keyword
MENU ▼
Read by QxMD icon Read
search

bulbar ALS

keyword
https://www.readbyqxmd.com/read/29779597/amyotrophic-lateral-sclerosis-and-the-respiratory-system
#1
REVIEW
Andrew T Braun, Candelaria Caballero-Eraso, Noah Lechtzin
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that always affects the respiratory muscles. It is characterized by degeneration of motor neurons in the brain and spinal cord. Respiratory complications are the most common causes of death in ALS and typically occur within 3 to 5 years of diagnosis. Because ALS affects both upper and lower motor neurons, it causes hyperreflexia, spasticity, muscle fasciculations, muscle atrophy, and weakness. It ultimately progresses to functional quadriplegia...
June 2018: Clinics in Chest Medicine
https://www.readbyqxmd.com/read/29774424/lingual-and-jaw-kinematic-abnormalities-precede-speech-and-swallowing-impairments-in-als
#2
Bridget J Perry, Rosemary Martino, Yana Yunusova, Emily K Plowman, Jordan R Green
Early identification of bulbar involvement in persons with ALS is critical for improving diagnosis and prognosis; however, efficacious diagnostic markers have not yet been identified. The purpose of this study was to determine whether kinematic changes of the tongue and jaw during swallowing, measured using 3D electromagnetic articulography (EMA), predate clinically identifiable symptoms of speech and swallowing impairment in persons diagnosed with ALS. Data were collected from 16 adults diagnosed with ALS and 18 neurotypical controls...
May 17, 2018: Dysphagia
https://www.readbyqxmd.com/read/29736957/functional-up-regulation-of-the-m-current-by-retigabine-contrasts-hyperexcitability-and-excitotoxicity-on-rat-hypoglossal-motoneurons
#3
Filippo Ghezzi, Laura Monni, Andrea Nistri
KEY POINT SUMMARY: Excessive neuronal excitability characterizes several neuropathological conditions including neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS). Hypoglossal motoneurons (HMs) that control tongue muscles, are extremely vulnerable to this disease and undergo damage and death when exposed to excessive glutamate extracellular concentration that causes excitotoxicity. Our laboratory devised an in vitro model of excitotoxicity obtained by pharmacological block of glutamate transporters...
May 7, 2018: Journal of Physiology
https://www.readbyqxmd.com/read/29699694/multi-disciplinary-clinical-protocol-for-the-diagnosis-of-bulbar-amyotrophic-lateral-sclerosis
#4
Rita Chiaramonte, Carmela Di Luciano, Ignazio Chiaramonte, Agostino Serra, Marco Bonfiglio
INTRODUCTION AND OBJECTIVES: The objective of this study was to examine the role of different specialists in the diagnosis of amyotrophic lateral sclerosis (ALS), to understand changes in verbal expression and phonation, respiratory dynamics and swallowing that occurred rapidly over a short period of time. MATERIALS AND METHODS: 22 patients with bulbar ALS were submitted for voice assessment, ENT evaluation, Multi-Dimensional Voice Program (MDVP), spectrogram, electroglottography, fiberoptic endoscopic evaluation of swallowing...
April 23, 2018: Acta Otorrinolaringológica Española
https://www.readbyqxmd.com/read/29687024/improved-stratification-of-als-clinical-trials-using-predicted-survival
#5
James D Berry, Albert A Taylor, Danielle Beaulieu, Lisa Meng, Amy Bian, Jinsy Andrews, Mike Keymer, David L Ennist, Bernard Ravina
Introduction: In small trials, randomization can fail, leading to differences in patient characteristics across treatment arms, a risk that can be reduced by stratifying using key confounders. In ALS trials, riluzole use (RU) and bulbar onset (BO) have been used for stratification. We hypothesized that randomization could be improved by using a multifactorial prognostic score of predicted survival as a single stratifier. Methods: We defined a randomization failure as a significant difference between treatment arms on a characteristic...
April 2018: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29679377/clinical-characteristics-of-long-term-survival-with-non-invasive-ventilation-and-factors-affecting-the-transition-to-invasive-ventilation-in-als
#6
Takahiko Hirose, Fumiharu Kimura, Hiroki Tani, Shin Ota, Akihiro Tsukahara, Eri Sano, Taro Shigekiyo, Yoshitsugu Nakamura, Kensuke Kakiuchi, Mikiko Motoki, Kiichi Unoda, Simon Ishida, Hideto Nakajima, Shigeki Arawaka
Introduction We evaluated post non-invasive ventilation survival and factors for the transition to tracheostomy in amyotrophic lateral sclerosis. Methods We analyzed 197 patients using a prospectively-collected database, with 114 patients since 2000. Results Of 114 patients, 59 patients underwent non-invasive ventilation (NIV), which prolonged the total median survival time to 43 months compared with 32 months without treatment. The best post-NIV survival was associated with a lack of bulbar symptoms, higher measured pulmonary function, and a slower rate of progression at diagnosis...
