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https://www.readbyqxmd.com/read/29125503/patient-preferences-for-treatments-of%C3%A2-neuromuscular-diseases-a-systematic-literature-review
#1
Erik Landfeldt, Josefin Edström, Peter Lindgren, Hanns Lochmüller
BACKGROUND: Treatment decisions of neuromuscular diseases involve weighing clinical benefits and risks, as well as impact on patient social life, work status, other activities of daily living, and health-related quality of life. OBJECTIVE: To conduct a systemic literature review of patient preferences for treatments of neuromuscular diseases. METHODS: We searched Embase, Web of Science, and PubMed for full-text articles reporting results from studies of patient preferences for treatments of neuromuscular diseases...
November 8, 2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/29123223/structural-basis-of-small-molecule-atpase-inhibition-of-a-human-mitotic-kinesin-motor-protein
#2
Hee-Won Park, Zhujun Ma, Haizhong Zhu, Shimin Jiang, Robert C Robinson, Sharyn A Endow
Kinesin microtubule motor proteins play essential roles in division, including attaching chromosomes to spindles and crosslinking microtubules for spindle assembly. Human kinesin-14 KIFC1 is unique in that cancer cells with amplified centrosomes are dependent on the motor for viable division because of its ability to cluster centrosomes and form bipolar spindles, but it is not required for division in almost all normal cells. Screens for small molecule inhibitors of KIFC1 have yielded several candidates for further development, but obtaining structural data to determine their sites of binding has been difficult...
November 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29117060/cognitive-development-1-year-after-infantile-critical-pertussis
#3
John T Berger, Michele E Villalobos, Amy E Clark, Richard Holubkov, Murray M Pollack, Robert A Berg, Joseph A Carcillo, Heidi Dalton, Rick Harrison, Kathleen L Meert, Christopher J L Newth, Thomas P Shanley, David L Wessel, Kanwaljeet J S Anand, Jerry J Zimmerman, Ronald C Sanders, Teresa Liu, Jeri S Burr, Douglas F Willson, Allan Doctor, J Michael Dean, Tammara L Jenkins, Carol E Nicholson
OBJECTIVES: Pertussis can cause life-threatening illness in infants. Data regarding neurodevelopment after pertussis remain scant. The aim of this study was to assess cognitive development of infants with critical pertussis 1 year after PICU discharge. DESIGN: Prospective cohort study. SETTING: Eight hospitals comprising the Eunice Kennedy Shriver National Institute for Child Health and Human Development Collaborative Pediatric Critical Care Research Network and 18 additional sites across the United States...
November 7, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29114969/apathy-in-rapid-eye-movement-sleep-behaviour-disorder-is-common-and-under-recognised
#4
Thomas R Barber, Kinan Muhammed, Daniel Drew, Michael Lawton, Marie Crabbe, Michal Rolinski, Timothy Quinnell, Zenobia Zaiwalla, Yoav Ben-Shlomo, Masud Husain, Michele Tm Hu
BACKGROUND: Apathy is an important neuropsychiatric feature of Parkinson's disease (PD), which often emerges before the onset of motor symptoms. Patients with rapid eye movement sleep behaviour disorder (RBD) have a high probability of developing Parkinson's disease in future. Neuropsychiatric problems are common in RBD, but apathy has not previously been detailed in this key prodromal population. METHODS: 88 patients with polysomnographically proven RBD, 65 patients with PD and 33 controls were assessed for apathy using the Lille Apathy Rating Scale (LARS)...
November 8, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29107592/generation-of-a-motor-nerve-organoid-with-human-stem-cell-derived-neurons
#5
Jiro Kawada, Shohei Kaneda, Takaaki Kirihara, Asif Maroof, Timothée Levi, Kevin Eggan, Teruo Fujii, Yoshiho Ikeuchi
During development, axons spontaneously assemble into a fascicle to form nerves and tracts in the nervous system as they extend within a spatially constrained path. However, understanding of the axonal fascicle has been hampered by lack of an in vitro model system. Here, we report generation of a nerve organoid composed of a robust fascicle of axons extended from a spheroid of human stem cell-derived motor neurons within our custom-designed microdevice. The device is equipped with a narrow channel providing a microenvironment that facilitates the growing axons to spontaneously assemble into a unidirectional fascicle...
