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SCLEROSIS LATERAL AMYOTROPHIC

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https://www.readbyqxmd.com/read/30114663/fasciculation-intensity-and-disease-progression-in-amyotrophic-lateral-sclerosis
#1
Jun Tsugawa, Thanuja Dharmadasa, Yan Ma, William Huynh, Steve Vucic, Matthew C Kiernan
OBJECTIVE: To investigate the association between the frequency and intensity of fasciculations with clinical measures of disease progression in amyotrophic lateral sclerosis (ALS). METHODS: Twenty-four consecutive patients with ALS underwent clinical review and neuromuscular ultrasound assessment to detect intensity of fasciculations. Results were correlated with clinical markers of disease severity, as measured by the ALS Functional Rating Scale-revised (ALSFRS-R) and rate of disease progression (ΔFS), in addition to assessment of cortical motor function...
August 2, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/30114551/three-new-case-reports-of-arteriovenous-malformation-related-amyotrophic-lateral-sclerosis
#2
Maria Chondrogianni, Marianna Bregianni, Frantzeska Frantzeskaki, Evangelos Giamarellos-Bourboulis, Evangelos Anagnostou, Evangelia Kararizou, Georgia Karadima, George Koutsis, Christos Moschovos, Anastasios Bonakis, Leonidas Stefanis
Despite recent advances in genetics, in most cases of Amyotrophic Lateral Sclerosis (ALS) no etiological factor can be identified. Cerebral Arteriovenous Malformations (AVMs) have been associated with ALS development in a few studies, but the nature of this connection is unclear. We report here 3 additional cases of young adults, who had undergone repeated embolizations for complex AVMs, and who then developed, after many years, ALS symptoms and signs. In two of these cases Vascular Endothelial Growth Factor (VEGF) levels were found to be extremely high, in contrast to previous reports...
August 9, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/30114473/unhealthy-gut-unhealthy-brain-the-role-of-the-intestinal-microbiota-in-neurodegenerative-diseases
#3
REVIEW
Lindsay Joy Spielman, Deanna Lynn Gibson, Andis Klegeris
The number of bacterial cells living within the human body is approximately equal to, or greater than, the total number of human cells. This dynamic population of microorganisms, termed the human microbiota, resides mainly within the gastrointestinal tract. It is widely accepted that highly diverse and stable microbiota promote overall human health. Colonization of the gut with maladaptive and pathogenic microbiota, a state also known as dysbiosis, is associated with a variety of peripheral diseases ranging from type 2 diabetes mellitus to cardiovascular and inflammatory bowel disease...
August 13, 2018: Neurochemistry International
https://www.readbyqxmd.com/read/30112417/itraq-based-proteomics-analysis-reveals-the-effect-of-rhubarb-in-rats-with-ischemic-stroke
#4
Xiangping Lin, Tao Liu, Pengfei Li, Zehui He, Yuanyuan Zhong, Hanjin Cui, Jiekun Luo, Yang Wang, Tao Tang
Background. Rhubarb, a traditional Chinese medicine, promotes viscera and remove blood stasis. Rhubarb is skilled in smoothening meridians, improving blood circulation which exhibits better efficacy on cerebral ischemic stroke. In this study, we aimed to analyze the underlying mechanisms of rhubarb which treated rats of middle cerebral artery occlusion (MCAO) model according to an iTRAQ-based proteomics and bioinformatics analysis. 30 rats were randomly allocated into three groups including sham group (SG), model group (MG), and rhubarb group (RG)...
