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https://www.readbyqxmd.com/read/29149772/natural-history-of-infantile-onset-spinal-muscular-atrophy
#1
Stephen J Kolb, Christopher S Coffey, Jon W Yankey, Kristin Krosschell, W David Arnold, Seward B Rutkove, Kathryn J Swoboda, Sandra P Reyna, Ai Sakonju, Basil T Darras, Richard Shell, Nancy Kuntz, Diana Castro, Julie Parsons, Anne M Connolly, Claudia A Chiriboga, Craig McDonald, W Bryan Burnette, Klaus Werner, Mathula Thangarajh, Perry B Shieh, Erika Finanger, Merit E Cudkowicz, Michelle M McGovern, D Elizabeth McNeil, Richard Finkel, Susan T Iannaccone, Edward Kaye, Allison Kingsley, Samantha R Renusch, Vicki L McGovern, Xueqian Wang, Phillip G Zaworski, Thomas W Prior, Arthur H M Burghes, Amy Bartlett, John T Kissel
OBJECTIVE: Infantile-onset spinal muscular atrophy (SMA) is the most common genetic cause of infant mortality, typically resulting in death prior to age 2. Clinical trials in this population require an understanding of disease progression and identification of meaningful biomarkers to hasten therapeutic development and predict outcomes. METHODS: A longitudinal, multi-center, prospective natural history study enrolled 26 SMA infants, and 27 control infants less than six months of age...
November 17, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/29149697/characteristics-of-motor-speech-phenotypes-in-multiple-sclerosis
#2
Jan Rusz, Barbora Benova, Hana Ruzickova, Michal Novotny, Tereza Tykalova, Jan Hlavnicka, Tomas Uher, Manuela Vaneckova, Michaela Andelova, Klara Novotna, Lucie Kadrnozkova, Dana Horakova
BACKGROUND: Motor speech disorders in multiple sclerosis (MS) are poorly understood and their quantitative, objective acoustic characterization remains limited. Additionally, little data regarding relationships between the severity of speech disorders and neurological involvement in MS, as well as the contribution of pyramidal and cerebellar functional systems on speech phenotypes, is available. METHODS: Speech data were acquired from 141 MS patients with Expanded Disability Status Scale (EDSS) ranging from 1 to 6...
November 8, 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29149655/one-year-after-ischemic-stroke-changes-in-leg-movement-path-stability-in-a-speed-accuracy-task-but-no-major-effects-on-the-hands
#3
Albertas Skurvydas, Vilma Juodzbaliene, Tomas Darbutas, Marius Brazaitis
First year after the stroke is essential for motor recovery. The main motor control strategy (i.e., faster movement production at the expense of lower movement accuracy and stability, or greater movement accuracy and stability at the expense of slower movement) selected by poststroke patients during a unilateral speed-accuracy task (SAT) remains unclear. We aimed to investigate the poststroke (12 months after stroke) effects on the trade-off between movement speed and accuracy, and intraindividual variability during a motor performance task...
November 14, 2017: Human Movement Science
https://www.readbyqxmd.com/read/29149213/optimal-design-and-control-of-an-electromechanical-transfemoral-prosthesis-with-energy-regeneration
#4
Farbod Rohani, Hanz Richter, Antonie J van den Bogert
In this paper, we present the design of an electromechanical above-knee active prosthesis with energy storage and regeneration. The system consists of geared knee and ankle motors, parallel springs for each motor, an ultracapacitor, and controllable four-quadrant power converters. The goal is to maximize the performance of the system by finding optimal controls and design parameters. A model of the system dynamics was developed, and used to solve a combined trajectory and design optimization problem. The objectives of the optimization were to minimize tracking error relative to human joint motions, as well as energy use...
2017: PloS One
https://www.readbyqxmd.com/read/29148855/effects-of-virtual-reality-based-training-with-bts-nirvana-on-functional-recovery-in-stroke-patients-preliminary-considerations
#5
Rosaria De Luca, Margherita Russo, Antonino Naro, Provvidenza Tomasello, Simona Leonardi, Floriana Santamaria, Latella Desireè, Alessia Bramanti, Giuseppe Silvestri, Placido Bramanti, Rocco Salvatore Calabrò
AIM OF THE STUDY: cognitive impairment occurs frequently in post-stroke patients. This study aimed to determine the effects of a virtual reality training (VRT) with BTs-Nirvana (BTsN) on the recovery of cognitive functions in stroke patients, using the Interactive-Semi-Immersive Program (I-SIP). MATERIALS AND METHODS: We enrolled 12 subjects (randomly divided into two groups: experimental group -EG; and control group -CG), who attended the Laboratory of Robotic and Cognitive Rehabilitation of IRCCS Neurolesi of Messina from January to June 2016...
