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https://www.readbyqxmd.com/read/28110362/adult-onset-intradural-spinal-teratoma-report-of-18-consecutive-cases-and-outcomes-in-a-single-center
#1
Wei Wan, Cheng Yang, Wangjun Yan, Tielong Liu, Xinghai Yang, Dianwen Song, Jianru Xiao
STUDY DESIGN: Eighteen consecutive patients with adult-onset intradural spinal teratoma underwent surgical treatment in our center from 1998 to 2013. BACKGROUND AND PURPOSE: Teratoma is defined as a neoplasm composed of elements derived from three germ cell layers (ectoderm, endoderm and mesoderm). Intraspinal teratoma is extremely rare and accounts for 0.2-0.5% of all spinal cord tumors. Moreover, teratoma occurs primarily in neonates and young children. Adult-onset intradural spinal teratoma is even rare...
January 21, 2017: European Spine Journal
https://www.readbyqxmd.com/read/28109863/disruption-of-cortical-arterial-network-is-associated-with-the-severity-of-transient-neurological-events-after-direct-bypass-surgery-in-adult-moyamoya-disease
#2
Yusuke Egashira, Keita Yamauchi, Yukiko Enomoto, Noriyuki Nakayama, Shinichi Yoshimura, Toru Iwama
OBJECTIVE: Transient neurological events (TNEs) frequently occur after revascularization in adult moyamoya disease (MMD). In the present study, we hypothesized that cortical arterial network disruption may be associated with TNE severity following bypass surgery. METHODS: This retrospective study included 76 hemispheres in 45 consecutive adult MMD patients who underwent direct revascularization surgery at our institution. We classified cortical arterial network disruption grade (NDG) into the following four categories based on angiography: NDG 0, > 90% of suprasylvian cortical branches of the middle cerebral artery demonstrated anterograde filling; NDG 1, 50% to 90%; NDG 2, < 50%; NDG 3, none...
January 18, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28109651/late-onset-krabbe-disease-due-to-the-new-galc-p-ala543pro-mutation-with-intriguingly-high-residual-galc-activity-in%C3%A2-vitro
#3
Inge Krägeloh-Mann, Klaus Harzer, Kevin Rostásy, Stefanie Beck-Wödl, Antje Bornemann, Judith Böhringer, Andrea Bevot, Verena Beck, Gisela Merkel, Maria Pechan
BACKGROUND: Krabbe disease (KD) is an inherited leukodystrophy due to a defect in the GALC gene which encodes the lysosomal galactosylceramide β-galactosidase (GALC). About two thirds of patients show the early onset form of KD dominated by cerebral demyelination leading to death in early infancy. Late onset forms include a spectrum of late infantile, juvenile and adult clinical courses. The deficiency of GALC leads to a galactosylceramide lipidosis in which lysosomal storage phenomena are seen almost only at the ultrastructural level...
January 6, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28109580/cardiac-transplantation-in-friedreich-ataxia-extended-follow-up
#4
Ashley McCormick, Julianna Shinnick, Kim Schadt, Rose Rodriguez, Linda Addonizio, Michio Hirano, Susan Perlman, Kimberly Y Lin, David R Lynch
Friedreich Ataxia (FRDA) is an autosomal recessive neurodegenerative disorder most commonly caused by guanine-adenine-adenine (GAA) trinucleotide repeat expansions in both alleles of the FXN gene. Although progressive ataxia remains the hallmark clinical feature, patients with FRDA are at high risk of developing cardiomyopathy, often resulting in premature death. There is no specific treatment for FRDA-associated cardiomyopathy; even in advanced cardiac failure cardiac transplantation is not commonly pursued...
January 10, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28109263/common-and-uncommon-neurological-manifestations-of-neuroborreliosis-leading-to-hospitalization
#5
Philipp Schwenkenbecher, Refik Pul, Ulrich Wurster, Josef Conzen, Kaweh Pars, Hans Hartmann, Kurt-Wolfram Sühs, Ludwig Sedlacek, Martin Stangel, Corinna Trebst, Thomas Skripuletz
BACKGROUND: Neuroborreliosis represents a relevant infectious disease and can cause a variety of neurological manifestations. Different stages and syndromes are described and atypical symptoms can result in diagnostic delay or misdiagnosis. The aim of this retrospective study was to define the pivotal neurological deficits in patients with neuroborreliosis that were the reason for admission in a hospital. METHODS: We retrospectively evaluated data of patients with neuroborreliosis...
