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https://www.readbyqxmd.com/read/28936169/movement-related-activity-of-human-subthalamic-neurons-during-a-reach-to-grasp-task
#1
Monika Pötter-Nerger, Rene Reese, Frank Steigerwald, Jan Arne Heiden, Jan Herzog, Christian K E Moll, Wolfgang Hamel, Uri Ramirez-Pasos, Daniela Falk, Maximilian Mehdorn, Christian Gerloff, Günther Deuschl, Jens Volkmann
The aim of the study was to record movement-related single unit activity (SUA) in the human subthalamic nucleus (STN) during a standardized motor task of the upper limb. We performed microrecordings from the motor region of the human STN and registered kinematic data in 12 patients with Parkinson's disease (PD) undergoing deep brain stimulation surgery (seven women, mean age 62.0 ± 4.7 years) while they intraoperatively performed visually cued reach-to-grasp movements using a grip device. SUA was analyzed offline in relation to different aspects of the movement (attention, start of the movement, movement velocity, button press) in terms of firing frequency, firing pattern, and oscillation...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/28930607/transcriptomics-in-amyotrophic-lateral-sclerosis
#2
Marios G Krokidis, Panagiotis Vlamos
Amyotrophic lateral sclerosis (ALS) is an adult-onset, incurable neurodegenerative disease characterized by the selective death of upper and lowers motor neurons in the spinal cord, brainstem and motor cortex, which ultimately leads to paralysis and death within 2-3 years of onset. ALS is poorly understood, although multiple studies have been proposed to explain the pathophysiological mechanisms of the disorder. The development of microarray technology, for simultaneous analysis of the transcriptional expression of thousands of genes, has provided new possibilities to get better insights into the pathogenesis of ALS, and most important, potential new candidate targets for novel treatments...
January 1, 2018: Frontiers in Bioscience (Elite Edition)
https://www.readbyqxmd.com/read/28926131/region-specific-impairment-of-the-cervical-spinal-cord-sc-in-amyotrophic-lateral-sclerosis-a-preliminary-study-using-sc-templates-and-quantitative-mri-diffusion-tensor-imaging-inhomogeneous-magnetization-transfer
#3
Henitsoa Rasoanandrianina, Aude-Marie Grapperon, Manuel Taso, Olivier M Girard, Guillaume Duhamel, Maxime Guye, Jean-Philippe Ranjeva, Shahram Attarian, Annie Verschueren, Virginie Callot
In this preliminary study, our objective was to investigate the potential of high-resolution anatomical imaging, diffusion tensor imaging (DTI) and conventional/inhomogeneous magnetization transfer imaging [magnetization transfer (MT)/inhomogeneous magnetization transfer (ihMT)] at 3 T, analyzed with template-extracted regions of interest, to measure the atrophy and structural changes of white (WM) and gray (GM) matter spinal cord (SC) occurring in patients with amyotrophic lateral sclerosis (ALS). Ten patients with ALS and 20 age-matched healthy controls were recruited...
September 19, 2017: NMR in Biomedicine
https://www.readbyqxmd.com/read/28926110/pacap-and-pac1r-are-differentially-expressed-in-motor-cortex-of-amyotrophic-lateral-sclerosis-patients-and-support-survival-of-ipsc-derived-motor-neurons
#4
Gabriele Bonaventura, Rosario Iemmolo, Agata Grazia D'Amico, Valentina La Cognata, Erminio Costanzo, Mario Zappia, Velia D'Agata, Francesca Luisa Conforti, Eleonora Aronica, Sebastiano Cavallaro
Amyotrophic lateral sclerosis (ALS) is a fatal and disabling neurodegenerative disease characterized by upper and lower motor neurons depletion. In our previous work, comprehensive genomic profiling of 41 motor cortex samples enabled to discriminate controls from sporadic ALS patients, and segregated these latter into two distinct subgroups (SALS1 and SALS2), each associated with different deregulated genes. In the present study, we focused our attention on two of them, Pituitary Adenylate Cyclase-Activating Polypeptide (PACAP) and its type 1 receptor (PAC1R), and validated the results of the transcriptome experiments by quantitative reverse transcription-polymerase chain reaction (qRT-PCR), immunohistochemistry and western blot analysis...
