keyword
MENU ▼
Read by QxMD icon Read
search

Primary lateral sclerosis

keyword
https://www.readbyqxmd.com/read/28229508/meditation-training-for-people-with-amyotrophic-lateral-sclerosis-a-randomized-clinical-trial
#1
F Pagnini, A Marconi, A Tagliaferri, G M Manzoni, R Gatto, V Fabiani, G Gragnano, G Rossi, E Volpato, P Banfi, A Palmieri, F Graziano, G Castelnuovo, M Corbo, E Molinari, N Riva, V Sansone, C Lunetta
BACKGROUND AND PURPOSE: Studies investigating psychological interventions for the promotion of well-being in people with amyotrophic lateral sclerosis (ALS) are lacking. The purpose of the current study was to examine the use of an ALS-specific mindfulness-based intervention for improving quality of life in this population. METHODS: A randomized, open-label and controlled clinical trial was conducted on the efficacy of an ALS-specific meditation programme in promoting quality of life...
February 23, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28214109/a-novel-mutation-in-trem2-gene-causing-nasu-hakola-disease-and-review-of-the-literature
#2
Efthimios Dardiotis, Vasileios Siokas, Eva Pantazi, Maria Dardioti, Dimitrios Rikos, Georgia Xiromerisiou, Aikaterini Markou, Dimitra Papadimitriou, Matthaios Speletas, Georgios M Hadjigeorgiou
Nasu-hakola disease (NHD) is a rare disease characterized by bone cysts and fractures, frontal lobe syndrome, and progressive presenile dementia. NHD may be the prototype of primary microglial disorders of the CNS or, as they have been coined, "microgliopathies". Mutations in TREM2 and TYROBP genes are known to cause NHD. Interestingly, recent evidence-associated rare genetic variants of TREM2 gene with increased risk of Alzheimer's disease, frontotemporal dementia, amyotrophic lateral sclerosis, and Parkinson's disease...
January 20, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28214014/controlling-the-digital-ulcerative-disease-in-systemic-sclerosis-is-associated-with-improved-hand-function
#3
Luc Mouthon, Patrick H Carpentier, Catherine Lok, Pierre Clerson, Virginie Gressin, Eric Hachulla, Alice Bérezné, Elisabeth Diot, Aurélie Khau Van Kien, Patrick Jego, Christian Agard, Anne-Bénédicte Duval-Modeste, Agnès Sparsa, Eve Puzenat, Marie-Aleth Richard
OBJECTIVES: Ischemic digital ulcers (DU) represent a major complication of systemic sclerosis (SSc). We investigated the impact of controlling the ulcerative disease on disability, pain, and quality of life in SSc patients receiving bosentan. METHODS: ECLIPSE (Study AC-052-517) is a 2-year prospective, multicenter, and observational study. Patients with SSc who experienced at least 1 DU in the previous year and received bosentan were included between October 2009 and March 2011...
January 13, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28213912/diagnostic-red-flags-steroid-treated-malignant-cns-lymphoma-mimicking-autoimmune-inflammatory-demyelination
#4
Alonso Barrantes-Freer, Aylin Sophie Engel, Odir Antonio Rodríguez-Villagra, Anne Winkler, Markus Bergmann, Christian Mawrin, Tania Kuempfel, Hannah Pellkofer, Imke Metz, Annalen Bleckmann, Silvia Hernández-Durán, Sven Schippling, Elisabeth J Rushing, Stephan Frank, Markus Glatzel, Jakob Matschke, Christian Hartmann, Guido Reifenberger, Wolf Müller, Hans-Ulrich Schildhaus, Wolfgang Brück, Christine Stadelmann
The presence of inflammation and demyelination in a central nervous system (CNS) biopsy points towards a limited, yet heterogeneous group of pathologies, of which multiple sclerosis (MS) represents one of the principal considerations. Inflammatory demyelination has also been reported in patients with clinically suspected primary central nervous system lymphoma (PCNSL), especially when steroids had been administered prior to biopsy acquisition. The histopathological changes induced by corticosteroid treatment can range from mild reduction to complete disappearance of lymphoma cells...
February 18, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28152040/distinct-repertoires-of-micrornas-present-in-mouse-astrocytes-compared-to-astrocyte-secreted-exosomes
#5
Ana Jovičić, Aaron D Gitler
BACKGROUND: Astrocytes are the most abundant cell type in the central nervous system (CNS) and secrete various factors that regulate neuron development, function and connectivity. microRNAs (miRNAs) are small regulatory RNAs involved in posttranslational gene regulation. Recent findings showed that miRNAs are exchanged between cells via nanovesicles called exosomes. In this study, we sought to define which miRNAs are contained within exosomes secreted by astrocytes. We also explored whether astroglial miRNA secretion via exosomes is perturbed in a mouse model of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease where astrocytes play a crucial role in driving disease progression...
