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https://www.readbyqxmd.com/read/29322259/csf-neurofilament-proteins-as-diagnostic-and-prognostic-biomarkers-for-amyotrophic-lateral-sclerosis
#1
Daniela Rossi, Paolo Volanti, Liliana Brambilla, Tiziana Colletti, Rossella Spataro, Vincenzo La Bella
Elevated cerebrospinal fluid (CSF), Neurofilament Light (NF-L) and phosphorylated Heavy (pNF-H) chain levels have been found in Amyotrophic Lateral Sclerosis (ALS), with studies reporting a correlation of both neurofilaments (NFs) with the disease progression. Here, we measured NF-L and pNF-H concentrations in the CSF of ALS patients from a single tertiary Center and investigated their relationship with disease-related variables. A total of 190 ALS patients (Bulbar, 29.9%; Spinal, 70.1%; M/F = 1.53) and 130 controls with mixed neurological diseases were recruited...
January 10, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29316850/demographics-and-clinical-characteristics-of-primary-lateral-sclerosis-case-series-and-a-review-of-literature
#2
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...
January 10, 2018: Neurodegenerative Disease Management
https://www.readbyqxmd.com/read/29298901/five-year-follow-up-and-outcomes-of-noninvasive-ventilation-in-subjects-with-neuromuscular-diseases
#3
Mi Ri Suh, Won Ah Choi, Dong Hyun Kim, Jang Woo Lee, Eun Young Kim, Seong-Woong Kang
INTRODUCTION: The purpose of this study was to investigate the 5-year outcomes of noninvasive ventilation (NIV) application in different neuromuscular disease (NMD) groups. METHODS: We categorized 180 subjects who had initiated NIV between March 2001 and August 2009 into 4 groups and followed them for > 5 y. The NIV maintenance rate and average duration, applying time, and forced vital capacity (FVC) were investigated at the time NIV was initiated and 5 y after NIV initiation in each group...
January 3, 2018: Respiratory Care
https://www.readbyqxmd.com/read/29260911/a-retrospective-investigation-of-the-relationship-between-baseline-covariates-and-rate-of-alsfrs-r-decline-in-als-clinical-trials
#4
Iyas Daghlas, Teresa E Lever, Emily Leary
The revised ALS functional rating scale (ALSFRS-R) is a longitudinal measure of global function commonly used to assess progression of amyotrophic lateral sclerosis (ALS), and as an endpoint in ALS clinical trials. Understanding how baseline covariates affect the rate of functional decline in ALS offers valuable information to clinical trialists. We used a mixed modeling approach in a retrospective study of the pooled resource open-Access ALS clinical trials database to elucidate the associations between baseline covariates and the rate of ALSFRS-R decline over time...
December 20, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29260601/an-association-study-between-scfd1-rs10139154-variant-and-amyotrophic-lateral-sclerosis-in-a-chinese-cohort
#5
Yongping Chen, Qingqing Zhou, Xiaojing Gu, Qianqian Wei, Bei Cao, Hui Liu, Yanbing Hou, Huifang Shang
BACKGROUND: A recent genome-wide association study (GWAS) demonstrated that the Sec1 family domain containing 1 (SCFD1) gene is associated with amyotrophic lateral sclerosis (ALS). The objective of our study was to investigate the association between the single nucleotide polymorphism (SNP) rs10139154 in the SCFD1 gene and ALS in a Chinese cohort. METHODS: A cohort of 1074 sporadic ALS (SALS) patients from the Department of Neurology at the West China Hospital of Sichuan University were genotyped for rs10139154 using a polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) analysis...
December 20, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29244892/alterations-in-the-masticatory-system-in-patients-with-amyotrophic-lateral-sclerosis
#6
Nina Riera-Punet, Jordi Martinez-Gomis, Andrés Paipa, Monica Povedano, Maria Peraire
AIMS: To determine the effect of amyotrophic lateral sclerosis (ALS) on aspects of masticatory function and to assess the relationship between ALS and the prevalence of traumatic mucosal lesions caused by oral self-injury. METHODS: A total of 153 ALS patients and 23 control subjects participated in this cross-sectional study. Clinical characteristics including site of onset, medication, type of feeding, and use of noninvasive mechanical ventilation were recorded...
