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Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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https://www.readbyqxmd.com/read/27899032/proteomic-profiling-of-the-spinal-cord-in-als-decreased-atp5d-levels-suggest-synaptic-dysfunction-in-als-pathogenesis
#1
Jooyeon Engelen-Lee, Anna M Blokhuis, Wim G M Spliet, R Jeroen Pasterkamp, Eleonora Aronica, Jeroen A A Demmers, Roel Broekhuizen, Giovanni Nardo, Niels Bovenschen, Leonard H Van Den Berg
BACKGROUND: We aimed to gain new insights into the pathogenesis of sporadic ALS (sALS) through a comprehensive proteomic analysis. METHODS: Protein profiles of the anterior and posterior horn in post-mortem spinal cord samples of 10 ALS patients and 10 controls were analysed using 2D-differential gel electrophoresis. The identified protein spots with statistically significant level changes and a spot ratio >2.0 were analysed by LC-MS/MS. RESULTS: In the posterior horn only 3 proteins were differentially expressed...
November 29, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27894192/alsuntangled-38-l-serine
#2
(no author information available yet)
No abstract text is available yet for this article.
November 29, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27894201/screening-for-cognitive-dysfunction-in-als-validation-of-the-edinburgh-cognitive-and-behavioural-als-screen-ecas-using-age-and-education-adjusted-normative-data
#3
Marta Pinto-Grau, Tom Burke, Katie Lonergan, Caroline McHugh, Iain Mays, Caoifa Madden, Alice Vajda, Mark Heverin, Marwa Elamin, Orla Hardiman, Niall Pender
BACKGROUND: Cognitive and behavioural changes are an important aspect in Amyotrophic Lateral Sclerosis (ALS). The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) briefly assesses these changes in ALS. OBJECTIVE: To validate the ECAS against a standardised neuropsychological battery and assess its sensitivity and specificity using age and education adjusted cut-off scores. METHOD: 30 incident ALS cases were assessed on both, ECAS and neuropsychological battery...
November 28, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27894191/identifying-behavioural-changes-in-als-validation-of-the-beaumont-behavioural-inventory-bbi
#4
Marwa Elamin, Marta Pinto-Grau, Tom Burke, Peter Bede, James Rooney, Meabhdh O'Sullivan, Katie Lonergan, Emma Kirby, Emma Quinlan, Nadia Breen, Alice Vajda, Mark Heverin, Niall Pender, Orla Hardiman
OBJECTIVE: Behavioural changes are an important part of amyotrophic lateral sclerosis (ALS). However, most tools do not account for the influence of motor impairment. Furthermore, they do not fully measure the broad range of behavioural changes specific to ALS. This study aimed to develop and validate an ALS specific behavioural inventory, the Beaumont Behavioural Inventory (BBI). METHODS: The BBI was validated in a cohort of ALS patients (n = 85) and 78 age-, gender-, and education-matched controls...
November 28, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27892707/is-firstly-diagnosed-als-really-als-results-of-a-population-based-study-with-long-term-follow-up
#5
Elisabetta Pupillo, Elisa Bianchi, Marco Poloni, Ettore Beghi
OBJECTIVE: To revise the first diagnosis of amyotrophic lateral sclerosis (ALS) in patients from a well-defined population. METHODS: Patients diagnosed with ALS in the years 1998-2002 and resident of Lombardy Region, Northern Italy were followed until death or April 30 2016 to assess long-term survival. During follow-up, the caring neurologists were asked to confirm the first diagnosis. Revised diagnoses were classified as confirmed and unconfirmed motor neuron disease (MND) with further specification where available...
November 28, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
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
#6
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/27892702/slowly-progressive-motor-neuron-disease-with-multi-system-involvement-related-to-p-e121g-sod1-mutation
#7
Guillaume Taieb, Anne Polge, Raul Juntas-Morales, Nicolas Pageot, Serge Lumbroso, Kevin Mouzat, William Camu
We report the third case of amyotrophic lateral sclerosis related to p.E121G Superoxide dismutase-1 (SOD1) mutation. Besides a sporadic presentation and a slow progressive course, as described in the 2 previously cases, our patient presented with prominent sensory and cerebellar signs. This case report strengthens that p.E121G should be considered as a causal mutation. Slowly upper and lower motor neuron degeneration, even with non-motor clinical features, should prompt a sequencing of SOD1.
November 28, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27892698/the-relationship-between-oestrogen-and-executive-functioning-in-als-females-with-emerging-frontotemporal-lobar-degeneration-ftld-supports-a-neuroendocrine-model-of-ftld-attenuation
#8
C Flaherty, J Kraft, A Brothers, M Harrison, R S Legro, A Manni, C Yang, Z Simmons
OBJECTIVE: The prevalence of ALS cognitive or behavioural impairment (ci or bi) consistent with Frontotemporal Degeneration (FTLD) approachs 50%, while ∼5-10% progress to dementia. Our goal was to explore ci and bi differencs between bulbar and limb onset, as well as the neuroprotective potential of oestrogen in emerging FTLD. METHODS: We applied Mann Whitney U to evaluate differences in cognitive and behavioural profiles between site of onset in 78 female and 83 male non-demented ALS participants classified by current consensus criteria with ci...