2017: PloS One
https://www.readbyqxmd.com/read/28150100/spontaneous-brain-activity-in-the-sensorimotor-cortex-in-amyotrophic-lateral-sclerosis-can-be-negatively-regulated-by-corticospinal-fiber-integrity
#6
Wataru Sako, Takashi Abe, Yuishin Izumi, Hiroki Yamazaki, Naoko Matsui, Masafumi Harada, Ryuji Kaji
Previous studies failed to detect reduced value of the amplitude of low frequency fluctuation (ALFF) derived from resting state functional magnetic resonance imaging in the primary motor cortex in amyotrophic lateral sclerosis (ALS) though primary motor cortex was mainly affected with ALS. We aimed to investigate the cause of masking the abnormality in the primary motor cortex in ALS and usefulness of ALFF for differential diagnosis among diseases showing muscle weakness. We enrolled ten patients with ALS and eleven disease controls showing muscle weakness...
February 1, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28144764/prospective-analysis-of-the-sealing-ability-of-the-enseal-%C3%A2-g2-articulating-tissue-sealer-and-transector-on-human-mesenteric-vessels-in-colorectal-surgery
#7
B Balachandran, G Melich, T Mustafa, S J Marecik, L M Prasad, M Gonzalez, S Sulo, F Dabbous, J J Park
BACKGROUND: The sealing and transection of mesenteric vessels is a crucial step in minimally invasive colorectal surgery. We examined the sealing quality of the ENSEAL(®) G2 Articulating Tissue Sealer in three different articulations in mesenteric vessels. METHODS: This was a prospective experimental study within a tertiary healthcare center, and 30 patients were recruited. Burst pressures for each specimen were measured as the primary outcome. Ten specimens at each of the three articulations were also histologically assessed for the quality of seal...
January 31, 2017: Techniques in Coloproctology
https://www.readbyqxmd.com/read/28139349/safety-and-efficacy-of-ozanezumab-in-patients-with-amyotrophic-lateral-sclerosis-a-randomised-double-blind-placebo-controlled-phase-2-trial
#8
Vincent Meininger, Angela Genge, Leonard H van den Berg, Wim Robberecht, Albert Ludolph, Adriano Chio, Seung H Kim, P Nigel Leigh, Matthew C Kiernan, Jeremy M Shefner, Claude Desnuelle, Karen E Morrison, Susanne Petri, Diane Boswell, Jane Temple, Rajat Mohindra, Matt Davies, Jonathan Bullman, Paul Rees, Arseniy Lavrov
BACKGROUND: Neurite outgrowth inhibitor A (Nogo-A) is thought to have a role in the pathophysiology of amyotrophic lateral sclerosis (ALS). A monoclonal antibody against Nogo-A showed a positive effect in the SOD1(G93A) mouse model of ALS, and a humanised form of this antibody (ozanezumab) was well tolerated in a first-in-human trial. Therefore, we aimed to assess the safety and efficacy of ozanezumab in patients with ALS. METHODS: This randomised, double-blind, placebo-controlled, phase 2 trial was done in 34 centres in 11 countries...
27, 2017: Lancet Neurology
https://www.readbyqxmd.com/read/28128448/chronic-infusion-of-sod1-g93a-astrocyte-secreted-factors-induces-spinal-motoneuron-degeneration-and-neuromuscular-dysfunction-in-healthy-rats
#9
U N Ramírez-Jarquín, F Rojas, B van Zundert, R Tapia
Amyotrophic lateral sclerosis is a fatal neurodegenerative disease and studies in vitro show that motoneuron degeneration is triggered by non-cell-autonomous mechanisms. However, whether soluble toxic factor(s) released by mutant superoxide dismutase 1 (SOD1) expressing astrocytes induces death of motoneurons and leads to motor dysfunction in vivo is not known. To directly test this, healthy adult rats were treated with conditioned media derived from primary mouse astrocytes (ACM) that express human (h) SOD1(G93A) (ACM-hG93A) via chronic osmotic pump infusion in the lumbar spinal cord...
