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ALS dysphagia

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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
#1
(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...
May 15, 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
#2
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
#3
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
#4
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
#5
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
#6
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 χ(2) test assessed differences between months/seasons. A two-step cluster analysis explored the significant survival independent factors...
March 10, 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
#7
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/28053507/riluzole-5-mg-ml-oral-suspension-for-optimized-drug-delivery-in-amyotrophic-lateral-sclerosis
#8
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
https://www.readbyqxmd.com/read/28029397/matrin-3-variants-are-frequent-in-italian-als-patients
#9
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/28009454/direct-estimation-for-adaptive-treatment-length-policies-methods-and-application-to-evaluating-the-effect-of-delayed-peg-insertion
#10
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/27965622/laryngeal-sensitivity-in-patients-with-amyotrophic-lateral-sclerosis
#11
Giovanni Ruoppolo, Emanuela Onesti, Maria Cristina Gori, Ilenia Schettino, Vittorio Frasca, Antonella Biasiotta, Carla Giordano, Marco Ceccanti, Chiara Cambieri, Antonio Greco, Costantino Eugenio Buonopane, Giorgio Cruccu, Marco De Vincentiis, Maurizio Inghilleri
Recent studies have shown the involvement of the sensory nervous system in patients with amyotrophic lateral sclerosis (ALS). The aim of our study was to investigate the correlation between the laryngeal sensitivity deficit and the type of ALS onset (bulbar or spinal) in a large series of 114 consecutive ALS patients. Participants were subdivided into two groups, bulbar and spinal ALS, according to the clinical onset of disease and submitted to a clinical and instrumental evaluation of swallowing, including a fiber-optic endoscopic evaluation of swallowing with sensory testing...
2016: Frontiers in Neurology
https://www.readbyqxmd.com/read/27876446/gastrostomy-tube-placement-is-safe-in-advanced-amyotrophic-lateral-sclerosis
#12
Manisha Kak, Naoum P Issa, Raymond P Roos, Bobbie Jean Sweitzer, Ori Gottlieb, Amy Guralnick, Steven R White, Carol E Semrad, Betty Soliven, Joumana Baroody, Kourosh Rezania
OBJECTIVES: To evaluate the safety and effect on survival of insertion of a gastrostomy tube (G-tube) in patients with amyotrophic lateral sclerosis (ALS) who have upright forced vital capacity (uFVC) ≤ 50% predicted. Current guidelines, which are based on higher rates of post-procedure complications in ALS patients with advanced respiratory dysfunction, have led to a recommendation to perform G-tube insertion before the FVC drops to <50% predicted, even when the patient has no significant dysphagia...
January 2017: Neurological Research
https://www.readbyqxmd.com/read/27759965/enteral-feeding-through-endoscopic-gastrostomy-in-amyotrophic-lateral-sclerosis-patients
#13
Gonçalo Nunes, Carla Adriana Santos, Miguel Grunho, Jorge Fonseca
BACKGROUND: Dysphagia is common in amyotrophic lateral sclerosis (ALS) and may result in malnutrition. Endoscopic gastrostomy (PEG) is recommended when oral feeding is unsafe. This work aims to assess the effectiveness and safety of PEG feeding on improving nutritional and prognostic parameters in ALS patients. METHODS: Observational and retrospective study using records from ALS patients referred for gastrostomy. Age, gender and mortality data were collected. NRS 2002, body mass index (BMI), serum albumin, transferrin and total cholesterol were recorded at the time of PEG (T0) and repeated after 3 months (T3)...
September 20, 2016: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/27569290/dysphagia-in-amyotrophic-lateral-sclerosis-relationships-between-disease-progression-and-fiberoptic-endoscopic-evaluation-of-swallowing
#14
Bruno Fattori, Gabriele Siciliano, Valentina Mancini, Luca Bastiani, Paolo Bongioanni, Elena Caldarazzo Ienco, Maria R Barillari, Salvatore O Romeo, Andrea Nacci
OBJECTIVE: Our aim was to evaluate the relationship between the disease severity of Amyotrophic Lateral Sclerosis (ALS) and the following parameters of Fiberoptic Endoscopic Evaluation of Swallowing (FEES): premature spillage, post-swallowing residue and aspiration. METHODS: We studied 202 patients (95 women and 107 men) with ALS; of these, 136 had spinal and 66 had bulbar onset. They were analyzed according to the Amyotrophic Lateral Sclerosis Functioning Rating Scale (ALSFRS) and the b-ALSFRS subscale (bulbar scale)...
