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https://www.readbyqxmd.com/read/28812416/role-of-branchiomotor-neurons-in-controlling-food-intake-of-zebrafish-larvae
#1
James R Allen, Kiran D Bhattacharyya, Emilia Asante, Badr Almadi, Kyle Schafer, Jeremy Davis, Jane Cox, Mark Voigt, John A Viator, Anand Chandrasekhar
The physical act of eating or feeding involves the coordinated action of several organs like eyes and jaws, and associated neural networks. Moreover, the activity of the neural networks controlling jaw movements (branchiomotor circuits) is regulated by the visual, olfactory, gustatory and hypothalamic systems, which are largely well characterized at the physiological level. By contrast, the behavioral output of the branchiomotor circuits and the functional consequences of disruption of these circuits by abnormal neural development are poorly understood...
August 16, 2017: Journal of Neurogenetics
https://www.readbyqxmd.com/read/28812061/behavioral-comorbidities-and-drug-treatments-in-a-zebrafish-scn1lab-model-of-dravet-syndrome
#2
Brian P Grone, Tiange Qu, Scott C Baraban
Loss-of-function mutations in SCN1A cause Dravet syndrome (DS), a catastrophic childhood epilepsy in which patients experience comorbid behavioral conditions, including movement disorders, sleep abnormalities, anxiety, and intellectual disability. To study the functional consequences of voltage-gated sodium channel mutations, we use zebrafish with a loss-of-function mutation in scn1lab, a zebrafish homolog of human SCN1A. Homozygous scn1lab(s552/s552) mutants exhibit early-life seizures, metabolic deficits, and early death...
July 2017: ENeuro
https://www.readbyqxmd.com/read/28812043/swallowing-markers-in-spinal-and-bulbar-muscular-atrophy
#3
Haruhiko Banno, Masahisa Katsuno, Keisuke Suzuki, Seiya Tanaka, Noriaki Suga, Atsushi Hashizume, Tomoo Mano, Amane Araki, Hirohisa Watanabe, Yasushi Fujimoto, Masahiko Yamamoto, Gen Sobue
OBJECTIVE: We examined the characteristics of dysphagia in spinal and bulbar muscular atrophy, a hereditary neuromuscular disease causing weakness of limb, facial, and oropharyngeal muscles via a videofluoroscopic swallowing study, and investigated the plausibility of using these outcome measures for quantitative analysis. METHODS: A videofluoroscopic swallowing study was performed on 111 consecutive patients with genetically confirmed spinal and bulbar muscular atrophy and 53 age- and sex-matched healthy controls...
August 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/28811697/role-of-iron-and-copper-in-the-pathogenesis-of-parkinson-s-disease
#4
Mohit Kumar Gangania, Jyoti Batra, Suman Kushwaha, Rachna Agarwal
Parkinson's disease (PD) is an old age disorder of basal ganglia which involves oligomerization of α-synuclein protein and formation of intercellular inclusions known as "Lewy bodies" in substantia nigra and caudate nuclei in brain which is progressive in nature. It is second most prevalent neurodegenerative disorder characterized by tremor at rest, muscle rigidity, slowness of movement (bradykinesia, akinesia), and changes in posture (instability). Both excess and deficiency in levels of transition metals (especially iron, copper) can be detrimental to the central nervous system...
July 2017: Indian Journal of Clinical Biochemistry: IJCB
https://www.readbyqxmd.com/read/28808252/altered-awareness-of-action-in-parkinson-s-disease-evaluations-by-explicit-and-implicit-measures
#5
Naho Saito, Keisuke Takahata, Hodaka Yamakado, Nobukatsu Sawamoto, Satoshi Saito, Ryosuke Takahashi, Toshiya Murai, Hidehiko Takahashi
Deficits in the integration of motor prediction and its feedback have been reported in Parkinson's disease. Conscious awareness of action is proposed to emerge under the integration of motor prediction and its feedback. Thus, it may lead to changes in the awareness of the authorship of action (in other words, the sense of agency) in Parkinson's disease. We have employed both explicit and implicit measures to assess the awareness of action in Parkinson's disease and matched controls. As an explicit measure, an action recognition task requiring explicit judgments was used...
