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ALS dysphagia

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https://www.readbyqxmd.com/read/29886477/distinct-clinical-features-and-outcomes-in-motor-neuron-disease-associated-with-behavioural-variant-frontotemporal-dementia
#1
Elena Cortés-Vicente, Janina Turon-Sans, Ellen Gelpi, Jordi Clarimón, Sergi Borrego-Écija, Oriol Dols-Icardo, Ignacio Illán-Gala, Alberto Lleó, Isabel Illa, Rafael Blesa, Ammar Al-Chalabi, Ricard Rojas-García
AIM: To determine the motor phenotype and outcome in a clinically ascertained group of patients with motor neuron disease (MND) and frontotemporal dementia (FTD). METHODS: This is an observational retrospective clinical study of patients fulfilling the clinical criteria for MND-FTD. A contemporary series of patients with amyotrophic lateral sclerosis (ALS) without dementia were included for comparison. Demographic, clinical, genetic, and neuropathological data were collected...
June 8, 2018: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/29599075/percutaneous-endoscopic-gastrostomy-in-patients-with-amyotrophic-lateral-sclerosis-mortality-and-complications
#2
J Carbó Perseguer, A Madejón Seiz, M Romero Portales, J Martínez Hernández, J S Mora Pardina, J García-Samaniego
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that causes severe dysphagia and weight loss. Percutaneous endoscopic gastrostomy (PEG) is currently the technique of choice for the enteral nutrition of these patients. OBJECTIVES: To analyse mortality and complications in a series of patients diagnosed with ALS who underwent PEG, and to evaluate factors related to patient survival after the procedure. MATERIAL AND METHODS: We performed a prospective, observational study including all patients diagnosed with ALS and treated by our hospital's Gastroenterology Department in the period 1997-2013...
March 26, 2018: Neurología: Publicación Oficial de la Sociedad Española de Neurología
https://www.readbyqxmd.com/read/29456900/a-barium-swallow-study-leading-to-an-incidental-finding-on-a-screening-colonoscopy
#3
Patricia Guzman Rojas, Chirin Orabi, Glenn Speth
Barolith is a mixture of firm feces with barium sulfate, and a frequent cause of obstruction of the appendiceal lumen that can result in appendicitis. Nonetheless, some other complications like aspiration, allergic reaction, and bowel obstruction have also been reported.  We present the case of a 71-year-old man with a history of amyotrophic lateral sclerosis (ALS), who came to the gastroenterology clinic complaining of intermittent loose stools and dysphagia to solids for the past months. The patient underwent a barium swallow study six days prior and was completely normal...
December 7, 2017: Curēus
https://www.readbyqxmd.com/read/29400682/swallowing-impairments-in-amyotrophic-lateral-sclerosis-and-myotonic-dystrophy-type-1-looking-for-the-portrait-of-dysphagic-patient-in-neuromuscular-diseases
#4
Elisa Andrenelli, Federica Lucia Galli, Rosaria Gesuita, Edlira Skrami, Francesco Ottavio Logullo, Leandro Provinciali, Marianna Capecci, Maria Gabriella Ceravolo, Michela Coccia
BACKGROUND: Dysphagia is a critical symptom of Neuromuscular Diseases and is often associated with considerable morbidity and mortality. OBJECTIVE: This study is designed to investigate the prevalence of dysphagia and to identify different clinical profiles of swallowing disorders in Myotonic Dystrophy type 1 (DM1) and Amyotrophic Lateral Sclerosis (ALS), the most common Neuromuscular Diseases in the adult age. METHODS: Consecutive DM1 and ALS patients from 2013 to 2015 referred to a Centre for Neuromuscular Disease were enrolled...
