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https://www.readbyqxmd.com/read/27892703/the-measurement-and-estimation-of-total-energy-expenditure-in-japanese-patients-with-als-a-doubly-labelled-water-method-study
#1
Toshio Shimizu, Kazuko Ishikawa-Takata, Akiko Sakata, Utako Nagaoka, Noriko Ichihara, Chiho Ishida, Yuki Nakayama, Tetsuo Komori, Masatoyo Nishizawa
Appropriate nutritional therapy has not been established for patients with amyotrophic lateral sclerosis (ALS). Our objective was to measure the total energy expenditure (TEE) and determine an equation to estimate the energy requirements for Japanese patients with ALS. Twenty-six Japanese patients with ALS participated in the study. The TEE was measured using the doubly labelled water (DLW) method for a 14-day period. Using a range of clinical parameters and multiple regression analyses, we determined an adequate equation to calculate TEE...
November 28, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27873462/mitochondrial-dysfunction-and-biogenesis-in-neurodegenerative-diseases-pathogenesis-and-treatment
#2
REVIEW
Mojtaba Golpich, Elham Amini, Zahurin Mohamed, Raymond Azman Ali, Norlinah Mohamed Ibrahim, Abolhassan Ahmadiani
Neurodegenerative diseases are a heterogeneous group of disorders that are incurable and characterized by the progressive degeneration of the function and structure of the central nervous system (CNS) for reasons that are not yet understood. Neurodegeneration is the umbrella term for the progressive death of nerve cells and loss of brain tissue. Because of their high energy requirements, neurons are especially vulnerable to injury and death from dysfunctional mitochondria. Widespread damage to mitochondria causes cells to die because they can no longer produce enough energy...
November 22, 2016: CNS Neuroscience & Therapeutics
https://www.readbyqxmd.com/read/27869378/pathogenese-und-klinik-der-rosazea-als-schl%C3%A3-ssel-f%C3%A3-r-eine-symptomorientierte-therapie
#3
M Reinholz, T Ruzicka, M Steinhoff, M Schaller, U Gieler, H Schöfer, B Homey, P Lehmann, T A Luger
Rosazea ist eine häufige chronisch-entzündliche Hauterkrankung, die typischerweise bei Erwachsenen vorkommt und das Gesicht betrifft. Synonyme der Rosazea sind Acne rosacea, Kupferfinne, Rotfinne, Couperose und Rosacea. Die Erkrankung ist durch einen chronischen und schubartigen Verlauf gekennzeichnet und wird durch ein genetisch prädisponiertes, multifaktorielles Geschehen bedingt. Ein vermehrtes Auftreten wird bei hellem Hauttyp und positiver Familienanamnese verzeichnet. Die charakteristischen Rosazea-Symptome manifestieren sich vorwiegend, aber nicht ausschließlich zentrofazial, wobei Stirn, Nase, Kinn und die Wangen maßgeblich betroffen sind...
December 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/27869375/aktueller-stand-der-systemischen-rosazea-therapie
#4
M Schaller, H Schöfer, B Homey, U Gieler, P Lehmann, T A Luger, T Ruzicka, M Steinhoff
Basierend auf den Daten zahlreicher Studien sind orale Tetracycline - und hier insbesondere Doxycyclin als Tetracyclin der zweiten Generation - die Grundpfeiler der systemischen Rosazea-Therapie. Bisher ist dafür jedoch nur Doxycyclin 40 mg in antientzündlicher Dosierung mit veränderter Wirkstofffreisetzung zugelassen. Seit Einführung der Therapie mit Doxycyclin einmal täglich in nicht antibiotischer Dosierung wird die orale Therapie häufiger als Erstbehandlung bei mittelschwerer bis schwerer papulopustulöser Rosazea verschrieben...
December 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/27869373/rosazea-management-update-%C3%A3-ber-allgemeine-ma%C3%A3-nahmen-und-topische-therapieoptionen
#5
M Schaller, H Schöfer, B Homey, M Hofmann, U Gieler, P Lehmann, T A Luger, T Ruzicka, M Steinhoff
Obwohl bislang für die Rosazea keine kurative Therapie besteht, können verschiedene Optionen zur Behandlung der Symptome und zur Vorbeugung von Exazerbationen empfohlen werden. Neben Selbsthilfemaßnahme wie der Vermeidung von Triggerfaktoren und einer geeigneten Hautpflege sollte das Rosazea-Management bei Patienten mit erythematöser und leichter bis schwerer papulopustulöser Rosazea die Anwendung topischer Präparate als First-Line-Therapie umfassen. Da Überlappungen der charakteristischen Rosazea-Symptome im klinischen Alltag die Regel sind, sollte die medikamentöse Therapie auf die individuellen Symptome zugeschnitten werden; auch eine Kombinationstherapie kann erforderlich sein...
