keyword
https://read.qxmd.com/read/38536565/ai-assisted-automatic-mri-based-tongue-volume-evaluation-in-motor-neuron-disease-mnd
#1
JOURNAL ARTICLE
Ina Vernikouskaya, Hans-Peter Müller, Albert C Ludolph, Jan Kassubek, Volker Rasche
PURPOSE: Motor neuron disease (MND) causes damage to the upper and lower motor neurons including the motor cranial nerves, the latter resulting in bulbar involvement with atrophy of the tongue muscle. To measure tongue atrophy, an operator independent automatic segmentation of the tongue is crucial. The aim of this study was to apply convolutional neural network (CNN) to MRI data in order to determine the volume of the tongue. METHODS: A single triplanar CNN of U-Net architecture trained on axial, coronal, and sagittal planes was used for the segmentation of the tongue in MRI scans of the head...
March 27, 2024: International Journal of Computer Assisted Radiology and Surgery
https://read.qxmd.com/read/38535081/the-contribution-of-functional-near-infrared-spectroscopy-fnirs-to-the-study-of-neurodegenerative-disorders-a-narrative-review
#2
REVIEW
Ioannis Liampas, Freideriki Danga, Panagiota Kyriakoulopoulou, Vasileios Siokas, Polyxeni Stamati, Lambros Messinis, Efthimios Dardiotis, Grigorios Nasios
Functional near-infrared spectroscopy (fNIRS) is an innovative neuroimaging method that offers several advantages over other commonly used modalities. This narrative review investigated the potential contribution of this method to the study of neurodegenerative disorders. Thirty-four studies involving patients with Alzheimer's disease (AD), mild cognitive impairment (MCI), frontotemporal dementia (FTD), Parkinson's disease (PD), or amyotrophic lateral sclerosis (ALS) and healthy controls were reviewed. Overall, it was revealed that the prefrontal cortex of individuals with MCI may engage compensatory mechanisms to support declining brain functions...
March 21, 2024: Diagnostics
https://read.qxmd.com/read/38534355/recent-research-trends-in-neuroinflammatory-and-neurodegenerative-disorders
#3
REVIEW
Jessica Cohen, Annette Mathew, Kirk D Dourvetakis, Estella Sanchez-Guerrero, Rajendra P Pangeni, Narasimman Gurusamy, Kristina K Aenlle, Geeta Ravindran, Assma Twahir, Dylan Isler, Sara Rukmini Sosa-Garcia, Axel Llizo, Alison C Bested, Theoharis C Theoharides, Nancy G Klimas, Duraisamy Kempuraj
Neuroinflammatory and neurodegenerative disorders including Alzheimer's disease (AD), Parkinson's disease (PD), traumatic brain injury (TBI) and Amyotrophic lateral sclerosis (ALS) are chronic major health disorders. The exact mechanism of the neuroimmune dysfunctions of these disease pathogeneses is currently not clearly understood. These disorders show dysregulated neuroimmune and inflammatory responses, including activation of neurons, glial cells, and neurovascular unit damage associated with excessive release of proinflammatory cytokines, chemokines, neurotoxic mediators, and infiltration of peripheral immune cells into the brain, as well as entry of inflammatory mediators through damaged neurovascular endothelial cells, blood-brain barrier and tight junction proteins...
March 14, 2024: Cells
https://read.qxmd.com/read/38534336/progressive-irreversible-proprioceptive-piezo2-channelopathy-induced-lost-forced-peripheral-oscillatory-synchronization-to-the-hippocampal-oscillator-may-explain-the-onset-of-amyotrophic-lateral-sclerosis-pathomechanism
#4
JOURNAL ARTICLE
Balázs Sonkodi
Amyotrophic lateral sclerosis (ALS) is a mysterious lethal multisystem neurodegenerative disease that gradually leads to the progressive loss of motor neurons. A recent non-contact dying-back injury mechanism theory for ALS proposed that the primary damage is an acquired irreversible intrafusal proprioceptive terminal Piezo2 channelopathy with underlying genetic and environmental risk factors. Underpinning this is the theory that excessively prolonged proprioceptive mechanotransduction under allostasis may induce dysfunctionality in mitochondria, leading to Piezo2 channelopathy...
March 12, 2024: Cells
https://read.qxmd.com/read/38533934/picking-out-progressive-pic-alterations-in-amyotrophic-lateral-sclerosis
#5
REVIEW
Andrew J Deutsch
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that causes motoneuron death. Motoneuron excitability dysfunction in ALS is suspected to contribute to motoneuron degeneration. Therefore, mechanisms underlying excitability dysfunction are being thoroughly investigated. A recent publication from Trajano et al. (Trajano, G. S., Orssatto, L. B. R., McCombe, P. A., Rivlin, W., Tang, L., & Henderson, R. D. The Journal of Physiology , 2023) examined temporal changes to persistent inward currents (PICs) in ALS patients...
