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https://www.readbyqxmd.com/read/28914354/comorbidity-of-dementia-with-amyotrophic-lateral-sclerosis-als-insights-from-a-large-multicenter-italian-cohort
#1
Francesca Trojsi, Mattia Siciliano, Cinzia Femiano, Gabriella Santangelo, Christian Lunetta, Andrea Calvo, Cristina Moglia, Kalliopi Marinou, Nicola Ticozzi, Gianluca Drago Ferrante, Carlo Scialò, Gianni Sorarù, Amelia Conte, Yuri M Falzone, Rosanna Tortelli, Massimo Russo, Valeria Ada Sansone, Adriano Chiò, Gabriele Mora, Barbara Poletti, Paolo Volanti, Claudia Caponnetto, Giorgia Querin, Mario Sabatelli, Nilo Riva, Giancarlo Logroscino, Sonia Messina, Antonio Fasano, Maria Rosaria Monsurrò, Gioacchino Tedeschi, Jessica Mandrioli
To assess the association, at diagnosis, between amyotrophic lateral sclerosis (ALS) and dementia in a large cohort of well-characterized Italian patients. We investigated the phenotypic profile of 1638 incident patients with definite, probable or laboratory-supported probable ALS, diagnosed from January 2009 to December 2013 in 13 Italian Referral Centers, located in 10 Italian Regions, and classified in two independent subsamples accounting for presence or not of dementia. The collected ALS features, including survival and other follow-up data, were compared between the two subgroups using a one-way analysis of variance and Chi-square test, as appropriate, logistic regression models and Kaplan-Meier survival analysis...
September 15, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28884711/-clinical-polymorphism-of-amyotrophic-lateral-sclerosis
#2
E A Kovrazhkina, O D Razinskaya, L V Gubsky
AIM: To clarify clinical polymorphism of amyotrophic lateral sclerosis (ALS). MATERIAL AND METHODS: The study was based on records of a hospital personalized register. Ninety-four patients, aged from 25 to 81 years, diagnosed with ALS according to El Escorial criteria were included. Electromyography and, if necessary, transcranial magnetic stimulation and magnetic-resonance tomography were used to confirm the diagnosis. Disease progression was assessed with the ARSFRS...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/28873374/characteristics-and-prognosis-of-oldest-old-subjects-with-amyotrophic-lateral-sclerosis
#3
Meira Dandaba, Philippe Couratier, Anaïs Labrunie, Marie Nicol, Bello Hamidou, Marie Raymondeau, Giancarlo Logroscino, Pierre Marie Preux, Benoît Marin
BACKGROUND: Amyotrophic Lateral Sclerosis (ALS) is an age-related neurodegenerative disease with unclear characteristics and prognosis in the oldest old (80 years and over). The aim of this study was to compare the oldest old and younger ALS patients in terms of clinical and socio-demographic characteristics, and prognosis. METHODS: ALS incident cases from the register of ALS in Limousin (FRALim), diagnosed between January 2000 and July 2013, were included. Descriptive and comparative analyses by age group were carried out...
September 5, 2017: Neuroepidemiology
https://www.readbyqxmd.com/read/28872913/post-hoc-analysis-of-randomised-placebo-controlled-double-blind-study-mci186-19-of-edaravone-mci-186-in-amyotrophic-lateral-sclerosis
#4
Koji Takei, Fumihiro Takahashi, Shawn Liu, Kikumi Tsuda, Joseph Palumbo
Post-hoc analyses of the ALS Functional Rating Scale-Revised (ALSFRS-R) score data, the primary endpoint in the 24-week double-blind placebo-controlled study of edaravone (MCI186-19, NCT01492686), were performed to confirm statistical robustness of the result. The previously reported original analysis had used a last observation carried forward (LOCF) method and also excluded patients with fewer than three completed treatment cycles. The post-hoc sensitivity analyses used different statistical methods as follows: 1) including all patients regardless of treatment cycles received (ALL LOCF); 2) a mixed model for repeated measurements (MMRM) analysis; and 3) the Combined Assessment of Function and Survival (CAFS) endpoint...
October 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28872912/an-assessment-of-treatment-guidelines-clinical-practices-demographics-and-progression-of-disease-among-patients-with-amyotrophic-lateral-sclerosis-in-japan-the-united-states-and-europe
#5
Koji Takei, Kikumi Tsuda, Fumihiro Takahashi, Manabu Hirai, Joseph Palumbo
BACKGROUND: There is an increasing clinical research focus on neuroprotective agents in amyotrophic lateral sclerosis (ALS). However, it is unclear how generalisable clinical study trial results are between different countries and regions. OBJECTIVE: To assess similarities and differences in clinical practice and treatment guidelines for ALS, and also to compare the demographics and rate of progression of disease in patients with ALS enrolled in clinical trials in Japan, the US, and Europe...