December 15, 2017: Journal of Oral & Facial Pain and Headache
https://www.readbyqxmd.com/read/29240971/functional-limitation-of-the-masticatory-system-in-patients-with-bulbar-involvement-in-amyotrophic-lateral-sclerosis
#7
Nina Riera-Punet, Jordi Martinez-Gomis, Eva Willaert, Monica Povedano, Maria Peraire
Amyotrophic lateral sclerosis (ALS) with bulbar dysfunction affects the motor neurons responsible for controlling the muscles in the jaw, face, soft palate, pharynx, larynx, and tongue. This cross-sectional study aimed to determine the functional limitation of the jaw in patients with ALS and bulbar dysfunction who had upper motor neurons (UMN), lower motor neurons (LMN) or balanced involvement. One hundred and fifty three patients with ALS and 23 controls were included. All participants answered using the eight-item Jaw Functional Limitation Scale (JFLS-8)...
December 14, 2017: Journal of Oral Rehabilitation
https://www.readbyqxmd.com/read/29214883/longitudinal-structural-changes-in-als-a-three-time-point-imaging-study-of-white-and-gray-matter-degeneration
#8
Peter Bede, Orla Hardiman
BACKGROUND: Cross-sectional imaging studies offer valuable pathological insights into the neurodegenerative changes of amyotrophic lateral sclerosis. However, clinical trials urgently require sensitive monitoring markers that can detect subtle progressive changes over relatively short periods of time. We have conducted a three time-point longitudinal study to explore anatomical patterns of disease spread and to determine whether MRI metrics capture longitudinal changes over four and eight-month intervals...
December 7, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29205054/best-practices-protocol-for-the-evaluation-of-bulbar-dysfunction-summary-recommendations-from-the-neals-bulbar-subcommittee-symposium
#9
Gary L Pattee, Emily K Plowman, Benjamin R Brooks, James D Berry, Nazem Atassi, Jennifer L Chapin, Kendrea Garand, Yana Yunusova, Courtney E Mcilduff, Eufrosina Young, John M Costello, Eric A Macklin, Eduardo R Locatelli, Vincenzo Silani, Daragh Heitzman, James Wymer, Stephen A Goutman, Deborah F Gelinas, Richard Smith, Bridget Perry, Paige Nalipinski, Kaila Stipancic, Meghan O'Brien, Stacey L Sullivan, Jordan Green
OBJECTIVE: The aim of this Symposium was to develop a consensus based, bulbar assessment protocol for implementation within NEALS clinics. METHODS: A one-day symposium, held in April 2017, was organized into Speech and Swallowing sections to establish summary recommendations for the assessment of bulbar dysfunction within each group. RESULTS: Summary recommendations included speech referrals and AAC evaluations at initial visit, CNS-BFS, maximum sustained phonation, and speaking rate...
December 5, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29189555/what-is-in-the-literature
#10
Mark B Bromberg
This edition of "What is in the Literature?" will focus on motor neuron disease (MND), including adult forms [amyotrophic lateral sclerosis (ALS), progressive muscular atrophy (PMA), primary lateral sclerosis (PLS), progressive bulbar palsy (PBA), and monomelic mononeuropathy (MMND)], and childhood forms [spinal muscle atrophy (SMA)].
