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Primary lateral sclerosis

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https://www.readbyqxmd.com/read/29785360/identical-patterns-of-cortico-efferent-tract-involvement-in-primary-lateral-sclerosis-and-amyotrophic-lateral-sclerosis-a-tract-of-interest-based-mri-study
#1
Hans-Peter Müller, Martin Gorges, Rebecca Kassubek, Johannes Dorst, Albert C Ludolph, Jan Kassubek
Background: There is an ongoing debate whether primary lateral sclerosis (PLS) should be regarded as an independent disease entity separate from amyotrophic lateral sclerosis (ALS) or as a slowly progressive variant of ALS. Objective: The study was designed to investigate specific white matter alterations in diffusion tensor imaging (DTI) data from PLS patients by a hypothesis-guided tract-of-interest-based approach compared with 'classical' ALS patients and healthy controls, in order to identify microstructural changes according to the neuropathologically defined ALS affectation pattern in vivo...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/29779213/differential-toxicity-of-tdp-43-isoforms-depends-on-their-sub-mitochondrial-localization-in-neuronal-cells
#2
Illari Salvatori, Alberto Ferri, Silvia Scaricamazza, Ilaria Giovannelli, Alessia Serrano, Simona Rossi, Nadia D'Ambrosi, Mauro Cozzolino, Andrea Di Giulio, Sandra Moreno, Cristiana Valle, Maria Teresa Carrì
TAR DNA binding protein 43 (TDP-43) is an RNA binding protein and a major component of protein aggregates found in Amyotrophic Lateral Sclerosis and several other neurodegenerative diseases. TDP-43 exists as a full length protein and as two shorter forms of 25 and 35 kDa. Full length mutant TDP-43s found in ALS patients re-localize from the nucleus to the cytoplasm and in part to mitochondria, where they exert a toxic role associated with neurodegeneration. However, induction of mitochondrial damage by TDP-43 fragments is yet to be clarified...
May 20, 2018: Journal of Neurochemistry
https://www.readbyqxmd.com/read/29772957/brain-machine-interfaces-powerful-tools-for-clinical-treatment-and-neuroscientific-investigations
#3
Marc W Slutzky
Brain-machine interfaces (BMIs) have exploded in popularity in the past decade. BMIs, also called brain-computer interfaces, provide a direct link between the brain and a computer, usually to control an external device. BMIs have a wide array of potential clinical applications, ranging from restoring communication to people unable to speak due to amyotrophic lateral sclerosis or a stroke, to restoring movement to people with paralysis from spinal cord injury or motor neuron disease, to restoring memory to people with cognitive impairment...
May 1, 2018: Neuroscientist: a Review Journal Bringing Neurobiology, Neurology and Psychiatry
https://www.readbyqxmd.com/read/29767817/-motor-neuron-diseases-clinical-and-genetic-differential-diagnostics
#4
REVIEW
M Regensburger, N Weidner, Z Kohl
The causes of degenerative disease of the upper and lower motor neurons are incompletely understood. In this review the current concepts in the clinical and genetic differential diagnostics of motor neuron diseases are presented. Hereditary spastic paraplegia, primary lateral sclerosis, spinal muscular atrophy and amyotrophic lateral sclerosis are explained, structured according to the affection of the upper and/or lower motor neuron. The substantial variability in the presentation and course of motor neuron diseases as well as the lack of specific laboratory tests hinder an early diagnosis...
May 16, 2018: Der Nervenarzt
https://www.readbyqxmd.com/read/29740862/integrated-mri-and-11-c-pbr28-pet-imaging-in-amyotrophic-lateral-sclerosis
#5
Mohamad J Alshikho, Nicole R Zürcher, Marco L Loggia, Paul Cernasov, Beverly Reynolds, Olivia Pijanowski, Daniel B Chonde, David Izquierdo Garcia, Caterina Mainero, Ciprian Catana, James Chan, Suma Babu, Sabrina Paganoni, Jacob M Hooker, Nazem Atassi
OBJECTIVE: To characterize [11 C]-PBR28 brain uptake using positron emission tomography (PET) in people with amyotrophic lateral sclerosis (ALS), and primary lateral sclerosis (PLS). We have previously shown increased [11 C]-PBR28 uptake in the precentral gyrus in a small group of ALS patients. Herein, we confirm our initial finding, study the longitudinal changes, and characterize the gray vs. white matter distribution of [11 C]-PBR28 uptake in a larger cohort of patients with ALS and PLS...