December 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/27863479/myasthenic-symptoms-in-anti-low-density-lipoprotein-receptor-related-protein-4-antibody-seropositive-amyotrophic-lateral-sclerosis-two-case-reports
#6
Hisashi Takahashi, Yu-Ichi Noto, Naoki Makita, Yukie Kushimura-Okada, Ryotaro Ishii, Akihiro Tanaka, Tomoyuki Ohara, Shunya Nakane, Osamu Higuchi, Masanori Nakagawa, Toshiki Mizuno
BACKGROUND: Myasthenic symptoms can be present in patients with amyotrophic lateral sclerosis (ALS). These symptoms have been considered to be caused by the degeneration of distal motor neurons and the neuromuscular junction (NMJ). Recent studies suggested that antibody to low-density lipoprotein receptor-related protein 4 (LRP4) was a pathogenic agent of myasthenia gravis (MG), and it was also detected in ALS patients. CASE PRESENTATION: Patient 1: A 58-year-old Japanese man developed progressive weakness and subsequent myasthenic symptoms including oculomotor disturbance...
November 18, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27862987/einsatz-und-wirksamkeit-von-systemtherapien-bei-erwachsenen-mit-schwerer-neurodermitis-erste-ergebnisse-des-deutschen-neurodermitis-registers-treatgermany
#7
Jochen Schmitt, Susanne Abraham, Freya Trautmann, Victoria Stephan, Regina Fölster-Holst, Bernhard Homey, Thomas Bieber, Natalija Novak, Michael Sticherling, Matthias Augustin, Andreas Kleinheinz, Peter Elsner, Stephan Weidinger, Thomas Werfel
HINTERGRUND: Versorgungsregister dienen der Erfassung des Einsatzes und der Wirksamkeit von Therapien unter realen Versorgungsbedingungen und sind als Basis einer evidenzbasierten Gesundheitsversorgung unverzichtbar. METHODIK: Das deutsche Neurodermitis-Register TREATgermany wurde als weltweit erstes Register für Patienten mit schwerer Neurodermitis 2011 initiiert. Erwachsene mit schwerer Neurodermitis (aktuelle/frühere antientzündliche Systemtherapie und/oder objektiver SCORAD ≥ 40) werden über einen Zeitraum von 24 Monaten prospektiv beobachtet...
November 10, 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/27854227/do-psychosocial-interventions-improve-quality-of-life-and-wellbeing-in-adults-with-neuromuscular-disorders-a-systematic-review-and-narrative-synthesis
#8
Elaine Walklet, Kate Muse, Jane Meyrick, Tim Moss
Quality of life and well-being are frequently restricted in adults with neuromuscular disorders. As such, identification of appropriate interventions is imperative. The objective of this paper was to systematically review and critically appraise quantitative studies (RCTs, controlled trials and cohort studies) of psychosocial interventions designed to improve quality of life and well-being in adults with neuromuscular disorders. A systematic review of the published and unpublished literature was conducted. Studies meeting inclusion criteria were appraised using a validated quality assessment tool and results presented in a narrative synthesis...
August 30, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27830784/decoding-als-from-genes-to-mechanism
#9
J Paul Taylor, Robert H Brown, Don W Cleveland
Amyotrophic lateral sclerosis (ALS) is a progressive and uniformly fatal neurodegenerative disease. A plethora of genetic factors have been identified that drive the degeneration of motor neurons in ALS, increase susceptibility to the disease or influence the rate of its progression. Emerging themes include dysfunction in RNA metabolism and protein homeostasis, with specific defects in nucleocytoplasmic trafficking, the induction of stress at the endoplasmic reticulum and impaired dynamics of ribonucleoprotein bodies such as RNA granules that assemble through liquid-liquid phase separation...
November 9, 2016: Nature
https://www.readbyqxmd.com/read/27826939/a-novel-iron-chelator-radical-scavenger-ameliorates-motor-dysfunction-and-improves-life-span-and-mitochondrial-biogenesis-in-sod1-g93a-als-mice
#10
Sagit Golko-Perez, Tamar Amit, Orit Bar-Am, Moussa B H Youdim, Orly Weinreb
The aim of the present study was to evaluate the therapeutic effect of the novel neuroprotective multitarget brain permeable monoamine oxidase inhibitor/iron chelating-radical scavenging drug, VAR10303 (VAR), co-administered with high-calorie/energy-supplemented diet (ced) in SOD1(G93A) transgenic amyotrophic lateral sclerosis (ALS) mice. Administration of VAR-ced was initiated after the appearance of disease symptoms (at day 88), as this regimen is comparable with the earliest time at which drug therapy could start in ALS patients...