August 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28807673/gpr55-a-therapeutic-target-for-parkinson-s-disease
#6
Marta Celorrio, Estefanía Rojo-Bustamante, Diana Fernández-Suárez, Elena Sáez, Ander Estella-Hermoso de Mendoza, Christa E Müller, María J Ramírez, Julen Oyarzábal, Rafael Franco, María S Aymerich
The GPR55 receptor is expressed abundantly in the brain, especially in the striatum, suggesting it might fulfill a role in motor function. Indeed, motor behavior is impaired in mice lacking GPR55, which also display dampened inflammatory responses. Abnormal-cannabidiol (Abn-CBD), a synthetic cannabidiol (CBD) isomer, is a GPR55 agonist that may serve as a therapeutic agent in the treatment of inflammatory diseases. In this study, we explored whether modulating GPR55 could also represent a therapeutic approach for the treatment of Parkinson's disease (PD)...
August 11, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28805585/understanding-and-treating-pain-syndromes-in-parkinson-s-disease
#7
Marialuisa Gandolfi, Christian Geroin, Angelo Antonini, Nicola Smania, Michele Tinazzi
Pain affects many people with Parkinson's disease (PD) and diminishes their quality of life. Different types of pain have been described, but their related pathophysiological mechanisms remain unclear. The aim of this chapter is to provide movement disorders specialists an update about the pathophysiology of pain and a practical guide for the management of pain syndromes in clinical practice. This chapter reviews current knowledge on the pathophysiological mechanisms of sensory changes and pain in PD, as well as assessment and treatment procedures to manage these symptoms...
2017: International Review of Neurobiology
https://www.readbyqxmd.com/read/28804474/a-longitudinal-electromyography-study-of-complex-movements-in-poststroke-therapy-1-heterogeneous-changes-despite-consistent-improvements-in-clinical-assessments
#8
Negin Hesam-Shariati, Terry Trinh, Angelica G Thompson-Butel, Christine T Shiner, Penelope A McNulty
Poststroke weakness on the more-affected side may arise from reduced corticospinal drive, disuse muscle atrophy, spasticity, and abnormal coordination. This study investigated changes in muscle activation patterns to understand therapy-induced improvements in motor-function in chronic stroke compared to clinical assessments and to identify the effect of motor-function level on muscle activation changes. Electromyography (EMG) was recorded from five upper limb muscles on the more-affected side of 24 patients during early and late therapy sessions of an intensive 14-day program of Wii-based Movement Therapy (WMT) and for a subset of 13 patients at 6-month follow-up...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28802148/inattentional-blindness-in-anesthesiology-a-simulation-study
#9
Anthony M-H Ho, Joseph Y C Leung, Glenio B Mizubuti, Leeanne H Contardi, Matthew T V Chan, Thomas S F Lo, Alex K T Lee
STUDY OBJECTIVES: Inattentional blindness is the psychological phenomenon of inability to see the unexpected even if it is in plain view. We hypothesized that anesthesiologists may overlook unexpected intraoperative events whereas medical students, lacking in intraoperative monitoring experience and knowledge, may be more likely to notice such events. DESIGN: A simulation study using a video of a simulated septic patient undergoing abdominal surgery. SETTING: A large academic center...
August 9, 2017: Journal of Clinical Anesthesia
https://www.readbyqxmd.com/read/28801146/bh4-deficiency-identified-in-a-neonatal-screening-program-for-hyperphenylalaninemia
#10
Cezar Antonio Abreu de Souza, Michelle Rosa Andrade Alves, Rosangelis Del Lama Soares, Viviane de Cássia Kanufre, Valéria de Melo Rodrigues, Rocksane de Carvalho Norton, Ana Lúcia Pimenta Starling, Marcos José Burle de Aguiar
OBJECTIVES: To show the general prevalence and to characterize tetrahydrobiopterin (BH4) deficiencies with hyperphenylalaninemia, identified by the Neonatal Screening Program of the State of Minas Gerais (NSPMG). METHODS: Descriptive study of patients with BH4 deficiency identified by the NSPMG. RESULTS: The prevalence found was 2.1 for 1,000,000 live births, with a frequency of 1.71% among hyperphenylalaninemias. There were four cases (40%) with 6-pyruvoyl-tetrahydropterin synthase (PTPS) deficiency, three with GTP cyclohydrolase I - autosomal recessive form (GTPCH I) deficiency, and three with dihydropteridine reductase (DHPR) deficiency (30% each)...