April 20, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29670892/bulbar-impairment-score-and-survival-of-stable-amyotrophic-lateral-sclerosis-patients-after-noninvasive-ventilation-initiation
#7
Jesús Sancho, Daniel Martínez, Enric Bures, José Luis Díaz, Alejandro Ponz, Emilio Servera
There is general agreement that noninvasive ventilation (NIV) prolongs survival in amyotrophic lateral sclerosis (ALS) and that the main cause of NIV failure is the severity of bulbar dysfunction. However, there is no evidence that bulbar impairment is a contraindication for NIV. The aim of this study was to determine the effect of bulbar impairment on survival in ALS patients with NIV. ALS patients for whom NIV was indicated were included. Those patients who refused NIV were taken as the control group. 120 patients who underwent NIV and 20 who refused NIV were included...
April 2018: ERJ Open Research
https://www.readbyqxmd.com/read/29666294/laryngeal-responses-to-mechanically-assisted-cough-in-progressing-amyotrophic-lateral-sclerosis
#8
Tiina M Andersen, Astrid Sandnes, Ove Fondenes, Roy M Nilsen, Ole-Bjørn Tysnes, John-Helge Heimdal, Hege H Clemm, Thomas Halvorsen, Maria Vollsœter, Ola D Røksund
BACKGROUND: Respiratory complications represent the major cause of death in amyotrophic lateral sclerosis (ALS). Noninvasive respiratory support is the mainstay therapy, but treatment becomes challenging as the disease progresses, possibly due to a malfunctioning larynx, which is the entrance to the airways. We studied laryngeal response patterns to mechanically assisted cough (mechanical insufflation-exsufflation) as ALS progresses. METHODS: This prospective longitudinal study of 13 consecutively included subjects with ALS were followed up during 2011-2016 with repeated tests of lung function, neurological status, and laryngeal responses to mechanical insufflation-exsufflation using video-recorded flexible transnasal fiberoptic laryngoscopy...
April 17, 2018: Respiratory Care
https://www.readbyqxmd.com/read/29659621/referral-bias-in-als-epidemiological-studies
#9
Giancarlo Logroscino, Benoit Marin, Marco Piccininni, Simona Arcuti, Adriano Chiò, Orla Hardiman, James Rooney, Stefano Zoccolella, Philippe Couratier, Pierre-Marie Preux, Ettore Beghi
BACKGROUND: Despite concerns about the representativeness of patients from ALS tertiary centers as compared to the ALS general population, the extent of referral bias in clinical studies remains largely unknown. Using data from EURALS consortium we aimed to assess nature, extent and impact of referral bias. METHODS: Four European ALS population-based registries located in Ireland, Piedmont, Puglia, Italy, and Limousin, France, covering 50 million person-years, participated...
2018: PloS One
https://www.readbyqxmd.com/read/29621978/high-frequency-of-the-tardbp-p-m337-v-mutation-among-south-eastern-chinese-patients-with-familial-amyotrophic-lateral-sclerosis
#10
Guo-Rong Xu, Wei Hu, Ling-Ling Zhan, Chong Wang, Liu-Qing Xu, Min-Ting Lin, Wan-Jin Chen, Ning Wang, Qi-Jie Zhang
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a devastating motor neuron disease characterized by substantial clinical and genetic heterogeneity. Thus far, only a few TARDBP-ALS families have been reported in China, and no mutation analysis has been reported in south-eastern China. METHODS: Seven index cases from ALS families negative for SOD1 and FUS mutations were screened by Sanger sequencing for TARDBP gene exons 2-6. TARDBP exon 6 was analysed in 215 sporadic ALS patients...
April 5, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29620511/gray-matter-volume-changes-over-the-whole-brain-in-the-bulbar-and-spinal-onset-amyotrophic-lateral-sclerosis-a-voxel-based-morphometry-study
#11
Zhi-Ye Chen, Meng-Qi Liu, Lin Ma
Objective To investigate cerebral structural signatures of the bulbar- and spinal-onset amyotrophic lateral sclerosis (ALS) using voxel-based morphometry on magnetic resonance imaging. Methods The MR structural images of the brain were obtained from 65 ALS patients (15 bulbar-onset, 50 spinal-onset) and 65 normal controls (NC) on a 3.0T MRI system. Gray matter (GM) volume changes were investigated by voxel-based morphometry, and the distribution of the brain regions with volume changes was compared between ALS and normal controls, as well as between bulbar-onset and spinal-onset ALS based on Neuromorphometrics atlas...
