82,866 results match your criteria Neuromuscular Scoliosis
Hum Mov Sci 2018 Dec 12;63:148-155. Epub 2018 Dec 12.
Laboratory of Neuromuscular Biomechanics, School of International Liberal Studies, Chukyo University, Nagoya, Japan. Electronic address:
Introduction: The present study investigated the effect of stretchable characteristics of elastic therapeutic tape and its elongation on surface electromyography (EMG) of knee extensor muscles during knee extension movements.
Methods: Nine healthy men performed knee extension movement with the application of normal elastic tape or highly stretchable tape and without the tapes (control). Tapes were applied on the anterior thigh to cross the knee joint with no elongation and elongation of 50 and 75% of the maximum stretchability. Read More
Arthritis Rheumatol 2018 Dec 15. Epub 2018 Dec 15.
Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy.
Objectives: Our aim was to evaluate expression of type I interferon (IFNα/β)- and type II IFN (IFNγ)-inducible genes in muscle biopsies of JDM patients and to correlate their expression levels with histological and clinical features.
Methods: Expression levels of IFN-inducible genes and pro-inflammatory cytokines were assessed by quantitative PCR in muscle biopsies of JDM patients (n = 39), Duchenne muscular dystrophy (DMD) patients (n = 24) and healthy controls (HCs) (n = 4). Histological and immunofluorescence studies were performed on muscle biopsy sections. Read More
Eur J Nucl Med Mol Imaging 2018 Dec 14. Epub 2018 Dec 14.
SyMPTOm PET/MRI Platform, Nuclear Medicine Department, Henri Mondor University Hospital, Assistance Publique-Hôpitaux de Paris, 51 avenue du Maréchal de Lattre de Tassigny, 94010, Créteil, France.
Eur J Hum Genet 2018 Dec 14. Epub 2018 Dec 14.
CHRU Montpellier, Laboratoire de Génétique moléculaire, Montpellier, France.
Next-generation sequencing (NGS) gene-panel-based analyses constitute diagnosis strategies which are adapted to the genetic heterogeneity within the field of myopathies, including more than 200 implicated genes to date. Nonetheless, important inter-laboratory diversity of gene panels exists at national and international levels, complicating the exchange of data and the visibility of the diagnostic offers available for referring neurologists. To address this issue, we here describe the initiative of the genetic diagnosis section of the French National Network for Rare Neuromuscular Diseases (Filière Nationale des Maladies Rares Neuromusculaires, FILNEMUS), which led to set up a consensual nationwide diagnostic strategy among the nine French genetic diagnosis laboratories using NGS for myopathies. Read More
BMJ Open 2018 Dec 14;8(12):e025123. Epub 2018 Dec 14.
Lysholm Department of Neuroradiology, The National Hospital for Neurology and Neurosurgery UCL Hospitals NHS Trust, London, UK.
Introduction: Central nervous system (CNS) gliomas are the most common primary intra-axial brain tumours and pose variable treatment response according to their grade, therefore, precise staging is mandatory. Histopathological analysis of surgical tumour samples is still deemed as the state-of-the-art staging technique for gliomas due to the moderate specificity of the available non-invasive imaging modalities. A recently evolved analysis of the tissue water diffusion properties, known as diffusional kurtosis imaging (DKI), is a dimensionless metric, which quantifies water molecules' degree of non-Gaussian diffusion, hence reflects tissue microenvironment's complexity by means of non-invasive diffusion-weighted MRI acquisitions. Read More
EMBO Mol Med 2018 Dec 14. Epub 2018 Dec 14.
MRC-Mitochondrial Biology Unit, University of Cambridge, Cambridge, UK
Loss-of-function mutations in , a gene exclusively found in higher eukaryotes, cause a characteristic type of cavitating leukoencephalopathy associated with mitochondrial cytochrome oxidase (COX) deficiency. Although the genetic association of APOPT1 pathogenic variants with isolated COX defects is now clear, the biochemical link between APOPT1 function and COX has remained elusive. We investigated the molecular role of APOPT1 using different approaches. Read More
J Pers Med 2018 Dec 13;8(4). Epub 2018 Dec 13.