October 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28870572/high-angular-resolution-diffusion-imaging-abnormalities-in-the-early-stages-of-amyotrophic-lateral-sclerosis
#6
Francesca Trojsi, Giuseppina Caiazzo, Federica Di Nardo, Michele Fratello, Gabriella Santangelo, Mattia Siciliano, Cinzia Femiano, Antonio Russo, Maria Rosaria Monsurrò, Mario Cirillo, Gioacchino Tedeschi, Fabrizio Esposito
OBJECTIVE: Using magnetic resonance (MR) high angular resolution diffusion imaging (HARDI), we aimed at revealing possible microstructural alterations in the early stage of amyotrophic lateral sclerosis (ALS), still not completely elucidated. METHODS: We studied 22 patients with ALS, in stages 1 or 2 according to the King's staging system, compared to 18 healthy controls (HCs). Statistical mapping of HARDI-derived parameters and tractography measures were performed using the Q-ball imaging diffusion data model...
September 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28864675/causal-inference-methods-to-study-gastric-tube-use-in-amyotrophic-lateral-sclerosis
#7
Erin McDonnell, David Schoenfeld, Sabrina Paganoni, Nazem Atassi
OBJECTIVE: To estimate effects of gastric tube (G-tube) on survival and quality of life (QOL) in people with amyotrophic lateral sclerosis (ALS) correcting for confounding by indication inherent in nonrandomized observational data. METHODS: To complement a recent causal inference analysis, which concluded that G-tube placement increases the hazard of death, permanent assisted ventilation, or tracheostomy by 28%, we fit causal inference models on a different sample of 481 patients with ALS enrolled in a recent clinical trial of ceftriaxone...
September 1, 2017: Neurology
https://www.readbyqxmd.com/read/28861032/mismatch-negativity-as-an-indicator-of-cognitive-sub-domain-dysfunction-in-amyotrophic-lateral-sclerosis
#8
Parameswaran Mahadeva Iyer, Kieran Mohr, Michael Broderick, Brighid Gavin, Tom Burke, Peter Bede, Marta Pinto-Grau, Niall P Pender, Russell McLaughlin, Alice Vajda, Mark Heverin, Edmund C Lalor, Orla Hardiman, Bahman Nasseroleslami
OBJECTIVE: To evaluate the utility of mismatch negativity (MMN), a neurophysiologic marker of non-motor cognitive processing, in amyotrophic lateral sclerosis (ALS). METHODS: 89 patients, stratified into 4 different phenotypic presentations of ALS (67 spinal-onset, 15 bulbar-onset, 7 ALS-FTD, 7 C9ORF72 gene careers), and 19 matched controls underwent 128-channel EEG data recording. Subjects were presented with standard auditory tones interleaved with pitch-deviant tones in three recording blocks...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28834910/application-of-different-ventilator-modes-in-patients-with-amyotrophic-lateral-sclerosis-according-to-certain-clinical-situations-a-case-report
#9
Donghwi Park
RATIONALE: Amyotrophic lateral sclerosis (ALS) is a rapidly progressing neurodegenerative disease that involves limb, axial, bulbar, and respiratory muscles. Fatigue and weakness of the respiratory muscles eventually induce respiratory insufficiency, which is one of the main causes of death in patients with ALS. In ALS patients with respiratory insufficiency, application of a ventilator is indispensable. Although there are various modes of ventilation, these modes are classified roughly into volume-controlled ventilation (VCV) and pressure-controlled ventilation (PCV)...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28813081/epidemiological-and-clinical-factors-impact-on-the-benefit-of-riluzole-in-the-survival-rates-of-patients-with-als
#10
Francis Meire Fávero, Mariana Callil Voos, Isac de Castro, Fátima Aparecida Caromano, Acary Souza Bulle Oliveira
Objective: To investigate the impact of epidemiological and clinical factors on the benefit of riluzole in patients with amyotrophic lateral sclerosis (ALS). Methods: The survival rate of 578 patients with ALS (1999-2011) was analyzed by descriptive statistics and Kaplan-Meier curves. Considering the median of the sample survival time (19 months), patients were divided in two groups: below (B19) and above the median (A19). Kaplan-Meier curves compared the survival rates of patients treated with riluzole and with patients who did not take the medication...
