keyword
https://read.qxmd.com/read/38642323/rare-association-between-spinocerebellar-ataxia-and-amyotrophic-lateral-sclerosis-a-case-series
#1
JOURNAL ARTICLE
Valerio Ferrari, Matteo Conti, Roberta Bovenzi, Rocco Cerroni, Mariangela Pierantozzi, Nicola B Mercuri, Alessandro Stefani
INTRODUCTION: In this work, we describe a new case of association between SCA2 and MND. CASE REPORT: A 58-year-old man who was diagnosed with spinocerebellar ataxia type 2 presented dysphagia and a significant decline in his ability to walk, with a reduction in autonomy and the need to use a wheelchair. We performed electromyography and electroneurography of the four limbs and of the cranial district and motor-evoked potentials to study upper and lower motor neurons...
April 20, 2024: Neurological Sciences
https://read.qxmd.com/read/38613054/use-of-muscle-ultrasonography-in-morphofunctional-assessment-of-amyotrophic-lateral-sclerosis-als
#2
JOURNAL ARTICLE
Juan J López-Gómez, Olatz Izaola-Jauregui, Laura Almansa-Ruiz, Rebeca Jiménez-Sahagún, David Primo-Martín, María I Pedraza-Hueso, Beatriz Ramos-Bachiller, Jaime González-Gutiérrez, Daniel De Luis-Román
UNLABELLED: Amyotrophic lateral sclerosis (ALS) is a progressive disease with a high prevalence of malnutrition that can influence prognosis. The main objective of this study is to compare the validity of muscle ultrasonography in the diagnosis of malnutrition and the prognosis of patients with ALS. METHODS: This is a prospective observational study that analyzes the nutritional status of patients at the beginning of nutritional monitoring. The morphofunctional assessment included the examination of anthropometric variables such as weight, height, body mass index (BMI), arm circumference, and calf circumference...
March 31, 2024: Nutrients
https://read.qxmd.com/read/38596011/serum-cytokines-profile-changes-in-amyotrophic-lateral-sclerosis
#3
JOURNAL ARTICLE
Chun-Zuan Xu, Xiao Huan, Su-Shan Luo, Hua-Hua Zhong, Chong-Bo Zhao, Yan Chen, Zhang-Yu Zou, Sheng Chen
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder, characterized by progressive limb weakness, dysphagia, dysphonia, and respiratory failure due to degeneration of upper and lower motor neurons. The pathogenesis of ALS is still unclear. Neuroinflammation has been found to be involved in its development and progression. Cytokines play a significant role in the inflammatory process. This study aims to identify novel biomarkers that may assist in the diagnosis of ALS...
April 15, 2024: Heliyon
https://read.qxmd.com/read/38575486/neurogenic-dysphagia
#4
REVIEW
Swapna K Chandran, Manon Doucet
This article provides an overview of neurogenic dysphagia, describing the evaluation and management of swallowing dysfunction in various neurologic diseases. The article will focus on stroke, Parkinson's disease, amyotrophic lateral sclerosis, and multiple sclerosis.
April 3, 2024: Otolaryngologic Clinics of North America
https://read.qxmd.com/read/38531942/timing-and-impact-of-percutaneous-endoscopic-gastrostomy-insertion-in-patients-with-amyotrophic-lateral-sclerosis-a-comprehensive-analysis
#5
JOURNAL ARTICLE
Bugyeong Son, Jisu Lee, Soorack Ryu, Yongsoon Park, Seung Hyun Kim
Dysphagia is common in amyotrophic lateral sclerosis (ALS) patients, often requiring percutaneous endoscopic gastrostomy (PEG) for enteral nutrition. We retrospectively analyzed data from 188 Korean patients with ALS who underwent PEG tube insertion at five-time points: symptom onset (t1 ), diagnosis (t2 ), recommended time for gastrostomy (t3 ), PEG insertion (t4 ), and one-year post-insertion (t5 ). The recommended time point for gastrostomy (T-rec for gastrostomy) was defined as the earlier time point between a weight loss of more than 10% and advanced dysphagia indicated by the ALSFRS-R swallowing subscore of 2 or less...