Northern Ireland Centre for Stratified Medicine, Altnagelvin Hospital Campus, Ulster University, Londonderry, BT47 6SB, Northern Ireland, UK.
Multiple genes and mechanisms of pathophysiology have been implicated in amyotrophic lateral sclerosis (ALS), suggesting it is a complex systemic disease. With this in mind, applying personalized medicine (PM) approaches to tailor treatment pipelines for ALS patients may be necessary. The modelling and analysis of molecular interaction networks could represent valuable resources in defining ALS-associated pathways and discovering novel therapeutic targets. Read More
Sleep 2018 Dec 14. Epub 2018 Dec 14.
Institute of Epidemiology and Social Medicine, Westfälische Wilhelms-Universität Münster, Münster, Germany.
Study Objectives: Periodic limb movements in sleep (PLMS) are frequent motor phenomena; however, population-based data are scarce. We assessed the prevalence of PLMS and factors associated with PLMS within two German population-based cohorts, the SHIP-TREND and BiDirect.
Methods: Single-night polysomnography was performed on 1107 subjects recruited from the general population (mean age: 52. Read More
J Electromyogr Kinesiol 2018 Dec 4;44:94-100. Epub 2018 Dec 4.
Human Performance Lab, Schulthess Clinic, Zurich, Switzerland. Electronic address:
Aim of this study was to investigate if knee extensor maximal voluntary contraction (MVC) torque and rate of torque development (RTD) deficits are accompanied by neuromuscular activation failure in patients with knee osteoarthritis (KOA). Nineteen patients with unilateral KOA completed gradual MVCs, from which MVC torque, voluntary activation and maximal EMG activity were recorded, and explosive MVCs, from which RTD and rate of EMG rise were recorded. For gradual MVCs, MVC torque (-28%), voluntary activation (-6%) and maximal EMG activity (-30%) were lower on the involved than on the uninvolved side (p < 0. Read More
Anesth Analg 2019 Jan;128(1):e16
Vet Med Sci 2018 Dec 13. Epub 2018 Dec 13.
Department of Internal Medicine, Faculty of Veterinary Medicine, University of Tehran, Tehran, Iran.
Tetanus is an acute, often fatal, infectious neuromuscular disease in all farmed mammals caused by Clostridium tetani. The disease is sporadic but outbreaks of tetanus have been described, as a result of wound contaminated with spores of C. tetani, which sporulates to the vegetative form and produce toxins. Read More
Muscle Nerve 2018 Dec 13. Epub 2018 Dec 13.
Department of Neurology, Aarhus University Hospital, Aarhus, Denmark.
Introduction: In Guillain-Barré Syndrome (GBS), patients often develop muscle atrophy from denervation and immobilization. We therefore conducted a pilot study of neuromuscular electrical stimulation (NMES) to evaluate feasibility, safety, and effect on muscle wasting in the early phase of GBS.
Methods: Seventeen patients were randomized to receive 20 minutes of muscle fiber stimulation followed by 40 minutes of MNES of the right or left quadriceps muscle with the untreated side as control. Read More
Compr Physiol 2018 Dec 13;9(1):127-148. Epub 2018 Dec 13.
Neuroscience and Mental Health Institute (NMHI), School of Molecular & Systems Medicine, University of Alberta, Edmonton, Alberta, Canada.
Neuroprostheses (NPs) are electrical stimulators that activate nerves, either to provide sensory input to the central nervous system (sensory NPs), or to activate muscles (motor NPs: MNPs). The first MNPs were belts with inbuilt batteries and electrodes developed in the 1850s to exercise the abdominal muscles. They became enormously popular among the general public, but as a result of exaggerated therapeutic claims they were soon discredited by the medical community. Read More
Curr Protoc Stem Cell Biol 2018 Dec 12:e75. Epub 2018 Dec 12.