September 19, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28914734/revisiting-the-concept-of-amyotrophic-lateral-sclerosis-as-a-multisystems-disorder-of-limited-phenotypic-expression
#5
Michael J Strong
PURPOSE OF REVIEW: The current review will examine the contemporary evidence that amyotrophic lateral sclerosis (ALS) is a syndrome in which the unifying feature is a progressive loss of upper and lower motor neuron function. RECENT FINDINGS: Although ALS is traditionally viewed as a neurodegenerative disorder affecting the motor neurons, there is considerable phenotypic heterogeneity and widespread involvement of the central nervous system. A broad range of both causative and disease modifying genetic variants are associated with both sporadic and familial forms of ALS...
September 13, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28912673/improving-the-delivery-of-sod1-antisense-oligonucleotides-to-motor-neurons-using-calcium-phosphate-lipid-nanoparticles
#6
Liyu Chen, Clare Watson, Marco Morsch, Nicholas J Cole, Roger S Chung, Darren N Saunders, Justin J Yerbury, Kara L Vine
Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease affecting the upper and lower motor neurons in the motor cortex and spinal cord. Abnormal accumulation of mutant superoxide dismutase I (SOD1) in motor neurons is a pathological hallmark of some forms of the disease. We have shown that the orderly progression of the disease may be explained by misfolded SOD1 cell-to-cell propagation, which is reliant upon its active endogenous synthesis. Reducing the levels of SOD1 is therefore a promising therapeutic approach...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28906030/pathological-and-immunoblot-analysis-of-phosphorylated-tdp-43-in-sporadic-amyotrophic-lateral-sclerosis-with-pallido-nigro-luysian-degeneration
#7
Akiko Uchino, Mieko Ogino, Junko Takahashi-Fujigasaki, Saori Oonuma, Naomi Kanazawa, Sabine Kajita, Masaaki Ichinoe, Masato Hasegawa, Kazutoshi Nishiyama, Shigeo Murayama
Transactivation response DNA-binding protein 43 kDa (TDP-43) is a key protein of sporadic amyotrophic lateral sclerosis (ALS), and phosphorylated form of TDP-43 (p-TDP-43) is a major pathological protein that accumulates in sporadic ALS. p-TDP-43 is found not only in primary motor neurons, but often propagates to non-motor systems as well. However, pallido-nigro-luysian (PNL) degeneration (PNLD) is rarely associated with ALS. We describe here a 68-year-old ALS patient presenting severe PNLD. He had difficulty walking due to poor movement of his right leg, and was diagnosed as having Parkinson's disease because of akinesia...
September 14, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28904104/the-superior-colliculus-and-the-steering-of-saccades-toward-a-moving-visual-target
#8
Laurent Goffart, Aaron Lee Cecala, Neeraj J Gandhi
Following the suggestion that a command encoding the current target location feeds the oculomotor system during interceptive saccades, we tested the involvement of the deep superior colliculus (dSC). Extracellular activity of 52 saccade-related neurons was recorded in three monkeys while they generated saccades to targets that were static or moving along the preferred axis, away from (outward) or toward a fixated target (inward) with a constant speed (20°/s). Vertical and horizontal motions were tested when possible...
September 13, 2017: Journal of Neurophysiology
https://www.readbyqxmd.com/read/28895473/neuroinflammation-in-amyotrophic-lateral-sclerosis-role-of-redox-dys-regulation
#9
Nadia D'Ambrosi, Mauro Cozzolino, Maria Teresa Carrì
SIGNIFICANCE: Amyotrophic lateral sclerosis (ALS) is due to degeneration of upper and lower motor neurons in the anterior horn of the spinal cord and in the motor cortex. Mechanisms leading to motor neuron death are complex and currently the disease is untreatable. Recent Advances. Work in genetic models of ALS indicates that an imbalance in the crosstalk that physiologically exists between motor neurons and the surrounding cells is eventually detrimental to motor neurons. In particular, the cascade of events collectively known as neuroinflammation and mainly characterized by a reactive phenotype of astrocytes and microglia, moderate infiltration of peripheral immune cells and elevated levels of inflammatory mediators has been consistently observed in motor regions of the central nervous system in sporadic and familial ALS, constituting a hallmark of the disease...