August 8, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/28800632/radar-a-novel-fast-screening-method-for-reading-difficulties-with-special-focus-on-dyslexia
#11
Ioannis Smyrnakis, Vassilios Andreadakis, Vassilios Selimis, Michail Kalaitzakis, Theodora Bachourou, Georgios Kaloutsakis, George D Kymionis, Stelios Smirnakis, Ioannis M Aslanides
Dyslexia is a developmental learning disorder of single word reading accuracy and/or fluency, with compelling research directed towards understanding the contributions of the visual system. While dyslexia is not an oculomotor disease, readers with dyslexia have shown different eye movements than typically developing students during text reading. Readers with dyslexia exhibit longer and more frequent fixations, shorter saccade lengths, more backward refixations than typical readers. Furthermore, readers with dyslexia are known to have difficulty in reading long words, lower skipping rate of short words, and high gaze duration on many words...
2017: PloS One
https://www.readbyqxmd.com/read/28796676/sleep-disorder-diagnosis-during-pregnancy-and-risk-of-preterm-birth
#12
Jennifer N Felder, Rebecca J Baer, Larry Rand, Laura L Jelliffe-Pawlowski, Aric A Prather
OBJECTIVE: To test the hypothesis that sleep disorder diagnosis would be associated with increased risk of preterm birth and to examine risk by gestational age, preterm birth type, and specific sleep disorder (insomnia, sleep apnea, movement disorder, and other). METHODS: In this observational study, participants were from a cohort of nearly 3 million women in California between 2007 and 2012. Inclusion criteria were women with singleton neonates liveborn between 20 and 44 weeks of gestation without chromosomal abnormalities or major structural birth defects linked to a hospital discharge database maintained by the California Office of Statewide Health Planning and Development and without mental illness during pregnancy...
August 4, 2017: Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28790021/deficient-striatal-adaptation-in-aminergic-and-glutamatergic-neurotransmission-is-associated-with-tardive-dyskinesia-in-non-human-primates-exposed-to-antipsychotic-drugs
#13
Catherine Lévesque, Giovanni Hernandez, Souha Mahmoudi, Frédéric Calon, Fabrizio Gasparini, Baltazar Gomez-Mancilla, Pierre J Blanchet, Daniel Lévesque
Tardive dyskinesia (TD) is a potentially disabling condition encompassing all delayed, persistent, and often irreversible abnormal involuntary movements arising in a fraction of subjects during long-term exposure to centrally acting dopamine receptor-blocking agents such as antipsychotic drugs and metoclopramide. However, the pathogenesis of TD has proved complex and remains elusive. To investigate the mechanism underlying the development of TD, we have chronically exposed 17 Cebus apella monkeys to typical (11) or atypical (6) antipsychotic drugs...
August 5, 2017: Neuroscience
https://www.readbyqxmd.com/read/28783910/effects-of-copper-on-the-survival-hatching-and-reproduction-of-a-pulmonate-snail-physa-acuta
#14
Lei Gao, Hai Doan, Bhanu Nidumolu, Anupama Kumar, Debra Gonzago
Acute and chronic bioassays provide essential basis for establishment of environmental quality standards. The effects of Cu on a pulmonate snail, Physa acuta, were investigated at a number of sublethal and lethal endpoints. Cu exposure suppressed movement and triggered an escape response in P. acuta at low and high concentrations, respectively, exerting acute toxic effects on adult snails exposed to a 96 h LC50 of 23.8 μg L(-1). Following 16 d exposure of Cu to the egg masses, successful hatching decreased with increasing Cu concentration...
October 2017: Chemosphere
https://www.readbyqxmd.com/read/28783058/evolution-of-diagnostic-tests-for-chronic-wasting-disease-a-naturally-occurring-prion-disease-of-cervids
#15
REVIEW
Nicholas J Haley, Jürgen A Richt
Since chronic wasting disease (CWD) was first identified nearly 50 years ago in a captive mule deer herd in the Rocky Mountains of the United States, it has slowly spread across North America through the natural and anthropogenic movement of cervids and their carcasses. As the endemic areas have expanded, so has the need for rapid, sensitive, and cost effective diagnostic tests-especially those which take advantage of samples collected antemortem. Over the past two decades, strategies have evolved from the recognition of microscopic spongiform pathology and associated immunohistochemical staining of the misfolded prion protein to enzyme-linked immunoassays capable of detecting the abnormal prion conformer in postmortem samples...
August 5, 2017: Pathogens
https://www.readbyqxmd.com/read/28782903/non-invasive-brain-stimulation-for-dystonia-therapeutic-implications
#16
REVIEW
R Erro, M Tinazzi, F Morgante, K P Bhatia
Dystonia is characterized by excessive muscle contractions giving rise to abnormal posture and involuntary twisting movements. Although dystonia syndromes are a heterogeneous group of disorders, certain pathophysiological mechanisms have been consistently identified across different forms. These pathophysiological mechanisms have subsequently been exploited for the development of non-invasive brain stimulation (NIBS) techniques able to modulate neural activity in one or more nodes of the putative network that is altered in dystonia, and the therapeutic role of NIBS has hence been suggested...