December 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/29181534/association-between-decline-in-slow-vital-capacity-and-respiratory-insufficiency-use-of-assisted-ventilation-tracheostomy-or-death-in-patients-with-amyotrophic-lateral-sclerosis
#11
Jinsy A Andrews, Lisa Meng, Sarah F Kulke, Stacy A Rudnicki, Andrew A Wolff, Michael E Bozik, Fady I Malik, Jeremy M Shefner
Importance: The prognostic value of slow vital capacity (SVC) in relation to respiratory function decline and disease progression in patients with amyotrophic lateral sclerosis (ALS) is not well understood. Objective: To investigate the rate of decline in percentage predicted SVC and its association with respiratory-related clinical events and mortality in patients with ALS. Design, Setting, and Participants: This retrospective study included 893 placebo-treated patients from 2 large clinical trials (EMPOWER and BENEFIT-ALS, conducted from March 28, 2011, to November 1, 2012, and from October 23, 2012, to March 21, 2014, respectively) and an ALS trial database (PRO-ACT, containing studies completed between 1990 and 2010) to investigate the rate of decline in SVC...
November 27, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/29174922/detecting-spinal-pyramidal-tract-of-amyotrophic-lateral-sclerosis-patients-with-diffusion-tensor-tractography
#12
Yusuke Fukui, Nozomi Hishikawa, Kota Sato, Yumiko Nakano, Ryuta Morihara, Jingwei Shang, Mami Takemoto, Yasuyuki Ohta, Toru Yamashita, Koji Abe
The objective of this study was to determine alteration of corticospinal tract in patients with amyotrophic lateral sclerosis (ALS) using diffusion tensor tractograhy (DTT) focusing on the cervical spinal cord (C5) and transcranial magnetic stimulation (TMS). We recruited 38 ALS, 6 spinal and bulbar muscular atrophy (SBMA), 7 spastic paraplegia (SP) patients, and 8 age-matched normal controls, and then ALS were divided into two subgroups according to their clinical type: 28 ALS-limb and 10 ALS-bulbar. DTT was performed using the diffusion tensor image (DTI) track module to reconstruct two fiber tracts via C5...
November 21, 2017: Neuroscience Research
https://www.readbyqxmd.com/read/29159848/speech-deterioration-in-amyotrophic-lateral-sclerosis-als-after-manifestation-of-bulbar-symptoms
#13
Tanja Makkonen, Hanna Ruottinen, Riitta Puhto, Mika Helminen, Johanna Palmio
BACKGROUND: The symptoms and their progression in amyotrophic lateral sclerosis (ALS) are typically studied after the diagnosis has been confirmed. However, many people with ALS already have severe dysarthria and loss of adequate speech at the time of diagnosis. Speech-and-language therapy interventions should be targeted timely based on communicative need in ALS. AIMS: To investigate how long natural speech will remain functional and to identify the changes in the speech of persons with ALS...
November 21, 2017: International Journal of Language & Communication Disorders
https://www.readbyqxmd.com/read/29134445/ptosis-and-bulbar-onset-an-unusual-phenotype-of-familial-als
#14
Fabiola De Marchi, L Corrado, E Bersano, M F Sarnelli, V Solara, S D'Alfonso, R Cantello, L Mazzini
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder of upper and lower motor neurons that usually spare the oculomotor nerves. Here, we describe a case of two siblings with a familial bulbar-onset ALS both with ptosis manifested at the onset of the disease.
November 13, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/29126546/changes-in-lingual-alveolar-contact-pressure-during-speech-over-six-months-in-amyotrophic-lateral-sclerosis
#15
Jeff Searl, Stephanie Knollhoff
PURPOSE: This study compared lingual-alveolar contact pressures during consonant (LACP-Sp) production between adults with and without amyotrophic lateral sclerosis (ALS) over three visits within six months and assessed the relationship between LACP-Sp and speech intelligibility. METHODS: Ten people with ALS and nine without read sentences with lingual-alveolar consonants embedded as LACP-Sp was recorded. Recordings were done at study entry, then three and six months after entry...