November 17, 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/29148558/ultrasound-propelled-nanowire-motors-enhance-asparaginase-enzymatic-activity-against-cancer-cells
#6
Murat Uygun, Beatriz Jurado-Sánchez, Deniz Aktas Uygun, Virendra Vikram Singh, Liangfang Zhang, Joseph Wang
Ultrasound-(US) propelled nanowires consisting of Au/Ni/Au/PEDOT-PPy-COOH segments are modified with asparaginase enzyme and applied as an effective anti-cancer agent. After immobilization of asparaginase onto the surface of the nanowire motors, the enzyme displays enhanced thermal and pH stabilities, improved resistance towards protease, and higher affinity for the substrate. The fast motion of the motor-carrying asparaginase leads to greatly accelerated biocatalytic depletion of asparagine and hence to a significantly enhanced inhibition efficacy against El4 lymphoma cancer cells (92%) as compared to free enzyme counterpart (17%) and other control groups...
November 17, 2017: Nanoscale
https://www.readbyqxmd.com/read/29148138/artificial-walking-technologies-to-improve-gait-in-cerebral-palsy-multichannel-neuromuscular-stimulation
#7
Jessica Rose, Katelyn Cahill-Rowley, Erin E Butler
Cerebral palsy (CP) is the most common childhood motor disability and often results in debilitating walking abnormalities, such as flexed-knee and stiff-knee gait. Current medical and surgical treatments are only partially effective in improving gait abnormalities and may cause significant muscle weakness. However, emerging artificial walking technologies, such as step-initiated, multichannel neuromuscular electrical stimulation (NMES), can substantially improve gait patterns and promote muscle strength in children with spastic CP...
November 2017: Artificial Organs
https://www.readbyqxmd.com/read/29148137/brain-computer-interfaces-with-multi-sensory-feedback-for-stroke-rehabilitation-a-case-study
#8
Danut C Irimia, Woosang Cho, Rupert Ortner, Brendan Z Allison, Bogdan E Ignat, Guenter Edlinger, Christoph Guger
Conventional therapies do not provide paralyzed patients with closed-loop sensorimotor integration for motor rehabilitation. This work presents the recoveriX system, a hardware and software platform that combines a motor imagery (MI)-based brain-computer interface (BCI), functional electrical stimulation (FES), and visual feedback technologies for a complete sensorimotor closed-loop therapy system for poststroke rehabilitation. The proposed system was tested on two chronic stroke patients in a clinical environment...
November 2017: Artificial Organs
https://www.readbyqxmd.com/read/29148080/genetic-risk-factors-for-perception-of-symptoms-in-gerd-an-observational-cohort-study
#9
A Patel, S Hasak, B D Nix, G S Sayuk, R D Newberry, C P Gyawali
BACKGROUND: Genetic polymorphisms in G-protein beta-3 subunit (GNβ3) and beta-2 adrenergic receptor (ADRB2) are associated with pain and gut hypersensitivity, which can overlap with gastroesophageal reflux disease (GERD). AIM: To evaluate relationships between single nucleotide polymorphisms (SNPs) within GNβ3 and ADRB2 systems, and reflux symptom burden, GERD phenotypes from ambulatory reflux monitoring, and quality of life. METHODS: Symptomatic adults undergoing ambulatory reflux testing were recruited and phenotyped based on acid burden and symptom reflux association; major oesophageal motor disorders and prior foregut surgery were exclusions...
November 17, 2017: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29147795/preoperative-pregabalin-has-no-effect-on-intraoperative-neurophysiological-monitoring-in-adolescents-undergoing-posterior-spinal-fusion-for-spinal-deformities-a-double-blind-randomized-placebo-controlled-clinical-trial
#10
Linda Helenius, Antti Puhakka, Tuula Manner, Olli Pajulo, Ilkka Helenius
PURPOSE: This study was designed to evaluate the effect of preoperative pregabalin on intraoperative neurophysiological monitoring in adolescents undergoing surgery for spinal deformities. METHODS: Thirty-one adolescents undergoing posterior spinal fusion were randomized to receive preoperatively either pregabalin 2 mg/kg twice daily or placebo. The ability to make reliable intraoperative neurophysiological measurements, transcranial motor (MEPs) and sensory evoked potentials (SSEP) was evaluated...