March 26, 2024: Scientific Reports
https://read.qxmd.com/read/38517530/dysphagia-assessments-as-criteria-in-the-decision-making-process-for-percutaneous-endoscopic-gastrostomy-placement-in-people-with-amyotrophic-lateral-sclerosis-a-systematic-review
#6
REVIEW
Ermioni Kotsia, Elizabeth Chroni, Anna Alexandropoulou, Claire Mills, Dimitra Veltsista, Zinovia Maria Kefalopoulou, Emilia Michou
To review the assessment methods of dysphagia as a criterion for the decision-making process for Percutaneous Endoscopic Gastrostomy (PEG) placement in patients with Amyotrophic Lateral Sclerosis (ALS). Systematic review. A search was conducted in three databases (EMBASE, CINAHL, PUBMED) in December 2022 and updated in July 2023. Two reviewers independently screened, selected, and extracted data. Study quality was appraised using the Joanna Briggs Institute Critical Appraisal Tools. Systematic review registration number in the International Prospective Register of Systematic Reviews (PROSPERO): CRD42022385461...
March 22, 2024: Dysphagia
https://read.qxmd.com/read/38436356/phenotypic-spectrum-of-inclusion-body-myositis
#7
REVIEW
Bhaskar Roy, Mazen M Dimachkie, Elie Naddaf
Inclusion body myositis (IBM) is a progressive, debilitating muscle disease commonly encountered in patients over the age of 50. IBM typically presents with asymmetric, painless, progressive weakness and atrophy of deep finger flexors and/or quadriceps muscle. Many patients with IBM develop dysphagia. However, atypical presentations of IBM with isolated dysphagia, asymptomatic hyper-CKemia, foot drop, proximal weakness, axial weakness, and facial diplegia have been reported. Other acquired and some inherited disorders may present similar to IBM, and this list gets more expansive when considering atypical presentations...
February 28, 2024: Clinical and Experimental Rheumatology
https://read.qxmd.com/read/38337170/supporting-people-with-motor-neuron-disease-mnd-to-make-decisions-about-gastrostomy-feeding-tube-placement-a-survey-of-uk-healthcare-professionals-practice-and-beliefs
#8
JOURNAL ARTICLE
Sean White, Alicia O'Cathain, Vanessa Halliday, Michael Bradburn, Christopher J McDermott
OBJECTIVE: Understand the practice and beliefs of healthcare professionals (HCPs) supporting the decision-making of people with MND (pwMND) about gastrostomy placement, including identifying differences between professions. METHODS: An online cross-sectional survey disseminated to HCPs who support the decision-making of pwMND about gastrostomy placement. RESULTS: A total of 139 participants completed the survey including representation from a range of healthcare professions...
February 9, 2024: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://read.qxmd.com/read/38332698/dynamic-suprahyoid-muscle-ultrasound-in-assessing-oropharyngeal-dysphagia-in-neurological-disorders
#9
JOURNAL ARTICLE
Joo Hye Sung, Seol-Hee Baek, Jin-Woo Park, Jung Hun Lee, Myeong Hun Son, Byung-Jo Kim
BACKGROUND: Appropriate evaluation and management of dysphagia are essential in neurological disorders. However, there is currently a lack of a simple yet reliable method for dysphagia evaluation. AIM: This study aimed to investigate the usefulness of new dynamic M-mode ultrasonography (US) parameters of suprahyoid muscle (SHM) to evaluate dysphagia. DESIGN: Prospective observational, cross-sectional study. SETTING: Inpatient setting at neurology department of tertiary medical center...
February 9, 2024: European Journal of Physical and Rehabilitation Medicine
https://read.qxmd.com/read/38264389/a-palliative-care-approach-to-amyotrophic-lateral-sclerosis
#10
JOURNAL ARTICLE
Cláudio Gouveia, Licínia Araújo, Susete Freitas, João Correia, Vilma Passos, Graciela Camacho, Luísa Gomes, Helena Fragoeiro, Cristiana Camacho, Beatriz Chambino
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a degenerative disease characterized by motor dysfunction. Currently, treatment options are limited and management is based mostly on symptom control and quality of life optimization, so palliative care plays a fundamental role. Our objective was to characterize the ALS population in Madeira Island that was referenced and/or followed by a palliative care unit over a five-year period. METHODS: Longitudinal, retrospective, descriptive, and observational study to analyze patients with ALS who were referred and/or followed by a palliative care unit during a five-year period, between 2017 and 2021...