November 14, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/29101795/impaired-frontal-lobe-functions-in-patients-with-parkinson-s-disease-and-psychosis
#6
Naveen Thota, Abhishek Lenka, Lija George, Shantala Hegde, Shyam Sundar Arumugham, Shweta Prasad, Albert Stezin, Nitish Kamble, Ravi Yadav, Pramod Kumar Pal
INTRODUCTION: Patients with Parkinson's disease (PD) may develop several non-motor symptoms (NMS). Psychosis is one of the debilitating NMS of PD. The neurobiology of psychosis is not fully understood. This study aims to compare the frontal lobe functions of PD patients with and without psychosis using the Frontal Assessment Battery (FAB). METHODOLOGY: This study included 69 patients with PD; 34 with psychosis (PD-P) and 35 without psychosis (PD-NP). Mini Mental Status Examination (MMSE) was used to screen for cognitive impairment...
October 28, 2017: Asian Journal of Psychiatry
https://www.readbyqxmd.com/read/29096367/electrochemical-immunosensors-for-the-detection-of-survival-motor-neuron-smn-protein-using-different-carbon-nanomaterials-modified-electrodes
#7
Shimaa Eissa, Nawal Alshehri, Anas M Abdel Rahman, Majed Dasouki, Khalid M Abu Salah, Mohammed Zourob
Spinal muscular atrophy is an untreatable potentially fatal hereditary disorder caused by loss-of-function mutations in the survival motor neuron (SMN) 1 gene which encodes the SMN protein. Currently, definitive diagnosis relies on the demonstration of biallelic pathogenic variants in SMN1 gene. Therefore, there is an urgent unmet need to accurately quantify SMN protein levels for screening and therapeutic monitoring of symptomatic newborn and SMA patients, respectively. Here, we developed a voltammetric immunosensor for the sensitive detection of SMN protein based on covalently functionalized carbon nanofiber-modified screen printed electrodes...
October 10, 2017: Biosensors & Bioelectronics
https://www.readbyqxmd.com/read/29092884/breastfeeding-and-motor-development-in-term-and-preterm-infants-in-a-longitudinal-us-cohort
#8
Kara A Michels, Akhgar Ghassabian, Sunni L Mumford, Rajeshwari Sundaram, Erin M Bell, Scott C Bello, Edwina H Yeung
Background: The relation between breastfeeding and early motor development is difficult to characterize because of the problems in existing studies such as incomplete control for confounding, retrospective assessment of infant feeding, and even the assessment of some motor skills too early.Objective: We sought to estimate associations between infant feeding and time to achieve major motor milestones in a US cohort.Design: The Upstate New York Infant Development Screening Program (Upstate KIDS Study) enrolled mothers who delivered live births in New York (2008-2010)...
November 1, 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/29089585/antibodies-against-peripheral-nerve-antigens-in-chronic-inflammatory-demyelinating-polyradiculoneuropathy
#9
Luis Querol, Ana M Siles, Roser Alba-Rovira, Agustín Jáuregui, Jérôme Devaux, Catherine Faivre-Sarrailh, Josefa Araque, Ricard Rojas-Garcia, Jordi Diaz-Manera, Elena Cortés-Vicente, Gisela Nogales-Gadea, Miquel Navas-Madroñal, Eduard Gallardo, Isabel Illa
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a heterogeneous disease in which diverse autoantibodies have been described but systematic screening has never been performed. Detection of CIDP-specific antibodies may be clinically useful. We developed a screening protocol to uncover novel reactivities in CIDP. Sixty-five CIDP patients and 28 controls were included in our study. Three patients (4.6%) had antibodies against neurofascin 155, four (6.2%) against contactin-1 and one (1.5%) against the contactin-1/contactin-associated protein-1 complex...