November 17, 2017: European Spine Journal
https://www.readbyqxmd.com/read/29147730/on-the-skilled-plantar-flexor-motor-action-and-unique-electromyographic-activity-of-ballet-dancers
#11
Sakiko Saito, Hiroki Obata, Mayumi Kuno-Mizumura, Kimitaka Nakazawa
The study aimed to compare the ability of dance and non-dance subjects to perform fine control of a simple heel-raising/lowering movement, and to determine if there are any differences in motor unit activity in the primary plantar flexor muscles during the movement. Subjects were instructed to accurately track a sinusoidal trace with a heel-raising and lowering movement at four controlled frequencies (1, 0.5, 0.25, and 0.125 Hz). The ankle joint angle was used to characterize movement errors from the target...
November 16, 2017: Experimental Brain Research. Experimentelle Hirnforschung. Expérimentation Cérébrale
https://www.readbyqxmd.com/read/29147661/an-eeg-tool-for-monitoring-patient-engagement-during-stroke-rehabilitation-a-feasibility-study
#12
Gadi Bartur, Katherin Joubran, Sara Peleg-Shani, Jean-Jacques Vatine, Goded Shahaf
Objective: Patient engagement is of major significance in neural rehabilitation. We developed a real-time EEG marker for attention, the Brain Engagement Index (BEI). In this work we investigate the relation between the BEI and temporary functional change during a rehabilitation session. Methods: First part: 13 unimpaired controls underwent BEI monitoring during motor exercise of varying levels of difficulty. Second part: 18 subacute stroke patients underwent standard motor rehabilitation with and without use of real-time BEI feedback regarding their level of engagement...
2017: BioMed Research International
https://www.readbyqxmd.com/read/29147635/global-and-targeted-pathway-impact-of-gliomas-on-white-matter-integrity-based-on-lobar-localization
#13
David R Ormond, Shawn D'Souza, John A Thompson
Primary brain tumors comprise 28% of all tumors and 80% of malignant tumors. Pathophysiology of high-grade gliomas includes significant distortion of white matter architecture, necrosis, the breakdown of the blood brain barrier, and increased intracranial pressure. Diffusion tensor imaging (DTI), a diffusion weighted imaging technique, can be used to assess white matter architecture. Use of DTI as a non-invasive pathophysiological tool to analyze glioma impact on white matter microstructure has yet to be fully explored...
September 7, 2017: Curēus
https://www.readbyqxmd.com/read/29147598/dbs-programming-an-evolving-approach-for-patients-with-parkinson-s-disease
#14
REVIEW
Aparna Wagle Shukla, Pam Zeilman, Hubert Fernandez, Jawad A Bajwa, Raja Mehanna
Deep brain stimulation (DBS) surgery is a well-established therapy for control of motor symptoms in Parkinson's disease. Despite an appropriate targeting and an accurate placement of DBS lead, a thorough and efficient programming is critical for a successful clinical outcome. DBS programming is a time consuming and laborious manual process. The current approach involves use of general guidelines involving determination of the lead type, electrode configuration, impedance check, and battery check. However there are no validated and well-established programming protocols...
2017: Parkinson's Disease
https://www.readbyqxmd.com/read/29147011/lower-serum-extracellular-superoxide-dismutase-levels-are-associated-with-polyneuropathy-in-recent-onset-diabetes
#15
Alexander Strom, Kirti Kaul, Jutta Brüggemann, Iris Ziegler, Ilka Rokitta, Sonja Püttgen, Julia Szendroedi, Karsten Müssig, Michael Roden, Dan Ziegler
Increased oxidative stress is implicated in the pathogenesis of experimental diabetic neuropathy, but translational evidence in recent-onset diabetes is scarce. We aimed to determine whether markers of systemic oxidative stress are associated with diabetic sensorimotor polyneuropathy (DSPN) in recent-onset diabetes. In this cross-sectional study, we measured serum concentrations of extracellular superoxide dismutase (SOD3), thiobarbituric acid reactive substances (TBARS), and reduced glutathione (GSH) in 107 type 1 and 215 type 2 diabetes patients from the German Diabetes Study baseline cohort and 37 glucose-tolerant individuals (controls)...