2018: NeuroRehabilitation
https://www.readbyqxmd.com/read/29379292/adherence-to-riluzole-in-patients-with-amyotrophic-lateral-sclerosis-an-observational-study
#5
Alessandro Introna, Eustachio D'Errico, Boris Modugno, Antonio Scarafino, Angela Fraddosio, Eugenio Distaso, Irene Tempesta, Antonella Mastronardi, Isabella Laura Simone
Objective: Riluzole is the first drug approved to treat amyotrophic lateral sclerosis (ALS). Recently, an oral suspension (OS) of riluzole was made available. Thus, the aim of our study was to evaluate the adherence to 2 formulations of riluzole in patients with ALS. Patients and methods: We enrolled 45 consecutive patients with ALS. At disease diagnosis, riluzole was prescribed in 2 different formulations depending on the severity of dysphagia (27/45 patients received tablets and 18/45 patients received OS)...
2018: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/29316850/demographics-and-clinical-characteristics-of-primary-lateral-sclerosis-case-series-and-a-review-of-literature
#6
Ramnath Santosh Ramanathan, Sandeep Rana
AIM: Primary lateral sclerosis (PLS) is a form of motor neuron disease involving only upper motor neurons. In some patients presenting as PLS, the disease progresses to involve lower motor neurons and thereby converting to amyotrophic lateral sclerosis (ALS). However, pure forms of PLS do exist. Our aim was to study epidemiological and clinical characteristics of pure PLS patients treated at our neuromuscular clinic. METHODS: We retrospectively reviewed 15 patients from July 2011 to October 2014 with PLS treated at the neuromuscular disorder clinic at our hospital...
February 2018: Neurodegenerative Disease Management
https://www.readbyqxmd.com/read/29241710/new-insights-into-the-gene-expression-associated-to-amyotrophic-lateral-sclerosis
#7
REVIEW
Daniela Recabarren-Leiva, Marcelo Alarcón
Amyotrophic lateral sclerosis (ALS) is the most prevalent neuromuscular disease worldwide. It is a lethal and progressive neurodegenerative disease, principally affecting motor neurons; patient clinical characteristics are muscle weakness, dysphagia and respiratory failure. The mean age is related to family history (40years, familial ALS or FALS) or with no family history (50years), but it is more common in people aged 60-69years. The cause of ALS is not known and it is not known yet why it affects some people and not others...
January 15, 2018: Life Sciences
https://www.readbyqxmd.com/read/29148035/late-age-onset-of-amyotrophic-lateral-sclerosis-is-often-not-considered-in-elderly-people
#8
E Broussalis, S Grinzinger, A B Kunz, M Killer-Oberpfalzer, E Haschke-Becher, H-P Hartung, J Kraus
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease causing an upper and lower motor neuron loss. It is neurology textbook knowledge that the mean age of onset is about 60 years. However, recent investigations show an increasing incidence in older persons. We therefore evaluated whether ALS is potentially not considered in elderly people with ALS symptoms, respectively, not recognized. MATERIALS AND METHODS: We included retrospectively all patients with ALS diagnoses after work-up that were admitted to our neurological and geriatric departments from 2007 to 2010 and collected their clinical data...
March 2018: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/29065369/the-fecal-microbiome-of-als-patients
#9
David Brenner, Andreas Hiergeist, Carolin Adis, Benjamin Mayer, André Gessner, Albert C Ludolph, Jochen H Weishaupt
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative motor neuron disease accompanied by both systemic and central nervous system-specific inflammation as well as deregulated energy metabolism. These potential pathogenetic factors have recently been found to mutually interact with the gut microbiota, raising the hypothesis of a link between microbiome alterations and ALS pathogenesis. The aim of our study was to assess whether ALS is associated with an altered composition of the fecal microbiota...
January 2018: Neurobiology of Aging
https://www.readbyqxmd.com/read/29054330/tongue-strength-in-patients-with-subacute-myelo-optico-neuropathy
#10
Satoshi Yamashita, Tatsuya Nakama, Mitsuharu Ueda, Shoji Honda, En Kimura, Masaaki Konagaya, Yukio Ando
Subacute myelo-optico-neuropathy (SMON) is a neurodegenerative disease that may be caused by overdose or prolonged oral administration of clioquinol. Recently, dysphagia has attracted attention as a complication of SMON. To investigate lingual control in SMON, we examined patients with SMON using assessments of maximum tongue pressure, compared with dysphagia-related diseases, such as sporadic inclusion body myositis (sIBM) and amyotrophic lateral sclerosis (ALS), and healthy volunteer. The mean maximum tongue pressure (Pmax ) in patients with SMON was 14...