November 28, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27819158/diagnostic-and-prognostic-significance-of-neurofilament-light-chain-nf-l-but-not-progranulin-and-s100b-in-the-course-of-amyotrophic-lateral-sclerosis-data-from-the-german-mnd-net
#9
Petra Steinacker, André Huss, Benjamin Mayer, Torsten Grehl, Julian Grosskreutz, Guntram Borck, Jens Kuhle, Dorothée Lulé, Thomas Meyer, Patrick Oeckl, Susanne Petri, Jochen Weishaupt, Albert C Ludolph, Markus Otto
There is a need for diagnostic, prognostic, and monitoring blood biomarkers for ALS. We aimed to analyse and compare proposed candidate markers for disease progression in the course of ALS. Blood samples were taken from 125 ALS patients, including nine patients with C9orf72 or SOD1 mutation, at regular intervals of six months. ALS patients were characterized by the ALS functional rating scale (ALSFRS-R) and the Edinburgh Cognitive and Behavioural ALS Screen (ECAS). We quantified neurofilament light chain (NF-L), S100B, and progranulin (PGRN) and analysed it in relation to disease progression...
November 5, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27813421/longitudinal-evaluation-of-upper-extremity-reachable-workspace-in-als-by-kinect-sensor
#10
Evan de Bie, Bjorn Oskarsson, Nanette C Joyce, Alina Nicorici, Gregorij Kurillo, Jay J Han
Our objective was to evaluate longitudinal changes in Microsoft Kinect measured upper extremity reachable workspace relative surface area (RSA) versus the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R), ALSFRS-R upper extremity sub-scale and Forced Vital Capacity (FVC) in a cohort of patients diagnosed with amyotrophic lateral sclerosis (ALS). Ten patients diagnosed with ALS (ages 52-76 years, ALSFRS-R: 8-41 at entry) were tested using single 3D depth sensor, Microsoft Kinect, to measure reachable workspace RSA across five visits spanning one year...
November 4, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27797285/validity-of-medico-administrative-data-related-to-amyotrophic-lateral-sclerosis-in-france-a-population-based-study
#11
Rosario Vasta, Farid Boumédiene, Philippe Couratier, Marie Nicol, Alessandra Nicoletti, Pierre-Marie Preux, Benoit Marin
The accuracy of French medico-administrative data concerning amyotrophic lateral sclerosis (ALS) is to date unknown. We aimed to assess the validity of hospital discharge data (HDD) and health insurance data (HID) related to ALS. A retrospective population-based study was performed. The French register of ALS in Limousin (FRALim) was used as gold standard (2000-2013 period). All patients discharged from the regional hospitals with a 'G12.2' code in their HDD (according to the International Classification of Disease-10th version) or having a G12 HID code were considered...
October 31, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27677562/serum-urate-at-trial-entry-and-als-progression-in-empower
#12
ÉIlis J O'Reilly, Dawei Liu, Donald R Johns, Merit E Cudkowicz, Sabrina Paganoni, Michael A Schwarzschild, Melanie Leitner, Alberto Ascherio
Our objective was to determine whether serum urate predicts ALS progression. A study population comprised adult participants of EMPOWER (n = 942), a phase III clinical trial to evaluate the efficacy of dexpramipexole to treat ALS. Urate was measured in blood samples collected during enrollment as part of the routine block chemistry. We measured outcomes by combined assessment of function and survival rank (CAFs), and time to death, by 12 months. Results showed that in females there was not a significant relation between urate and outcomes...
September 28, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27652840/epilepsy-and-electroencephalographic-abnormalities-in-c9orf72-repeat-expansion
#13
Margherita Capasso, Francesca Anzellotti, Roberta Di Giacomo, Marco Onofrj
A patient has been recently described with frontotemporal cognitive decline and C9orf72 repeat expansion who presented abnormally slowed background and photoparoxysmal response at electroencephalographic (EEG) recording. Our data, based on five patients with repeat expansions in C9orf72 and EEG recordings suggest that abnormally slowed background and epilepsy represent previously unrecognized features of the C9orf72 phenotype in some patients.
September 21, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27629284/survival-in-amyotrophic-lateral-sclerosis-patients-on-non-invasive-ventilation-what-can-we-do-more
#14
Antonio M Esquinas, Giancarlo Garuti, Giulia Michela Pellegrino, Giuseppe Francesco Sferrazza Papa
No abstract text is available yet for this article.
September 15, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27629154/corrigendum
#15
(no author information available yet)
No abstract text is available yet for this article.
September 14, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27891837/corrigendum
#16
(no author information available yet)
No abstract text is available yet for this article.
November 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27827537/session-1-joint-opening-session
#17
(no author information available yet)
No abstract text is available yet for this article.
November 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27827536/poster-communications
#18
(no author information available yet)
No abstract text is available yet for this article.
November 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27825267/session-subject-index
#19
(no author information available yet)
No abstract text is available yet for this article.
November 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27825266/session-author-index
#20
(no author information available yet)
No abstract text is available yet for this article.
November 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
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