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Lou Gehrig's Disease

Inês do Carmo G Golçalves, Wiebke A Rehorst, Min Jeong Kye
Recent findings indicate an important role for RNA-mediated gene expression in motor neuron diseases, including amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). ALS, also known as Lou Gehrig's disease, is an adult-onset progressive neurodegenerative disorder, whereby SMA or "children's Lou Gehrig's disease" is considered a pediatric neurodevelopmental disorder. Despite the differences in genetic causes, both ALS and SMA share common phenotypes; dysfunction/loss of motor neurons that eventually leads to muscle weakness and atrophy...
August 15, 2016: CNS & Neurological Disorders Drug Targets
Paul Mehta, Wendy Kaye, Leah Bryan, Theodore Larson, Timothy Copeland, Jennifer Wu, Oleg Muravov, Kevin Horton
PROBLEM/CONDITION: Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig's disease, is a progressive and fatal neuromuscular disease for which no cure or viable treatment has been identified. ALS, like most noncommunicable diseases, is not a nationally notifiable disease in the United States. The prevalence of ALS in the United States during 2010-2011 was estimated to be 3.9 cases per 100,000 persons in the general population. Updated prevalence estimates are needed to help monitor disease status, better understand etiology, and identify risk factors for ALS...
2016: MMWR. Surveillance Summaries: Morbidity and Mortality Weekly Report. Surveillance Summaries
Saurabh Kumar, Pallavi Aga, Aakansha Gupta, Neera Kohli
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig disease, is a chronic degenerative neurologic disease and is characterized by the selective involvement of the motor system. Usually, patients present with upper motor neuron (UMN) and lower motor neuron compromise. Degeneration of the UMN in the cerebral cortex is one of the main pathologic changes in ALS. These changes usually affect corticospinal tracts leading to degeneration of the fibers which show characteristic hyperintensities along the tracts leading to the "wine glass sign...
January 2016: Journal of Pediatric Neurosciences
Hélio A G Teive, Plínio M G Lima, Francisco M B Germiniani, Renato P Munhoz
The use of eponyms in neurology remains controversial, and important questions have been raised about their appropriateness. Different approaches have been taken, with some eponyms being excluded, others replaced, and new ones being created. An example is Hallervorden-Spatz syndrome, which has been replaced by neurodegeneration with brain iron accuulatium (NBIA). Amiothoplic lateral sclerosys (ALS), for which the eponym is Charcot's disease, has been replaced in the USA by Lou Gehrig's disease. Guillain-Barré syndrome (GBS) is an eponym that is still the subject of controversy, and various different names are associated with it...
May 2016: Arquivos de Neuro-psiquiatria
(no author information available yet)
May is Amyotrophic Lateral Sclerosis (ALS) Awareness Month. ALS, also known as Lou Gehrig's disease, is a progressive, fatal, neurodegenerative disorder of upper and lower motor neurons. The cause of ALS is not known, and no cure exists. Persons with ALS usually die within 2-5 years of diagnosis.
2016: MMWR. Morbidity and Mortality Weekly Report
Yeun Ju Kim, Maria Luisa Guzman-Hernandez, Eva Wisniewski, Nicolas Echeverria, Tamas Balla
Phospholipase C (PLC)-mediated hydrolysis of the limited pool of plasma membrane (PM) phosphatidylinositol 4,5-bisphosphate [PtdIns(4,5)P2] requires replenishment from a larger pool of phosphatidylinositol (PtdIns) via sequential phosphorylation by PtdIns 4-kinases and phosphatidylinositol 4-phosphate (PtdIns4P) 5-kinases. Since PtdIns is synthesized in the endoplasmic reticulum (ER) and PtdIns(4,5)P2 is generated in the PM, it has been postulated that PtdIns transfer proteins (PITPs) provide the means for this lipid transfer function...
February 2016: Biochemical Society Transactions
Maitham Ahmed Al-Sammak, Douglas G Rogers, Kyle D Hoagland
The cyanobacterial neurotoxin β-N-methylamino-L-alanine (BMAA) is considered to be an "excitotoxin," and its suggested mechanism of action is killing neurons. Long-term exposure to L-BMAA is believed to lead to neurodegenerative diseases including Parkinson's and Alzheimer's diseases and amyotrophic lateral sclerosis (Lou Gehrig's disease). Objectives of this study were to determine the presumptive median lethal dose (LD50), the Lowest-Observed-Adverse-Effect Level (LOAEL), and histopathologic lesions caused by the naturally occurring BMAA isomer, L-BMAA, in mice...
