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Clinical electrophysiology

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https://www.readbyqxmd.com/read/28542676/detailed-clinical-phenotype-and-molecular-genetic-findings-in-cln3-associated-isolated-retinal-degeneration
#1
Cristy A Ku, Sarah Hull, Gavin Arno, Ajoy Vincent, Keren Carss, Robert Kayton, Douglas Weeks, Glenn W Anderson, Ryan Geraets, Camille Parker, David A Pearce, Michel Michaelides, Robert E MacLaren, Anthony G Robson, Graham E Holder, Elise Heon, F Lucy Raymond, Anthony T Moore, Andrew R Webster, Mark E Pennesi
Importance: Mutations in genes traditionally associated with syndromic retinal disease are increasingly found to cause nonsyndromic inherited retinal degenerations. Mutations in CLN3 are classically associated with juvenile neuronal ceroid lipofuscinosis, a rare neurodegenerative disease with early retinal degeneration and progressive neurologic deterioration, but have recently also been identified in patients with nonsyndromic inherited retinal degenerations. To our knowledge, detailed clinical characterization of such cases has yet to be reported...
May 25, 2017: JAMA Ophthalmology
https://www.readbyqxmd.com/read/28542544/development-and-characterization-of-an-experimental-model-of-diet-induced-metabolic-syndrome-in-rabbit
#2
Oscar Julián Arias-Mutis, Vannina G Marrachelli, Amparo Ruiz-Saurí, Antonio Alberola, Jose Manuel Morales, Luis Such-Miquel, Daniel Monleon, Francisco J Chorro, Luis Such, Manuel Zarzoso
Metabolic syndrome (MetS) has become one of the main concerns for public health because of its link to cardiovascular disease. Murine models have been used to study the effect of MetS on the cardiovascular system, but they have limitations for studying cardiac electrophysiology. In contrast, the rabbit cardiac electrophysiology is similar to human, but a detailed characterization of the different components of MetS in this animal is still needed. Our objective was to develop and characterize a diet-induced experimental model of MetS that allows the study of cardiovascular remodeling and arrhythmogenesis...
2017: PloS One
https://www.readbyqxmd.com/read/28538254/acute-demyelinating-polyneuropathy-as-presentation-of-hereditary-neuropathy-with-liability-to-pressure-palsies-in-a-patient-who-exercised-regularly-in-the-army
#3
Malathi Perugula, Gulshan Uppal, Miguel Chuquilin
Acute generalized neuropathy as the presenting manifestation of hereditary neuropathy with liability to pressure palsies (HNPP) is rare. We report a 19-year-old Army recruit who exercised regularly for 9 months and presented with 2 weeks of numbness, tingling, and weakness in both upper and lower extremities, starting 2 weeks after influenza vaccination and 1 day after vigorous exercise. Based on acute onset, clinical examination and electrophysiological findings, Guillain-Barre syndrome was diagnosed, and intravenous immunoglobulin was administered with minimal improvement...
June 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28538252/concurrent-paraspinous-myopathy-and-myasthenia-gravis
#4
Alissa E Romano, Zaid Al-Qudah, Henry J Kaminski, Bashar Katirji, Karim Salame
Paraspinous myopathy is a rare neuromuscular disorder characterized by selective involvement of the cervical, thoracic, or lumbar muscles. Leading clinical features include a bent spine or dropped head (antecollis). In myasthenia gravis (MG), patients may have camptocormia secondary to neuromuscular junction dysfunction of the paraspinal muscles, and this condition usually responds to acetylcholinesterase inhibitors or immunosuppressive treatments. However, concomitant MG and paraspinous myopathy with histologic and electrophysiologic evidence of myopathic changes of the paraspinal muscles has only been reported twice in the literature...
June 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28537000/advances-in-clinical-cardiology-2016-a-summary-of-the-key-clinical-trials
#5
REVIEW
Alastair Gray, Conor McQuillan, Ian B A Menown
INTRODUCTION: The findings of many new cardiology clinical trials over the last year have been published or presented at major international meetings. This paper aims to describe and place in context a summary of the key clinical trials in cardiology presented between January and December 2016. METHODS: The authors reviewed clinical trials presented at major cardiology conferences during 2016 including the American College of Cardiology (ACC), European Association for Percutaneous Cardiovascular Interventions (EuroPCR), European Society of Cardiology (ESC), European Association for the Study of Diabetes (EASD), Transcatheter Cardiovascular Therapeutics (TCT), and the American Heart Association (AHA)...
