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Dementia in Motor Neuron Disease

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New insights into Wnt signaling alterations in amyotrophic lateral sclerosis: a potential therapeutic target?

Carlos Gonzalez-Fernandez, Pau González, Francisco Javier Rodríguez,

Amyotrophic lateral sclerosis is a fatal neurodegenerative disorder characterized by upper and lower motor neuron degeneration, which leads to progressive paralysis of skeletal muscles and, ultimately, respiratory failure between 2-5 years after symptom onset. Unfortunately, currently accepted treatments for amyotrophic lateral sclerosis are extremely scarce and only provide modest benefit. ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(9):1580-1589]

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RT-QuIC-based detection of alpha-synuclein seeding activity in brains of dementia with Lewy Body patients and of a transgenic mouse model of synucleinopathy.

Jung-Youn Han, Hyung-Sup Jang, Alison J E Green, Young Pyo Choi,

RT-QuIC is a shaking-based cyclic amplification technique originally developed in the prion field to detect minute amounts of scrapie prion protein (PrPSc). In this study, we applied the RT-QuIC assay to investigate a-synuclein (a-syn) seeding activity in brains of Dementia with Lewy Body (DLB) patients and in brains of G2-3 ... Read more >>

Prion (Prion)
[2020, 14(1):88-94]

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The roles of atypical protein kinase Cs (aPKCs) in the nervous system: targets for neuroregeneration?

Dennis Dik-Long Chau, Kwok-Fai Lau,

Neural Regen Res (Neural regeneration research)
[2020, 15(8):1475-1476]

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Exercise promotes recovery after motoneuron injury via hormonal mechanisms.

Cory Chew, Dale R Sengelaub,

Injuries to spinal motoneurons manifest in a variety of forms, including damage to peripheral axons, neurodegenerative disease, or direct insult centrally. Such injuries produce a variety of negative structural and functional changes in both the directly affected and neighboring motoneurons. Exercise is a relatively simple behavioral intervention that has been ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(8):1373-1376]

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Type XIX collagen: a promising biomarker from the basement membranes.

Ana C Calvo, Laura Moreno, Leticia Moreno, Janne M Toivonen, Raquel Manzano, Nora Molina, Miriam de la Torre, Tresa López, Francisco J Miana-Mena, María J Muñoz, Pilar Zaragoza, Pilar Larrodé, Alberto García-Redondo, Rosario Osta,

Among collagen members in the collagen superfamily, type XIX collagen has raised increasing interest in relation to its structural and biological roles. Type XIX collagen is a Fibril-Associated Collagen with Interrupted Triple helices member, one main subclass of collagens in this superfamily. This collagen contains a triple helix composed of ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(6):988-995]

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Novel small molecule TRVA242 targets neuromuscular junction in amyotrophic lateral sclerosis.

Poulomee Bose,

Neural Regen Res (Neural regeneration research)
[2020, 15(6):1041-1042]

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Atypical antipsychotics, more than just an antipsychotic.

Hiram Tendilla-Beltran, Gonzalo Flores,

Neural Regen Res (Neural regeneration research)
[2020, 15(8):1477-1478]

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VRK1 variants in two Portuguese unrelated patients with childhood-onset motor neuron disease.

Daniela Pimenta Silva, Mariana Soeiro E Sá, Fernando Silveira, Susana Pinto, Marta Gromicho, Ana Berta Sousa, Miguel Leão, Mamede De Carvalho,

VRK1 encodes a serine/protein kinase possibly involved in pathways related to amyotrophic lateral sclerosis (ALS) pathogenesis. Pathogenic variants in VRK1 have been related to different phenotypes. We describe the clinical phenotype of two unrelated Portuguese patients with different VRK1 variants. Both patients presented a bilateral distal weakness in lower limbs ... Read more >>

Amyotroph Lateral Scler Frontotemporal Degener (Amyotrophic lateral sclerosis & frontotemporal degeneration)
[2020, :1-5]

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Primary lateral sclerosis: diagnosis and management.

Martin R Turner, Kevin Talbot,

Primary lateral sclerosis (PLS) is a rare neurodegenerative disorder at the upper motor neurone extreme of the spectrum of motor neurone disease. The diagnosis is clinical and based on the characteristic features of slowly progressive spasticity beginning in the lower limbs, or more rarely with spastic dysarthria, typically presenting around ... Read more >>

Pract Neurol (Practical neurology)
[2020, :]

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Aggressive FUS-Mutant Motor Neuron Disease Without Profound Spinal Cord Pathology.

Yan Chen Wongworawat, Yin Allison Liu, Ravi Raghavan, Charles L White, Robin Dietz, Craig Zuppan, Jeffrey Rosenfeld,

A 29-year-old man presented with rapidly progressive severe neck weakness, asymmetrical bilateral upper extremity weakness, bulbar dysfunction, profound muscle wasting, and weight loss. Within 1 year, his speech became unintelligible, he became gastrostomy- and tracheostomy/ventilator-dependent, and wheelchair bound. Electrophysiology suggested motor neuron disease. Whole exome sequencing revealed a heterozygous pathogenic ... Read more >>

J. Neuropathol. Exp. Neurol. (Journal of neuropathology and experimental neurology)
[2020, 79(4):365-369]

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Characteristic Features of FUS Inclusions in Spinal Motor Neurons of Sporadic Amyotrophic Lateral Sclerosis.