September 12, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/28878620/autophagy-dysregulation-in-als-when-protein-aggregates-get-out-of-hand
#10
REVIEW
Nandini Ramesh, Udai Bhan Pandey
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that results from the loss of upper and lower motor neurons. One of the key pathological hallmarks in diseased neurons is the mislocalization of disease-associated proteins and the formation of cytoplasmic aggregates of these proteins and their interactors due to defective protein quality control. This apparent imbalance in the cellular protein homeostasis could be a crucial factor in causing motor neuron death in the later stages of the disease in patients...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28878414/atlanto-axial-malformation-in-an-adult-quarter-horse-gelding
#11
Robert Cole, Jennifer Taintor, Reid Hanson
An adult gelding was evaluated for bilateral intermittent forelimb lameness of approximately 2 years duration. The horse was found to have grade 2/5 upper motor neuron-general proprioception ataxia with no cranial nerve deficits. During radiographic and gross necropsy examinations a novel atlanto-axial malformation of possible congenital etiology was found.
September 2017: Canadian Veterinary Journal. la Revue Vétérinaire Canadienne
https://www.readbyqxmd.com/read/28871262/role-of-neuroinflammation-in-amyotrophic-lateral-sclerosis-cellular-mechanisms-and-therapeutic-implications
#12
REVIEW
Jia Liu, Fei Wang
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects upper motor neurons (MNs) comprising the corticospinal tract and lower MNs arising from the brain stem nuclei and ventral roots of the spinal cord, leading to fatal paralysis. Currently, there are no effective therapies for ALS. Increasing evidence indicates that neuroinflammation plays an important role in ALS pathogenesis. The neuroinflammation in ALS is characterized by infiltration of lymphocytes and macrophages, activation of microglia and reactive astrocytes, as well as the involvement of complement...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28871219/p2x7-receptor-activation-modulates-autophagy-in-sod1-g93a-mouse-microglia
#13
Paola Fabbrizio, Susanna Amadio, Savina Apolloni, Cinzia Volonté
Autophagy and inflammation play determinant roles in the pathogenesis of Amyotrophic Lateral Sclerosis (ALS), an adult-onset neurodegenerative disease characterized by deterioration and final loss of upper and lower motor neurons (MN) priming microglia to sustain neuroinflammation and a vicious cycle of neurodegeneration. Given that extracellular ATP through P2X7 receptor constitutes a neuron-to-microglia alarm signal implicated in ALS, and that P2X7 affects autophagy in immune cells, we have investigated if autophagy can be directly triggered by P2X7 activation in primary microglia from superoxide dismutase 1 (SOD1)-G93A mice...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28870058/technologically-advanced-assessment-of-upper-limb-spasticity-a-pilot-study
#14
Federico Posteraro, Simona Crea, Stefano Mazzoleni, Mihai Berteanu, Ileana Ciobanu, Nicola Vitiello, Marco Cempini, Sabata Gervasio, Natalie Mrachacz-Kersting
BACKGROUND: Spasticity is a muscle disorder associated with upper motor neuron syndrome occurring in neurological disorders, such as stroke, multiple sclerosis, spinal cord injury and others. It influences the patient's rehabilitation, interfering with function, limiting independence, causing pain and producing secondary impairments, such as contractures or other complications. Due to the heterogeneity of clinical signs of spasticity, there is no agreement on the most appropriate assessment and measurement modality for the evaluation of treatment outcomes...
