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SCLEROSIS LATERAL AMYOTROPHIC

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https://www.readbyqxmd.com/read/30540963/axon-seq-decodes-the-motor-axon-transcriptome-and-its-modulation-in-response-to-als
#1
Jik Nijssen, Julio Aguila, Rein Hoogstraaten, Nigel Kee, Eva Hedlund
Spinal motor axons traverse large distances to innervate target muscles, thus requiring local control of cellular events for proper functioning. To interrogate axon-specific processes we developed Axon-seq, a refined method incorporating microfluidics, RNA sequencing (RNA-seq), and bioinformatic quality control. We show that the axonal transcriptome is distinct from that of somas and contains fewer genes. We identified 3,500-5,000 transcripts in mouse and human stem cell-derived spinal motor axons, most of which are required for oxidative energy production and ribogenesis...
December 11, 2018: Stem Cell Reports
https://www.readbyqxmd.com/read/30539901/monitoring-value-of-multimodal-magnetic-resonance-imaging-in-disease-progression-of-amyotrophic-lateral-sclerosis-a-prospective-observational-study
#2
Dong-Chao Shen, Yin-Yan Xu, Bo Hou, Hong-Fei Tai, Kang Zhang, Shuang-Wu Liu, Zhi-Li Wang, Feng Feng, Ming-Sheng Liu, Li-Ying Cui
Background: Ongoing efforts have been made to identify new neuroimaging markers to track amyotrophic lateral sclerosis (ALS) progression. This study aimed to explore the monitoring value of multimodal magnetic resonance imaging (MRI) in the disease progression of ALS. Methods: From September 2015 to March 2017, ten patients diagnosed with ALS in Peking Union Medical College Hospital completed head MRI scans at baseline and during follow-up. Multimodal MRI analyses, including gray matter (GM) volume measured by voxel-based morphometry; cerebral blood flow (CBF) evaluated by arterial spin labeling; functional connectivity, including low-frequency fluctuation (fALFF) and regional homogeneity (ReHo), measured by resting-state functional MRI; and integrity of white-matter (WM) fiber tracts evaluated by diffusion tensor imaging, were performed in these patients...
December 20, 2018: Chinese Medical Journal
https://www.readbyqxmd.com/read/30539846/network-strategy-to-investigate-differential-pathways-in-sporadic-amyotrophic-lateral-sclerosis
#3
Li-Hong Han, Xiao-Yan Fan, Hong Guo, Wei Wei, Mao-Meng Chen, Shu-Fang Yan
Objective: The objective of this paper was to investigate differential pathways in sporadic amyotrophic lateral sclerosis (SALS) based on pathway network analysis. Materials and Methods: To achieve this goal, first, differentially expressed genes (DEGs) between SALS and normal controls were identified, and a target network was defined as DEGs correlated interactions from the search tool for the retrieval of interacting genes/proteins (STRING). Second, topological centrality analysis was conducted on the target network to identify hub genes and hub network...
December 2018: Journal of Cancer Research and Therapeutics
https://www.readbyqxmd.com/read/30538761/traditional-korean-medicine-treatment-for-patients-with-wilting-disorder-a-literature-review-of-in-vivo-studies
#4
REVIEW
Sung-Jin Kim, Yeon-Cheol Park, Yong-Hyeon Baek, Byung-Kwan Seo
Wilting disorder is an abnormal condition characterized by weakness and paralysis of the upper and lower extremities. Pathogenesis and treatment target of the disorder are unclear; hence, allopathic treatment is generally used to relieve the symptoms. To investigate the treatment mechanism and effect of Traditional Korean Medicine (TKM) in patients with wilting disorder, we reviewed in vivo studies that focused on the effect of TKM on the main symptoms of wilting disorder and treatment of the diseases that can cause these symptoms...
2018: Evidence-based Complementary and Alternative Medicine: ECAM
https://www.readbyqxmd.com/read/30538137/sexuality-and-intimacy-in-als-systematic-literature-review-and-future-perspectives
#5
Barbara Poletti, Laura Carelli, Federica Solca, Rita Pezzati, Andrea Faini, Nicola Ticozzi, Hiroshi Mitsumoto, Vincenzo Silani
Several features of amyotrophic lateral sclerosis (ALS) impact on sexuality and intimate relationship; however, the issue has received poor attention so far. We performed a systematic literature review in order to provide an up-to-date account of sexuality in ALS. References were identified by searches of PubMed, Web of Science, Scopus and PsycINFO (1970-2017, English literature). The following were the key terms: 'sexual' OR 'sexuality' OR 'intimacy' OR 'marital' AND 'ALS' OR 'Amyotrophic Lateral Sclerosis' OR 'Motor Neuron Disease' OR 'MND'...