Kensuke Ikenaka, Shinsuke Ishigaki, Yohei Iguchi, Kaori Kawai, Yusuke Fujioka, Satoshi Yokoi, Rehab F Abdelhamid, Seiichi Nagano, Hideki Mochizuki, Masahisa Katsuno, Gen Sobue,

Alterations of RNA metabolism caused by mutations in RNA-binding protein genes, such as transactivating DNA-binding protein-43 (TDP-43) and fused in sarcoma (FUS), have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS). Unlike the accumulation of TDP43, which is accepted as a pathological hall mark of sporadic ALS (sALS), ... Read more >>

J. Neuropathol. Exp. Neurol. (Journal of neuropathology and experimental neurology)
[2020, 79(4):370-377]

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The multifaceted role of kinases in amyotrophic lateral sclerosis: genetic, pathological and therapeutic implications.

Wenting Guo, Tijs Vandoorne, Jolien Steyaert, Kim A Staats, Ludo Van Den Bosch,

Amyotrophic lateral sclerosis is the most common degenerative disorder of motor neurons in adults. As there is no cure, thousands of individuals who are alive at present will succumb to the disease. In recent years, numerous causative genes and risk factors for amyotrophic lateral sclerosis have been identified. Several of ... Read more >>

Brain (Brain : a journal of neurology)
[2020, :]

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IN VITRO AND IN VIVO MODELS OF AMYOTROPHIC LATERAL SCLEROSIS: AN UPDATED OVERVIEW.

Auderlan M Gois, Deise M F Mendonça, Marco Aurelio M Freire, Jose R Santos,

Amyotrophic Lateral Sclerosis (ALS) is a progressive, neurodegenerative disease characterized by loss of upper motor neurons (UMN) and lower motor neurons (LMN). Disease affects people all over the world and is more prevalent in men. Patients with ALS develop extensive muscle wasting, paralysis and ultimately death, with a median survival ... Read more >>

Brain Res. Bull. (Brain research bulletin)
[2020, :]

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Rifampicin quinone pretreatment improves neuronal survival by modulating microglia inflammation induced by α-synuclein.

Leonardo Acuna, Natalia S Corbalán, Rita Raisman-Vozari,

Neural Regen Res (Neural regeneration research)
[2020, 15(8):1473-1474]

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Exosomes as mediators of neuron-glia communication in neuroinflammation.

María Pascual, Francesc Ibáñez, Consuelo Guerri,

In recent years, a type of extracellular vesicles named exosomes has emerged that play an important role in intercellular communication under physiological and pathological conditions. These nanovesicles (30-150 nm) contain proteins, RNAs and lipids, and their internalization by bystander cells could alter their normal functions. This review focuses on recent ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(5):796-801]

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Upper motor neuron burden measurement in motor neuron diseases: Does one scale fit all?

Suma Babu,

Muscle Nerve (Muscle & nerve)
[2020, 61(4):431-432]

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The Loss of TBK1 Kinase Activity in Motor Neurons or in All Cell Types Differentially Impacts ALS Disease Progression in SOD1 Mice.

Valeria Gerbino, Esther Kaunga, Junqiang Ye, Daniele Canzio, Sean O'Keeffe, Noam D Rudnick, Paolo Guarnieri, Cathleen M Lutz, Tom Maniatis,

DNA sequence variants in the TBK1 gene associate with or cause sporadic or familial amyotrophic lateral sclerosis (ALS). Here we show that mice bearing human ALS-associated TBK1 missense loss-of-function mutations, or mice in which the Tbk1 gene is selectively deleted in motor neurons, do not display a neurodegenerative disease phenotype. ... Read more >>

Neuron (Neuron)
[2020, :]

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Fabry Disease With Concomitant Lewy Body Disease.

Kelly Del Tredici, Albert C Ludolph, Simone Feldengut, Christian Jacob, Heinz Reichmann, Jürgen R Bohl, Heiko Braak,

Although Gaucher disease can be accompanied by Lewy pathology (LP) and extrapyramidal symptoms, it is unknown if LP exists in Fabry disease (FD), another progressive multisystem lysosomal storage disorder. We aimed to elucidate the distribution patterns of FD-related inclusions and LP in the brain of a 58-year-old cognitively unimpaired male ... Read more >>

J. Neuropathol. Exp. Neurol. (Journal of neuropathology and experimental neurology)
[2020, 79(4):378-392]

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Altered skeletal muscle glucose-fatty acid flux in amyotrophic lateral sclerosis (ALS)

Frederik Steyn, Siobhan Kirk, Tesfaye Tefera, Teresa Xie, Timothy Tracey, Dean Kelk, Elyse Wimberger, Fleur Garton, Llion Roberts, Sarah Chapman, Jeff Coombes, Matthew Leevy, Alberto Ferri, Cristiana Valle, Frédérique René, Jean-Philippe Loeffler, Pamela McCombe, Robert Henderson, Shyuan Ngo,

Abstract Amyotrophic lateral sclerosis (ALS) is characterized by the degeneration of upper and lower motor neurons, yet an increasing number of studies in both mouse models and patients with ALS suggest that altered metabolic homeostasis is a feature of disease. Pre-clinical and clinical studies have shown that modulation of energy ... Read more >>

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Rapid Eye Movement Sleep Behavior Disorder and Neurodegenerative Diseases: An Update.

