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Clinical electrophysiology

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https://www.readbyqxmd.com/read/30006059/novel-mutations-in-hint1-gene-cause-the-autosomal-recessive-axonal-neuropathy-with-neuromyotonia
#1
Zhangyang Wang, Jie Lin, Kai Qiao, Shuang Cai, Victor W Zhang, Chongbo Zhao, Jiahong Lu
Autosomal recessive axonal neuropathy with neuromyotonia (ARAN-NM) is a rare form of hereditary neuropathy. Mutations in HINT1 gene have been identified to be the cause of this disorder. We report two unrelated patients who presented gait impairment, progressive distal muscle weakness and atrophy, neuromyotonia and foot deformities. Electrophysiological studies showed axonal motor neuropathy and neuromyotonic discharges. Using Next-generation sequencing, we identified two homozygous mutations, NM_005340.6: c...
July 10, 2018: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/30005736/effect-of-electroacupuncture-on-porcine-cardiac-excitability-induced-by-left-stellate-ganglion-stimulation
#2
Tatsuo Takamiya, Yukiko Kubo, Peyman Benharash, Wei Zhou
Augmentation of cardiac sympathetic tone has been shown to induce ventricular arrhythmias. Acupuncture has been clinically used to treat hypertension, angina pectoris, and atrial arrhythmias. However, the effects of acupuncture on ventricular electrophysiology and autonomic tone remain unknown. We hypothesized that acupuncture attenuates cardiac excitability and corrects the imbalance of autonomic tone during sympathetic hyperactivity. Fourteen Yorkshire pigs were randomized to electroacupuncture (EA, 2 Hz, 0...
September 2018: Autonomic Neuroscience: Basic & Clinical
https://www.readbyqxmd.com/read/30004124/central-sensitization-of-the-spino-parabrachial-amygdala-pathway-that-outlasts-a-brief-nociceptive-stimulus
#3
Sarah A Kissiwaa, Elena E Bagley
Pain is an important defence against dangers in our environment, however some clinical conditions produce pain that outlasts this useful role and persist even after the injury has healed. The experience of pain consists of somatosensory elements of intensity and location, negative emotional/aversive feelings and subsequent restrictions on lifestyle due to learned association between certain activities and pain. The amygdala contributes negative emotional value to nociceptive sensory information and forms the association between an aversive response and the environment in which it occurs...
July 13, 2018: Journal of Physiology
https://www.readbyqxmd.com/read/30003857/diagnostic-biomarkers-of-epilepsy
#4
Chiara Sueri, Sara Gasparini, Simona Balestrini, Angelo Labate, Antonio Gambardella, Emilio Russo, Antonio Leo, Silvia Casarotto, Francesca Pittau, Michele Trimboli, Vittoria Cianci, Michele Ascoli, Salvatore M Cavalli, Giulia Ferrigno, Umberto Aguglia, Edoardo Ferlazzo
BACKGROUND: diagnostic biomarkers of epilepsy are objectively measurable variables associated to the development of epilepsy or the propensity to generate seizures. Identification of biomarkers could be helpful for differential diagnosis and for tailored therapeutic approaches. OBJECTIVE: this review focuses on diagnostic biomarkers of epilepsy, including genetic, serological, neuroimaging and electrophysiological variables. METHODS: references were mainly identified through PubMed search until December 2017 and backtracking of references in pertinent studies...
July 12, 2018: Current Pharmaceutical Biotechnology
https://www.readbyqxmd.com/read/30003814/review-article-gfrr-r-2017-0429-r1-oxidative-stress-and-atrial-fibrillation-an-update
#5
Panagiotis Korantzopoulos, Konstantinos Letsas, Nikolaos Fragakis, Gary Tse, Tong Liu
Atrial remodeling involves electrophysiological and structural abnormalities that promote the development and perpetuation of atrial fibrillation. Experimental and clinical data indicate that oxidative stress is implicated in the pathophysiology of atrial remodeling. The mechanistic links between atrial remodeling and oxidative stress are complex with several underlying diseases and conditions may affect these pathways. Therefore, the development of antioxidant interventions in this setting remains difficult...