December 11, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/30537912/deficits-in-emotion-recognition-as-markers-of-frontal-behavioral-dysfunction-in-amyotrophic-lateral-sclerosis
#6
Aldrin Pedroza Martins, Laura de Godoy Rousseff Prado, Patricia Lillo, Eneida Mioshi, Antônio Lúcio Teixeira, Leonardo Cruz de Souza
OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with prominent motor symptoms. Patients with ALS may also manifest frontal behavior symptoms and cognitive decline, including impairment in facial emotion recognition. The authors aimed to investigate whether deficits in emotion recognition were associated with frontal behavior symptoms in ALS. METHODS: Participants were patients with probable or definite sporadic ALS (N=21; male:female ratio, 11:10; median age, 62 years; median disease duration, 3 years) and age-matched and education-matched healthy control subjects (N=25; male:female ratio, 14:11; median age, 61 years)...
December 12, 2018: Journal of Neuropsychiatry and Clinical Neurosciences
https://www.readbyqxmd.com/read/30536581/safety-tolerability-and-pharmacodynamics-of-intrathecal-injection-of-recombinant-human-hgf-kp-100-in-subjects-with-amyotrophic-lateral-sclerosis-a-phase-i-trial
#7
Hitoshi Warita Md PhD, Masaaki Kato Md PhD, Ryuta Asada PhD, Atsuko Yamashita PhD, Daichika Hayata, Kiichi Adachi PhD, Masashi Aoki Md PhD
Hepatocyte growth factor is an endogenous pleiotropic factor shown to act as a potent neuroprotectant against disease progression in animal models of amyotrophic lateral sclerosis, which is a devastating, adult-onset motor neuron disease. To evaluate the safety, tolerability, and pharmacokinetics of recombinant 5-residue-deleted human hepatocyte growth factor (KP-100) injected intrathecally through an implantable catheter connected to a subcutaneous port, we conducted a first-in-human phase I trial of intrathecal KP-100 in 15 Japanese patients with amyotrophic lateral sclerosis...
December 11, 2018: Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/30534373/rare-tbk1-variants-in-patients-with-frontotemporal-dementia-and-amyotrophic-lateral-sclerosis-in-a-chinese-cohort
#8
Bin Jiao, Qiying Sun, Zhenhua Yuan, Junling Wang, Lin Zhou, Xinxiang Yan, Beisha Tang, Lu Shen
Background: The TANK-Binding Kinase 1 ( TBK1 ) gene has recently been identified as the third or fourth most frequent cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). The aim of this study was to assess the genetic contribution of TBK1 in a Chinese cohort. Methods: A total of 270 cases with ALS, FTD, or their combination were recruited into this study. All the coding exons of TBK1 and intron-exon boundaries were sequenced using Sanger sequencing...
2018: Translational Neurodegeneration
https://www.readbyqxmd.com/read/30534107/biomarker-supervised-g-csf-filgrastim-response-in-als-patients
#9
Siw Johannesen, Bettina Budeus, Sebastian Peters, Sabine Iberl, Anne-Louise Meyer, Tina Kammermaier, Eva Wirkert, Tim-Henrik Bruun, Verena C Samara, Wilhelm Schulte-Mattler, Wolfgang Herr, Armin Schneider, Jochen Grassinger, Ulrich Bogdahn
Objective: To evaluate safety, tolerability and feasibility of long-term treatment with Granulocyte-colony stimulating factor (G-CSF), a well-known hematopoietic stem cell factor, guided by assessment of mobilized bone marrow derived stem cells and cytokines in the serum of patients with amyotrophic lateral sclerosis (ALS) treated on a named patient basis. Methods: 36 ALS patients were treated with subcutaneous injections of G-CSF on a named patient basis and in an outpatient setting. Drug was dosed by individual application schemes (mean 464 Mio IU/month, range 90-2160 Mio IU/month) over a median of 13...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/30533572/neat1-and-paraspeckles-in-neurodegenerative-diseases-a-missing-lnc-found
#10
REVIEW
Haiyan An, Non G Williams, Tatyana A Shelkovnikova
Neurodegenerative diseases are among the most common causes of disability worldwide. Although neurodegenerative diseases are heterogeneous in both their clinical features and the underlying physiology, they are all characterised by progressive loss of specific neuronal populations. Recent experimental evidence suggests that long non-coding RNAs (lncRNAs) play important roles in the CNS in health and disease. Nuclear Paraspeckle Assembly Transcript 1 (NEAT1) is an abundant, ubiquitously expressed lncRNA, which forms a scaffold for a specific RNA granule in the nucleus, or nuclear body, the paraspeckle...
