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Cell Mol Bioeng 2022 Jun 7;15(3):255-265. Epub 2022 Apr 7.

Department of Mechanical and Aerospace Engineering, Sapienza University of Rome, Via Eudossiana 18, 00184 Rome, Italy.

Introduction: The neuromuscular junction (NMJ) is a chemical synapse responsible for converting electrical pulses generated by the motor neuron into electrical activity in muscle fibers, and is severely impaired in various diseases, such as Amyotrophic Lateral Sclerosis (ALS). Here, we proposed a novel technique to measure, for the first time, NMJ functionality in isotonic conditions, which better reflect muscle physiological activity.

Methods: We employed the testing technique, studied a proper placing of two pairs of wire electrodes for nerve and muscle stimulation, developed an extensive testing protocol, and proposed a novel parameter, the Isotonic Neurotransmission Failure (INF), to properly capture the impairments in neurotransmission during isotonic fatigue. Read More

Acta Myol 2022 Mar 31;41(1):30-36. Epub 2022 Mar 31.

Neuromuscular Pediatric Unit, UOC di Neuropsichiatria dell'età pediatrica, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy.

Neuromuscular diseases are rare and usually chronic progressive disorders that require a multidisciplinary clinical evaluation and functional monitoring. The patient-physician relationship and therapies are also key elements to be provided. The COVID-19 pandemic dramatically changed the way patients' health was managed and national health care services underwent a radical reorganization. Read More

J Pers Med 2022 Mar 19;12(3). Epub 2022 Mar 19.

Department of Medical and Surgical Sciences, University of Catanzaro "Magna Graecia", 88100 Catanzaro, Italy.

There is a lack of data on patient and diagnostic factors for prognostication of complete recovery in patients with peripheral facial palsy. Thus, the aim of this study was to evaluate the role of a telerehabilitave enhancement through the description of a case report with the use of short-wave diathermy and neuromuscular electrical stimulation combined to facial proprioceptive neuromuscular facilitation (PNF) rehabilitation in unrecovered facial palsy, in a COVID-19 pandemic scenario describing a paradigmatic telerehabilitation report. A 43-year-old woman underwent a facial rehabilitation plan consisting of a synergistic treatment with facial PNF rehabilitation, short-wave diathermy, and neuromuscular electrical stimulation (12 sessions lasting 45 min, three sessions/week for 4 weeks). Read More

Front Med (Lausanne) 2022 21;9:827416. Epub 2022 Feb 21.

Faculty of Health and Medical Sciences, The University of Western Australia, Perth, WA, Australia.

ATP Binding Cassette Subfamily A Member 3 (ABCA-3) is a lipid transporter protein highly expressed in type-II alveolar (AT-II) cells. Mutations in can result in severe respiratory disease in infants and children. To study ABCA-3 deficiency , primary AT-II cells would be the cell culture of choice although sample accessibility is limited. Read More

Clin Orthop Relat Res 2022 06 23;480(6):1191-1204. Epub 2022 Feb 23.

Clinical Laboratory for Bionic Extremity Reconstruction, Department of Plastic, Reconstructive and Aesthetic Surgery, Medical University of Vienna, Vienna, Austria.

Background: Currently used prosthetic solutions in upper extremity amputation have limited functionality, owing to low information transfer rates of neuromuscular interfacing. Although surgical innovations have expanded the functional potential of the residual limb, available interfaces are inefficacious in translating this potential into improved prosthetic control. There is currently no implantable solution for functional interfacing in extremity amputation which offers long-term stability, high information transfer rates, and is applicable for all levels of limb loss. Read More

J Vis Exp 2021 12 8(178). Epub 2021 Dec 8.

Genethon, 91000, Evry, France; Université Paris-Saclay, Univ Evry, Inserm, Genethon, Integrare research unit UMR_S951, 91000, Evry, France;

Neuromuscular junctions (NMJs) are highly specialized synapses between lower motor neurons and skeletal muscle fibers that play an essential role in the transmission of molecules from the nervous system to voluntary muscles, leading to contraction. They are affected in many human diseases, including inherited neuromuscular disorders such as Duchenne muscular dystrophy (DMD), congenital myasthenic syndromes (CMS), spinal muscular atrophy (SMA), and amyotrophic lateral sclerosis (ALS). Therefore, monitoring the morphology of neuromuscular junctions and their alterations in disease mouse models represents a valuable tool for pathological studies and preclinical assessment of therapeutic approaches. Read More

BMC Neurol 2021 Sep 25;21(1):371. Epub 2021 Sep 25.

Department of Neurosurgery, the First Affiliated Hospital of Nanchang University, No.17, Yongwai Street, Jiangxi Province, 330006, Nanchang, China.

Background: Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder of neuromuscular transmission. The objective was to examine the efficacy and safety of 3,4-diaminopyridine (3,4-DAP) in patients with LEMS.

Methods: We searched several databases to identify relevant studies, including PubMed, EMBASE, Web of Science, MEDLINE, Cochrane Neuromuscular Disease Group Specialized Register and the Cochrane Central Register of Controlled Trials(CENTRAL). Read More

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 05 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

Risk Anal 2021 02 21;41(2):273-288. Epub 2020 Aug 21.

Institute for Disease Modeling, P.O. Box 23350, Seattle, WA, 98102.

In Pakistan, annual poliovirus investment decisions drive quantities of supplemental immunization campaigns districts receive. In this article, we assess whether increased spending on poliovirus surveillance is associated with greater likelihood of correctly identifying districts at high risk of polio with assignment of an elevated "risk ranking." We reviewed programmatic documents from Pakistan for the period from 2012-2017, recording whether districts had been classified as "high risk" or "low risk" in each year. 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