July 13, 2018: Free Radical Research
https://www.readbyqxmd.com/read/30002598/complement-c5a-alters-the-membrane-potential-of-neutrophils-during-hemorrhagic-shock
#6
David A C Messerer, Stephanie Denk, Karl J Föhr, Rebecca Halbgebauer, Christian K Braun, Felix Hönes, Julia Harant, Michael Fauler, Manfred Frick, Benedikt L Nußbaum, Peter Radermacher, Sebastian Hafner, Markus S Huber-Lang
Background: Polymorphonuclear granulocytes (PMN) play a crucial role in host defense. Physiologically, exposure of PMN to the complement activation product C5a results in a protective response against pathogens, whereas in the case of systemic inflammation, excessive C5a substantially impairs neutrophil functions. To further elucidate the inability of PMN to properly respond to C5a, this study investigates the role of the cellular membrane potential of PMN in response to C5a. Methods: Electrophysiological changes in cellular and mitochondrial membrane potential and intracellular pH of PMN from human healthy volunteers were determined by flow cytometry after exposure to C5a...
2018: Mediators of Inflammation
https://www.readbyqxmd.com/read/30001496/sex-differences-in-cardiac-electrophysiology
#7
Ursula Ravens
Women have a longer QT-interval than men and this difference appears to evolve after puberty suggesting that sex hormones have an influence on cardiac electrophysiology. Sex hormones do in fact regulate cardiac ion channels via genomic and non-genomic pathways. Women are at greater risk for life threatening arrhythmias under conditions that prolong QT interval. In addition women exhibit greater sensitivity to QT interval prolonging drugs. Female sex has also an impact in propensity to cardiovascular disease, including atrial fibrillation...
July 12, 2018: Canadian Journal of Physiology and Pharmacology
https://www.readbyqxmd.com/read/29998508/genetic-and-phenotypic-profile-of-112-patients-with-x-linked-charcot-marie-tooth-disease-type-1
#8
Jun-Hui Yuan, Yusuke Sakiyama, Akihiro Hashiguchi, Masahiro Ando, Yuji Okamoto, Akiko Yoshimura, Yujiro Higuchi, Hiroshi Takashima
BACKGROUND: X-linked Charcot-Marie-Tooth disease type 1 (CMTX1), caused by mutations in gap junction protein beta 1 (GJB1), is characterized by various central nervous system symptoms and gender differences of clinical severity. The aim of this study is to identify the frequency and mutation spectrum of CMTX1 patients in Japan, and to demonstrate their phenotypic diversities. METHODS: Using three high-throughput sequencing systems, we performed targeted gene panel sequencing on 1,483 unrelated index patients with suspected CMT disease...
July 11, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29997009/a-novel-three-base-pair-deletion-in-domain-two-of-the-cardiac-sodium-channel-causes-brugada-syndrome
#9
Boon Yew Tan, Luokai Wang, Mahesh Uttamchandani, Hector Barajas-Martinez, Robert Dumaine, Nathalie Morin, Chi Keong Ching, Kah Leng Ho, Daniel Thuan Tee Chong, Weien Chow, Eric Peng Huat Yap, Shabbir Moochhala, Dan Hu, Rita Yu Yin Yong, Wee Siong Teo
INTRODUCTION: Mutations within SCN5A are found in a significant proportion (15-30%) of Brugada syndrome (BrS) cases and impair sodium transport across excitable cardiac cells that mediate ventricular contractions. Genetic testing offers a means to clinically assess and manage affected individuals and their family members. METHODS AND RESULTS: The proband at age 44 years old exhibited a syncopal event during exercise, and presented later with a spontaneous type-I BrS pattern on 12‑lead resting electrocardiogram (ECG)...
July 2018: Journal of Electrocardiology
https://www.readbyqxmd.com/read/29996615/-a-study-of-clinical-and-genetic-characteristics-of-a-leber-hereditary-optic-neuropathy-family-with-the-heteroplasmic-m-14484t-c-mutation
#10
Y Sun, K Lei, Z L Xu, Y Geng
Objective: To study clinical and genetic characteristics of a Leber hereditary optic neuropathy (LHON) family with the heteroplasmic m.14484T>C mutation. Methods: A cross-sectional study. The objects of the study included a 31-year-old male LHON patient with the heteroplasmic m.14484T>C mutation (the proband) who visited Department of Ophthalmology in the Affiliated Central Hospital of Qingdao University in March 2015 and other 36 matrilineal relatives in a four-generation family (12 males and 24 females aged 2-81 years, median 27 years)...