Department of Cellular and Molecular Medicine, University of Ottawa, Ottawa, Ontario, Canada.
We have established an in vitro model of the human congenital heart defect (CHD)-associated mutation NKX2.5 R141C. We describe the use of the hanging drop method to differentiate Nkx2. Read More
Pediatr Pulmonol 2018 Dec 12. Epub 2018 Dec 12.
Department of Pediatrics, Ohio State University, Columbus, Ohio.
Background: Spinal Muscular Atrophy type 1 (SMA1) is a rare genetic neuromuscular disease where 75% of SMA1 patients die/require permanent-ventilation by 13.6 months. This study assessed the health outcomes of SMA1 infants treated with AVXS-101 gene replacement therapy. Read More
Acta Anaesthesiol Scand 2018 Dec 11. Epub 2018 Dec 11.
Department of Anaesthesiology, Herlev and Gentofte Hospital, University of Copenhagen, Herlev, Denmark.
Background: Mivacurium is a short-acting non-depolarizing muscle relaxant, which is hydrolyzed by butyrylcholinesterase. The neuromuscular block (NMB) can be antagonized with cholinesterase inhibitors (CHEI), but the short duration of action of mivacurium questions the need. This systematic review evaluated if the use of CHEIs (neostigmine, pyridostigmine or edrophonium) facilitates reversal of mivacurium-induced NMB. Read More
Pediatr Pulmonol 2018 Dec 11. Epub 2018 Dec 11.
Department of Pediatric Rehabilitation Medicine, Kennedy Krieger Institute, Baltimore, Maryland.
Objective: The objective of this retrospective review was to determine the utility of polysomnography (PSG) in influencing the decision to decannulate pediatric patients with brain and spinal cord injuries in an inpatient rehabilitation hospital setting.
Methods: Between 2010 and 2016, data were collected on pediatric patients with brain and/or spinal cord injuries who had PSG performed with the goal of decannulation. Patients underwent a decannulation protocol involving toleration of continuous tracheostomy capping and bedside tracheoscopy by otolaryngology. Read More
EJNMMI Res 2018 Dec 13;8(1):110. Epub 2018 Dec 13.
Department of Nuclear Medicine and Molecular Imaging, Division of Nuclear Medicine, KU Leuven, Herestraat 49, 3000, Leuven, Belgium.
Background: F-FDG brain PET measures metabolic changes in neurodegenerative disorders and may discriminate between different diseases even at an early stage. The objective of this study was to classify patients with amyotrophic lateral sclerosis (ALS) and Parkinson plus syndromes (PP). To this end, different approaches were evaluated using generalized linear models and corresponding glucose metabolic brain patterns. Read More
eNeurologicalSci 2018 Dec 22;13:38-39. Epub 2018 Nov 22.
Department of Neurology, University of Florida, Gainesville, FL 32610, United States.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired disorder of peripheral nerves and nerve roots. Its cause is unknown, but recently antibodies to nodal and paranodal proteins have been discovered in a small subset of CIDP patients. These contactin and neurofascin-related immune-mediated neuropathies are thought to be variants of CIDP and often respond suboptimally to standard therapy. Read More
Radiol Case Rep 2019 Mar 5;14(3):309-314. Epub 2018 Dec 5.
Department of Medical, Surgical, Neurological, Metabolic Sciences, and Aging, 2nd Division of Neurology, Center for Rare Diseases and InterUniversity Center for Research in Neurosciences, University of Campania "Luigi Vanvitelli", Via Sergio Pansini, 80131 Naples, Italy.