November 8, 2016: Neurotoxicity Research
https://www.readbyqxmd.com/read/27822919/cell-based-therapies-for-amyotrophic-lateral-sclerosis-motor-neuron-disease
#11
REVIEW
S Fadilah Abdul Wahid, Zhe Kang Law, Nor Azimah Ismail, Raymond Azman Ali, Nai Ming Lai
BACKGROUND: Amyotrophic lateral sclerosis (ALS), which is also known as motor neuron disease (MND) is a fatal disease associated with rapidly progressive disability, for which no definitive treatment as yet exists. Current treatment regimens largely focus on relieving symptoms to improve the quality of life of those affected. Based on data from preclinical studies, cell-based therapy is a promising treatment for ALS/MND. OBJECTIVES: To assess the effects of cell-based therapy for people with ALS/MND, compared with placebo or no additional treatment...
November 8, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27815397/new-strategy-that-delays-progression-of-amyotrophic-lateral-sclerosis-in-g1h-g93a-transgenic-mice-oral-administration-of-xanthine-oxidoreductase-inhibitors-that-are-not-substrates-for-the-purine-salvage-pathway
#12
Shinsuke Kato, Masako Kato, Teruo Kusano, Takeshi Nishino
Amyotrophic lateral sclerosis (ALS), Lou Gehrig's disease, is a progressive fatal neurodegenerative disease that involves both upper and lower motor neurons. We orally administered 4 xanthine oxidoreductase (XOR) inhibitors to G1H-G93A mice carrying 25 transgene copy numbers of human mutant G93A superoxide dismutase 1, from 80 days of age. Three nonpurine-analogue inhibitors (TEI-6720: Febuxostat, Y-700 and FYX-051), but not allopurinol with a purine analogue ring (pyrazolo pyrimidine ring), significantly delayed disease onset, prolonged survival and the duration of disease stages, improved clinical signs, and alleviated weight loss...
November 4, 2016: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/27814735/gene-expression-profiles-and-protein-protein-interaction-networks-in-amyotrophic-lateral-sclerosis-patients-with-c9orf72-mutation
#13
Meena Kumari Kotni, Mingzhu Zhao, Dong-Qing Wei
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that involves the death of neurons. ALS is associated with many gene mutations as previously studied. In order to explore the molecular mechanisms underlying ALS with C9orf72 mutation, gene expression profiles of ALS fibroblasts and control fibroblasts were subjected to bioinformatics analysis. Genes with critical functional roles can be detected by a measure of node centrality in biological networks. In gene co-expression networks, highly connected genes called as candidate hubs have been associated with key disease-related pathways...
November 5, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27788555/-ventilatory-support-and-management-of-secretions-in-amyotrophic-lateral-sclerosis
#14
M Boentert, P Young
The term amyotrophic lateral sclerosis (ALS) comprises a group of motor neuron diseases which are characterized by rapid disease progression and poor prognosis which is mostly due to severe respiratory muscle weakness and its sequelae. Since causative treatment options are limited it is crucial to offer comprehensive symptomatic therapies to affect patients. Symptoms of respiratory muscle weakness, sleep-disordered breathing and, subsequently, chronic hypercapnic respiratory failure are known to severely affect health-related quality of life and social functioning of patients with ALS...
October 2016: Fortschritte der Neurologie-Psychiatrie
https://www.readbyqxmd.com/read/27784774/safety-of-intrathecal-autologous-adipose-derived-mesenchymal-stromal-cells-in-patients-with-als
#15
Nathan P Staff, Nicolas N Madigan, Jonathan Morris, Mark Jentoft, Eric J Sorenson, Greg Butler, Dennis Gastineau, Allan Dietz, Anthony J Windebank
OBJECTIVE: To determine the safety of intrathecal autologous adipose-derived mesenchymal stromal cell treatment for amyotrophic lateral sclerosis (ALS). METHODS: Participants with ALS were enrolled and treated in this phase I dose-escalation safety trial, ranging from 1 × 10(7) (single dose) to 1 × 10(8) cells (2 monthly doses). After intrathecal treatments, participants underwent standardized follow-up, which included clinical examinations, revised ALS Functional Rating Scale (ALSFRS-R) questionnaire, blood and CSF sampling, and MRI of the neuroaxis...