July 11, 2018: [Zhonghua Yan Ke za Zhi] Chinese Journal of Ophthalmology
https://www.readbyqxmd.com/read/29995258/virtual-thorough-qt-tqt-trial-extrapolation-of-in-vitro-cardiac-safety-data-to-in-vivo-situation-using-multi-scale-physiologically-based-ventricular-cell-wall-model-exemplified-with-tolterodine-and-fesoterodine
#11
Nikunjkumar Patel, Barbara Wisniowska, Sebastian Polak
QT interval prolongation typically assessed with dedicated clinical trials called thorough QT/QTc (TQT) studies is used as surrogate to identify the proarrhythmic risk of drugs albeit with criticism in terms of cost-effectiveness in establishing the actual risk of torsade de pointes (TdP). Quantitative systems toxicology and safety (QSTS) models have potential to quantitatively translate the in vitro cardiac safety data to clinical level including simulation of TQT trials. Virtual TQT simulations have been exemplified with use of two related drugs tolterodine and fesoterodine...
July 11, 2018: AAPS Journal
https://www.readbyqxmd.com/read/29994993/electrophysiological-characterisation-of-central-sensitisation-in-canine-spontaneous-osteoarthritis
#12
James R Hunt, Megan Goff, Helen Jenkins, John Harris, Toby G Knowles, B Duncan X Lascelles, Masataka Enomoto, Mike Mendl, Becky Whay, Joanna C Murrell
In man, central sensitisation (CS) contributes to the pain of osteoarthritis (OA). Dogs with spontaneous OA may also exhibit CS. Electrophysiological reflex measurements are more objective than behavioural assessments, and can be used to evaluate CS in preclinical and clinical studies. It was hypothesised that dogs suffering from OA would exhibit electrophysiological characteristics indicative of CS, associated with reduced diffuse noxious inhibitory controls (DNIC). 117 client owned dogs were recruited to the study...
July 9, 2018: Pain
https://www.readbyqxmd.com/read/29993485/electrophysiological-muscle-classification-using-multiple-instance-learning-and-unsupervised-time-and-spectral-domain-analysis
#13
Tahereh Kamali, Daniel W Stashuk
OBJECTIVE: Electrophysiological muscle classification (EMC) is a crucial step in the diagnosis of neuromuscular disorders. Existing quantitative techniques are not sufficiently robust and accurate to be reliably clinically used. Here, EMC is modelled as a multiple instance learning (MIL) problem and a system to infer unsupervised motor unit potential (MUP) labels and create supervised muscle classifications is presented. METHODS: The system has five main steps: 1) MUP representation using morphological, stability, and near fiber parameters as well as spectral features extracted from wavelet coefficients; 2) MUP feature selection using unsupervised Laplacian scores; 3) MUP clustering using neighborhood distance entropy consistency to find representations of MUP normality and abnormality; 4) Muscle representation by embedding its MUP cluster associations in a feature vector; and 5) Muscle classification using support vector machines or random forests...
February 5, 2018: IEEE Transactions on Bio-medical Engineering
https://www.readbyqxmd.com/read/29993409/transfer-learning-from-simulations-on-a-reference-anatomy-for-ecgi-in-personalised-cardiac-resynchronization-therapy
#14
Sophie Giffard-Roisin, Herve Delingette, Thomas Jackson, Jessica Webb, Lauren Fovargue, Jack Lee, C Aldo Rinaldi, Reza Razavi, Nicholas Ayache, Maxime Sermesant
GOAL: Non-invasive cardiac electrophysiology (EP) model personalisation has raised interest for instance in the scope of predicting EP cardiac resynchronization therapy (CRT) response. However, the restricted clinical applicability of current methods is due in particular to the limitation to simple situations and the important computational cost. METHODS: We propose in this manuscript an approach to tackle these two issues. First, we analyse more complex propagation patterns (multiple onsets and scar tissue) using relevance vector regression and shape dimensionality reduction on a large simulated database...
May 23, 2018: IEEE Transactions on Bio-medical Engineering
https://www.readbyqxmd.com/read/29992405/non-contact-mapping-in-cardiac-electrophysiology
#15
REVIEW
Marcus Wieczorek
Catheter ablation of atrial and ventricular arrhythmias is now considered a standard technology for selected patients. In some patients, however, cure of the arrhythmia is hampered by the complexity of the arrhythmia or the way the arrhythmia presents in the electrophysiological laboratory: some focal atrial and ventricular arrhythmias are difficult to induce using electrical stimulation or medical provocation. Precise mapping of these arrhythmias is challenging or even impossible by contact mapping, while other arrhythmias are poorly tolerated and need early termination...