March 30, 2018: Chinese Medical Sciences Journal, Chung-kuo i Hsüeh K'o Hsüeh Tsa Chih
https://www.readbyqxmd.com/read/29608902/effectiveness-of-home-based-exercises-without-supervision-by-physical-therapists-for-patients-with-early-stage-amyotrophic-lateral-sclerosis-a-pilot-study
#12
Kosuke Kitano, Takashi Asakawa, Naoto Kamide, Keisuke Yorimoto, Masaki Yoneda, Yutaka Kikuchi, Makoto Sawada, Tetsuo Komori
OBJECTIVE: This study aimed to verify the effects of structured home-based exercises without supervision by a physical therapist on patients with early-stage amyotrophic lateral sclerosis (ALS). DESIGN: This is a historical controlled study that is part of a multicenter collaborative study. SETTING: Rehabilitation departments at general hospitals and outpatient clinics with a neurology department in Japan. PARTICIPANTS: Twenty-one patients with ALS were enrolled and designated as the Home-EX group, and they performed unsupervised home-based exercises...
March 30, 2018: Archives of Physical Medicine and Rehabilitation
https://www.readbyqxmd.com/read/29599075/percutaneous-endoscopic-gastrostomy-in-patients-with-amyotrophic-lateral-sclerosis-mortality-and-complications
#13
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/29598923/prognosis-for-patients-with-amyotrophic-lateral-sclerosis-development-and-validation-of-a-personalised-prediction-model
#14
Henk-Jan Westeneng, Thomas P A Debray, Anne E Visser, Ruben P A van Eijk, James P K Rooney, Andrea Calvo, Sarah Martin, Christopher J McDermott, Alexander G Thompson, Susana Pinto, Xenia Kobeleva, Angela Rosenbohm, Beatrice Stubendorff, Helma Sommer, Bas M Middelkoop, Annelot M Dekker, Joke J F A van Vugt, Wouter van Rheenen, Alice Vajda, Mark Heverin, Mbombe Kazoka, Hannah Hollinger, Marta Gromicho, Sonja Körner, Thomas M Ringer, Annekathrin Rödiger, Anne Gunkel, Christopher E Shaw, Annelien L Bredenoord, Michael A van Es, Philippe Corcia, Philippe Couratier, Markus Weber, Julian Grosskreutz, Albert C Ludolph, Susanne Petri, Mamede de Carvalho, Philip Van Damme, Kevin Talbot, Martin R Turner, Pamela J Shaw, Ammar Al-Chalabi, Adriano Chiò, Orla Hardiman, Karel G M Moons, Jan H Veldink, Leonard H van den Berg
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive, fatal motor neuron disease with a variable natural history. There are no accurate models that predict the disease course and outcomes, which complicates risk assessment and counselling for individual patients, stratification of patients for trials, and timing of interventions. We therefore aimed to develop and validate a model for predicting a composite survival endpoint for individual patients with ALS. METHODS: We obtained data for patients from 14 specialised ALS centres (each one designated as a cohort) in Belgium, France, the Netherlands, Germany, Ireland, Italy, Portugal, Switzerland, and the UK...
March 26, 2018: Lancet Neurology
https://www.readbyqxmd.com/read/29577526/the-assessment-of-bulbar-function-in-amyotrophic-lateral-sclerosis-validation-of-a-self-report-scale-cns-bfs
#15
Richard A Smith, Eric A Macklin, Kathleen J Myers, Gary L Pattee, Kimberly L Goslin, Gregg D Meekins, Jordan R Green, Jeremy M Shefner, Erik P Pioro
BACKGROUND: Impaired bulbar functions of speech and swallowing are among the most serious consequences of amyotrophic lateral sclerosis (ALS). Despite this, clinical trials in ALS have rarely emphasized bulbar function as an endpoint. When symptomatic benefit is measured, the rater-administered ALSFRS-R functional rating scale or various quality of life measures are commonly used. Accordingly, we sought to evaluate the utility of measures specific to bulbar function in ALS. METHODS: We assessed bulbar functions in 120 ALS patients, with clinicians first making direct observations of the degree of speech, swallowing, and salivation impairment in these subjects...