December 2023: Curēus
https://read.qxmd.com/read/38262892/dysphagia-in-patients-with-sickle-cell-disease-an-understudied-problem
#11
JOURNAL ARTICLE
Candice J Adams-Mitchell, Wally R Smith, Diana J Wilkie
Dysphagia which is defined as disordered swallowing is well known as one of the most common and dangerous symptoms of many diseases, including neurological disorders such as Parkinson's disease, amyotrophic lateral sclerosis, myasthenia gravis, and most commonly, stroke. Strokes are a potentially devastating complication of sickle cell disease (SCD), the most common genetic hemoglobinopathy worldwide, yet little is known about dysphagia as it relates to SCD. Thus, the purposes of this article are to review briefly the primary causes and health consequences of dysphagia, to highlight the relevance of dysphagia to SCD, to review what little is known about dysphagia in SCD, to recommend, based on our consensus and the available literature, when to screen, evaluate, and monitor dysphagia in patients with SCD, and to outline unanswered questions where research on dysphagia in SCD might improve health outcomes...
January 22, 2024: Journal of the National Medical Association
https://read.qxmd.com/read/38226086/dental-management-of-patients-with-amyotrophic-lateral-sclerosis
#12
REVIEW
Nasser AlMadan, Ali AlMajed, Mohammed AlAbbad, Fadhel AlNashmi, Abdulmohsen Aleissa
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that affects the upper and lower motor neurons with upper and lower motor neuron manifestations. It is divided into two variants: a spinal onset and a bulbar onset. The first starts as focal muscle weakness and wasting that spreads with disease progression, while the second phenotype presents with dysarthria, dysphonia, and dysphagia. Moreover, an extra-motor manifestation could be reported with the most commonly reported symptoms being the change in cognition and sleep disorder...
December 2023: Curēus
https://read.qxmd.com/read/38202163/management-and-treatment-for-dysphagia-in-neurodegenerative-disorders
#13
REVIEW
Rumi Ueha, Carmel Cotaoco, Kenji Kondo, Tatsuya Yamasoba
Patients with neurodegenerative disorders (NDDs) often experience functional dysphagia, which may involve dysfunction in a specific phase of swallowing or in the entire process. This review outlines the approach to dysphagia in the setting of NDDs. Distinguishing the etiology of dysphagia can be difficult, and it is important to always look out for signs pointing to NDD as the cause. Thorough diagnostic work-up is essential, and it includes a comprehensive history and physical examination, alongside swallowing function tests, such as fiberoptic endoscopic evaluation of swallowing, videofluoroscopic swallowing study, and high-resolution manometry...
December 27, 2023: Journal of Clinical Medicine
https://read.qxmd.com/read/38143551/bulbar-onset-amyotrophic-lateral-sclerosis-in-an-african-american-older-adult
#14
Arielle Kasindi, Kathy Jo Carstarphen
Background: Amyotrophic lateral sclerosis (ALS), a fatal neuromuscular disease, affects the motor tracts and anterior horn cells of the spinal cord, causing both upper and lower motor neuron dysfunction. ALS typically involves progressive peripheral weakness and mobility issues. Case Report: An African American male in his early 70s presented to his primary care provider (PCP) with bulbar weakness and urinary tract symptoms. At presentation, and even later in the disease course, he ambulated well and did not report any limb issues...
2023: Ochsner Journal
https://read.qxmd.com/read/37941924/radiologically-inserted-gastrostomy-tube-in-neurological-disease-a-retrospective-study
#15
JOURNAL ARTICLE
Brittany Marie Zaita, Abheek Ghosh, Sean Lee, Aislynn Raymond, Tanvir Agnihotri, Nabeel M Akhter
OBJECTIVES: This study aimed to compare the safety and efficacy of balloon and non-balloon (or dilator) gastrostomy devices in radiologically inserted gastrostomy (RIG) for patients with neurological disease. MATERIAL AND METHODS: A retrospective analysis of 152 patients was conducted at a tertiary care hospital from July 2017 to September 2020. 104 and 48 patients were included in the balloon and non-balloon groups, respectively. The frequency of complications per specific neurological indication as well as the breakdown of the different complications pertaining to each indication was recorded for analysis...
2023: Journal of Clinical Imaging Science
https://read.qxmd.com/read/37816542/airway-clearance-strategies-and-secretion-management-in-amyotrophic-lateral-sclerosis
#16
REVIEW
Kristen L McHenry
Amyotrophic lateral sclerosis (ALS) is a rare, neurodegenerative motor neuron disease that affects voluntary muscle movement. Often, difficulty in coughing, breathing, and swallowing are sequela associated with the condition, and the presence of bulbar muscle predominant weakness results in deleterious effects on airway clearance and secretion management. This narrative review will provide practical guidance for clinicians treating this population. Cough insufficiency in this population typically manifests as a prolonged, slow, weak cough effort that impedes the clearability of secretions and airway protection...