May 8, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29739754/dexpramipexole-as-an-oral-steroid-sparing-agent-in-hypereosinophilic-syndromes
#6
Sandhya R Panch, Michael E Bozik, Thomas Brown, Michelle Makiya, Calman Prussin, Donald G Archibald, Gregory T Hebrank, Mary Sullivan, Xiaoping Sun, Lauren Wetzler, JeanAnne Ware, Michael P Fay, Cynthia E Dunbar, Steven I Dworetzky, Paneez Khoury, Irina Maric, Amy D Klion
Hypereosinophilic syndromes (HES) are a heterogeneous group of disorders characterized by peripheral eosinophilia and eosinophil-related end organ damage. Whereas most patients respond to glucocorticoid (GC) therapy, high doses are often necessary and side effects are common. Dexpramipexole (KNS 760704), an orally bioavailable synthetic amino-benzothiazole, showed an excellent safety profile and was coincidentally noted to significantly decrease absolute eosinophil counts (AEC) in a phase 3 trial for amyotrophic lateral sclerosis...
May 8, 2018: Blood
https://www.readbyqxmd.com/read/29731706/phosphorylation-of-threonine-175-tau-in-the-induction-of-tau-pathology-in-amyotrophic-lateral-sclerosis-frontotemporal-spectrum-disorder-als-ftsd-a-review
#7
REVIEW
Alexander J Moszczynski, Matthew A Hintermayer, Michael J Strong
Approximately 50-60% of all patients with amyotrophic lateral sclerosis (ALS) will develop a deficit of frontotemporal function, ranging from frontotemporal dementia (FTD) to one or more deficits of neuropsychological, speech or language function which are collectively known as the frontotemporal spectrum disorders of ALS (ALS-FTSD). While the neuropathology underlying these disorders is most consistent with a widespread alteration in the metabolism of transactive response DNA-binding protein 43 (TDP-43), in both ALS with cognitive impairment (ALSci) and ALS with FTD (ALS-FTD; also known as MND-FTD) there is evidence for alterations in the metabolism of the microtubule associated protein tau...
2018: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/29728608/active-nuclear-import-and-passive-nuclear-export-are-the-primary-determinants-of-tdp-43-localization
#8
Emile S Pinarbasi, Tolga Cağatay, Ho Yee Joyce Fung, Ying C Li, Yuh Min Chook, Philip J Thomas
ALS (Amyotrophic Lateral Sclerosis) is a neurodegenerative disease characterized by the redistribution of the RNA binding protein TDP-43 in affected neurons: from predominantly nuclear to aggregated in the cytosol. However, the determinants of TDP-43 localization and the cellular insults that promote redistribution are incompletely understood. Here, we show that the putative Nuclear Export Signal (NES) is not required for nuclear egress of TDP-43. Moreover, when the TDP-43 domain which contains the putative NES is fused to a reporter protein, YFP, the presence of the NES is not sufficient to mediate nuclear exclusion of the fusion protein...
May 4, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29720226/increased-a20-e3-ubiquitin-ligase-interactions-in-bid-deficient-glia-attenuate-tlr3-and-tlr4-induced-inflammation
#9
Sinéad Kinsella, Michael Fichtner, Orla Watters, Hans-Georg König, Jochen H M Prehn
BACKGROUND: Chronic pro-inflammatory signaling propagates damage to neural tissue and affects the rate of disease progression. Increased activation of Toll-like receptors (TLRs), master regulators of the innate immune response, is implicated in the etiology of several neuropathologies including amyotrophic lateral sclerosis, Alzheimer's disease, and Parkinson's disease. Previously, we identified that the Bcl-2 family protein BH3-interacting domain death agonist (Bid) potentiates the TLR4-NF-κB pro-inflammatory response in glia, and specifically characterized an interaction between Bid and TNF receptor associated factor 6 (TRAF6) in microglia in response to TLR4 activation...
May 2, 2018: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/29716660/a-blended-psychosocial-support-program-for-partners-of-patients-with-amyotrophic-lateral-sclerosis-and-progressive-muscular-atrophy-protocol-of-a-randomized-controlled-trial
#10
Jessica de Wit, Anita Beelen, Constance H C Drossaert, Ruud Kolijn, Leonard H van den Berg, Johanna M A Visser-Meily, Carin D Schröder
BACKGROUND: Informal caregivers of patients with Amyotrophic Lateral Sclerosis (ALS) or Progressive Muscular Atrophy (PMA) face stressful demands due to severe impairments and prospect of early death of the patients they care for. Caregivers often experience feelings of psychological distress and caregiver burden, but supportive interventions are lacking. The objective of this study is to investigate the effectiveness of a psychosocial support program aimed at enhancing feelings of control over caregiving tasks and reducing psychological distress...