X-linked adrenoleukodystrophy (X-ALD) is a rare inherited metabolic disease affecting the nervous system and the adrenal glands. It is caused by a mutation of the gene, resulting in the impaired degradation of very long-chain fatty acids and their subsequent accumulation in several organs and tissues. X-ALD is notable for its high phenotypical variability, that includes isolated adrenocortical insufficiency, slowly progressive myelopathy with paraparesis, ataxia, and peripheral neuropathy to severe childhood cerebral forms. Read More
Front Neurol 2018 28;9:1027. Epub 2018 Nov 28.
Molecular Genetics Laboratory UILDM, Santa Lucia Foundation, Rome, Italy.
Facioscapulohumeral muscular dystrophy (FSHD) is a neuromuscular disorder which is typically transmitted by an autosomal dominant pattern, although reduced penetrance and sporadic cases caused by mutations, are often observed. FSHD may be caused by a contraction of a repetitive element, located on chromosome 4 (4q35). This locus is named and consists of 11 to more than 100 repeated units (RU). Read More
Nat Commun 2018 Dec 13;9(1):5303. Epub 2018 Dec 13.
MIT Media Lab, Center for Extreme Bionics, Massachusetts Institute of Technology, Cambridge, 02139, MA, USA.
Optogenetics has been used to orchestrate temporal- and tissue-specific control of neural tissues and offers a wealth of unique advantages for neuromuscular control. Here, we establish a closed-loop functional optogenetic stimulation (CL-FOS) system to control ankle joint position in murine models. Using the measurement of either joint angle or fascicle length as a feedback signal, we compare the controllability of CL-FOS to closed-loop functional electrical stimulation (CL-FES) and demonstrate significantly greater accuracy, lower rise times and lower overshoot percentages. Read More
Orv Hetil 2018 Dec;159(50):2144-2149
Fogászati és Szájsebészeti Klinika, Pécsi Tudományegyetem, Klinikai Központ Pécs, Dischka Győző u. 5., 7621.
From an anatomic and functional point of view, the stomatognathic system and the upper cervical spine are closely connected. Together with the complex neuromuscular relationships, this generates an important field of cooperation between dentists and orthopedics. The aim of this case report was to demonstrate the improvements of temporomandibular joint (TMJ) function after orthopedic surgery in case of a patient with idiopathic scoliosis. Read More
Nutrients 2018 Dec 7;10(12). Epub 2018 Dec 7.
Research Centre for Musculoskeletal Science & Sports Medicine, Department of Exercise and Sport Science, Manchester Metropolitan University Cheshire, Crewe CW1 5DU, UK.
The aim of this study was to determine the response to an oral glucose tolerance test (OGTT) in adult males with Becker muscular dystrophy (BMD) and Duchenne muscular dystrophy (DMD), and to investigate whether body composition contributes to any variance in the glucose response. Twenty-eight adult males with dystrophinopathy (BMD, = 13; DMD, = 15) and 12 non-dystrophic controls, ingested 75 g oral anhydrous glucose solution. Fingertip capillary samples were assessed for glucose at 30-min intervals over 2-h post glucose ingestion. Read More
Medicine (Baltimore) 2018 Dec;97(49):e13460
Federal University of Rio de Janeiro.
Rationale: Myasthenia gravis (MG) is an uncommon autoimmune disease mediated by antibodies that attack the postsynaptic acetylcholine receptors (AchRs) at the neuromuscular junction, causing fluctuating muscle weakness, aggravated with use, and relieved with rest.
Patient Concerns: A 32-year-old woman with a diagnosis of MG based on clinical findings, eletroneuromyography, and brain magnetic resonance imaging (MRI) was admitted to our hospital with dysphagia, dysphonia, diplopia, and intense weakness, associated with a 2-day viral gastroenteritis.
Diagnoses: Physical examination revealed globally reduced deep tendon reflexes, and tetraparesis, with muscle strength grade 4 in the left limbs and grade 2 in the right limbs. Read More
Medicine (Baltimore) 2018 Dec;97(49):e13424
Department of Surgery.