November 2017: Journal of Communication Disorders
https://www.readbyqxmd.com/read/29113448/can-oxidation-reduction-potential-of-cerebrospinal-fluid-be-a-monitoring-biomarker-in-amyotrophic-lateral-sclerosis
#16
Milos Opacic, Zorica Stevic, Vladimir Bascarevic, Miroslav Zivic, Mihajlo Spasic, Ivan Spasojevic
The monitoring of progression in amyotrophic lateral sclerosis (ALS) relies on clinical outcome measures that take months to interpret, such as revised ALS functional rating scale (ALSFRS-R) score, with no approved biomarkers. A number of clinical studies have documented the involvement of oxidative stress in ALS pathology. Pertinent to this, we propose to evaluate oxidation-reduction potential (ORP) of cerebrospinal fluid (CSF) as a potential indicator of ALS progression. The case/control study included 24 patients with neurological non-neurodegenerative disorders (controls), and 82 ALS patients with different degrees of disease (ALSFRS-R score: 21-47)...
November 7, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/29084402/health-status-perspectives-in-amyotrophic-lateral-sclerosis
#17
Susana Pinto, Mamede de Carvalho
BACKGROUND/AIMS: The global perception of the health status (HS) of amyotrophic lateral sclerosis (ALS) patients before the initial diagnosis has not been addressed previously. METHODS: We recorded the following at the first visit, before diagnostic information: (1) visual analog scale (VAS) of the EQ-5D; (2) the revised ALS functional rating scale (ALSFRS- R), bulbar (ALSFRSb), upper limb (ALSFRSul), lower limb (ALSFRSll), and respiratory (RofALSFRS-R) subscores; and (3) forced and slow vital capacities...
October 31, 2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/29082510/brain-signal-intensity-changes-as-biomarkers-in-amyotrophic-lateral-sclerosis
#18
Juan F Vázquez-Costa, Miguel Mazón, Joan Carreres-Polo, David Hervás, Jordi Pérez-Tur, Luis Martí-Bonmatí, Teresa Sevilla
OBJECTIVES: To evaluate the contribution of the demographical, clinical, analytical and genetic factors to brain signal intensity changes in T2-weighted MR images in amyotrophic lateral sclerosis (ALS) patients and controls. METHODS: Susceptibility-weighted and FLAIR sequences were obtained in a 3T MR scanner. Iron-related hypointensities in the motor cortex (IRhMC) and hyperintensities of the corticospinal tract (HCT) were qualitatively scored. Age, gender, family history and clinical variables were recorded...
October 29, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/29073458/resting-state-fmri-correlates-of-theory-of-mind-impairment-in-amyotrophic-lateral-sclerosis
#19
Francesca Trojsi, Federica Di Nardo, Gabriella Santangelo, Mattia Siciliano, Cinzia Femiano, Carla Passaniti, Giuseppina Caiazzo, Michele Fratello, Mario Cirillo, Maria Rosaria Monsurrò, Fabrizio Esposito, Gioacchino Tedeschi
Theory of Mind (ToM), the ability to recognize thoughts and emotions of another, may be one of the cognitive domains affected in amyotrophic lateral sclerosis (ALS), a neurodegenerative disease now recognized as a multi-system disorder. The present study aimed to identify early dysfunctions of brain resting state functional magnetic resonance imaging (RS-fMRI) networks in a group of ALS patients longitudinally explored for impairment of "cognitive" and "affective" ToM subcomponents. RS-fMRI connectivity was investigated in a group of 21 patients with ALS (i...
October 3, 2017: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/29067136/amyotrophic-lateral-sclerosis-an-acupuncture-approach
#20
Poovadan Sudhakaran
Background: Amyotrophic lateral sclerosis (ALS) is an adult onset neurodegenerative disorder that has no curative treatment and is usually fatal. Modern medicine treatment is mostly supportive. Acupuncture has much more to offer by way of symptomatic relief and improving quality of life (QoL). Useful points for the treatment of bulbar paralysis, paralysis of upper and lower extremities, and correction of underlying Disharmony Patterns are discussed and an illustrative case is presented to demonstrate the acupuncture approach for treating ALS...
October 1, 2017: Medical Acupuncture
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