November 17, 2017: Experimental & Molecular Medicine
https://www.readbyqxmd.com/read/29146863/modeling-search-movements-of-an-insect-s-front-leg
#16
Tibor I Tóth, Eva Berg, Silvia Daun
Beside locomotion, search movements are another important type of motor activity of insects. They are very often performed by the front legs of the animals. They consist of cyclic stereotypical leg movements that can be modified by sensory signals. The details of the local organization of these movements have however not yet been studied. In this paper, we, using an appropriate variant of our existing one-leg model, present a scheme of how these searching movements might be organized and performed on the level of local neuromuscular control networks...
November 2017: Physiological Reports
https://www.readbyqxmd.com/read/29146650/benefits-and-risks-of-iron-interventions-in-children-brisc-protocol-for-a-three-arm-parallel-group-randomised-controlled-field-trial-in-bangladesh
#17
Mohammed Imrul Hasan, Sheikh Jamal Hossain, Sabine Braat, Michael John Dibley, Jane Fisher, Sally Grantham-McGregor, Fahmida Tofail, Julie A Simpson, Shams Ei Arifeen, Jena Hamadani, Beverley-Ann Biggs, Sant-Rayn Pasricha
INTRODUCTION: Anaemia is a major global health problem affecting about 43% of preschool children globally and 60% of 6-24-month-old children in rural Bangladesh, half of which is attributed to iron deficiency (ID). Although WHO recommends universal supplementation with iron or home fortification with iron-containing multiple micronutrient powders (MMPs) to children under 2 years, evidence for benefits of these interventions on childhood development (a key rationale for these interventions) and harms (especially infection) remains limited...
November 15, 2017: BMJ Open
https://www.readbyqxmd.com/read/29146598/structural-analyses-unravel-the-molecular-mechanism-of-cyclic-di-gmp-regulation-of-bacterial-chemotaxis-via-a-pilz-adaptor-protein
#18
Xin-Fu Yan, Lingyi Xin, Jackie Tan Yen, Yukai Zeng, Shengyang Jin, Qing Wei Cheang, Rachel Andrea Chea Yuen Fong, Keng-Hwee Chiam, Zhao-Xun Liang, Yong-Gui Gao
The bacterial second messenger cyclic di-GMP (c-di-GMP) has emerged as a prominent mediator of bacterial physiology, motility and pathogenicity. Cdi-GMP often regulates the function of its protein targets through a unique mechanism that involves a discrete PilZ adaptor protein. However, the molecular mechanism in c-di-GMP-mediated protein regulation is unclear. Here, we present the structure of the PilZ adaptor protein MapZ co-crystallized in complex with c-di-GMP and its protein target CheR1, a chemotaxis-regulating methyltransferase in Pseudomonas aeruginosa This co-crystal structure, together with the structure of free CheR1, revealed that the binding of c-di-GMP induces dramatic structural changes in MapZ that are crucial for CheR1 binding...
November 16, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29146468/tdcs-changes-in-motor-excitability-are-specific-to-orientation-of-current-flow
#19
Vishal Rawiji, Matteo Ciocca, André Zacharia, David Soares, Dennis Truong, Marom Bikson, John Rothwell, Sven Bestmann
BACKGROUND: Measurements and models of current flow in the brain during transcranial Direct Current Stimulation (tDCS) indicate stimulation of regions in-between electrodes. Moreover, the folded cortex results in local fluctuations in current flow intensity and direction, and animal studies suggest current flow direction relative to cortical columns determines response to tDCS. METHODS: Here we test this idea by using Transcranial Magnetic Stimulation Motor Evoked Potentials (TMS-MEP) to measure changes in corticospinal excitability following tDCS applied with electrodes aligned orthogonal (across) or parallel to M1 in the central sulcus...
November 7, 2017: Brain Stimulation
https://www.readbyqxmd.com/read/29146111/microrna-expression-patterns-in-human-anterior-cingulate-and-motor-cortex-a-study-of-dementia-with-lewy-bodies-cases-and-controls
#20
Peter T Nelson, Wang-Xia Wang, Sarah A Janse, Katherine L Thompson
OVERVIEW: MicroRNAs (miRNAs) have been implicated in neurodegenerative diseases including Parkinson's disease and Alzheimer's disease (AD). Here, we evaluated the expression of miRNAs in anterior cingulate (AC; Brodmann area [BA] 24) and primary motor (MO; BA 4) cortical tissue from aged human brains in the University of Kentucky AD Center autopsy cohort, with a focus on dementia with Lewy bodies (DLB). METHODS: RNA was isolated from gray matter of brain samples with pathology-defined DLB, AD, AD+DLB, and low-pathology controls, with n=52 cases initially included (n=23 with DLB), all with low (<4hrs) postmortem intervals...
November 13, 2017: Brain Research
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