Purpose: Many studies have demonstrated the advantage of maintaining intraoperative deep neuromuscular block (NMB) with sugammadex. This trial is designed to evaluate the impact of muscle relaxation during laparoscopic subtotal gastrectomy on the oncological benefits, particularly in obese patients with gastric cancer.
Materials And Methods: This is a double-blind, randomized controlled multicenter prospective trial. Read More
Clin Immunol 2018 Dec 10. Epub 2018 Dec 10.
Department of Medicine A, Tel Hashomer, Israel; Sackler School of Medicine, Ramat Aviv, Israel; The Zabludowicz Center for Autoimmune Diseases, Tel-Hashomer, Israel; The Dr. Pinchas Borenstein Talpiot Medical Leadership Program 2013, Sheba Medical Center, Tel-Hashomer, Israel. Electronic address:
Background: Paraneoplastic neurological syndromes (PNS) are a group of syndromes that affect the central and peripheral neuromuscular system in association with cancer. Specific antibodies may assist in the diagnosis of PNS. The antibodies tested can be classified into those directed against intracellular neuronal proteins ("well characterized" PNS: Hu, Yo, RI, CV2, amphiphysin, Ma1, Ma2) and those directed against neural surface antigens (autoimmune encephalitis syndromes: NMDA, AMPA, LGI1, CASPR2, GABAR). Read More
PLoS Genet 2018 Dec 13;14(12):e1007845. Epub 2018 Dec 13.
Institute of Molecular Biology and Medicine, Laboratory Biology of the Cell Nucleus, Université Libre de Bruxelles (ULB), Gosselies, Belgium.
Nucleoporins build the nuclear pore complex (NPC), which, as sole gate for nuclear-cytoplasmic exchange, is of outmost importance for normal cell function. Defects in the process of nucleocytoplasmic transport or in its machinery have been frequently described in human diseases, such as cancer and neurodegenerative disorders, but only in a few cases of developmental disorders. Here we report biallelic mutations in the nucleoporin NUP88 as a novel cause of lethal fetal akinesia deformation sequence (FADS) in two families. Read More
Am J Physiol Gastrointest Liver Physiol 2018 Dec 13. Epub 2018 Dec 13.
Cattedra di Gastroenterologia, IRCCS Ospedale Maggiore, Italy.
Background: Jackhammer esophagus (JE) is a hypercontractile disorder, the pathogenesis of which is incompletely understood. Multiple rapid swallows (MRS) and rapid drink challenge (RDC) are complementary tests used during high resolution manometry (HRM) that evaluate inhibitory and excitatory neuromuscular function and latent obstruction respectively. Methods&aim. Read More
J Athl Train 2018 Dec 13. Epub 2018 Dec 13.
Department of Rehabilitation Sciences, School of Health Professions, Texas Tech University, Lubbock.
Context: The abdominal-bracing maneuver, a volitional preemptive abdominal contraction (VPAC) strategy, is commonly used during resisted shoulder exercises. How VPAC affects shoulder-muscle function during resisted shoulder exercise is unknown.
Objective: To identify the effects of VPAC on selected parascapular and glenohumeral muscles during specific shoulder exercises with or without resistance. Read More
Sports Med 2018 Dec 12. Epub 2018 Dec 12.
Sports Performance Research Institute New Zealand (SPRINZ), Auckland University of Technology, Auckland, New Zealand.
Ther Adv Neurol Disord 2018 3;11:1756286418809588. Epub 2018 Nov 3.
Department of Neurosciences, Reproductive and Odontostomatological Sciences, University Federico II of Naples, Naples, Italy.
Background: Ataluren was approved for the treatment of nmDMD, both the efficacy and safety have been previously reported only from clinical trials but no report exists about real-life experience.
Patient/methods: we describe three Italian children with nmDMD treated with ataluren for 1 year. Measurements were made every 3 months and was evaluated the 6-Minute Walking Distance (6MWD). Read More
Front Physiol 2018 27;9:1620. Epub 2018 Nov 27.