September 4, 2017: European Journal of Physical and Rehabilitation Medicine
https://www.readbyqxmd.com/read/28860970/failure-to-deliver-and-translate-new-insights-into-rna-dysregulation-in-als
#15
REVIEW
Alyssa N Coyne, Benjamin L Zaepfel, Daniela C Zarnescu
Amyotrophic Lateral Sclerosis (ALS) is a progressive and fatal neurodegenerative disease affecting both upper and lower motor neurons. The molecular mechanisms underlying disease pathogenesis remain largely unknown. Multiple genetic loci including genes involved in proteostasis and ribostasis have been linked to ALS providing key insights into the molecular mechanisms underlying disease. In particular, the identification of the RNA binding proteins TDP-43 and fused in sarcoma (FUS) as causative factors of ALS resulted in a paradigm shift centered on the study of RNA dysregulation as a major mechanism of disease...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28855684/massive-transcriptome-sequencing-of-human-spinal-cord-tissues-provides-new-insights-into-motor-neuron-degeneration-in-als
#16
Anna Maria D'Erchia, Angela Gallo, Caterina Manzari, Susanna Raho, David S Horner, Matteo Chiara, Alessio Valletti, Italia Aiello, Francesca Mastropasqua, Loredana Ciaccia, Franco Locatelli, Francesco Pisani, Grazia Paola Nicchia, Maria Svelto, Graziano Pesole, Ernesto Picardi
ALS is a devastating and debilitating human disease characterized by the progressive death of upper and lower motor neurons. Although much effort has been made to elucidate molecular determinants underlying the onset and progression of the disorder, the causes of ALS remain largely unknown. In the present work, we have deeply sequenced whole transcriptome from spinal cord ventral horns of post-mortem ALS human donors affected by the sporadic form of the disease (which comprises ~90% of the cases but which is less investigated than the inherited form of the disease)...
August 30, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28852382/mitochondrial-quality-control-in-amyotrophic-lateral-sclerosis-towards-a-common-pathway
#17
REVIEW
Bilal Khalil, Jean-Charles Liévens
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder characterized by loss of upper and lower motor neurons. Different mechanisms contribute to the disease initiation and progression, including mitochondrial dysfunction which has been proposed to be a central determinant in ALS pathogenesis. Indeed, while mitochondrial defects have been mainly described in ALS-linked SOD1 mutants, it is now well established that mitochondria become also dysfunctional in other ALS conditions. In such context, the mitochondrial quality control system allows to restore normal functioning of mitochondria and to prevent cell death, by both eliminating and replacing damaged mitochondrial components or by degrading the entire organelle through mitophagy...
July 2017: Neural Regeneration Research
https://www.readbyqxmd.com/read/28839321/late-onset-hirayama-disease-presenting-with-ulnar-neuropathy-a-case-report
#18
Meral Bilgilisoy Filiz, Tuncay Cakir, Naciye Fusun Toraman, Sebnem Koldas DoGan, Iclal Erdem Toslak, Hilmi Uysal
Hirayama disease (HD) was first reported by Hirayama et al. in 1959. The disease is considered as a type of benign focal motor neuron disease that primarily affects upper limbs of young males. In this case report, we present a man aged 40-years with rapidly progressive weakness and atrophy in his left hand. The findings of nerve conduction studies were consistent with left ulnar neuropathy at the elbow. Flexion magnetic resonance imaging (MRI) revealed minimal enlargement of the posterior epidural space and anterior displacement of the spinal cord...
August 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28833372/axonal-components-of-nerves-innervating-the-human-arm
#19
Bernhard Gesslbauer, Laura A Hruby, Aidan D Roche, Dario Farina, Roland Blumer, Oskar C Aszmann
OBJECTIVE: Axons traveling within the brachial plexus are responsible for the dexterous control of human arm and hand movements. Despite comprehensive knowledge on the topographical anatomy of nerves innervating the human upper limbs, the definite quantity of sensory and motor axons within this neural network remains elusive. Our aim was to perform a quantitative analysis of the axonal components of human upper limb nerves based on highly specific molecular features from spinal cord level to the terminal nerves at wrist level...
August 18, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28828608/eaat2-and-the-molecular-signature-of-amyotrophic-lateral-sclerosis
#20
Lauren Taylor Rosenblum, Davide Trotti
Amyotrophic lateral sclerosis (ALS) is a rapid and fatal neurodegenerative disease, primarily affecting upper and lower motor neurons. It is an extremely heterogeneous disease in both cause and symptom development, and its mechanisms of pathogenesis remain largely unknown. Excitotoxicity, a process caused by excessive glutamate signaling, is believed to play a substantial role, however. Excessive glutamate release, changes in postsynaptic glutamate receptors, and reduction of functional astrocytic glutamate transporters contribute to excitotoxicity in ALS...
2017: Advances in Neurobiology
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