January 21, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28108958/biocompatibility-study-of-three-distinct-carbon-pastes-for-application-as-electrode-material-in-neural-stimulations-and-recordings
#6
Melinda Varga, Paul Wolff, Klaus-Juergen Wolter
Neural interfaces hold great promise for research and treatment of a wide variety of neurological diseases. Medical electrodes are designed to interface with the nervous system and provide control signals for neural prostheses. We fabricated previously a hook-up neural electrode. Here we investigate the in vitro cytotoxicity of three commercial carbon pastes used for printing the conductor tracks of this electrode. At first, the carbon pastes were characterized with respect to their microstructure and chemical composition...
February 2017: Journal of Materials Science. Materials in Medicine
https://www.readbyqxmd.com/read/28108584/astrocytic-transporters-in-alzheimer-s-disease
#7
REVIEW
Chris Ugbode, Yuhan Hu, Benjamin Whalley, Chris Peers, Marcus Rattray, Mark L Dallas
Astrocytes play a fundamental role in maintaining the health and function of the central nervous system. Increasing evidence indicates that astrocytes undergo both cellular and molecular changes at an early stage in neurological diseases, including Alzheimer's disease (AD). These changes may reflect a change from a neuroprotective to a neurotoxic phenotype. Given the lack of current disease-modifying therapies for AD, astrocytes have become an interesting and viable target for therapeutic intervention. The astrocyte transport system covers a diverse array of proteins involved in metabolic support, neurotransmission and synaptic architecture...
February 1, 2017: Biochemical Journal
https://www.readbyqxmd.com/read/28108397/aav9-igf1-protects-tdp-25-cells-from-apoptosis-and-oxidative-stress-partly-via-up-regulating-the-expression-of-vegf-in-vitro
#8
Zhongyao Li, Weisong Duan, Can Cui, Yaling Liu, Chunyan Li, Yakun Liu
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease lacking of curable treatments to date. Adeno-associated virus (AAV) vectors make gene therapy an effective strategy in treating neurological disorders. Despite Kaspar and colleagues have showed that AAV-IGF1 delivery successfully prolonged the survival of SOD1G93A mice, whether IGF-1 act as a protective role in the TDP-43 mutant model still have not been reported. In this study, we proved that AAV9 vector mediated expression of human wild-type IGF-1 protected TDP-25 cells from apoptosis and oxidative stress...
January 17, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28107809/plasma-protein-profiling-for-potential-biomarkers-in-the-early-diagnosis-of-alzheimer-s-disease
#9
Yuki Kitamura, Ryoko Usami, Sahoko Ichihara, Hirotaka Kida, Masayuki Satoh, Hidekazu Tomimoto, Mariko Murata, Shinji Oikawa
OBJECTIVES: Alzheimer's disease (AD) is the most common cause of dementia in elderly persons. Since the pathology of AD develops slowly from a preclinical or early phase into a fully expressed clinical syndrome, at the time of diagnosis the disease has been progressing for many years. To facilitate the early diagnosis of AD, we performed protein profiling of blood in patients with mild AD as defined by the Functional Assessment Staging (FAST) scale. METHODS: Plasma samples from mild AD patients and healthy controls were analyzed using two-dimensional differential gel electrophoresis (2D-DIGE) combined with matrix-assisted laser desorption ionization time-of-flight tandem mass spectrometry (MALDI-TOF/TOF/MS) followed by peptide mass fingerprinting...
January 20, 2017: Neurological Research
https://www.readbyqxmd.com/read/28107736/crystal-structures-binding-interactions-and-adme-evaluation-of-brain-penetrant-n-substituted-indazole-5-carboxamides-as-subnanomolar-selective-monoamine-oxidase-b-and-dual-mao-a-b-inhibitors
#10
Nikolay T Tzvetkov, Hans-Georg Stammler, Beate Neumann, Silvia Hristova, Liudmil Antonov, Marcus Gastreich
The pharmacological and physicochemical analysis of structurally optimized N-alkyl-substituted indazole-5-carboxamides, developed as potential drug and radioligand candidates for the treatment and diagnosis of Parkinson's disease (PD) and other neurological disorders, is reported. Recent efforts have been focused on the development of subnanomolar potent, selective MAO-B (N1-alkyl-substituted compounds 12a-14a and 15) and dual active MAO-A/B (N2-methylated compounds 12b-14b) inhibitors with nanomolar potency towards MAO-B and moderately active against MAO-A enzyme, respectively...