Technische Universität Kaiserslautern, Fachgebiet Sportwissenschaft, Kaiserslautern, Germany.
Poor posture in childhood and adolescence is held responsible for the occurrence of associated disorders in adult age. This study aimed to verify whether body posture in adolescence can be enhanced through the improvement of neuromuscular performance, attained by means of targeted strength, stretch, and body perception training, and whether any such improvement might also transition into adulthood. From a total of 84 volunteers, the posture development of 67 adolescents was checked annually between the age of 14 and 20 based on index values in three posture situations. Read More
Pediatr Pulmonol 2019 Jan 7;54(1):53-60. Epub 2018 Dec 7.
Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Objectives: Tracheostomy is indicated for very-low-birth-weight infants (VLBWIs) with prolonged respiratory problems during the perinatal period. The objective of this study is to clarify the epidemiology and risk factors in VLBWIs with tracheostomy after birth in Japan.
Methods: A total of 40 806 VLBWIs were registered in the Neonatal Research Network of Japan database from 2003 to 2012. Read More
Clin Kidney J 2018 Dec 16;11(6):822-831. Epub 2018 Jul 16.
Renal Service, Veterans Affairs Medical Center, Washington, DC, USA.
Skeletal muscle wasting has gained interest as a primary consequence of chronic kidney disease (CKD) due to the relationship between skeletal muscle mass, mortality and major adverse cardiovascular events in this population. The combination of reductions in physical function, skeletal muscle performance and skeletal muscle mass places individuals with CKD at greater risk of sarcopenia. Therefore the monitoring of skeletal muscle composition and function may provide clinical insight into disease progression. Read More
Front Neurol 2018 22;9:986. Epub 2018 Nov 22.
COGNAC-G (COGNition and ACtion Group), Université Paris Descartes-CNRS UMR-MD-SSA, Paris, France.
In the present experiments, multiple balance perturbations were provided by unpredictable support-surface translations in various directions and velocities. The aim of this study was to distinguish the passive and the active phases during the pre-impact period of a fall. It was hypothesized that it should be feasible if one uses a specific quantitative kinematic analysis to evaluate the dispersion of the body segments trajectories across trials. Read More
Front Neurol 2018 20;9:985. Epub 2018 Nov 20.
Division of Pulmonary and Critical Care Medicine, School of Medicine, Johns Hopkins University, Baltimore, MD, United States.
Invasive procedures were previously developed for measuring pharyngeal collapsibility in rodents during expiration, when declining neuromuscular activity makes the airway unstable. We developed a non-invasive approach for streamlining collapsibility measurements by characterizing responses in physiologic markers of dynamic expiratory airflow obstruction to negative nasal pressure challenges. Anesthetized mice were instrumented to monitor upper airway pressure-flow relationships with head-out plethysmography while nasal pressure was ramped down from ~ +5 to -20 cm HO over several breaths. Read More
Front Aging Neurosci 2018 22;10:387. Epub 2018 Nov 22.
School of Human Kinetics, University of Ottawa, Ottawa, ON, Canada.
Falling is one of the primary concerns for people with Parkinson's Disease and occurs predominately during dynamic movements, such as walking. Several methods have been proposed to quantify dynamic balance and to assess fall risk. However, no consensus has been reached concerning which method is most appropriate for examining walking balance during unperturbed and perturbed conditions, particularly in Parkinson's Disease individuals. Read More
Front Hum Neurosci 2018 20;12:432. Epub 2018 Nov 20.
Department of Physical Medicine and Rehabilitation, College of Medicine, Yeungnam University, Daegu, South Korea.