March 27, 2024: Journal of Neurophysiology
https://read.qxmd.com/read/38533726/conformational-fingerprinting-with-raman-spectroscopy-reveals-protein-structure-as-a-translational-biomarker-of-muscle-pathology
#6
JOURNAL ARTICLE
James J P Alix, Maria Plesia, Alexander P Dudgeon, Catherine A Kendall, Channa Hewamadduma, Marios Hadjivassiliou, Gráinne S Gorman, Robert W Taylor, Christopher J McDermott, Pamela J Shaw, Richard J Mead, John C Day
Neuromuscular disorders are a group of conditions that can result in weakness of skeletal muscles. Examples include fatal diseases such as amyotrophic lateral sclerosis and conditions associated with high morbidity such as myopathies (muscle diseases). Many of these disorders are known to have abnormal protein folding and protein aggregates. Thus, easy to apply methods for the detection of such changes may prove useful diagnostic biomarkers. Raman spectroscopy has shown early promise in the detection of muscle pathology in neuromuscular disorders and is well suited to characterising the conformational profiles relating to protein secondary structure...
March 27, 2024: Analyst
https://read.qxmd.com/read/38533300/a-brief-literature-review-of-low-level-laser-therapy-for-treating-amyotrophic-lateral-sclerosis-and-confirmation-of-its-effectiveness
#7
REVIEW
Sergey V Moskvin
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with a steadily progressive course due to the death of central and peripheral motor neurons responsible for voluntary movements. Low-level laser therapy (LLLT) is a treatment method unique in its universality and efficacy, particularly for neurodegenerative diseases. METHODS: In this review, we discuss the effect and application of LLLT in the treatment of ALS. A literature search for English and Russian publications for the keywords "Amyotrophic Lateral Sclerosis", "Low-Level Laser Therapy" was performed using PubMed, Scopus, Google Scholar, Web of Science and Russian Science Citation Index (RSCI) databases...
2024: BioMedicine
https://read.qxmd.com/read/38531942/timing-and-impact-of-percutaneous-endoscopic-gastrostomy-insertion-in-patients-with-amyotrophic-lateral-sclerosis-a-comprehensive-analysis
#8
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/38531462/edaravone-counteracts-redox-and-metabolic-disruptions-in-an-emerging-zebrafish-model-of-sporadic-als
#9
JOURNAL ARTICLE
Nuno A S Oliveira, Brígida R Pinho, Joana Pinto, Paula Guedes de Pinho, Jorge M A Oliveira
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease in which the death of motor neurons leads to loss of muscle function. Additionally, cognitive and circadian disruptions are common in ALS patients, contributing to disease progression and burden. Most ALS cases are sporadic, and environmental exposures contribute to their aetiology. However, animal models of these sporadic ALS cases are scarce. The small vertebrate zebrafish is a leading organism to model neurodegenerative diseases; previous studies have proposed bisphenol A (BPA) or β-methylamino-l-alanine (BMAA) exposure to model sporadic ALS in zebrafish, damaging motor neurons and altering motor responses...
March 24, 2024: Free Radical Biology & Medicine
https://read.qxmd.com/read/38528673/fluctuating-salience-in-those-living-with-genetic-risk-of-motor-neuron-disease-a-qualitative-interview-study
#10
JOURNAL ARTICLE
Jade Howard, Fadhila Mazanderani, Karen Forrest Keenan, Martin R Turner, Louise Locock
BACKGROUND: Motor neuron disease (MND) (also known as amyotrophic lateral sclerosis) is a life-limiting neurodegenerative condition. In up to 20% of people with MND, a pathogenic variant associated with autosomal dominant inheritance can be identified. Children of people carrying a pathogenic variant have a 50% chance of inheriting this and a higher, although harder to predict, chance of developing the disease compared to the general adult population. This paper explores the experience of living with the genetic risk of MND...