2018: BioMed Research International
https://www.readbyqxmd.com/read/30112276/integrated-imaging-of-11-c-pbr28-pet-mr-diffusion-and-magnetic-resonance-spectroscopy-1-h-mrs-in-amyotrophic-lateral-sclerosis
#5
Eva-Maria Ratai, Mohamad J Alshikho, Nicole R Zürcher, Marco L Loggia, Catherine L Cebulla, Paul Cernasov, Beverly Reynolds, Jennifer Fish, Raghav Seth, Suma Babu, Sabrina Paganoni, Jacob M Hooker, Nazem Atassi
Objective: To determine the relationship between brain tissue metabolites measured by in vivo magnetic resonance spectroscopy (1 H-MRS), and glial activation assessed with [11 C]-PBR28 uptake in people with amyotrophic lateral sclerosis (ALS). Methods: Forty ALS participants were evaluated clinically using the revised ALS functional rating scale (ALSFRS-R) and upper motor neuron burden (UMNB). All participants underwent simultaneous brain [11 C]-PBR28 PET and MR imaging including diffusion tensor imaging to acquire fractional anisotropy (FA)...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/30110532/investigation-of-anti-sod1-antibodies-yields-new-structural-insight-into-sod1-misfolding-and-surprising-behavior-of-the-antibodies-themselves
#6
Ryan S Atlasi, Ravinder Malik, Christian I Corrales, Laura Tzeplaeff, Julian P Whitelegge, Neil R Cashman, Gal Bitan
Mutations in Cu/Zn-superoxide dismutase (SOD1) gene are linked to 10-20% of familial amyotrophic lateral sclerosis (fALS) cases. The mutations cause misfolding and self-assembly of SOD1 into toxic oligomers and aggregates, resulting in motor neuron degeneration. The molecular mechanisms underlying SOD1 aggregation and toxicity are unclear. Characterization of misfolded SOD1 is particularly challenging due to its metastable nature. Antibodies against misfolded SOD1 are useful tools for this purpose, provided their specificity and selectivity are well characterized...
August 15, 2018: ACS Chemical Biology
https://www.readbyqxmd.com/read/30109997/-mutations-of-g38r-and-d40g-cause-amyotrophic-lateral-sclerosis-by-reducing-annexin-a11-protein-stability
#7
Di Liao, Qiao Liao, Cao Huang, Fangfang Bi
To explore the role of the mutations G38R and D40G of Annexin A11 (ANXA11) in the onset of amyotrophic lateral sclerosis (ALS).
 Methods: The plasmids expressing ANXA11 wild type protein, ANXA11 G38R protein and ANXA11 D40G protein were constructed, respectively. The recombinant plasmids were then transfected into HEK293 cells respectively followed by cycloheximide (CHX) treatment for 0, 2, 4 and 8 h. The protein expressions of ANXA11 wild type, ANXA11 G38R and ANXA11 D40G mutations were determined by Western blot...
June 28, 2018: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/30109267/noncoding-repeat-expansions-for-als-in-japan-are-associated-with-the-atxn8os-gene
#8
Makito Hirano, Makoto Samukawa, Chiharu Isono, Kazumasa Saigoh, Yusaku Nakamura, Susumu Kusunoki
Objective: To assess the contribution of noncoding repeat expansions in Japanese patients with amyotrophic lateral sclerosis (ALS). Methods: Sporadic ALS in Western countries is frequently associated with noncoding repeat expansions in the C9ORF72 gene. Spinocerebellar ataxia type 8 (SCA8) is another noncoding repeat disease caused by expanded CTA/CTG repeats in the ATXN8OS gene. Although the involvement of upper and lower motor neurons in SCA8 has been reported, a positive association between SCA8 and ALS remains unestablished...
August 2018: Neurology. Genetics
https://www.readbyqxmd.com/read/30108476/performance-differences-using-a-vibro-tactile-p300-bci-in-lis-patients-diagnosed-with-stroke-and-als
#9
Alexander Heilinger, Rupert Ortner, Vincenzo La Bella, Zulay R Lugo, Camille Chatelle, Steven Laureys, Rossella Spataro, Christoph Guger
Patients with locked-in syndrome (LIS) are typically unable to move or communicate and can be misdiagnosed as patients with disorders of consciousness (DOC). Behavioral assessment scales are limited in their ability to detect signs of consciousness in this population. Recent research has shown that brain-computer interface (BCI) technology could supplement behavioral scales and allows to establish communication with these severely disabled patients. In this study, we compared the vibro-tactile P300 based BCI performance in two groups of patients with LIS of different etiologies: stroke ( n = 6) and amyotrophic lateral sclerosis (ALS) ( n = 9)...