January 11, 2017: European Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28107381/in-vivo-two-photon-imaging-of-astrocytes-in-gfap-gfp-transgenic-mice
#11
Dongjun Guo, Jia Zou, Nicholas Rensing, Michael Wong
Astrocytes play important roles in normal brain function and neurological diseases. In vivo two-photon excitation laser scanning microscopy has the potential to reveal rapid, dynamic structural changes in cells in a variety of physiological and pathological conditions. The type of in vivo imaging method has been shown to affect the plasticity of dendritic spines of neurons, but the optimal in vivo imaging methods of astrocytes have not been established. We compared open-skull and thinned-skull imaging methods for two-photon laser microscopy of live astrocytes in neocortex of GFAP-GFP transgenic mice...
2017: PloS One
https://www.readbyqxmd.com/read/28107234/comparison-between-acrylic-cage-and-polyetheretherketone-peek-cage-in-single-level-anterior-cervical-discectomy-and-fusion-a-randomized-clinical-trial
#12
Majid R Farrokhi, Zahra Nikoo, Mehrnaz Gholami, Khadijeh Hosseini
STUDY DESIGN: Prospective, single-blind randomized-controlled clinical study. OBJECTIVE: To compare polyetheretherketone (PEEK) cage with a novel Acrylic cage to find out which fusion cage yielded better clinical outcomes following single-level anterior cervical discectomy and fusion (ACDF). SUMMARY OF BACKGROUND DATA: ACDF is considered a standard neurosurgical treatment for degenerative diseases of cervical intervertebral disks. There are many options, including bone grafts, bone cement, and spacers made of titanium, carbon fiber, and synthetic materials, used to restore physiological disk height and enhance spinal fusion, but the ideal device, which would provide immediate structural support and subsequent osteointegration and stability, has not been identified yet...
February 2017: Clinical Spine Surgery
https://www.readbyqxmd.com/read/28107174/biological-bases-of-human-musicality
#13
Carla Perrone-Capano, Floriana Volpicelli, Umberto di Porzio
Music is a universal language, present in all human societies. It pervades the lives of most human beings and can recall memories and feelings of the past, can exert positive effects on our mood, can be strongly evocative and ignite intense emotions, and can establish or strengthen social bonds. In this review, we summarize the research and recent progress on the origins and neural substrates of human musicality as well as the changes in brain plasticity elicited by listening or performing music. Indeed, music improves performance in a number of cognitive tasks and may have beneficial effects on diseased brains...
January 20, 2017: Reviews in the Neurosciences
https://www.readbyqxmd.com/read/28106800/frequency-and-pathological-phenotype-of-bovine-astrovirus-ch13-neuros1-infection-in-neurologically-diseased-cattle-towards-assessment-of-causality
#14
Senija Selimovic-Hamza, Céline L Boujon, Monika Hilbe, Anna Oevermann, Torsten Seuberlich
Next-generation sequencing (NGS) has opened up the possibility of detecting new viruses in unresolved diseases. Recently, astrovirus brain infections have been identified in neurologically diseased humans and animals by NGS, among them bovine astrovirus (BoAstV) CH13/NeuroS1, which has been found in brain tissues of cattle with non-suppurative encephalitis. Only a few studies are available on neurotropic astroviruses and a causal relationship between BoAstV CH13/NeuroS1 infections and neurological disease has been postulated, but remains unproven...
January 18, 2017: Viruses
https://www.readbyqxmd.com/read/28106787/anti-nmda-receptor-encephalitis-and-vaccination
#15
Hsiuying Wang
Anti-N-methyl-d-aspartate (Anti-NMDA) receptor encephalitis is an acute autoimmune neurological disorder. The cause of this disease is often unknown, and previous studies revealed that it might be caused by a virus, vaccine or tumor. It occurs more often in females than in males. Several cases were reported to be related to vaccination such as the H1N1 vaccine and tetanus/diphtheria/pertussis and polio vaccines. In this study, we reported an anti-NMDA receptor encephalitis case that may be caused by Japanese encephalitis vaccination...