December 2018: Non-Coding RNA Research
https://www.readbyqxmd.com/read/30533570/long-non-coding-rnas-and-als-still-much-to-do
#11
REVIEW
Stella Gagliardi, Cecilia Pandini, Maria Garofalo, Matteo Bordoni, Orietta Pansarasa, Cristina Cereda
Alterations in RNA metabolism play an important role in Amyotrophic Lateral Sclerosis (ALS) pathogenesis. The literature has described, so far, a small number of long non coding RNAs (lncRNAs) associated to ALS demonstrating that how there is still much to do to identify and understand their role in ALS. This class of RNAs may offer numerous starting points for new investigations about pathogenic mechanism involved in ALS disease. In this review, we have collected all the presented data about lncRNAs and ALS to offer an overview about this class of non-coding RNAs and their possible role in ALS disease...
December 2018: Non-Coding RNA Research
https://www.readbyqxmd.com/read/30533567/non-coding-rna-in-c9orf72-related-amyotrophic-lateral-sclerosis-and-frontotemporal-dementia-a-perfect-storm-of-dysfunction
#12
REVIEW
Andrew G L Douglas
A hexanucleotide repeat expansion in the first intron/promoter region of C9orf72 is the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Both sense and antisense transcripts exist at the C9orf72 locus but the function of the antisense lncRNA is unknown. RNA toxicity of the transcribed repeat expansion has been implicated in the pathogenesis of C9orf72 -related ALS/FTD, not only through direct sequestration of important RNA binding proteins but also indirectly through non-ATG dependent translation into dipeptide repeats...
December 2018: Non-Coding RNA Research
https://www.readbyqxmd.com/read/30531427/triple-stimulation-technique-in-amyotrophic-lateral-sclerosis
#13
Yue Wang, Han Wang, Li-Ying Cui
PURPOSE: To identify upper motor neuron (UMN) dysfunction using the triple stimulation technique (TST) in amyotrophic lateral sclerosis (ALS). METHODS: Fifty ALS and 42 non-ALS patients were examined clinically, using conventional transcranial magnetic stimulation and TST. RESULTS: For ALS patients presenting with UMN in tested limb, the TST amplitude ratio was abnormal in 25 of 28 patients (89.3%). For ALS patients without UMN signs, 6 of 22 patients (27...
December 7, 2018: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/30531015/safety-of-intrathecal-injection-of-wharton-s-jelly-derived-mesenchymal-stem-cells-in-amyotrophic-lateral-sclerosis-therapy
#14
Monika Barczewska, Mariusz Grudniak, Stanisław Maksymowicz, Tomasz Siwek, Tomasz Ołdak, Katarzyna Jezierska-Woźniak, Dominika Gładysz, Wojciech Maksymowicz
Animal experiments have confirmed that mesenchymal stem cells can inhibit motor neuron apoptosis and inflammatory factor expression and increase neurotrophic factor expression. Therefore, mesenchymal stem cells have been shown to exhibit prospects in the treatment of amyotrophic lateral sclerosis. However, the safety of their clinical application needs to be validated. To investigate the safety of intrathecal injection of Wharton's jelly-derived mesenchymal stem cells in amyotrophic lateral sclerosis therapy, 43 patients (16 females and 27 males, mean age of 57...
February 2019: Neural Regeneration Research
https://www.readbyqxmd.com/read/30531003/oxidative-stress-the-lowest-common-denominator-of-multiple-diseases
#15
REVIEW
Veronika Matschke, Carsten Theiss, Johann Matschke
Oxygen is essential to the human life and life of all aerobic organisms. The complete oxidation of nutrients for the biological energy supply is one of the most important prerequisites for the formation of higher life forms. However, cells that benefit from oxidative respiration also suffer from reactive oxygen species because they adapted to oxygen as an energy source. Healthy cells balance the formation and elimination of reactive oxygen species thereby creating and keeping reactive oxygen species-homeostasis...