August 25, 2016: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/27534658/correlating-factors-in-the-recommendation-of-feeding-tubes-in-the-nutritional-management-of-amyotrophic-lateral-sclerosis
#15
Caitlin S Jackson-Tarlton, Timothy J Benstead, Steve Doucette
Amyotrophic lateral sclerosis (ALS) is associated with nutritional deficits. Gastrostomy tubes are often inserted in patients with ALS to supplement or replace oral intake. The aim of this study was to better understand the practices of gastrostomy tube insertion in patients with ALS. Pre-collected de-identified data were obtained from the Canadian Neuromuscular Disease Registry (CNDR). Feeding tube status was compared with markers of dysphagia, respiratory compromise, and weight status in both univariate and multivariate analysis by employing odds ratios...
October 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27514792/hypoparathyroidism-a-rare-mimic-of-amyotrophic-lateral-sclerosis
#16
Haris Hakeem, Masood Uz Zaman, Sara Khan
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) carries a grim prognosis. Various ALS mimics have been reported and should be excluded before confirming this diagnosis. METHODS: We report the case of a 61-year-old man who presented with progressively worsening limb weakness and dysphagia. His examination showed mixed upper and lower motor neuron signs without sensory impairment. ALS was suspected, however, atypical diffuse pain prompted diagnostic work-up to exclude other causes...
March 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/27454127/complications-with-fiberoptic-endoscopic-evaluation-of-swallowing-in-2-820-examinations
#17
Andrea Nacci, Jacopo Matteucci, Salvatore Osvaldo Romeo, Stefania Santopadre, Maria Denise Cavaliere, Maria Rosaria Barillari, Stefano Berrettini, Bruno Fattori
OBJECTIVE: To perform a retrospective evaluation of the incidence of complications and adverse events during fiberoptic endoscopic evaluation of swallowing (FEES) in 2,820 examinations. PATIENTS AND METHODS: Subjects included in- and outpatients at Pisa University Hospital referred for FEES by other physicians due to dysphagia symptoms. Neurologic diseases were the most commonly diagnosed conditions in the patients tested (48.3%). Informed consent was obtained from all subjects...
2016: Folia Phoniatrica et Logopaedica
https://www.readbyqxmd.com/read/27372362/swallowing-characteristics-in-amyotrophic-lateral-sclerosis
#18
Mansi Pankaj Jani, Geeta Bharat Gore
BACKGROUND: Motor neurone disease also commonly known as Amyotrophic Lateral Sclerosis (ALS) is a neurological condition which affects various motor functions of the body. Dysphagia (disordered swallowing) is commonly seen in patients with ALS having bulbar symptoms. OBJECTIVES: Research reveals presence of dysphagia in patients with ALS at various stages of swallowing using instrumental assessment. However, very few studies have been done focussing on clinical profiling of swallowing in these patients...
June 27, 2016: NeuroRehabilitation
https://www.readbyqxmd.com/read/27188850/amyotrophic-lateral-sclerosis-in-northern-spain-40-years-later-what-has-changed
#19
Javier Riancho, Pablo Lozano-Cuesta, Ana Santurtún, Pascual Sánchez-Juan, José Manuel López-Vega, José Berciano, José Miguel Polo
BACKGROUND: In the last years different studies have reported an increase of amyotrophic lateral sclerosis (ALS) incidence, highlighting the role of the environment in this disease. This prompted us to review ALS cases diagnosed at our hospital in the last decade and to compare them with a previous ALS series reported in our region 30 years ago. METHODS: We reviewed those ALS cases diagnosed at our centre between 2004 and 2013. Subsequently, we compared them with the previous series regarding clinical and epidemiological features...
2016: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/27151083/the-interaction-between-breathing-and-swallowing-in-amyotrophic-lateral-sclerosis
#20
Nazan Simsek Erdem, Kamil Karaali, Ali Ünal, Ferah Kızılay, Candan Öğüş, Hilmi Uysal
The aim of the study is to determine the association between respiratory swallow patterns in amyotrophic lateral sclerosis (ALS) patients. Furthermore, it aims to clarify the role of the dysphagia limit in defining the relationship between swallowing disorders and respiratory disorders. Functional rating scales were used to describe swallowing and respiratory function. Swallowing was observed using the dysphagia limit. Dysphagia limit is the volume at which a second or more swallows are required to swallow the whole bolus...
December 2016: Acta Neurologica Belgica
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