August 2017: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/28762856/exploring-the-diagnosis-delay-and-als-functional-impairment-at-diagnosis-as-relevant-criteria-for-clinical-trial-enrolment
#11
Bello Hamidou, Benoit Marin, Geraldine Lautrette, Marie Nicol, William Camu, Philippe Corcia, Marie-Christine Arnes-Bes, Christine Tranchant, Pierre Clavelou, Didier Hannequin, Giroud Maurice, Katell Beauvais, Jean-Christophe Antoine, Véronique Danel-Brunaud, Fausto Viader, Pierre-Marie Preux, Philippe Couratier
Objectives were: i) to describe the phenotypic heterogeneity of incident amyotrophic lateral sclerosis (ALS) patients diagnosed in 2012 in French ALS centres; ii) to look at the associations between ALSFRS-R score and ALSFRS-R slope (ΔFS) at time of diagnosis with diagnosis delay, ALS phenotypes and Airlie House diagnosis criteria (AHDC); iii) to describe the rate of progression on ΔFS, according to diagnosis delay. METHODS: Incident ALS cases diagnosed in French ALS centres were included...
August 1, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28748847/clinical-features-of-isolated-bulbar-palsy-of-amyotrophic-lateral-sclerosis-in-chinese-population
#12
Hua-Gang Zhang, Lu Chen, Lu Tang, Nan Zhang, Dong-Sheng Fan
BACKGROUND: Progressive bulbar palsy (PBP) is a classic phenotype of bulbar onset amyotrophic lateral sclerosis (ALS) with more rapid progression and worse prognosis. However, as an often under-understood variant of ALS, isolated bulbar palsy (IBP) appears to progress more slowly and has a relatively benign prognosis. This study aimed to investigate the natural course and clinical features of IBP in Chinese population and to compare them with those of PBP. METHODS: The clinical data of patients with bulbar onset ALS were collected from January 2009 to December 2013...
August 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28741375/comparison-of-slow-and-forced-vital-capacities-on-ability-to-predict-survival-in-als
#13
Susana Pinto, Mamede de Carvalho
INTRODUCTION: Slow (SVC) and forced (FVC) vital capacities are the most used pulmonary function tests in amyotrophic lateral sclerosis (ALS). It is unknown if they equally predict survival in ALS. The aim of the present study was to compare both measures in predicting survival in this disease. METHODS: Consecutive definite/probable ALS patients (2000-2014) in whom respiratory tests were performed at baseline and four months later were included. All patients were evaluated with the revised ALS functional rating scale (ALSFRS-R), respiratory (RofALSFRS-R), bulbar (ALSFRSb), upper and lower limb subscores, SVC, FVC, maximal inspiratory (MIP) and expiratory (MEP) pressures...
July 25, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28738414/impact-of-amyotrophic-lateral-sclerosis-on-slow-tonic-myofiber-composition-in-human-extraocular-muscles
#14
Anton E Tjust, Adam Danielsson, Peter M Andersen, Thomas Brännström, Fatima Pedrosa Domellöf
Purpose: To analyze the proportion and cross-sectional area of myofibers containing myosin heavy chain slow-twitch (MyHCI) and myosin heavy chain slow tonic (MyHCsto) in extraocular muscles of autopsied amyotrophic lateral sclerosis (ALS) patients with either spinal or bulbar site of disease onset. Methods: Whole-muscle cross sections from the middle portion of the medial rectus were labeled with antibodies against MyHCI or MyHCsto and laminin. Myofibers labeled with the MyHC antibodies (MyHCI+sto) and the total number of myofibers were quantified in the orbital and global layer of 6 control individuals and 18 ALS patients...
July 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28717355/correlation-of-creatine-kinase-levels-with-clinical-features-and-survival-in-amyotrophic-lateral-sclerosis
#15
Hongfei Tai, Liying Cui, Yuzhou Guan, Mingsheng Liu, Xiaoguang Li, Dongchao Shen, Dawei Li, Bo Cui, Jia Fang, Qingyun Ding, Kang Zhang, Shuangwu Liu
OBJECTIVE: To evaluate serum creatine kinase (CK) levels of amyotrophic lateral sclerosis (ALS) patients and to explore the relationship between CK levels and the clinical characteristics and survival prognosis of ALS patients. METHODS: We analyzed the CK levels of 185 ALS patients who underwent long-term follow-up. The relationship between CK levels and clinical features including sex, age, disease duration, site of onset, body mass index (BMI), serum creatinine (Cr), and spontaneous electromyographic activity was analyzed by univariate analysis and multiple linear regression...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28710326/cortical-influences-drive-amyotrophic-lateral-sclerosis
#16
REVIEW
Andrew Eisen, Heiko Braak, Kelly Del Tredici, Roger Lemon, Albert C Ludolph, Matthew C Kiernan
The early motor manifestations of sporadic amyotrophic lateral sclerosis (ALS), while rarely documented, reflect failure of adaptive complex motor skills. The development of these skills correlates with progressive evolution of a direct corticomotoneuronal system that is unique to primates and markedly enhanced in humans. The failure of this system in ALS may translate into the split hand presentation, gait disturbance, split leg syndrome and bulbar symptomatology related to vocalisation and breathing, and possibly diffuse fasciculation, characteristic of ALS...