Feng Zhang, Long Niu, Xinyao Liu, Yufei Liu, Song Li, Huan Yu, Weidong Le,

Rapid eye movement sleep behavior disorder (RBD) is a sleep behavior disorder characterized by abnormal behaviors and loss of muscle atonia during rapid eye movement (REM) sleep. RBD is generally considered to be associated with synucleinopathies, such as Parkinson’s disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy ... Read more >>

Aging Dis (Aging and Disease)
[2020, 11(2):315-326]

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The Role of Sex and Sex Hormones in Neurodegenerative Diseases.

Elisabetta Vegeto, Alessandro Villa, Sara Della Torre, Valeria Crippa, Paola Rusmini, Riccardo Cristofani, Mariarita Galbiati, Adriana Maggi, Angelo Poletti,

Neurodegenerative diseases (NDs) are a wide class of disorders of the central nervous system (CNS) with unknown etiology. Several factors were hypothesized to be involved in the pathogenesis of these diseases, including genetic and environmental factors. Many of these diseases show a sex prevalence and sex steroids were shown to ... Read more >>

Endocr. Rev. (Endocrine reviews)
[2020, 41(2):]

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Fast-tracking regenerative medicine for traumatic brain injury.

Brooke Bonsack, Matt Heyck, Chase Kingsbury, Blaise Cozene, Nadia Sadanandan, Jea-Young Lee, Cesar V Borlongan,

Traumatic brain injury remains a global health crisis that spans all demographics, yet there exist limited treatment options that may effectively curtail its lingering symptoms. Traumatic brain injury pathology entails a progression from primary injury to inflammation-mediated secondary cell death. Sequestering this inflammation as a means of ameliorating the greater ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(7):1179-1190]

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The transcription factor Nurr1 is up-regulated in amyotrophic lateral sclerosis patients and SOD1-G93A mice.

Valeria Valsecchi, Marina Boido, Francesca Montarolo, Michela Guglielmotto, Simona Perga, Serena Martire, Santina Cutrupi, Andrea Iannello, Nadia Gionchiglia, Elena Signorino, Andrea Calvo, Giuseppe Fuda, Adriano Chiò, Antonio Bertolotto, Alessandro Vercelli,

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that affects both lower and upper motor neurons (MNs) in the central nervous system (CNS). ALS etiology is highly multifactorial and multifarious, and an effective treatment is still lacking. Neuroinflammation is a hallmark of ALS and could be targeted to develop new ... Read more >>

Dis Model Mech (Disease Models & Mechanisms)
[2020, :]

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Mirror Movements in Amyotrophic Lateral Sclerosis: A Combined Study Using Diffusion Tensor Imaging and Transcranial Magnetic Stimulation.

Matthias Wittstock, Nora Wilde, Annette Grossmann, Elisabeth Kasper, Stefan Teipel,

Objective: Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disorder predominantly affecting the motor system. In a number of patients, mirror movements (MMs) suggest involvement of transcallosal fiber tracts in conjunction with upper motor neuron involvement. The aim of the study was to elucidate functional and structural alterations of callosal integrity ... Read more >>

Front Neurol (Frontiers in Neurology)
[2020, 11:164]

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Spinal cord hypermetabolism extends to skeletal muscle in amyotrophic lateral sclerosis: a computational approach to [18F]-fluorodeoxyglucose PET/CT images.

Matteo Bauckneht, Rita Lai, Alberto Miceli, Daniela Schenone, Vanessa Cossu, Maria Isabella Donegani, Stefano Raffa, Anna Borra, Stefano Marra, Cristina Campi, Annamaria Orengo, Anna Maria Massone, Alberto Tagliafico, Claudia Caponnetto, Corrado Cabona, Angelina Cistaro, Adriano Chiò, Silvia Morbelli, Flavio Nobili, Gianmario Sambuceti, Michele Piana, Cecilia Marini,

PURPOSE:Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease leading to neuromuscular palsy and death. We propose a computational approach to [18F]-fluorodeoxyglucose (FDG) PET/CT images to analyze the structure and metabolic pattern of skeletal muscle in ALS and its relationship with disease aggressiveness. MATERIALS AND METHODS:A computational 3D method was used ... Read more >>

EJNMMI Res (EJNMMI research)
[2020, 10(1):23]

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