May 23, 2017: Advances in Therapy
https://www.readbyqxmd.com/read/28536920/clinicopathological-features-of-multiple-mononeuropathy-associated-with-systemic-lupus%C3%A2-erythematosus-a-multicenter-study
#6
Elodie Rivière, Fleur Cohen Aubart, Thierry Maisonobe, François Maurier, Christophe Richez, Bruno Gombert, Marie Gousseff, Daniel Adoue, Alexis Mathian, Miguel Hié, Julien Haroche, Zahir Amoura
Multiple mononeuropathy (MM) occurs rarely during systemic lupus erythematosus (SLE) but may lead to major disability. The aim of this study was to investigate the clinic-pathological presentations of MM during SLE, as well as long-term outcomes. We conducted a multicentric retrospective study that included patients receiving a diagnosis of MM during SLE. Ten patients were included (8 women and 2 men, median age at MM diagnosis: 40.4 years). SLE was diagnosed before MM in 9/10 patients (median time 8.2 years)...
May 23, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28536523/positive-modulatory-interactions-of-nmda-receptor-glun1-2b-ligand-binding-domains-attenuate-antagonists-activity
#7
Douglas Bledsoe, Ceyhun Tamer, Ivana Mesic, Christian Madry, Bradley G Klein, Bodo Laube, Blaise M Costa
N-methyl D-aspartate receptors (NMDAR) play crucial role in normal brain function and pathogenesis of neurodegenerative and psychiatric disorders. Functional tetra-heteromeric NMDAR contains two obligatory GluN1 subunits and two identical or different non-GluN1 subunits that include six different gene products; four GluN2 (A-D) and two GluN3 (A-B) subunits. The heterogeneity of subunit combination facilities the distinct function of NMDARs. All GluN subunits contain an extracellular N-terminal Domain (NTD) and ligand binding domain (LBD), transmembrane domain (TMD) and an intracellular C-terminal domain (CTD)...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28534728/hearing-loss-and-age-induced-changes-in-the-central-auditory-system-measured-by-the-p3-response-to-small-changes-in-frequency
#8
Kathy R Vander Werff, Kerrie L Nesbitt
BACKGROUND: Recent behavioral studies have suggested that individuals with sloping audiograms exhibit localized improvements in frequency discrimination in the frequency region near the drop in hearing. Auditory-evoked potentials may provide evidence of such cortical plasticity and reorganization of frequency maps. PURPOSE: The objective of this study was to evaluate electrophysiological evidence of cortical plasticity related to cortical frequency representation and discrimination abilities in older individuals with high-frequency sensorineural hearing loss (SNHL)...
May 2017: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/28529158/evaluation-of-direct-and-cell-mediated-triple-gene-therapy-in-spinal-cord-injury-in-rats
#9
Rustem Robertovich Islamov, Andrey Alexandrovich Izmailov, Mikhail Evgenyevich Sokolov, Philip Olegovich Fadeev, Farid Vagizovich Bashirov, Anton Alexandrovich Eremeev, Gulnara Ferdinantovna Shaymardanova, Maxim Michaylovich Shmarov, Boris Savelyevich Naroditskiy, Yuri Alexandrovich Chelyshev, Igor Aleksandrovich Lavrov, András Palotás
Current treatment options for spinal cord injury (SCI) are scarce. One of the most promising innovative approaches include gene-therapy, however no single gene has so far been shown to be of clinical relevance. This study investigates the efficacy of various combinations of vascular endothelial growth factor (VEGF), glial cell-derived neurotrophic factor (GDNF), angiogenin (ANG) and neuronal cell adhesion molecule (NCAM) in rats. Multiple therapeutic genes were administered intrathecally either via adenoviral vectors or by using genetically modified human umbilical cord blood mononuclear cells (hUCBMCs)...