July 10, 2018: Herzschrittmachertherapie & Elektrophysiologie
https://www.readbyqxmd.com/read/29989510/the-visible-heart%C3%A2-project-and-methodologies-novel-use-for-studying-cardiac-monophasic-action-potentials-and-evaluating-their-underlying-mechanisms
#16
Megan M Schmidt, Paul A Iaizzo
This review describes the utilization of Visible Heart® methodologies for electrophysiologic studies, specifically in the investigation of monophasic action potential (MAP) recordings, with the aim to facilitate new catheter/device design and development that may lead to earlier diagnosis, treatment, and ultimately a higher quality of life for patients with atrial fibrillation. Areas Covered: We describe the historically proposed mechanisms behind which electrode is responsible for the MAP recording, new catheters for recording these signals, and how Visible Heart methodologies can be utilized to develop and test new technologies for electrophysiologic investigations...
July 10, 2018: Expert Review of Medical Devices
https://www.readbyqxmd.com/read/29989175/evaluation-of-left-ventricular-false-tendons-in-children-with-idiopathic-left-ventricular-tachycardia
#17
Anthony Merlocco, David W Brown, Kimberlee Gauvreau, Douglas Y Mah, John K Triedman, Mark E Alexander, Edward P Walsh
BACKGROUND: Left ventricular false tendons (FT) traverse the ventricular cavity and are thought to have some association with idiopathic left ventricular tachycardia (ILVT). However, reported prevalence of FT varies widely, making correlation difficult. Superior echocardiographic windows of pediatric patients may permit better analysis of FT in ILVT. Our study describes the relationship between FT and ILVT in young patients. METHODS: Retrospective case-control study of 30 ILVT patients with 98 controls compared for FT...
July 10, 2018: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/29989041/interesting-case-of-narrow-qrs-tachycardia-with-atrioventricular-dissociation
#18
Krishna Kumar Mohanan Nair, Narayanan Namboodiri, Hiren Kevadiya, Ajitkumar Valaparambil
A 30-year-old man with no structural heart disease has been evaluated for paroxysmal palpitation with documented regular narrow QRS tachycardia that has not responded to intravenous adenosine. Surface electrocardiogram has not shown any pre-excitation. He has been taken for an electrophysiology study after informed consent. Diagnostic catheters were placed at the coronary sinus, His bundle region, and right ventricle. During catheter manipulation a regular narrow QRS tachycardia with incomplete right bundle branch block morphology and normal QRS axis similar to the clinical tachycardia got induced...
July 2018: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/29988378/feasibility-of-nitrogen-doped-ultrananocrystalline-diamond-microelectrodes-for-electrophysiological-recording-from-neural-tissue
#19
Yan T Wong, Arman Ahnood, Matias I Maturana, William Kentler, Kumaravelu Ganesan, David B Grayden, Hamish Meffin, Steven Prawer, Michael R Ibbotson, Anthony N Burkitt
Neural prostheses that can monitor the physiological state of a subject are becoming clinically viable through improvements in the capacity to record from neural tissue. However, a significant limitation of current devices is that it is difficult to fabricate electrode arrays that have both high channel counts and the appropriate electrical properties required for neural recordings. In earlier work, we demonstrated nitrogen doped ultrananocrystalline diamond (N-UNCD) can provide efficacious electrical stimulation of neural tissue, with high charge injection capacity, surface stability and biocompatibility...
2018: Frontiers in Bioengineering and Biotechnology
https://www.readbyqxmd.com/read/29988336/the-limbic-reticular-coupling-theory-of-memory-processing-in-the-brain-and-its-greater-compatibility-over-other-theories
#20
Zi-Jian Cai
The limbic-reticular coupling theory suggests that the hippocampus and amygdala regulate such descending limbic structures as the mammillary bodies, septum, hypothalamus and epithalamus to regulate the ascending noradrenergic, serotonergic, dopaminergic and cholinergic systems, performing declarative memory consolidation and recall. Recent studies have revealed that, less sensitive to familiarity, the hippocampus functions via the fornix, mammillary bodies and hypothalamus for memory recall. Lesions to the thalamic nuclei were complicated with damage to adjacent fornix, stria medullaris and habenula, simultaneously destroying two kinds of structures respectively for familiarity and recall...
April 2018: Dementia & Neuropsychologia
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