October 31, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29082509/combining-olfactory-test-and-motion-analysis-sensors-in-parkinson-s-disease-preclinical-diagnosis-a-pilot-study
#10
C Maremmani, F Cavallo, C Purcaro, G Rossi, S Salvadori, E Rovini, D Esposito, A Pieroni, S Ramat, P Vanni, B Fattori, G Meco
OBJECTIVES: Preclinical diagnosis of Parkinson's disease (PD) is nowadays a topic of interest as the neuropathological process could begin years before the appearance of motor symptoms. Several symptoms, among them hyposmia, could precede motor features in PD. In the preclinical phase of PD, a subclinical reduction in motor skills is highly likely. In this pilot study, we investigate a step-by-step method to achieve preclinical PD diagnosis. MATERIAL AND METHODS: We used the IOIT (Italian Olfactory Identification Test) to screen a population of healthy subjects...
October 29, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/29075188/high-yield-of-adult-oligodendrocyte-lineage-cells-obtained-from-meningeal-biopsy
#11
Sissi Dolci, Annachiara Pino, Valeria Berton, Pau Gonzalez, Alice Braga, Marta Fumagalli, Elisabetta Bonfanti, Giorgio Malpeli, Francesca Pari, Stefania Zorzin, Clelia Amoroso, Denny Moscon, Francisco J Rodriguez, Guido Fumagalli, Francesco Bifari, Ilaria Decimo
Oligodendrocyte loss can lead to cognitive and motor deficits. Current remyelinating therapeutic strategies imply either modulation of endogenous oligodendrocyte precursors or transplantation of in vitro expanded oligodendrocytes. Cell therapy, however, still lacks identification of an adequate source of oligodendrocyte present in adulthood and able to efficiently produce transplantable cells. Recently, a neural stem cell-like population has been identified in meninges. We developed a protocol to obtain high yield of oligodendrocyte lineage cells from one single biopsy of adult rat meningeal tissue...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/29073112/dynein-dynactin-is-necessary-for-anterograde-transport-of-mbp-mrna-in-oligodendrocytes-and-for-myelination-in-vivo
#12
Amy L Herbert, Meng-Meng Fu, Catherine M Drerup, Ryan S Gray, Breanne L Harty, Sarah D Ackerman, Thomas O'Reilly-Pol, Stephen L Johnson, Alex V Nechiporuk, Ben A Barres, Kelly R Monk
Oligodendrocytes in the central nervous system produce myelin, a lipid-rich, multilamellar sheath that surrounds axons and promotes the rapid propagation of action potentials. A critical component of myelin is myelin basic protein (MBP), expression of which requires anterograde mRNA transport followed by local translation at the developing myelin sheath. Although the anterograde motor kinesin KIF1B is involved in mbp mRNA transport in zebrafish, it is not entirely clear how mbp transport is regulated. From a forward genetic screen for myelination defects in zebrafish, we identified a mutation in actr10, which encodes the Arp11 subunit of dynactin, a critical activator of the retrograde motor dynein...
October 24, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29070904/a-new-low-cost-compact-auto-phoropter-for-refractive-assessment-in-developing-countries
#13
Babak Amirsolaimani, Gholam Peyman, Jim Schwiegerling, Arkady Bablumyan, N Peyghambarian
Using a phoropter to measure the refractive error is one of the most commonly used methods by ophthalmologists and optometrists. Here, we demonstrate design and fabrication of a portable automatic phoropter with no need for patient's feedback. The system is based on three tunable-focus fluidic lenses and thin-film holographic optical elements to perform automatic refractive error measurement and provide a diagnostic prescription without supervision. Three separate lenses are deployed to correct the defocus and astigmatism...
October 25, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29070544/excess-iron-intake-as-a-factor-in-growth-infections-and-development-of-infants-and-young-children
#14
REVIEW
Bo Lönnerdal
The provision of iron via supplementation or the fortification of foods has been shown to be effective in preventing and treating iron deficiency and iron deficiency anemia in infants and young children. However, iron is a pro-oxidative element and can have negative effects on biological systems even at moderate amounts. An increasing number of studies have reported adverse effects of iron that was given to infants and young-children populations who initially were iron replete. These effects include decreased growth (both linear growth and weight), increased illness (usually diarrhea), interactions with other trace elements such as copper and zinc, altered gut microbiota to more pathogenic bacteria, increased inflammatory markers, and impaired cognitive and motor development...