May 18, 2017: Brain Research Bulletin
https://www.readbyqxmd.com/read/28527317/fully-automatic-segmentation-of-left-ventricular-anatomy-in-3-d-lge-mri
#10
Tanja Kurzendorfer, Christoph Forman, Michaela Schmidt, Christoph Tillmanns, Andreas Maier, Alexander Brost
The current challenge for electrophysiology procedures, targeting the left ventricle, is the localization and qualification of myocardial scar. Late gadolinium enhanced magnetic resonance imaging (LGE-MRI) is the current gold standard to visualize regions of myocardial infarction. Commonly, a stack of 2-D images is acquired of the left ventricle in short-axis orientation. Recently, 3-D LGE-MRI methods were proposed that continuously cover the whole heart with a high resolution within a single acquisition. The acquisition promises an accurate quantification of the myocardium to the extent of myocardial scarring...
May 8, 2017: Computerized Medical Imaging and Graphics: the Official Journal of the Computerized Medical Imaging Society
https://www.readbyqxmd.com/read/28525682/secondary-sulcus-fixed-foldable-iol-implantation-with-25-g-infusion-in-patients-with-previous-ppv-after-open-globe-injury
#11
Tiangeng He, Caiyun You, Song Chen, Xiangda Meng, Yuanyuan Liu, Hua Yan
PURPOSE: To evaluate the safety and efficacy of secondary sulcus-fixed foldable intraocular lens (IOL) implantation through a clear corneal incision with 25-G infusion in patients with previous pars plana vitrectomy (PPV) after open-globe injury, and to analyze postoperative outcomes and prognostic factors of treatment. METHODS: Clinical data of 89 eyes of 89 patients with open-globe injury who underwent secondary sulcus-fixed foldable IOL implantation through a clear corneal incision with 25-G infusion after vitrectomy in our hospital between January 2008 and June 2015 were retrospectively analyzed...
May 16, 2017: European Journal of Ophthalmology
https://www.readbyqxmd.com/read/28525585/the-effects-of-gender-on-electrical-therapies-for-the-heart-physiology-epidemiology-and-access-to-therapies-a-report-from-the-xii-congress-of-the-italian-association-on-arrhythmology-and-cardiostimulation-aiac
#12
Giuseppe Boriani, Stefano Lorenzetti, Elisabetta Cerbai, Giuseppe Oreto, Gabriele Bronzetti, Vincenzo Livio Malavasi, Alessandro Biffi, Luigi Padeletti, Gianluca Botto, Igor Diemberger
The difference between men and women is clear even just by looking at an electrocardiogram: females present higher resting heart rate, a shorter QRS complex length and greater corrected QT interval. The development of these differences from pubertal age onward suggests that sexual hormones play a key role, although their effect is far from being completely understood. Different incidences between sexes have been reported for many arrhythmias, both ventricular and supraventricular, and also for sudden cardiac death...
May 19, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28514704/guillain-barr%C3%A3-syndrome-variants-forms-fruste-reclassification-with-new-criteria
#13
Fu Liong Hiew, Rahmansah Ramlan, Shanthi Viswanathan, Santhi Puvanarajah
OBJECTIVES: This study aimed to evaluate the clinical and electrophysiological characteristics of various distinctive classical and localised Guillain-Barré syndrome (GBS) subtypes. PATIENTS AND METHODS: Clinical characteristics and electrophysiological data of sixty-one consecutive patients admitted between 2012 and 2015 were systematically analysed and reclassified according to the new GBS clinical classification. Neurophysiology was evaluated with Hadden et al...
May 4, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28512434/evaluation-of-a-rapid-anisotropic-model-for-ecg-simulation
#14
Simone Pezzuto, Peter Kal'avský, Mark Potse, Frits W Prinzen, Angelo Auricchio, Rolf Krause
State-of-the-art cardiac electrophysiology models that are able to deliver physiologically motivated activation maps and electrocardiograms (ECGs) can only be solved on high-performance computing architectures. This makes it nearly impossible to adopt such models in clinical practice. ECG imaging tools typically rely on simplified models, but these neglect the anisotropic electric conductivity of the tissue in the forward problem. Moreover, their results are often confined to the heart-torso interface. We propose a forward model that fully accounts for the anisotropic tissue conductivity and produces the standard 12-lead ECG in a few seconds...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28509703/pathway-for-the-management-of-atrial-fibrillation-and-atrial-flutter
#15
Eyal Herzog, Edgar Argulian, Steven B Levy, Emad F Aziz
Atrial fibrillation (AF) is the most common cardiac rhythm disturbance encountered in clinical practice, and its prevalence is increasing as the population ages. The American College of Cardiology, the American Heart Association, and the European Society of Cardiology established guidelines for the management of patients with AF. Atrial flutter (Afl) is less common and is often associated with or preceded by AF or occurs in an isolated pattern. A major limitation of the currently published guidelines for the management of patients with AF and Afl is their complexity, the fact that official guidelines are published separately for each of these arrhythmias, and that they were published several years ago...