May 2, 2018: BMC Psychology
https://www.readbyqxmd.com/read/29710121/association-of-retinal-ganglion-cell-layer-thickness-with-future-disease-activity-in-patients-with-clinically-isolated-syndrome
#11
Hanna G Zimmermann, Benjamin Knier, Timm Oberwahrenbrock, Janina Behrens, Catherina Pfuhl, Lilian Aly, Miriam Kaminski, Muna-Miriam Hoshi, Svenja Specovius, René M Giess, Michael Scheel, Mark Mühlau, Judith Bellmann-Strobl, Klemens Ruprecht, Bernhard Hemmer, Thomas Korn, Friedemann Paul, Alexander U Brandt
Importance: Clinically isolated syndrome (CIS) describes a first clinical incident suggestive of multiple sclerosis (MS). Identifying patients with CIS who have a high risk of future disease activity and subsequent MS diagnosis is crucial for patient monitoring and the initiation of disease-modifying therapy. Objective: To investigate the association of retinal optical coherence tomography (OCT) results with future disease activity in patients with CIS. Design, Setting, and Participants: This prospective, longitudinal cohort study took place between January 2011 and May 2017 at 2 German tertiary referral centers...
April 23, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/29706605/hypermetabolism-in-als-is-associated-with-greater-functional-decline-and-shorter-survival
#12
Frederik J Steyn, Zara A Ioannides, Ruben P A van Eijk, Susan Heggie, Kathryn A Thorpe, Amelia Ceslis, Saman Heshmat, Anjali K Henders, Naomi R Wray, Leonard H van den Berg, Robert D Henderson, Pamela A McCombe, Shyuan T Ngo
OBJECTIVE: To determine the prevalence of hypermetabolism, relative to body composition, in amyotrophic lateral sclerosis (ALS) and its relationship with clinical features of disease and survival. METHODS: Fifty-eight patients with clinically definite or probable ALS as defined by El Escorial criteria, and 58 age and sex-matched control participants underwent assessment of energy expenditure. Our primary outcome was the prevalence of hypermetabolism in cases and controls...
April 29, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29679161/present-and-future-of-ultra-high-field-mri-in-neurodegenerative-disorders
#13
REVIEW
Graziella Donatelli, Roberto Ceravolo, Daniela Frosini, Michela Tosetti, Ubaldo Bonuccelli, Mirco Cosottini
PURPOSE OF REVIEW: With a high signal-to-noise ratio, unparalleled spatial resolution, and improved contrasts, ultra-high field MR (≥ 7 T) has great potential in depicting the normal radiological anatomy of smaller structures in the brain and can also provide more information about morphological, quantitative, and metabolic changes associated with a wide range of brain disorders. By focusing attention on specific brain regions believed to be associated with early pathological change, or by more closely inspecting recognized foci of brain pathology, ultra-high field MR can improve the accuracy and sensitivity of neuroimaging...
April 20, 2018: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/29678369/impaired-corticomuscular-and-interhemispheric-cortical-beta-oscillation-coupling-in-amyotrophic-lateral-sclerosis
#14
Malcolm Proudfoot, Freek van Ede, Andrew Quinn, Giles L Colclough, Joanne Wuu, Kevin Talbot, Michael Benatar, Mark W Woolrich, Anna C Nobre, Martin R Turner
OBJECTIVES: The neural activity of the primary motor cortex is variably synchronised with contralateral peripheral electromyographic signals, which is thought to facilitate long-range communication through the motor system. Such corticomuscular coherence (CMC) is typically observed in the beta-band (15-30 Hz) range during steady force production. We aimed to measure pathological alteration to CMC resulting from ALS. METHODS: CMC was appraised during a forearm grip task in 17 ALS patients contrasted against age-matched healthy controls...
April 6, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29666705/the-role-of-moderate-aerobic-exercise-as-determined-by-cardiopulmonary-exercise-testing-in-als
#15
Anna Caroline Marques Braga, Anabela Pinto, Susana Pinto, Mamede de Carvalho
Introduction: The efficacy of cardiopulmonary exercise testing (CPET) to determining exercise intensity has not been established in Amyotrophic Lateral Sclerosis (ALS). We studied this intervention. Methods: We included 48 ALS patients randomized in 2 groups: G1 ( n = 24), exercise intensity leveled by CPET; G2 ( n = 24), standard care limited by fatigue, during 6 months. ALS functional scale (ALSFRS-R) and forced vital capacity (FVC) were performed every 3 months; CPET was done at admission ( T 1) and 6 months later ( T 2)...