: Neuromuscular electrical stimulation (NMES) is a popular rehabilitative modality to improve motor function of the extremities and trunk. In this study, we investigated changes of hand function and the contralateral corticospinal tract (CST) with treatment by NMES on the finger extensor muscles for 2 weeks, using serial diffusion tensor tractography (DTT). : Thirteen right handed normal subjects were recruited. Read More
J Neural Eng 2018 Nov 1. Epub 2018 Nov 1.
Department of Bioengineering, Imperial College London, London, SW7 2AZ, UNITED KINGDOM OF GREAT BRITAIN AND NORTHERN IRELAND.
Objective: Surface electromyography (EMG) is currently used as a control signal for active prostheses in amputees who underwent targeted muscle reinnervation (TMR) surgery. Recent research has shown that it is possible to access the spiking activity of spinal motor neurons from multi-channel surface EMG. In this study, we propose the use of multi-channel epimysial EMG electrodes as an interface for decoding motor neurons activity following TMR. Read More
J Neural Eng 2018 Oct 23. Epub 2018 Oct 23.
Biomedical Engineering, Case Western Reserve University, Wickenden Building, Cleveland, Ohio, 44106, UNITED STATES.
Objective: Neuromuscular stimulation is a therapeutic approach to treat impairments such as stroke or pain, although the resulting inputs to the nervous system due to the stimulation are not well quantified. Stimulation activates both afferents and efferents, and the resulting neural activity is a mix of the effects of both: the changes in afferent activity due to efferent physiological actions plus the alterations due to afferent stimulation. This study quantitatively describes the resulting Golgi tendon organ Ib afferent activity in response to mixed afferent and efferent stimulation. Read More
Tissue Barriers 2018 Dec 7:1-22. Epub 2018 Dec 7.
a Neuromuscular Immunopathology Research Laboratory, Division of Neuromuscular Disease, Department of Neurology , University of Alabama at Birmingham , Birmingham , AL , USA.
The human blood-nerve barrier (BNB) formed by endoneurial microvascular endothelial cells, serves to maintain the internal microenvironment in peripheral nerves required for normal axonal signal transduction to and from the central nervous system. The mechanisms of human BNB formation in health and disease are not fully elucidated. Prior work established a sufficient role for glial-derived neurotrophic factor (GDNF) in enhancing human BNB biophysical properties following serum withdrawal in vitro via RET-tyrosine kinase-dependent cytoskeletal remodeling. Read More
Sleep Breath 2018 Dec 6. Epub 2018 Dec 6.
Division of Cardiology, Pulmonology and Vascular Medicine, Heinrich-Heine University Medical Center Duesseldorf, Moorenstraße 5, 40225, Düsseldorf, Germany.
Purpose: This study tested the hypothesis that a reduction of pulmonary congestion achieved by a reduction of mitral regurgitation (MR) severity in heart failure (HF) patients is associated with reduced event lengths of sleep-disordered breathing (SDB).
Methods: We prospectively enrolled 20 consecutive HF patients who underwent MitraClip implantation. Patients underwent cardiorespiratory polygraphic recording prior to and after percutaneous mitral valve repair (PMVR). Read More
JMIR Res Protoc 2018 Dec 6;7(12):e12339. Epub 2018 Dec 6.
Department of Rehabilitation Medicine, Keio University School of Medicine, Tokyo, Japan.
Background: We developed a brain-machine interface (BMI) system for poststroke patients with severe hemiplegia to detect event-related desynchronization (ERD) on scalp electroencephalogram (EEG) and to operate a motor-driven hand orthosis combined with neuromuscular electrical stimulation. ERD arises when the excitability of the ipsi-lesional sensorimotor cortex increases.
Objective: The aim of this study was to evaluate our hypothesis that motor training using this BMI system could improve severe hemiparesis that is resistant to improvement by conventional rehabilitation. Read More
Neuroimage Clin 2018 Nov 28. Epub 2018 Nov 28.