March 25, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29572281/biomarker-based-analysis-of-preclinical-progression-in-spinal-and-bulbar-muscular-atrophy
#16
Yasuhiro Hijikata, Atsushi Hashizume, Shinichiro Yamada, Tomonori Inagaki, Daisuke Ito, Akihiro Hirakawa, Keisuke Suzuki, Naoki Atsuta, Takashi Tsuboi, Makoto Hattori, Akihiro Hori, Haruhiko Banno, Gen Sobue, Masahisa Katsuno
OBJECTIVE: To identify a candidate biomarker reflecting biological changes during the preclinical progression of spinal and bulbar muscular atrophy (SBMA). METHODS: We analyzed longitudinal changes in biochemical parameters obtained during health examinations before and after the diagnosis of SBMA. We estimated trajectories of clinical markers across years from the onset of weakness using linear mixed models and compared these trajectories with those estimated for male healthy controls and patients with amyotrophic lateral sclerosis (ALS) and Parkinson disease (PD)...
March 23, 2018: Neurology
https://www.readbyqxmd.com/read/29566377/the-primitive-palmomental-reflex-in-amyotrophic-lateral-sclerosis
#17
Alfonsa Claudia Taiello, Rossella Spataro, Vincenzo La Bella
BACKGROUND AND PURPOSE: The palmomental reflex (PMR) is a primitive reflex that might be released due to inhibition in adulthood. It has been associated with several neurodegenerative conditions. The aim of the present study was to evaluate the frequency of PMR in amyotrophic lateral sclerosis (ALS). PATIENTS AND METHODS: Non-demented ALS patients (n = 179) were recruited. Two groups of disease controls were enrolled: (a) non-demented patients with other neurological disorders (NC; n = 86, mean age 60 ± 14 years); (b) healthy subjects, healthy controls (HC; n = 175, mean age 61 ± 12 years)...
March 22, 2018: European Neurology
https://www.readbyqxmd.com/read/29547678/characterization-of-patients-with-amyotrophic-lateral-sclerosis-attending-the-muscular-dystrophy-association-supported-clinics-in-puerto-rico
#18
Brenda Deliz, Kathya Ramos, Cynthia M Pérez
OBJECTIVE: To evaluate the sociodemographic characteristics and clinical and functional profile of amyotrophic lateral sclerosis (ALS) patients evaluated at Puerto Rico's Muscular Dystrophy Association-supported (MDA) clinics. METHODS: A retrospective review of 76 medical records of ALS patients evaluated at any of four MDA-sponsored clinics in Puerto Rico. RESULTS: The mean age of diagnosis was 57.4 ± 11.1 yrs. Most of the patients (52.3%) were women...
March 2018: Puerto Rico Health Sciences Journal
https://www.readbyqxmd.com/read/29492846/k-atp-channel-expression-and-genetic-polymorphisms-associated-with-progression-and-survival-in-amyotrophic-lateral-sclerosis
#19
José M Vidal-Taboada, Marco Pugliese, Maria Salvadó, Josep Gámez, Nicole Mahy, Manuel J Rodríguez
The ATP-sensitive potassium (KATP ) channel directly regulates the microglia-mediated inflammatory response following CNS injury. To determine the putative role of the KATP channel in amyotrophic lateral sclerosis (ALS) pathology, we investigated whether ALS induces changes in KATP channel expression in the spinal cord and motor cortex. We also characterized new functional variants of human ABCC8, ABCC9, KCNJ8, and KCNJ11 genes encoding for the KATP channel and analyzed their association with ALS risk, rate of progression, and survival in a Spanish ALS cohort...
February 28, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29491329/-an-early-history-of-japanese-amyotrophic-lateral-sclerosis-als-related-diseases-and-the-current-development
#20
Koji Abe
The present review focuses an early history of Japanese amyotrophic lateral sclerosis (ALS)-related diseases and the current development. In relation to foreign previous reports, five topics are introduced and discussed on ALS with dementia, ALS/Parkinsonism dementia complex (ALS/PDC), familial ALS (FALS), spinal bulbar muscular atrophy (SBMA), and multisystem involvement especially in cerebellar system of ALS including ALS/SCA (spinocerebellar ataxia) crossroad mutation Asidan. This review found the great contribution of Japanese reports on the above five topics, and confirmed the great development of ALS-related diseases over the past 120 years...
March 28, 2018: Rinshō Shinkeigaku, Clinical Neurology
keyword
keyword
88466
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"