April 2024: Health Expectations: An International Journal of Public Participation in Health Care and Health Policy
https://read.qxmd.com/read/38527450/cerebral-tau-deposition-in-co-morbid-progressive-supranuclear-palsy-and-amyotrophic-lateral-sclerosis-an-18f-flortaucipir-and-7t-mri-study
#11
JOURNAL ARTICLE
Ian Cheong, Yong Du, Gwenn Smith, Jun Hua, Xu Li, Alexander Pantelyat
Introduction Progressive supranuclear palsy (PSP) is a four-repeat tauopathy characterized by multiple clinicopathologic subtypes. Advanced neuroimaging techniques have shown an early ability to distinguish PSP subtypes non-invasively for improved diagnosis. This study utilized tau-PET imaging and MRI techniques at 7 Tesla (7T) to determine the neuroimaging profile of a participant with comorbid PSP and amyotrophic lateral sclerosis (ALS). Method [18F]-flortaucipir PET imaging was performed on one participant with PSP-ALS, one participant with typical PSP (Richardson's syndrome; PSP-RS), and 15 healthy control volunteers...
March 25, 2024: Neuro-degenerative Diseases
https://read.qxmd.com/read/38526287/osmr-is-a-potential-driver-of-inflammation-in-amyotrophic-lateral-sclerosis
#12
JOURNAL ARTICLE
Wenzhi Chen, Shishi Jiang, Shu Li, Cheng Li, Renshi Xu
JOURNAL/nrgr/04.03/01300535-202419110-00031/figure1/v/2024-03-08T184507Z/r/image-tiff Amyotrophic lateral sclerosis is a neurodegenerative disease, and the molecular mechanism underlying its pathology remains poorly understood. However, inflammation is known to play an important role in the development of this condition. To identify driver genes that affect the inflammatory response in amyotrophic lateral sclerosis, as well as potential treatment targets, it is crucial to analyze brain tissue samples from patients with both sporadic amyotrophic lateral sclerosis and C9orf72-related amyotrophic lateral sclerosis...
November 1, 2024: Neural Regeneration Research
https://read.qxmd.com/read/38525704/emerging-evidence-of-golgi-stress-signaling-for-neuropathies
#13
REVIEW
Remina Shirai, Junji Yamauchi
The Golgi apparatus is an intracellular organelle that modifies cargo, which is transported extracellularly through the nucleus, endoplasmic reticulum, and plasma membrane in order. First, the general function of the Golgi is reviewed and, then, Golgi stress signaling is discussed. In addition to the six main Golgi signaling pathways, two pathways that have been increasingly reported in recent years are described in this review. The focus then shifts to neurological disorders, examining Golgi stress reported in major neurological disorders, such as Alzheimer's disease, Parkinson's disease, and Huntington's disease...
March 7, 2024: Neurology International
https://read.qxmd.com/read/38524582/constipation-in-patients-with-motor-neuron-disease-a-retrospective-longitudinal-study
#14
JOURNAL ARTICLE
Yuki Yamamoto, Koji Fujita, Hiroki Yamazaki, Shotaro Haji, Yusuke Osaki, Yuishin Izumi
BACKGROUND: Constipation has been recently recognized as a complication associated with motor and autonomic dysfunction in patients with motor neuron disease (MND), typified by amyotrophic lateral sclerosis (ALS). However, the long-term characteristics of constipation remain unclear in patients with MND. We longitudinally investigated the prevalence and risk factors of constipation in a consecutive cohort of patients with MND. METHODS: Data from Japanese patients with MND enrolled in a single-center registry from June 2017 to December 2021 were retrospectively investigated...
March 30, 2024: Heliyon
https://read.qxmd.com/read/38524401/amyotrophic-lateral-sclerosis-and-pain-a-narrative-review-from-pain-assessment-to-therapy
#15
REVIEW
Vincenzo Pota, Pasquale Sansone, Sara De Sarno, Caterina Aurilio, Francesco Coppolino, Manlio Barbarisi, Francesco Barbato, Marco Fiore, Gianluigi Cosenza, Maria Beatrice Passavanti, Maria Caterina Pace
Amyotrophic lateral sclerosis (ALS) is the most frequent neurodegenerative disease of the motor system that affects upper and lower motor neurons, leading to progressive muscle weakness, spasticity, atrophy, and respiratory failure, with a life expectancy of 2-5 years after symptom onset. In addition to motor symptoms, patients with ALS have a multitude of nonmotor symptoms; in fact, it is currently considered a multisystem disease. The purpose of our narrative review is to evaluate the different types of pain, the correlation between pain and the disease's stages, the pain assessment tools in ALS patients, and the available therapies focusing above all on the benefits of cannabis use...