Sorbonne Université, CNRS, INSERM, Laboratoire d'Imagerie Biomédicale, Paris, France; APHP, Département de Neurologie, Hôpital Pitié-Salpêtrière, Centre référent SLA, Paris, France; Northern Ireland Centre for Stratified Medicine, Biomedical Sciences Research Institute Ulster University, C-TRIC, Altnagelvin Hospital, Derry, Londonderry, United Kingdom. Electronic address:
Spinal muscular atrophy (SMA) type III and IV are autosomal recessive, slowly progressive lower motor neuron syndromes. Nevertheless, wider cerebral involvement has been consistently reported in mouse models. The objective of this study is the characterisation of spinal and cerebral pathology in adult forms of SMA using multimodal quantitative imaging. Read More
Rev Esp Geriatr Gerontol 2018 Dec 3. Epub 2018 Dec 3.
Servicio de Geriatría, Hospital Universitario de la Santa Creu, Vic, Barcelona, España; Grupo de Investigación en Cronicidad de la Catalunya Central (C3RG), España.
Myasthenia gravis (MG), amyotrophic lateral sclerosis and Guillain-Barre syndrome (GBS) have been classically considered as exceptional or unusual diseases in people with a geriatric profile. Over the past 25 years, several population-based studies have been conducted in the Osona area (Barcelona), which, for the first time, has led to describing the high global incidences in the elderly, especially those over 80 years-old. The results suggest the possibility of underdiagnosis of these neuromuscular diseases in the elderly, a fact that could be especially relevant in the case of MG and GBS, since they are 2potentially reversible entities with high mortality in the event of underdiagnosis and absence of treatment. Read More
BMC Pulm Med 2018 Dec 6;18(1):190. Epub 2018 Dec 6.
Department of Anesthesiology and Intensive Therapy, Semmelweis University, 1082 Üllői út 78B, Budapest, Hungary.
Background: Home mechanical ventilation is an established treatment for chronic respiratory failure resulting in improved survival and quality of life. Technological advancement, evolving health care reimbursement systems and newly implemented national guidelines result in increased utilization worldwide. Prevalence shows great geographical variations and data on East-Central European practice has been scarce to date. Read More
Neuropediatrics 2018 Dec 12. Epub 2018 Dec 12.
Department of Medical Genetics, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
X-linked myotubular myopathy (XLMTM) is a rare inherited neuromuscular disorder associated with mutations in the gene on the Xq28 region. We report a severely affected girl with XLMTM, caused by maternally inherited 661 kb Xq28 microduplication identified by chromosomal microarray analysis and confirmed also on DNA from muscle biopsy with a custom-designed X-chromosome-specific microarray. X-inactivation analysis revealed a skewed inactivation pattern on the proband's muscle biopsy. Read More
Elife 2018 Dec 12;7. Epub 2018 Dec 12.
Department of Zoology, University of Cambridge, Cambridge, United Kingdom.
Reactive oxygen species (ROS) have been extensively studied as damaging agents associated with ageing and neurodegenerative conditions. Their role in the nervous system under non-pathological conditions has remained poorly understood. Working with the larval locomotor network, we show that in neurons ROS act as obligate signals required for neuronal activity-dependent structural plasticity, of both pre- and postsynaptic terminals. Read More
Dev Biol 2019 Jan 25;445(1):37-53. Epub 2018 Oct 25.
Department of Developmental and Cell Biology, School of Biological Sciences, University of California, Irvine, Irvine, CA 92697-2300, USA. Electronic address:
Analysis of mutants that affect formation and function of the Drosophila larval neuromuscular junction (NMJ) has provided valuable insight into genes required for neuronal branching and synaptic growth. We report that NMJ development in Drosophila requires both the Drosophila ortholog of FNDC3 genes; CG42389 (herein referred to as miles to go; mtgo), and CCT3, which encodes a chaperonin complex subunit. Loss of mtgo function causes late pupal lethality with most animals unable to escape the pupal case, while rare escapers exhibit an ataxic gait and reduced lifespan. Read More