October 25, 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/29066856/modelling-acrylamide-acute-neurotoxicity-in-zebrafish-larvae
#15
Eva Prats, Cristian Gómez-Canela, Shani Ben-Lulu, Tamar Ziv, Francesc Padrós, Daniel Tornero, Natàlia Garcia-Reyero, Romà Tauler, Arie Admon, Demetrio Raldúa
Acrylamide (ACR), a type-2 alkene, may lead to a synaptopathy characterized by ataxia, skeletal muscles weakness and numbness of the extremities in exposed human and laboratory animals. Currently, only the mildly affected patients undergo complete recovery, and identification of new molecules with therapeutic bioactivity against ACR acute neurotoxicity is urgently needed. Here, we have generated a zebrafish model for ACR neurotoxicity by exposing 5 days post-fertilization zebrafish larvae to 1 mM ACR for 3 days...
October 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29028904/considerations-for-clinical-neuropsychological-evaluation-in-amyotrophic-lateral-sclerosis
#16
Susan C Woolley, Beth K Rush
The clinical neuropsychologist has the opportunity to be uniquely involved in the evaluation and treatment of individuals with amyotrophic lateral sclerosis (ALS). We review the current literature that defines cognitive and behavioral symptoms in ALS, including current knowledge of the neuropathological and genetic underpinning for these symptoms. There are unique considerations for clinical neuropsychological evaluation and clinical research in ALS and we highlight these in this review. Specifically, we shed light on special factors that contribute to our understanding of cognitive and behavioral impairment in ALS, including co-morbid symptoms, differential diagnosis, and considerations for longitudinal tracking of phenotypes...
November 1, 2017: Archives of Clinical Neuropsychology: the Official Journal of the National Academy of Neuropsychologists
https://www.readbyqxmd.com/read/29021962/first-year-metabolic-control-guidelines-and-their-impact-on-future-metabolic-control-and-neurocognitive-functioning-in-children-with-pku
#17
Alicia de la Parra, María Ignacia García, Valerie Hamilton, Carolina Arias, Juan Francisco Cabello, Verónica Cornejo
There is a consensus on the importance of early and life-long treatment for PKU patients. Still, differences exist on target blood phenylalanine (Phe) concentrations for children with PKU in different countries and treatment centers. For the first time, long-term metabolic control and child development and cognitive functioning is compared between children with mean phenylalanine concentrations under 240 μmol/L (group A), between 240 and 360 μmol/L (group B) or over 360 μmol/L (group C) during their first year of life...
December 2017: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/29021837/the-bssg-rat-model-of-parkinson-s-disease-progressing-towards-a-valid-predictive-model-of-disease
#18
REVIEW
Jackalina M Van Kampen, Harold A Robertson
ABSTRACT: Parkinson's disease (PD) is a neurodegenerative disorder, classically considered a movement disorder. A great deal is known about the anatomical connections and neuropathology and pharmacological changes of PD, as they relate to the loss of dopaminergic function and the appearance of cardinal motor symptoms. Our understanding of the role of dopamine in PD has led to the development of effective pharmacological treatments of the motor symptoms in the form of dopamine replacement therapy using levodopa and dopaminergic agonists...
September 2017: EPMA Journal
https://www.readbyqxmd.com/read/29020570/two-dimensional-solutions-in-a-multi-dimensional-world-a-commentary-on-effect-of-touch-screen-tablet-use-on-fine-motor-development-of-young-children
#19
Franzina Coutinho
No abstract text is available yet for this article.
October 20, 2017: Physical & Occupational Therapy in Pediatrics
https://www.readbyqxmd.com/read/29018544/verbal-augmented-feedback-in-the-rehabilitation-of-lower-extremity-musculoskeletal-dysfunctions-a-systematic-review
#20
REVIEW
Marianne Storberget, Linn Helen J Grødahl, Suzanne Snodgrass, Paulette van Vliet, Nicola Heneghan
BACKGROUND: Verbal augmented feedback (VAF) is commonly used in physiotherapy rehabilitation of individuals with lower extremity musculoskeletal dysfunction or to induce motor learning for injury prevention. Its effectiveness for acquisition, retention and transfer of learning of new skills in this population is unknown. OBJECTIVES: First, to investigate the effect of VAF for rehabilitation and prevention of lower extremity musculoskeletal dysfunction. Second, to determine its effect on motor learning and the stages of acquisition, retention and transfer in this population...
2017: BMJ Open Sport & Exercise Medicine
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