July 14, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28680390/the-role-of-the-heat-shock-protein-b8-hspb8-in-motoneuron-diseases
#17
REVIEW
Paola Rusmini, Riccardo Cristofani, Mariarita Galbiati, Maria E Cicardi, Marco Meroni, Veronica Ferrari, Giulia Vezzoli, Barbara Tedesco, Elio Messi, Margherita Piccolella, Serena Carra, Valeria Crippa, Angelo Poletti
Amyotrophic lateral sclerosis (ALS) and spinal and bulbar muscular atrophy (SBMA) are two motoneuron diseases (MNDs) characterized by aberrant protein behavior in affected cells. In familial ALS (fALS) and in SBMA specific gene mutations lead to the production of neurotoxic proteins or peptides prone to misfold, which then accumulate in form of aggregates. Notably, some of these proteins accumulate into aggregates also in sporadic ALS (sALS) even if not mutated. To prevent proteotoxic stresses detrimental to cells, misfolded and/or aggregated proteins must be rapidly removed by the protein quality control (PQC) system...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28640860/the-path-to-specialist-multidisciplinary-care-in-amyotrophic-lateral-sclerosis-a-population-based-study-of-consultations-interventions-and-costs
#18
Miriam Galvin, Padhraig Ryan, Sinead Maguire, Mark Heverin, Caoifa Madden, Alice Vajda, Charles Normand, Orla Hardiman
BACKGROUND: Amyotrophic Lateral Sclerosis (ALS) is a devastating neurological condition that requires coordinated, multidisciplinary clinical management. ALS is prone to misdiagnosis as its signs and symptoms may be non-specific, which may prolong patients' journey to multidisciplinary ALS care. METHODS: Using chart review and national register data, we have detailed the journey of a national cohort of ALS patients (n = 155) from the time of first symptom to presentation at a multidisciplinary clinic (MDC)...
2017: PloS One
https://www.readbyqxmd.com/read/28613097/dynamic-muscle-ultrasound-identifies-upper-motor-neuron-involvement-in-amyotrophic-lateral-sclerosis
#19
Yu-Ichi Noto, Neil Simon, Kazumoto Shibuya, José M Matamala, Thanuja Dharmadasa, Matthew C Kiernan
OBJECTIVE: The aim of the present study was to elucidate the pattern of change in bulbar muscles using ultrasound in patients diagnosed with amyotrophic lateral sclerosis (ALS). METHODS: Changes in the mylohyoid and geniohyoid muscle complex (mylohyoid-geniohyoid-muscle-complex) thickness were recorded while swallowing 5 ml of water using M-mode ultrasound in 30 ALS patients compared to 20 healthy controls. The ratio of mylohyoid-geniohyoid-muscle-complex thickness as determined by the maximum thickness of mylohyoid-geniohyoid-muscle-complex during swallowing divided by thickness at rest, was compared between ALS patients and controls, with the correlation between thickness ratio, echogenicity and clinical parameters assessed...
February 10, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28608303/assessment-of-the-factorial-validity-and-reliability-of-the-alsfrs-r-a-revision-of-its-measurement-model
#20
Leonhard A Bakker, Carin D Schröder, Michael A van Es, Paul Westers, Johanna M A Visser-Meily, Leonard H van den Berg
The amyotrophic lateral sclerosis functional rating scale-revised (ALSFRS-R) is a widely used primary outcome measure in amyotrophic lateral sclerosis (ALS) clinical practice and clinical trials. ALSFRS-R items cannot, however, validly be summed to obtain a total score, but constitute domain scores reflecting a profile of disease severity. Currently, there are different measurement models for estimating domain scores. The objective of the present study is, therefore, to derive the measurement model that best fits the data for a valid and uniform estimation of ALSFRS-R domain scores...
July 2017: Journal of Neurology
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