2018: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/30106482/cough-peak-flow-decline-rate-predicts-survival-in-patients-with-amyotrophic-lateral-sclerosis
#10
Chiharu Matsuda, Toshio Shimizu, Yuki Nakayama, Michiko Haraguchi
INTRODUCTION: This study investigated the prognostic significance of the cough peak flow (CPF) decline rate in patients with amyotrophic lateral sclerosis (ALS). METHODS: Thirty-four patients with ALS participated. We examined CPF, forced vital capacity (FVC), body mass index, and the revised ALS Functional Rating Scale (ALSFRS-R), and followed patients to death, tracheostomy or non-invasive ventilator dependency. We analyzed the relationship between the rate of decline of each parameter and survival prognosis...
August 14, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/30105628/neuromuscular-junction-morphology-and-gene-dysregulation-in-the-wobbler-model-of-spinal-neurodegeneration
#11
Whitney A Ratliff, Jessica N Saykally, Michael J Kane, Bruce A Citron
Amyotrophic lateral sclerosis (ALS) is the most common adult-onset neuromuscular disease for which there is currently no effective treatment. The progression of ALS includes loss of motor neurons controlling the voluntary muscles, with much of this loss occurring at the neuromuscular junction. In an effort to better understand changes at the neuromuscular junction, we utilized the wobbler mouse model of motor neuron loss. We examined biceps and end plate morphologies and monitored selected factors involved in end plate function...
August 13, 2018: Journal of Molecular Neuroscience: MN
https://www.readbyqxmd.com/read/30105395/retinoids-and-amyotrophic-lateral-sclerosis-reply
#12
Gabriele Nagel, Dietrich Rothenbacher, Albert C Ludolph
No abstract text is available yet for this article.
August 13, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/30105376/retinoids-and-amyotrophic-lateral-sclerosis
#13
Javier Riancho, Francisco J Gil-Bea, Adolfo López de Munaín
No abstract text is available yet for this article.
August 13, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/30104996/combinatory-biomarker-use-of-cortical-thickness-munix-and-alsfrs-r-at-baseline-and-in-longitudinal-courses-of-individual-patients-with-amyotrophic-lateral-sclerosis
#14
Anna M Wirth, Andrei Khomenko, Dobri Baldaranov, Ines Kobor, Ohnmar Hsam, Thomas Grimm, Siw Johannesen, Tim-Henrik Bruun, Wilhelm Schulte-Mattler, Mark W Greenlee, Ulrich Bogdahn
Objective: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative process affecting upper and lower motor neurons as well as non-motor systems. In this study, precentral and postcentral cortical thinning detected by structural magnetic resonance imaging (MRI) were combined with clinical (ALS-specific functional rating scale revised, ALSFRS-R) and neurophysiological (motor unit number index, MUNIX) biomarkers in both cross-sectional and longitudinal analyses. Methods: The unicenter sample included 20 limb-onset classical ALS patients compared to 30 age-related healthy controls...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/30103356/controlling-of-smart-home-system-based-on-brain-computer-interface
#15
Qiang Gao, Xuewen Zhao, Xiao Yu, Yu Song, Zhe Wang
BACKGROUND: Brain computer interface (BCI) technology is a communication and control approach. Up to now many studies have attempted to develop an EEG-based BCI system to improve the quality of life of people with severe disabilities, such as amyotrophic lateral sclerosis (ALS), paralysis, brain stroke and so on. The proposed BCIBSHS could help to provide a new way for supporting life of paralyzed people and elderly people. OBJECTIVE: The goal of this paper is to explore how to set up a cost-effective and safe-to-use online BCIBSHS to recognize multi-commands and control smart devices based on SSVEP...