2018: Neurology Research International
https://www.readbyqxmd.com/read/29661904/increased-cerebral-functional-connectivity-in-als-a-resting-state-magnetoencephalography-study
#16
Malcolm Proudfoot, Giles L Colclough, Andrew Quinn, Joanne Wuu, Kevin Talbot, Michael Benatar, Anna C Nobre, Mark W Woolrich, Martin R Turner
OBJECTIVE: We sought to assess cortical function in amyotrophic lateral sclerosis (ALS) using noninvasive neural signal recording. METHODS: Resting-state magnetoencephalography was used to measure power fluctuations in neuronal oscillations from distributed cortical parcels in 24 patients with ALS and 24 healthy controls. A further 9 patients with primary lateral sclerosis and a group of 15 asymptomatic carriers of genetic mutations associated with ALS were also studied...
April 17, 2018: Neurology
https://www.readbyqxmd.com/read/29650983/myotube-elasticity-of-an-amyotrophic-lateral-sclerosis-mouse-model
#17
Béla Varga, Marta Martin-Fernandez, Cécile Hilaire, Ana Sanchez-Vicente, Julie Areias, Céline Salsac, Frédéric J G Cuisinier, Cédric Raoul, Frédérique Scamps, Csilla Gergely
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that affects the motor system leading to generalized paralysis and death of patients. The understanding of early pathogenic mechanisms will help to define early diagnostics criteria that will eventually provide basis for efficient therapeutics. Early symptoms of ALS usually include muscle weakness or stiffness. Therefore, mechanical response of differentiated myotubes from primary cultures of mice, expressing the ALS-causing SOD1 G93A mutation, was examined by atomic force microscopy...
April 12, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29649360/glycerolipid-headgroups-control-rate-and-mechanism-of-sod1-aggregation-and-accelerate-fibrillization-of-slowly-aggregating-als-mutants
#18
Sanaz Rasouli, Alireza Abdolvahabi, Corbin M Croom, Devon Plewman, Yunhua Shi, Bryan F Shaw
Interactions between superoxide dismutase-1 (SOD1) and lipid membranes might be directly involved in the toxicity and intercellular propagation of aggregated SOD1 in amyotrophic lateral sclerosis (ALS), but the chemical details of lipid-SOD1 interactions and their effects on SOD1 aggregation remain unclear. This paper determined the rate and mechanism of nucleation of fibrillar apo-SOD1 catalyzed by liposomal surfaces with identical hydrophobic chains (RCH2 (O2 C18 H33 )2 ), but headgroups of different net charge and hydrophobicity (i...
April 12, 2018: ACS Chemical Neuroscience
https://www.readbyqxmd.com/read/29627873/disease-modifying-treatment-in-progressive-multiple-sclerosis
#19
REVIEW
John Robert Ciotti, Anne Haney Cross
PURPOSE OF REVIEW: Multiple sclerosis (MS) is an immune-mediated disorder that affects the central nervous system (CNS), often first affecting people in early adulthood. Although most MS patients have a relapsing-remitting course (RRMS) at disease onset, a substantial proportion later develop chronic progression, termed secondary progressive MS (SPMS). Approximately 10% of MS patients experience chronic progression from disease onset, termed primary progressive multiple sclerosis (PPMS)...
April 7, 2018: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/29608902/effectiveness-of-home-based-exercises-without-supervision-by-physical-therapists-for-patients-with-early-stage-amyotrophic-lateral-sclerosis-a-pilot-study
#20
Kosuke Kitano, Takashi Asakawa, Naoto Kamide, Keisuke Yorimoto, Masaki Yoneda, Yutaka Kikuchi, Makoto Sawada, Tetsuo Komori
OBJECTIVE: This study aimed to verify the effects of structured home-based exercises without supervision by a physical therapist on patients with early-stage amyotrophic lateral sclerosis (ALS). DESIGN: This is a historical controlled study that is part of a multicenter collaborative study. SETTING: Rehabilitation departments at general hospitals and outpatient clinics with a neurology department in Japan. PARTICIPANTS: Twenty-one patients with ALS were enrolled and designated as the Home-EX group, and they performed unsupervised home-based exercises...
March 30, 2018: Archives of Physical Medicine and Rehabilitation
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