January 18, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28106101/the-systematic-analysis-of-coding-and-long-non-coding-rnas-in-the-sub-chronic-and-chronic-stages-of-spinal-cord-injury
#16
Raquel Cuevas-Diaz Duran, Han Yan, Yiyan Zheng, Xingfan Huang, Raymond Grill, Dong H Kim, Qilin Cao, Jia Qian Wu
Spinal cord injury (SCI) remains one of the most debilitating neurological disorders and the majority of SCI patients are in the chronic phase. Previous studies of SCI have usually focused on few genes and pathways at a time. In particular, the biological roles of long non-coding RNAs (lncRNAs) have never been characterized in SCI. Our study is the first to comprehensively investigate alterations in the expression of both coding and long non-coding genes in the sub-chronic and chronic stages of SCI using RNA-Sequencing...
January 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28105729/whole-genome-grey-and-white-matter-dna-methylation-profiles-in-dorsolateral-prefrontal-cortex
#17
Jose Vicente Sanchez-Mut, Holger Heyn, Enrique Vidal, Raúl Delgado-Morales, Sebastian Moran, Sergi Sayols, Juan Sandoval, Isidre Ferrer, Manel Esteller, Johannes Gräff
The brain's neocortex is anatomically organized into grey and white matter, which are mainly composed by neuronal and glial cells, respectively. The neocortex can be further divided in different Brodmann areas according to their cytoarchitectural organization, which are associated with distinct cortical functions. There is increasing evidence that brain development and function are governed by epigenetic processes, yet their contribution to the functional organization of the neocortex remains incompletely understood...
January 20, 2017: Synapse
https://www.readbyqxmd.com/read/28105569/the-spectrum-of-niemann-pick-type-c-disease-in-greece
#18
Irene Mavridou, Evangelia Dimitriou, Marie T Vanier, Lluisa Vilageliu, Daniel Grinberg, Philippe Latour, Athina Xaidara, Lilia Lycopoulou, Sevasti Bostantjopoulou, Dimitrios Zafeiriou, Helen Michelakakis
Niemann-Pick type C disease (NPC) is a neurovisceral lysosomal storage disease caused by mutations in either the NPC1 or the NPC2 gene. It is a cellular lipid trafficking disorder characterized by the accumulation of unesterified cholesterol and various sphingolipids in the lysosomes and late endosomes, and it exhibits a broad clinical spectrum. Today, over 420 disease-causing mutations have been identified in the NPC1 and the NPC2 genes. We present the clinical, biochemical, and molecular findings in 14 cases diagnosed in Greece during the last 28 years...
January 20, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28105556/chikungunya-infection-presenting-as-mild-encephalitis-with-a-reversible-lesion-in-the-splenium-a-case-report
#19
Kadam Nagpal, Puneet Agarwal, Amit Kumar, Rajashekhar Reddi
Chikungunya fever is an Aedes mosquito-transmitted infection caused by chikungunya virus, an RNA virus in the family Togaviridae. The disease is characteristically manifested as fever, arthralgia, and/or rash. Various neurological manifestations like meningoencephalitis, myelitis, and myeloneuropathy have been mentioned in various reports. We present a rare case of chikungunya fever presenting with mild encephalitis with a reversible lesion of the splenium (MERS), which showed complete clinical and radiological recovery...
January 19, 2017: Journal of Neurovirology
https://www.readbyqxmd.com/read/28105280/glun2a-selective-pyridopyrimidinone-series-of-nmdar-positive-allosteric-modulators-with-an-improved-in-vivo-profile
#20
Elisia Villemure, Matthew Volgraf, Yu Jiang, Guosheng Wu, Cuong Q Ly, Po-Wai Yuen, Aijun Lu, Xifeng Luo, Mingcui Liu, Shun Zhang, Patrick J Lupardus, Heidi J A Wallweber, Bianca M Liederer, Gauri Deshmukh, Emile Plise, Suzanne Tay, Tzu-Ming Wang, Jesse E Hanson, David H Hackos, Kimberly Scearce-Levie, Jacob B Schwarz, Benjamin D Sellers
The N-methyl-d-aspartate receptor (NMDAR) is an ionotropic glutamate receptor, gated by the endogenous coagonists glutamate and glycine, permeable to Ca(2+) and Na(+). NMDAR dysfunction is associated with numerous neurological and psychiatric disorders, including schizophrenia, depression, and Alzheimer's disease. Recently, we have disclosed GNE-0723 (1), a GluN2A subunit-selective and brain-penetrant positive allosteric modulator (PAM) of NMDARs. This work highlights the discovery of a related pyridopyrimidinone core with distinct structure-activity relationships, despite the structural similarity to GNE-0723...
January 12, 2017: ACS Medicinal Chemistry Letters
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