

Neuromuscul Disord 2020 11 14;30(11):897-903. Epub 2020 Oct 14.

Department of Neurology, Faculdade de Medicina da Universidade de São Paulo (FMUSP), Av. Dr. Enéas Carvalho Aguiar 255, 05403-900 São Paulo SP, Brazil. Electronic address:

This study was designed to analyze the sensitivity, specificity, and accuracy of jitter parameters combined with repetitive nerve stimulation (RNS) in congenital myasthenic syndrome (CMS), chronic progressive external ophthalmoplegia (CPEO), and congenital myopathies (CM). Jitter was obtained with a concentric needle electrode during voluntary activation of the Orbicularis Oculi muscle in CMS (n = 21), CPEO (n = 20), and CM (n = 18) patients and in controls (n = 14). RNS (3 Hz) was performed in six different muscles for all patients (Abductor Digiti Minimi, Tibialis Anterior, upper Trapezius, Deltoideus, Orbicularis Oculi, and Nasalis). Read More

IEEE Trans Neural Syst Rehabil Eng 2020 07;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

Muscle Nerve 2020 09 10;62(3):333-343. Epub 2020 Jun 10.

Department of Neurosciences, Case Western Reserve University, Cleveland, Ohio.

Introduction: Our aim in this study was to identify the prevalence and clinical characteristics of LRP4/agrin-antibody-positive double-seronegative myasthenia gravis (DNMG).

Methods: DNMG patients at 16 sites in the United States were tested for LRP4 and agrin antibodies, and the clinical data were collected.

Results: Of 181 DNMG patients, 27 (14. Read More

Anesth Analg 2020 08;131(2):378-386

From the Department of Anesthesia, Critical Care and Pain Medicine.

The morbidity, mortality, and blistering pace of transmission of infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has led to an unprecedented worldwide health crisis. Coronavirus disease 2019 (COVID-19), the disease produced by SARS-CoV-2 infection, is remarkable for persistent, severe respiratory failure requiring mechanical ventilation that places considerable strain on critical care resources. Because recovery from COVID-19-associated respiratory failure can be prolonged, tracheostomy may facilitate patient management and optimize the use of mechanical ventilators. Read More

Anesth Analg 2020 08;131(2):351-364

Department of Anesthesia, McGill University, Montreal, Canada.

Health care systems are belligerently responding to the new coronavirus disease 2019 (COVID-19). The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a specific condition, whose distinctive features are severe hypoxemia associated with (>50% of cases) normal respiratory system compliance. When a patient requires intubation and invasive ventilation, the outcome is poor, and the length of stay in the intensive care unit (ICU) is usually 2 or 3 weeks. 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