2024: Behavioural Neurology
https://read.qxmd.com/read/38522911/the-clinical-practice-guideline-for-the-management-of-amyotrophic-lateral-sclerosis-in-japan-update-2023
#16
JOURNAL ARTICLE
Makoto Urushitani, Hitoshi Warita, Naoki Atsuta, Yuishin Izumi, Osamu Kano, Toshio Shimizu, Yuki Nakayama, Yugo Narita, Hiroyuki Nodera, Takuji Fujita, Koichi Mizoguchi, Mitsuya Morita, Masashi Aoki
Amyotrophic lateral sclerosis (ALS) is an adult-onset intractable motor neuron disease characterized by selective degeneration of cortical neurons in the frontotemporal lobe and motor neurons in the brainstem and spinal cord. Impairment of these neural networks causes progressive muscle atrophy and weakness that spreads throughout the body, resulting in life-threatening bulbar palsy and respiratory muscle paralysis. However, no therapeutic strategy has yet been established to halt ALS progression. Although evidence for clinical practice in ALS remains insufficient, novel research findings have steadily accumulated in recent years...
March 23, 2024: Rinshō Shinkeigaku, Clinical Neurology
https://read.qxmd.com/read/38522514/tryptophan-residues-in-tdp-43-and-sod1-modulate-the-cross-seeding-and-toxicity-of-sod1
#17
JOURNAL ARTICLE
Edward Pokrishevsky, Michéle G DuVal, Luke McAlary, Sarah Louadi, Silvia Pozzi, Andrei Roman, Steven S Plotkin, Anke Dijkstra, Jean-Pierre Julien, W Ted Allison, Neil R Cashman
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease of motor neurons. Neuronal superoxide dismutase-1 (SOD1) inclusion bodies are characteristic of familial ALS with SOD1 mutations, while a hallmark of sporadic ALS is inclusions containing aggregated wild-type TAR DNA-binding protein 43 (TDP-43). We show here that co-expression of mutant or wild-type TDP-43 with SOD1 leads to misfolding of endogenous SOD1 and aggregation of SOD1 reporter protein SOD1G85R -GFP in human cell cultures, and promotes synergistic axonopathy in zebrafish...
March 22, 2024: Journal of Biological Chemistry
https://read.qxmd.com/read/38522245/remote-pulmonary-function-testing-allows-for-early-identification-of-need-for-non-invasive-ventilation-in-a-subset-of-persons-with-als
#18
JOURNAL ARTICLE
Andrew Geronimo, Zachary Simmons
The traditional ALS multidisciplinary clinical practice of quarterly respiratory assessment may leave some individuals in danger of developing untreated respiratory insufficiency between visits or beginning non-invasive ventilation (NIV) later than would be optimal. Remote, or home-based, pulmonary function testing (rPFT) allows patients with ALS to perform regular respiratory testing at more frequent intervals in the home. The aim of this study was to determine the clinical benefit of weekly rPFT compared to standard, quarterly in-clinic respiratory assessments: the number of individuals with earlier identification of NIV need, the magnitude of this advance notice, and the individual factors predicting benefit...
March 19, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38522244/timing-initiation-of-non-invasive-ventilation-in-management-of-als
#19
EDITORIAL
Michael Swash
No abstract text is available yet for this article.
March 19, 2024: Journal of the Neurological Sciences
https://read.qxmd.com/read/38521060/single-cell-dissection-of-the-human-motor-and-prefrontal-cortices-in-als-and-ftld
#20
JOURNAL ARTICLE
S Sebastian Pineda, Hyeseung Lee, Maria J Ulloa-Navas, Raleigh M Linville, Francisco J Garcia, Kyriakitsa Galani, Erica Engelberg-Cook, Monica C Castanedes, Brent E Fitzwalter, Luc J Pregent, Mahammad E Gardashli, Michael DeTure, Diana V Vera-Garcia, Andre T S Hucke, Bjorn E Oskarsson, Melissa E Murray, Dennis W Dickson, Myriam Heiman, Veronique V Belzil, Manolis Kellis
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) share many clinical, pathological, and genetic features, but a detailed understanding of their associated transcriptional alterations across vulnerable cortical cell types is lacking. Here, we report a high-resolution, comparative single-cell molecular atlas of the human primary motor and dorsolateral prefrontal cortices and their transcriptional alterations in sporadic and familial ALS and FTLD. By integrating transcriptional and genetic information, we identify known and previously unidentified vulnerable populations in cortical layer 5 and show that ALS- and FTLD-implicated motor and spindle neurons possess a virtually indistinguishable molecular identity...
March 14, 2024: Cell
keyword
keyword
13186
1
2
Fetch more papers »
Fetching more papers... Fetching...
Remove bar
Read by QxMD icon Read
×

Save your favorite articles in one place with a free QxMD account.

×

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"

We want to hear from doctors like you!

Take a second to answer a survey question.