August 7, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28781201/seven-tesla-mri-of-the-substantia-nigra-in-patients-with-rapid-eye-movement-sleep-behavior-disorder
#17
Daniela Frosini, Mirco Cosottini, Graziella Donatelli, Mauro Costagli, Laura Biagi, Claudio Pacchetti, Michele Terzaghi, Pietro Cortelli, Dario Arnaldi, Enrica Bonanni, Michela Tosetti, Ubaldo Bonuccelli, Roberto Ceravolo
INTRODUCTION: Susceptibility-weighted imaging of the substantia nigra (SN) both at 7 and 3 Tesla (T) has shown high accuracy in distinguishing patients with Parkinson's disease (PD) and healthy subjects (HS). Patients with rapid eye movement (REM) behavior disorder (RBD) can develop synucleinopathies, and such risk is higher with dopamine transporter single photon emission tomography (123I-FP-CIT SPECT) evidence of nigro-striatal dysfunction. We aimed at evaluating SN 7T magnetic resonance imaging (7T-MRI) in patients with RBD and determining the agreement between MRI and 123I-FP-CIT SPECT...
August 2, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28781028/hypoplastic-hippocampus-in-atypical-rett-syndrome-with-a-novel-foxg1-mutation
#18
Kotoha Harada, Mayumi Yamamoto, Yukihiko Konishi, Kaori Koyano, Satoru Takahashi, Masanori Namba, Takashi Kusaka
The forkhead box G1 (FOXG1) gene encodes a brain-specific transcription factor and is associated with a congenital variant of atypical Rett syndrome (RTT); several FOXG1 mutations have been identified. The congenital variant of RTT shows a hypoplastic corpus callosum, delayed myelination, and frontal and temporal atrophy. Although no report has described a hippocampal abnormality in humans, the current study suggests that FOXG1 also regulates neurogenesis in the postnatal hippocampus. In the present case, severe developmental delay was observed in a patient with a congenital variant of RTT from about 4months, in conjunction with acquired microcephaly, hypotonia, limited motor function, absent purposeful hand use, and repetitive jerky movements of the upper limbs...
August 3, 2017: Brain & Development
https://www.readbyqxmd.com/read/28779867/neurological-complications-of-childhood-cancer
#19
Lauren Weaver, Ayman Samkari
Though the treatment of pediatric cancers has come a long way, acute and chronic effects of cancer are still affecting the life of many children. These effects may be caused not only by the malignancy itself but also by the interventions used for the purpose of treatment. This article focuses primarily on the indirect effects of pediatric cancers and their treatment on the central and peripheral nervous system. Chemotherapy, radiation, and stem cell transplantation cause an immune-compromised state and place the patient at risk of infection, the leading cause of mortality in pediatric cancer...
February 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28777491/identification-of-stac3-variants-in-non-native-american-families-with-overlapping-features-of-carey-fineman-ziter-syndrome-and-moebius-syndrome
#20
Aida Telegrafi, Bryn D Webb, Sarah M Robbins, Carlos E Speck-Martins, David FitzPatrick, Leah Fleming, Richard Redett, Andreas Dufke, Gunnar Houge, Jeske J T van Harssel, Alain Verloes, Angela Robles, Irini Manoli, Elizabeth C Engle, Ethylin W Jabs, David Valle, John Carey, Julie E Hoover-Fong, Nara L M Sobreira
Horstick et al. (2013) previously reported a homozygous p.Trp284Ser variant in STAC3 as the cause of Native American myopathy (NAM) in 5 Lumbee Native American families with congenital hypotonia and weakness, cleft palate, short stature, ptosis, kyphoscoliosis, talipes deformities, and susceptibility to malignant hyperthermia (MH). Here we present two non-Native American families, who were found to have STAC3 pathogenic variants. The first proband and her affected older sister are from a consanguineous Qatari family with a suspected clinical diagnosis of Carey-Fineman-Ziter syndrome (CFZS) based on features of hypotonia, myopathic facies with generalized weakness, ptosis, normal extraocular movements, cleft palate, growth delay, and kyphoscoliosis...
August 4, 2017: American Journal of Medical Genetics. Part A
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