November 22, 2016: Neurology
https://www.readbyqxmd.com/read/27769241/therapeutic-effect-of-berberine-on-tdp-43-related-pathogenesis-in-ftld-and-als
#16
Cheng-Fu Chang, Yi-Chao Lee, Kuen-Haur Lee, Hui-Ching Lin, Chia-Ling Chen, Che-Kun James Shen, Chi-Chen Huang
BACKGROUND: In the central nervous system regions of the sporadic and familial FTLD and ALS patients, TDP-43 has been identified as the major component of UBIs inclusions which is abnormally hyperphosphorylated, ubiquitinated, and cleaved into C-terminal fragments to form detergent-insoluble aggregates. So far, the effective drugs for FTLD and ALS neurodegenerative diseases are yet to be developed. Autophagy has been demonstrated as the major metabolism route of the pathological TDP-43 inclusions, hence activation of autophagy is a potential therapeutic strategy for TDP-43 pathogenesis in FTLD and ALS...
October 21, 2016: Journal of Biomedical Science
https://www.readbyqxmd.com/read/27752511/%C3%AE-car-igf-1-vector-targeting-of-motor-neurons-ameliorates-disease-progression-in-als-mice
#17
Ioanna Eleftheriadou, Ioannis Manolaras, Elaine E Irvine, Michael Dieringer, Antonio Trabalza, Nicholas D Mazarakis
OBJECTIVE: We have previously described the generation of coxsackievirus and adenovirus receptor (α CAR)-targeted vector, and shown that intramuscular delivery in mouse leg muscles resulted in specific retrograde transduction of lumbar-motor neurons (MNs). Here, we utilized the α CAR-targeted vector to investigate the in vivo neuroprotective effects of lentivirally expressed IGF-1 for inducing neuronal survival and ameliorating the neuropathology and behavioral phenotypes of the SOD1(G93A) mouse model of ALS...
October 2016: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/27716798/microneurotrophins-improve-survival-in-motor-neuron-astrocyte-co-cultures-but-do-not-improve-disease-phenotypes-in-a-mutant-sod1-mouse-model-of-amyotrophic-lateral-sclerosis
#18
Kelly E Glajch, Laura Ferraiuolo, Kaly A Mueller, Matthew J Stopford, Varsha Prabhkar, Achille Gravanis, Pamela J Shaw, Ghazaleh Sadri-Vakili
Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease caused by loss of motor neurons. ALS patients experience rapid deterioration in muscle function with an average lifespan of 3-5 years after diagnosis. Currently, the most effective therapeutic only extends lifespan by a few months, thus highlighting the need for new and improved therapies. Neurotrophic factors (NTFs) are important for neuronal development, maintenance, and survival. NTF treatment has previously shown efficacy in pre-clinical ALS models...
2016: PloS One
https://www.readbyqxmd.com/read/27714895/gingival-stromal-cells-as-an-in-vitro-model-cannabidiol-modulates-genes-linked-with-amyotrophic-lateral-sclerosis
#19
Thangavelu Soundara Rajan, Domenico Scionti, Francesca Diomede, Gianpaolo Grassi, Federica Pollastro, Adriano Piattelli, Lucio Cocco, Placido Bramanti, Emanuela Mazzon, Oriana Trubiani
Research in recent years has extensively investigated the therapeutic efficacy of mesenchymal stromal cells in regenerative medicine for many neurodegenerative diseases at preclinical and clinical stages. However, the success rate of stem cell therapy remains less at translational phase. Lack of relevant animal models that potentially simulate the molecular etiology of human pathological symptoms might be a reason behind such poor clinical outcomes associated with stem cell therapy. Apparently, self-renewal and differentiation ability of mesenchymal stem cells may help to study the early developmental signaling pathways connected with the diseases, such as Alzheimer's disease, Amyotrophic lateral sclerosis (ALS), etc...
October 7, 2016: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/27704280/distinct-conformers-of-transmissible-misfolded-sod1-distinguish-human-sod1-fals-from-other-forms-of-familial-and-sporadic-als
#20
Jacob I Ayers, Jeffrey Diamond, Adriana Sari, Susan Fromholt, Ahmad Galaleldeen, Lyle W Ostrow, Jonathan D Glass, P John Hart, David R Borchelt
Evidence of misfolded wild-type superoxide dismutase 1 (SOD1) has been detected in spinal cords of sporadic ALS (sALS) patients, suggesting an etiological relationship to SOD1-associated familial ALS (fALS). Given that there are currently a number of promising therapies under development that target SOD1, it is of critical importance to better understand the role of misfolded SOD1 in sALS. We previously demonstrated the permissiveness of the G85R-SOD1:YFP mouse model for MND induction following injection with tissue homogenates from paralyzed transgenic mice expressing SOD1 mutations...
December 2016: Acta Neuropathologica
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