July 27, 2018: Technology and Health Care: Official Journal of the European Society for Engineering and Medicine
https://www.readbyqxmd.com/read/30103325/three-vcp-mutations-in-patients-with-frontotemporal-dementia
#16
Tsz Hang Wong, Cyril Pottier, David Hondius, Lieke H H Meeter, Jeroen G J van Rooij, Shami Melhem, The Netherlands Brain Bank, Rick van Minkelen, Cornelia M van Duijn, Annemieke J M Rozemuller, Harro Seelaar, Rosa Rademakers, John C van Swieten
Valosin-containing protein (VCP) is involved in multiple cellular activities. Mutations in VCP lead to heterogeneous clinical presentations including inclusion body myopathy with Paget's disease of the bone, frontotemporal dementia and amyotrophic lateral sclerosis, even in patients carrying the same mutation. We screened a cohort of 48 patients with familial frontotemporal dementia (FTD) negative for MAPT, GRN, and C9orf72 mutations for other known FTD genes by using whole exome sequencing. In addition, we carried out targeted sequencing of a cohort of 37 patients with frontotemporal lobar degeneration with Transactive response DNA-binding protein 43 (TDP-43) subtype from the Netherlands Brain bank...
August 9, 2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/30102919/the-analysis-of-underivatized-%C3%AE-methylamino-l-alanine-bmaa-bama-aeg-2-4-dab-in-pteropus-mariannus-mariannus-specimens-using-hilic-lc-ms-ms
#17
Amanda J Foss, Neil Chernoff, Mark T Aubel
β-Methylamino-L-alanine (BMAA) has been identified as the potential cause of the amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) observed in the Chamorro people of Guam. The principal hypothesis for BMAA exposure and intoxication relies on the biomagnification of BMAA in flying fox specimens ingested by the Chamorro people. Although high levels of BMAA were quantitated in flying fox specimens utilizing liquid chromatography-fluorescence (LC-FL), there have not been any confirmatory analyses conducted to date...
August 10, 2018: Toxicon: Official Journal of the International Society on Toxinology
https://www.readbyqxmd.com/read/30101862/-motor-neuron-heterogeneity-and-selective-vulnerability-in-als
#18
Hidemi Misawa, Yuta Morisaki
Different and selective vulnerability among motor neuron subtypes are a fundamental, but unexplained, feature of amyotrophic lateral sclerosis (ALS): fast-fatigable (FF) motor neurons are the most vulnerable, and fast fatigue-resistant/slow (FR/S) motor neurons are relatively resistant. We identified that osteopontin (OPN) can serve as a marker of FR/S motor neurons, whereas matrix metalloproteinase-9 (MMP9) is expressed by FF motor neurons in mice. In SOD1G93A ALS model mice, as the disease progressed, OPN was secreted and accumulated as granular deposits in the extracellular matrix...
2018: Nihon Yakurigaku Zasshi. Folia Pharmacologica Japonica
https://www.readbyqxmd.com/read/30101496/riluzole-and-edaravone-a-tale-of-two-amyotrophic-lateral-sclerosis-drugs
#19
REVIEW
Manoj Kumar Jaiswal
Over the past decades, a multitude of experimental drugs have been shown to delay disease progression in preclinical animal models of amyotrophic lateral sclerosis (ALS) but failed to show efficacy in human clinical trials or are still waiting for approval under Phase I-III trials. Riluzole, a glutamatergic neurotransmission inhibitor, is the only drug approved by the USA Food and Drug Administration for ALS treatment with modest benefits on survival. Recently, an antioxidant drug, edaravone, developed by Mitsubishi Tanabe Pharma was found to be effective in halting ALS progression during early stages...
August 12, 2018: Medicinal Research Reviews
https://www.readbyqxmd.com/read/30100896/communication-matters-pitfalls-and-promise-of-hightech-communication-devices-in-palliative-care-of-severely-physically-disabled-patients-with-amyotrophic-lateral-sclerosis
#20
REVIEW
Katharina Linse, Elisa Aust, Markus Joos, Andreas Hermann
Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease, leading to progressive paralysis, dysarthria, dysphagia, and respiratory disabilities. Therapy is mostly focused on palliative interventions. During the course of the disease, verbal as well as nonverbal communicative abilities become more and more impaired. In this light, communication has been argued to be "the essence of human life" and crucial for patients' quality of life. High-tech augmentative and alternative communication (HT-AAC) technologies such as eyetracking based computer devices and brain-computer-interfaces provide the possibility to maintain caregiver-independent communication and environmental control even in the advanced disease state of ALS...
2018: Frontiers in Neurology
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