A Journey with Amyotrophic Lateral Sclerosis

ABSTRACT: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease. Motor neurons are affected in certain patterns, such as cervical, thoracic, lumbar, and bulbar (facial) regions. Although initial presentations can vary, eventually upper and lower motor neurons are lost in the two types of ALS: familial and sporadic. A case study highlighting the reality of living with bulbar ALS relays Sister A's journey from early to late ALS. Etiology, pathophysiology, diagnosis, treatment, and clinical care are discussed.
Source: Journal of Christian Nursing - Category: Nursing Tags: Feature: CE Connection: practice Source Type: research

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In this study, HCY was observed to sensitize the neuromuscular junction to ROS-induced depression of spontaneous transmission frequency, an effect we found to be mediated by a N-methyl-D-aspartate receptor (NMDAR) and nitric oxide (NO). The NMDAR antagonist D, L-2-amino-5-phosphonopentanoic acid prevented the HCY-induced sensitization to oxidative stress. Disrupting NO activity with either the nitric oxide synthase I antagonist Nω-nitro-L-arginine methyl ester hydrochloride or the NO scavenger 2-(4-carboxyphenyl)-4,4,5,5-tetramethyl-imidazoline-1-oxyl-3-oxide potassium salt also prevented sensitization. Moreover, rep...
Source: NeuroReport - Category: Neurology Tags: Cellular, Molecular and Developmental Neuroscience Source Type: research
This article discusses the preclinical pharmacology, pharmacokinetics, safety profile, clinical studies and drug interactions of edaravone (Radicava) in ALS. PMID: 29998226 [PubMed - in process]
Source: Drugs of Today - Category: Drugs & Pharmacology Tags: Drugs Today (Barc) Source Type: research
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) represent two ends of a disease spectrum with shared clinical, genetic and pathological features. These include near ubiquitous pathological inclusions of the RNA binding protein (RBP) TDP-43, and often the presence of a GGGGCC expansion in theC9ORF72 (C9) gene. Previously we reported that the sequestration of hnRNP H altered the splicing of target transcripts in C9ALS patients (Conlon et al. 2016). Here we show that this signature also occurs in half of 50 post-mortem sporadic, non-C9 ALS/FTD brains. Furthermore, and equally surprisingly, these 'like-C9...
Source: eLife - Category: Biomedical Science Tags: Biochemistry and Chemical Biology Human Biology and Medicine Source Type: research
Noninvasive ventilator support using bi-level positive airway pressure/continuous positive airway pressure (BiPAP/CPAP) is commonly utilized for chronic medical conditions like sleep apnea and neuromuscular disorders like amyotrophic lateral sclerosis (ALS) that lead to weakness of respiratory muscles. Generic masks come in standard sizes and are often perceived by patients as being uncomfortable, ill-fitting, and leaky. A significant number of patients are unable to tolerate the masks and eventually stop using their devices. The goal of this project is to develop custom-fit masks to increase comfort, decrease air leakage,...
Source: Journal of Medical Devices, Transactions of the ASME - Category: Medical Devices Source Type: research
Publication date: Available online 12 July 2018Source: NeuroscienceAuthor(s): Andrea Markovinovic, Tereza Ljutic, Louis-Charles Béland, Ivana MuniticAbstractMutations in a ubiquitin (Ub)-binding adaptor protein optineurin have been found in amyotrophic lateral sclerosis (ALS), a neurodegenerative disease with a prominent neuroinflammatory component. Unlike more frequent ALS mutations which cause disease by gaining toxic properties such as aggregation, mutated optineurin is thought to cause disease by loss-of-function, highlighting its neuroprotective role. Optineurin regulates inflammatory signalling by acting as a ...
Source: Neuroscience - Category: Neuroscience Source Type: research
Nishad Khamankar, Grant Coan, Barry Weaver, Cassie S. Mitchell
Source: Frontiers in Neurology - Category: Neurology Source Type: research
There are several suspected infectious causes of amyotrophic lateral sclerosis (ALS) or motor neurone disease including HIV-1 and species of Brucella, Cyanobacteria and Schistosoma. The increased rates and clusters of ALS in amateur and professional outdoor sports players including rugby, football and soccer players suggest a microorganism present in the grass, dirt and dust they play on and in may be a causative factor. The probable zoonosis Mycobacterium avium subspecies paratuberculosis (MAP) is heavily excreted in an infected domestic ruminant ’s feces or manure and is extensively distributed throughout the soil ...
Source: Medical Hypotheses - Category: Biomedical Science Authors: Source Type: research
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Source: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration - Category: Neurology Authors: Source Type: research
Publication date: October 2018Source: Clinica Chimica Acta, Volume 485Author(s): Kuo-Hsuan Chang, Mei-Ling Cheng, Mu-Chun Chiang, Chiung-Mei ChenAbstractOxidative stress is commonly involved in the pathogenesis of various neurodegenerative diseases, such as Alzheimer’s disease, Parkinson’s disease, Huntington’s disease and amyotrophic lateral sclerosis. Therefore, lipophilic antioxidants, such as vitamin A, carotinoids, vitamin E, coenzyme Q10, docosahexaenoic acid and eicosapentaenoic acid, have received increasing attention as therapeutic and preventive intervention for neurodegenerative diseases. Altho...
Source: Clinica Chimica Acta - Category: Laboratory Medicine Source Type: research
ConclusionWe present a detailed clinical and laboratory analysis of the patient. This case report will emphasize the importance of evaluating anti-MuSK and anti-LRP4 antibodies even in patients with anti-AChR antibodies.
Source: Clinica Chimica Acta - Category: Laboratory Medicine Source Type: research
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