January 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28872920/open-label-24-week-extension-study-of-edaravone-mci-186-in-amyotrophic-lateral-sclerosis
#11
(no author information available yet)
We aimed to explore the longer-term efficacy and safety of edaravone in an active-treatment extension period following the double-blind period of the second phase III study. Patients who met all the following criteria (scores ≥2 points on all 12 items of the revised amyotrophic lateral sclerosis functional rating scale [ALSFRS-R], forced vital capacity ≥80%, definite or probable ALS, and disease duration ≤2 years) were randomised to 60 mg intravenous edaravone or placebo for six cycles in the double-blind period, and then offered the opportunity to proceed to this 24-week open-label extension period...
October 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28807987/use-of-noninvasive-ventilation-during-feeding-tube-placement
#12
REVIEW
Paolo Banfi, Eleonora Volpato, Chiara Valota, Salvatore D'Ascenzo, Chiara Bani Alunno, Agata Lax, Antonello Nicolini, Nicola Ticozzi, Vincenzo Silani, John R Bach
Parenteral nutrition is indicated in amyotrophic lateral sclerosis (ALS) when dysphagia, loss of appetite, and difficulty protecting the airways cause malnutrition, severe weight loss, dehydration, and increased risk of aspiration pneumonia. The aim of this review is to compare percutaneous endoscopic gastrostomy (PEG), radiologically inserted G-tube (RIG), and percutaneous radiologic gastrostomy (PRG) in patients with ALS, performed with or without noninvasive ventilation (NIV). We searched PubMed, MEDLINE, EMBASE, the Cochrane Central Register of Controlled Trials (CENTRAL), the EBSCO Online Research Database, and Scopus up to December 2015...
August 14, 2017: Respiratory Care
https://www.readbyqxmd.com/read/28522181/safety-and-efficacy-of-edaravone-in-well-defined-patients-with-amyotrophic-lateral-sclerosis-a-randomised-double-blind-placebo-controlled-trial
#13
RANDOMIZED CONTROLLED TRIAL
(no author information available yet)
BACKGROUND: In a previous phase 3 study in patients with amyotrophic lateral sclerosis (ALS), edaravone did not show a significant difference in the Revised ALS Functional Rating Scale (ALSFRS-R) score compared with placebo. Post-hoc analysis of these data revealed that patients in an early stage with definite or probable diagnosis of ALS, defined by the revised El Escorial criteria, who met a select set of inclusion criteria showed a greater magnitude of effect than did the full study population...
July 2017: Lancet Neurology
https://www.readbyqxmd.com/read/28443187/treatment-with-penicillin-g-and-hydrocortisone-reduces-als-associated-symptoms-a-case-series-of-three-patients
#14
Bert Tuk, Harmen Jousma, Pieter J Gaillard
Three male Caucasian patients with ALS were admitted to the hospital due to progressive dysphagia and dysarthria. During two 21-day courses of penicillin G and hydrocortisone, these patients' dysphagia and dysarthria resolved. The patient's other ALS-associated symptoms also improved, including respiratory function, coordination, walking, and muscle strength. This is the first report of a treatment with a protocol for treating dysphagia, dysarthria, respiratory depression and other ALS-related symptoms. Furthermore, the observations are consistent with the recent hypothesis that the successful treatment of ALS symptoms with this treatment course in six patients with syphilitic ALS was not directly due to the treatment of syphilis; but that the administered penicillin G and/or hydrocortisone treated these patients' ALS symptoms due the off-target pharmacological activity of penicillin G and/or hydrocortisone...