January 27, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28114839/detecting-neuronal-dysfunction-of-hand-motor-cortex-in-als-a-mrsi-study
#10
Yuzhou Wang, Xiaodi Li, Wenming Chen, Zhanhang Wang, Yan Xu, Jingpan Luo, Hanbo Lin, Guijun Sun
BACKGROUND: Although hand motor cortex (HMC) has been constantly used for identification of primary motor cortex in magnetic resonance spectroscopy (MRS) studies of amyotrophic lateral sclerosis (ALS), neurochemical profiles of HMC have never been assessed independently. As HMC has a constant location and the clinic-anatomic correlation between hand motor function and HMC has been established, we hypothesize that HMC may serve as a promising region of interest in diagnosing ALS. PATIENTS AND METHODS: Fourteen ALS patients and 14 age- and gender-matched healthy controls (HC) were recruited in this study...
January 23, 2017: Somatosensory & Motor Research
https://www.readbyqxmd.com/read/28095923/nadph-oxidase-in-brain-injury-and-neurodegenerative-disorders
#11
REVIEW
Merry W Ma, Jing Wang, Quanguang Zhang, Ruimin Wang, Krishnan M Dhandapani, Ratna K Vadlamudi, Darrell W Brann
Oxidative stress is a common denominator in the pathology of neurodegenerative disorders such as Alzheimer's disease, Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis, and multiple sclerosis, as well as in ischemic and traumatic brain injury. The brain is highly vulnerable to oxidative damage due to its high metabolic demand. However, therapies attempting to scavenge free radicals have shown little success. By shifting the focus to inhibit the generation of damaging free radicals, recent studies have identified NADPH oxidase as a major contributor to disease pathology...
January 17, 2017: Molecular Neurodegeneration
https://www.readbyqxmd.com/read/28076984/percutaneous-endoscopic-gastrostomy-body-weight-loss-and-survival-in-amyotrophic-lateral-sclerosis-a-population-based-registry-study
#12
Antonio Fasano, Nicola Fini, Diana Ferraro, Laura Ferri, Marco Vinceti, Jessica Mandrioli
OBJECTIVE: To assess the role of percutaneous endoscopic gastrostomy (PEG) insertion, and its timing, on ALS survival, and to study prognostic factors of survival before and after PEG placement in a population-based setting. METHODS: In this observational population-based, registry study, we enrolled patients with newly- diagnosed ALS, according to the El Escorial revised criteria, who were resident in the Emilia Romagna Region, and who developed severe dysphagia needing enteral nutritional support...
January 11, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28070747/enhanced-bulbar-function-in-amyotrophic-lateral-sclerosis-the-nuedexta-treatment-trial
#13
Richard Smith, Erik Pioro, Kathleen Myers, Michael Sirdofsky, Kimberly Goslin, Gregg Meekins, Hong Yu, James Wymer, Merit Cudkowicz, Eric A Macklin, David Schoenfeld, Gary Pattee
The goal of this randomized, blinded, crossover clinical trial was to determine whether Nuedexta (dextromethorphan and quinidine) enhanced speech, swallowing, and salivation in patients with ALS. Sixty patients with amyotrophic lateral sclerosis (ALS) received either Nuedexta or placebo for 28 to 30 days, followed by a 10 to 15-day washout period. Subsequently, patients were switched to the opposite treatment arm for the remaining days of the trial. The primary endpoint was a reduction in the self-report Center for Neurologic Study Bulbar Function Scale (CNS-BFS) score...
January 9, 2017: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
https://www.readbyqxmd.com/read/28067943/gamma-aminobutyric-acid-gaba-modulators-for-amyotrophic-lateral-sclerosis-motor-neuron-disease
#14
REVIEW
Andrea Diana, Rita Pillai, Paolo Bongioanni, Aidan G O'Keeffe, Robert G Miller, Dan H Moore
BACKGROUND: Imbalance of gamma aminobutyric acid (GABA) and related modulators has been implicated as an important factor in the pathogenesis of amyotrophic lateral sclerosis (ALS), which is also known as motor neuron disease (MND). In this context, the role and mechanism of action of gabapentin and baclofen have been extensively investigated, although with conflicting results. This is the first systematic review to assess clinical trials of GABA modulators for the treatment of ALS. OBJECTIVES: To examine the efficacy of gabapentin, baclofen, or other GABA modulators in delaying the progression of ALS, and to evaluate adverse effects of these interventions...