January 24, 2024: Respiratory Care
https://read.qxmd.com/read/37814618/short-term-outcome-of-plasma-adsorption-therapy-in-amyotrophic-lateral-sclerosis
#17
JOURNAL ARTICLE
Bin Li, Wei Zhang, Shaoxin Zhong, Jianyi Pan, Xiaohong Wang, Hequn Zou, Xianrui Dou
BACKGROUND: To observe the short-term outcome of plasma adsorption PA therapy in amyotrophic lateral sclerosis (ALS). METHODS: 28 cases of als patients were recruited in this study, of which 20 were male and 8 were female with a mean age of 53.21±9.07 years and the average course of 33±23.35 months. The clinical manifestations were limb weakness (N=27), muscular atrophy (N=27), muscular tremor (N=5), dysphagia (N=12) and dysarthria (N=12). The clinical data of the patients recruited were graded by Amyotrophic Lateral Sclerosis Functional Rating Scale Revised (ALSFRSR) : <10 (N=1), 11-20 (N=4), 21-30 (N=6), 31-40 (N=12), >40 (N=5)...
August 25, 2023: Journal of Medical Biochemistry
https://read.qxmd.com/read/37785360/daily-adaptive-vs-non-adaptive-external-beam-radiation-therapy-with-concurrent-chemotherapy-for-locally-advanced-non-small-cell-lung-cancer-nsclc-a-prospective-randomized-trial-of-an-individualized-approach-for-toxicity-reduction-artia-lung
#18
JOURNAL ARTICLE
D N Stanley, J Harms, A J Kole, M C Dobelbower, C McCann, L Levine, K Russell, A M McDonald
PURPOSE/OBJECTIVE(S): Radiotherapy (RT) with concurrent chemotherapy is a standard treatment for Stage III NSCLC. However, radiation pneumonitis incidence increases rapidly with volume of lung irradiated, and esophagitis increases with dose to the esophagus. Both conditions can have adverse impacts on patients' quality of life. Daily online adaptive RT (ART) may allow for reduced normal tissue doses due to smaller margins around the target, as well as target size reduction with tumor response...
October 1, 2023: International Journal of Radiation Oncology, Biology, Physics
https://read.qxmd.com/read/37760880/acoustic-voice-analysis-as-a-useful-tool-to-discriminate-different-als-phenotypes
#19
JOURNAL ARTICLE
Giammarco Milella, Diletta Sciancalepore, Giada Cavallaro, Glauco Piccirilli, Alfredo Gabriele Nanni, Angela Fraddosio, Eustachio D'Errico, Damiano Paolicelli, Maria Luisa Fiorella, Isabella Laura Simone
Approximately 80-96% of people with amyotrophic lateral sclerosis (ALS) become unable to speak during the disease progression. Assessing upper and lower motor neuron impairment in bulbar regions of ALS patients remains challenging, particularly in distinguishing spastic and flaccid dysarthria. This study aimed to evaluate acoustic voice parameters as useful biomarkers to discriminate ALS clinical phenotypes. Triangular vowel space area (tVSA), alternating motion rates (AMRs), and sequential motion rates (SMRs) were analyzed in 36 ALS patients and 20 sex/age-matched healthy controls (HCs)...
August 31, 2023: Biomedicines
https://read.qxmd.com/read/37682589/effectiveness-of-home-based-telerehabilitation-interventions-for-dysphagia-in-patients-with-head-and-neck-cancer-systematic-review
#20
REVIEW
Wenwen Yang, Yifei Du, Mengran Chen, Sufang Li, Fan Zhang, Peiyang Yu, Xiaoxia Xu
BACKGROUND: Multimodal treatment-induced dysphagia has serious negative effects on survivors of head and neck cancer. Owing to advances in communication technologies, several studies have applied telecommunication-based interventions that incorporate swallowing exercises, education, monitoring, feedback, self-management, and communication. It is especially urgent to implement home-based remote rehabilitation in the context of the COVID-19 pandemic. However, the optimal strategy and effectiveness of remote interventions are unclear...
September 8, 2023: Journal of Medical Internet Research
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