June 2017: Critical Pathways in Cardiology
https://www.readbyqxmd.com/read/28509608/differential-diagnosis-and-recovery-of-acute-bilateral-foot-drop-in-a-patient-with-a-history-of-low-back-pain-a-case-report
#16
Melanie Lomaglio, Bob Canale
BACKGROUND AND PURPOSE: Acute bilateral foot drop is rare and may be due to peripheral or central lesions. The purpose of this case report was to describe the differential diagnosis and recovery of a patient with low back pain (LBP) that awoke with bilateral foot drop. CASE DESCRIPTION: A 39-year-old man with a history of LBP awoke with a steppage gait pattern. Spinal imaging and tapping were negative for sinister pathologies. A subsequent history taken by the physical therapist uncovered that the patient had previously taken a narcotic and slept in a kneeling position to relieve his LBP...
May 16, 2017: Physiotherapy Theory and Practice
https://www.readbyqxmd.com/read/28508737/the-future-for-long-chain-n-3-pufa-in-the-prevention-of-coronary-heart-disease-do-we-need-to-target-non-fish-eaters
#17
W L Hall
Dietary guidelines in many countries include a recommendation to consume oily fish, mainly on the basis of evidence from prospective cohort studies that fish consumption is cardioprotective. However, average intakes are very low in a large proportion of the UK population. Some groups, such as vegans and vegetarians, purposely omit fish (along with meat) from their diet resulting in zero or trace intakes of long chain (LC) n-3 PUFA. Although the efficacy of dietary fish oil supplementation in the prevention of CVD has been questioned in recent years, the balance of evidence indicates that LC n-3 PUFA exert systemic pleiotropic effects through their influence on gene expression, cell signalling, membrane fluidity and by conversion to specialised proresolving mediators; autacoid lipid mediators that resolve inflammatory events...
May 16, 2017: Proceedings of the Nutrition Society
https://www.readbyqxmd.com/read/28507738/mark-e-josephson-clinical-investigator
#18
EDITORIAL
John M Miller
Mark E Josephson entered the world of clinical cardiac electrophysiology (EP) almost at its inception (1972); with so much to learn and so many directions one could take, he dived into the field with unbridled enthusiasm and an uncommon - perhaps almost unique - aptitude for asking questions and finding ways to answer them. Few aspects of EP escaped his indelible influence. In this short paper, I will attempt to touch on some of the high points of his astounding career as a clinical investigator.
April 2017: Arrhythmia & Electrophysiology Review
https://www.readbyqxmd.com/read/28507528/cognitive-impairments-in-occupational-burnout-error-processing-and-its-indices-of-reactive-and-proactive-control
#19
Krystyna Golonka, Justyna Mojsa-Kaja, Magda Gawlowska, Katarzyna Popiel
The presented study refers to cognitive aspects of burnout as the effects of long-term work-related stress. The purpose of the study was to investigate electrophysiological correlates of burnout to explain the mechanisms of the core burnout symptoms: exhaustion and depersonalization/cynicism. The analyzed error-related electrophysiological markers shed light on impaired cognitive mechanisms and the specific changes in information-processing in burnout. In the EEG study design (N = 80), two components of error-related potential (ERP), error-related negativity (ERN), and error positivity (Pe), were analyzed...
2017: Frontiers in Psychology
https://www.readbyqxmd.com/read/28506602/ventricular-tachycardia-score-a-novel-method-for-wide-qrs-complex-tachycardia-differentiation-explained
#20
Marek Jastrzębski, Piotr Kukla, Danuta Czarnecka
Philosophy, merits and limitations of a novel method for wide QRS complex tachycardia differentiation, based on a scoring system and called the ventricular tachycardia (VT) score, were explained. The following criteria were assigned one point: initial dominant R wave in V1; initial r>40 ms in V1 or V2; notched S in V1; initial R wave in aVR; lead II RWPT≥50 ms; and absence of an RS in leads V1-V6. Atrioventricular dissociation (including fusion/capture beats and partial dissociation) was assigned two points...
April 25, 2017: Journal of Electrocardiology
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