January 9, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28045953/double-blind-controlled-randomized-trial-of-cyclophosphamide-versus-methylprednisolone-in-secondary-progressive-multiple-sclerosis
#15
Bruno Brochet, Mathilde S A Deloire, Paul Perez, Timothé Loock, Louise Baschet, Marc Debouverie, Sophie Pittion, Jean-Christophe Ouallet, Pierre Clavelou, Jérôme de Sèze, Nicolas Collongues, Patrick Vermersch, Hélène Zéphir, Giovanni Castelnovo, Pierre Labauge, Christine Lebrun, Mikael Cohen, Aurélie Ruet
BACKGROUND: Therapeutic options are limited in secondary progressive multiple sclerosis (SPMS). Open-label studies suggested efficacy of monthly IV cyclophosphamide (CPM) without induction for delaying progression but no randomized trial was conducted so far. OBJECTIVE: To compare CPM to methylprednisolone (MP) in SPMS. METHODS: Randomized, double-blind clinical trial on two parallel groups. Patient with SPMS, with a documented worsening of the Expanded Disability Status Scale (EDSS) score during the last year and an EDSS score between 4·0 and 6·5 were recruited and received one intravenous infusion of treatment (CPM: 750 mg /m2 body surface area-MP: 1g) every four weeks for one year, and every eight weeks for the second year...
2017: PloS One
https://www.readbyqxmd.com/read/28032330/cerebrospinal-fluid-cytotoxicity-does-not-affect-survival-in-amyotrophic-lateral-sclerosis
#16
L Galán, J Matías-Guiu, J A Matias-Guiu, M Yáñez, V Pytel, A Guerrero-Sola, A Vela-Souto, J A Arranz-Tagarro, U Gómez-Pinedo, A G García
OBJECTIVES: Cerebrospinal fluid (CSF) from some patients with amyotrophic lateral sclerosis (ALS) has been demonstrated to significantly reduce the neuronal viability of primary cell cultures of motor neurons. We aimed to study the potential clinical consequences associated with the cytotoxicity of CSF in a cohort of patients with ALS. METHODS: We collected CSF from thirty-one patients with ALS. We analysed cytotoxicity by incubating it into the primary cultures of motor cortex neurons...
December 29, 2016: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/28029397/matrin-3-variants-are-frequent-in-italian-als-patients
#17
Giuseppe Marangi, Serena Lattante, Paolo Niccolò Doronzio, Amelia Conte, Giorgio Tasca, Mauro Monforte, Agata Katia Patanella, Giulia Bisogni, Emiliana Meleo, Salvatore La Spada, Marcella Zollino, Mario Sabatelli
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by loss of motor neurons in the primary motor cortex, brainstem, and spinal cord. Recently, missense variants in MATR3 were identified in familial and sporadic ALS patients, but very few additional ALS patients have been reported so far. The p.S85C MATR3 variant was previously associated to a different phenotype, namely a distal myopathy associated with dysphagia and dysphonia. Here, we assessed the contribution of MATR3 variants in a cohort of 322 Italian ALS patients...
October 6, 2016: Neurobiology of Aging
https://www.readbyqxmd.com/read/28012605/corrigendum-to-review-of-amyotrophic-lateral-sclerosis-parkinson-s-and-alzheimer-s-diseases-helps-further-define-proteomic-events-of-the-novel-paradigm-for-alzheimer-s-with-metals-as-primary-disease-cause-med-hypotheses-85-2015-779-790
#18
https://www.readbyqxmd.com/read/28009454/direct-estimation-for-adaptive-treatment-length-policies-methods-and-application-to-evaluating-the-effect-of-delayed-peg-insertion
#19
Xin Lu, Brent A Johnson
Dysphagia is a primary cause of death among patients diagnosed with amyotrophic lateral sclerosis (ALS), and percutaneous endoscopic gastrostomy (PEG) is a procedure to insert a tube into the stomach to assist or replace oral feeding. It is believed that PEG is beneficial and, generally, earlier insertion is preferable to later. However, gathering clinical evidence to support these beliefs on the use and timing of PEG is challenging because controlled clinical trials are not feasible and clinical endpoints are confounded with PEG in observational data...
December 23, 2016: Biometrics
https://www.readbyqxmd.com/read/28003278/clinical-spectrum-of-amyotrophic-lateral-sclerosis-als
#20
Leslie I Grad, Guy A Rouleau, John Ravits, Neil R Cashman
Amyotrophic lateral sclerosis (ALS) is primarily characterized by progressive loss of motor neurons, although there is marked phenotypic heterogeneity between cases. Typical, or "classical," ALS is associated with simultaneous upper motor neuron (UMN) and lower motor neuron (LMN) involvement at disease onset, whereas atypical forms, such as primary lateral sclerosis and progressive muscular atrophy, have early and predominant involvement in the UMN and LMN, respectively. The varying phenotypes can be so distinctive that they would seem to have differing biology...
December 21, 2016: Cold Spring Harbor Perspectives in Medicine
keyword
keyword
22590
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"