February 2019: Neural Regeneration Research
https://www.readbyqxmd.com/read/30530996/amyotrophic-lateral-sclerosis-a-complex-syndrome-that-needs-an-integrated-research-approach
#16
REVIEW
Javier Riancho, Francisco J Gil-Bea, Ana Santurtun, Adolfo López de Munaín
Amyotrophic lateral sclerosis, the most common neurodegenerative disease affecting motor neurons, lacks an effective treatment. A small fraction of amyotrophic lateral sclerosis cases have a familial origin, related to mutations in causative genes, while the vast majority of amyotrophic lateral sclerosis cases are considered to be sporadic, resulting from the interaction between genes and environmental factors in predisposed individuals. During the past few years, dozens of drugs have been postulated as promising strategies for the disease after showing some beneficial effects in preclinical cellular and murine models...
February 2019: Neural Regeneration Research
https://www.readbyqxmd.com/read/30530995/amyotrophic-lateral-sclerosis-as-a-synaptopathy
#17
REVIEW
Matthew J Fogarty
The synapse is an incredibly specialized structure that allows for the coordinated communication of information from one neuron to another. When assembled into circuits, steady streams of excitatory and inhibitory synaptic activity shape neural outputs. At the organismal level, ensembles of neural networks underlie behavior, emotion and memory. Disorder or dysfunctions of synapses, a synaptopathy, may underlie a host of developmental and degenerative neurological conditions. There is a possibility that amyotrophic lateral sclerosis may be a result of a synaptopathy within the neuromotor system...
February 2019: Neural Regeneration Research
https://www.readbyqxmd.com/read/30530797/tau-imaging-detects-distinctive-distribution-of-tau-pathology-in-als-pdc-on-the-kii-peninsula
#18
Hitoshi Shinotoh, Hitoshi Shimada, Yasumasa Kokubo, Kenji Tagai, Fumitoshi Niwa, Soichiro Kitamura, Hironobu Endo, Maiko Ono, Yasuyuki Kimura, Shigeki Hirano, Maya Mimuro, Masanori Ichise, Naruhiko Sahara, Ming-Rong Zhang, Tetsuya Suhara, Makoto Higuchi
OBJECTIVE: To characterize the distribution of tau pathology in patients with amyotrophic lateral sclerosis/parkinsonism dementia complex on the Kii Peninsula (Kii ALS/PDC) by tau PET using [11 C]PBB3 as ligand. METHODS: This is a cross-sectional study of 5 patients with ALS/PDC and one asymptomatic participant with a dense family history of ALS/PDC from the Kii Peninsula who took part in this study. All were men, and their age was 76 ± 8 (mean ± SD) years. Thirteen healthy men (69 ± 6 years) participated as healthy controls (HCs)...
December 7, 2018: Neurology
https://www.readbyqxmd.com/read/30528837/pregnancy-5-years-after-onset-of-amyotrophic-lateral-sclerosis-symptoms-a-case-report-and-review-of-the-literature
#19
Ludmila Barcelos Porto, Anne Maria Lefebvre Berndl
BACKGROUND: Pregnancy in patients with amyotrophic lateral sclerosis (ALS) is extremely rare and often results in delivery of a healthy baby when patients are in the early stages of the disease. CASE: This report describes the case of a successful pregnancy 5 years after ALS onset. Significant worsening of weakness, unsteady balance, and dysphagia were noticed around the third trimester. A healthy child was delivered at term by planned CS. After delivery the patient developed remarkable weakness, dysphagia, and dysarthria...
December 7, 2018: Journal of Obstetrics and Gynaecology Canada: JOGC, Journal D'obstétrique et Gynécologie du Canada: JOGC
https://www.readbyqxmd.com/read/30528741/motor-unit-number-index-munix-of-hand-muscles-is-a-disease-biomarker-for-adult-spinal-muscular-atrophy
#20
René Günther, Christoph Neuwirth, Jan Christoph Koch, Paul Lingor, Nathalie Braun, Robert Untucht, Daniel Petzold, Markus Weber, Andreas Hermann
OBJECTIVE: There is still insufficient knowledge about natural history in adult spinal muscular atrophy, thus valid markers for treatment and disease monitoring are urgently needed. METHODS: We studied hand muscle innervation pattern of 38 adult genetically confirmed 5q spinal muscular atrophy (SMA) patients by the motor unit number index (MUNIX) method. Data were compared to healthy controls and amyotrophic lateral sclerosis (ALS) patients and systematically correlated to typical disease-relevant scores and other clinical as well as demographic characteristics...
November 24, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
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