2017: F1000Research
https://www.readbyqxmd.com/read/28377742/dysphagia-in-amyotrophic-lateral-sclerosis-impact-on-patient-behavior-diet-adaptation-and-riluzole-management
#15
Emanuela Onesti, Ilenia Schettino, Maria Cristina Gori, Vittorio Frasca, Marco Ceccanti, Chiara Cambieri, Giovanni Ruoppolo, Maurizio Inghilleri
This retrospective study aimed to investigate the clinical features associated with deteriorated swallow in amyotrophic lateral sclerosis (ALS) patients with spinal and bulbar onset, describe the modification of diet and liquid intake, and assess the impact of dysphagia on the use of riluzole. One hundred forty-five patients were observed periodically every 3-6 months. They underwent routinely fiberoptic endoscopic evaluation of swallowing (FEES) and spirometry; dysphagia severity was classified according to the Penetration Aspiration Scale and the Pooling score (P-score) integrated with other parameters such as sensation, collaboration, and age (P-SCA score)...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28346382/the-balloon-based-manometry-evaluation-of-swallowing-in-patients-with-amyotrophic-lateral-sclerosis
#16
Jerzy Tomik, Barbara Tomik, Sebastian Gajec, Piotr Ceranowicz, Małgorzata Pihut, Rafał Olszanecki, Paweł Stręk, Jacek Składzień
The aim of the study was to analyse the disturbances of the oro-pharyngeal swallowing phase of dysphagia in amyotrophic lateral sclerosis (ALS) patients with the use of specific manometric measurements and to evaluate their plausible association with the duration of the disease. Seventeen patients with ALS were evaluated with manometric examinations of the oral and pharyngeal part of the gastrointestinal tract. Tests were carried out by using the oesophageal balloon-based method with four balloon transducers located 5 cm away from each other...
March 27, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28319880/the-importance-of-the-reproducibility-of-oropharyngeal-swallowing-in-amyotrophic-lateral-sclerosis-an-electrophysiological-study
#17
G Cosentino, E Alfonsi, L Mainardi, E Alvisi, F Brighina, F Valentino, B Fierro, G Sandrini, G Bertino, M Berlangieri, R De Icco, M Fresia, A Moglia
OBJECTIVE: To investigate electrophysiologically the reproducibility of oropharyngeal swallowing in patients with ALS. METHODS: We enrolled 26 ALS patients, both with and without clinical signs of dysphagia, and 30 age-matched controls. The reproducibility of the electrophysiological signals related to the oral phase (electromyographic activity of the submental/suprahyoid muscles) and the pharyngeal phase (laryngeal-pharyngeal mechanogram) of swallowing across repeated swallows was assessed...
May 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28281365/seasons-and-als-time-of-death
#18
Susana Pinto, Mamede De Carvalho
INTRODUCTION: Respiratory complications are the main cause of death in amyotrophic lateral sclerosis (ALS). Season-associated-death risk was not addressed before. OBJECTIVE: To assess month/season-associated death risk in ALS. METHODS: We included all patients followed in our unit who died before 1 January 2016, excluding those with uncertain information. A χ2 test assessed differences between months/seasons. A two-step cluster analysis explored the significant survival independent factors...
May 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28076984/percutaneous-endoscopic-gastrostomy-body-weight-loss-and-survival-in-amyotrophic-lateral-sclerosis-a-population-based-registry-study
#19
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...
May 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28053507/riluzole-5-mg-ml-oral-suspension-for-optimized-drug-delivery-in-amyotrophic-lateral-sclerosis
#20
Ann Margaret Dyer, Alan Smith
The aim of the present work is to extensively evaluate the pharmaceutical attributes of currently available riluzole presentations. The article describes the limitations and risks associated with the administration of crushed tablets, including the potential for inaccurate dosing and reduced rate of absorption when riluzole is administered with high-fat foods, and the advantages that a recently approved innovative oral liquid form of riluzole confers on amyotrophic lateral sclerosis (ALS) patients. The article further evaluates the patented and innovative controlled flocculation technology used in the pseudoplastic suspension formulation to reduce the oral anesthesia seen with crushed tablets, resulting in optimized drug delivery for riluzole...
2017: Drug Design, Development and Therapy
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