2015: Journal of Toxicology
Sabrina Paganoni, Chafic Karam, Nanette Joyce, Richard Bedlack, Gregory T Carter
BACKGROUND: Amyotrophic lateral sclerosis (ALS or Lou Gehrig's disease) is a neurodegenerative disease that results in progressive muscle weakness and wasting. There is no known cure and the disease is uniformly fatal. PURPOSE: This review discusses current concepts in ALS care, from breaking the diagnosis to end-of-life care. People with ALS have several multidisciplinary needs due to a complex and dynamic disease process. They benefit from rehabilitation interventions that are individualized and have the goal of optimizing independence, function, and safety...
2015: NeuroRehabilitation
Jingsong Zhou, Jianxun Yi, Lynda Bonewald
Amyotrophic lateral sclerosis (ALS), also called Lou Gehrig's disease, is a fatal neuromuscular disorder characterized by degeneration of motor neurons and by skeletal muscle atrophy. Although the death of motor neurons is a pathological hallmark of ALS, the potential role of other organs in disease progression remains to be elucidated. Skeletal muscle and bone are the two largest organs in the human body. They are responsible not only for locomotion but also for maintaining whole body normal metabolism and homeostasis...
October 2015: Current Osteoporosis Reports
Agavni Petrosyan, I-Hui Hsieh, John P Phillips, Kourosh Saberi
Mutation of the human gene superoxide dismutase (hSOD1) is associated with the fatal neurodegenerative disease familial amyotrophic lateral sclerosis (Lou Gehrig's disease). Selective overexpression of hSOD1 in Drosophila motorneurons increases lifespan to 140% of normal. The current study was designed to determine resistance to lifespan decline and failure of sensorimotor functions by overexpressing hSOD1 in Drosophila's motorneurons. First, we measured the ability to maintain continuous flight and wingbeat frequency (WBF) as a function of age (5 to 50 days)...
March 2015: Genetics and Molecular Biology
Caroline M O Volpe, Jose A Nogueira-Machado
Amyotrophic Lateral Sclerosis (ALS) or Lou Gehrig's disease is an axonopathy with adultonset, progressive and irreversible degeneration of upper and lower motor neurons. Around 90% of ALS is considered as sporadic ALS (sALS) without apparent genetic cause while in the familial type of ALS (fALS) at least one affected blood relative needs to be identified. Both sALS and fALS show similar progression and pathological profile. Biochemical and immunological roles have been reported for both types of ALS. It has been suggested that mutation in SOD1 gene would be responsible for the oxidative stress and neurotoxicity...
2015: Recent Patents on Endocrine, Metabolic & Immune Drug Discovery
Kang Mi Pang, Ji Woon Park
This article reports a case of masticatory muscle pain and progressive limited mouth opening secondary to amyotrophic lateral sclerosis (ALS), popularly known as Lou Gehrig's disease. The symptoms were first mistaken as those of temporomandibular disorders, before fatty degeneration of all masticatory muscles were discovered on magnetic resonance imaging (MRI). ALS should be considered in the differential diagnosis process when the patient presents with longstanding progressive mouth opening limitation associated with pain...
2015: Journal of Oral & Facial Pain and Headache
Michael Collins
No abstract text is available yet for this article.
January 27, 2015: JAMA: the Journal of the American Medical Association
Udensi K Udensi, Paul B Tchounwou
Oxidative stress (OS) has been characterized by an imbalance between the production of reactive oxygen species (ROS) and a biological system's ability to repair oxidative damage or to neutralize the reactive intermediates including peroxides and free radicals. High ROS production has been associated with significant decrease in antioxidant defense mechanisms leading to protein, lipid and DNA damage and subsequent disruption of cellular functions. In humans, OS has been reported to play a role in the pathogenesis of neurodegenerative diseases such as Alzheimer's disease, Huntington's disease, Lou Gehrig's disease, multiple sclerosis and Parkinson's disease, as well as atherosclerosis, autism, cancer, heart failure, and myocardial infarction...
2014: Journal of Experimental & Clinical Cancer Research: CR
Muhamed Hadzipasic, Babak Tahvildari, Maria Nagy, Minjuan Bian, Arthur L Horwich, David A McCormick
Amyotrophic lateral sclerosis (ALS; Lou Gehrig's disease) affects motor neurons (MNs) in the brain and spinal cord. Understanding the pathophysiology of this condition seems crucial for therapeutic design, yet few electrophysiological studies in actively degenerating animal models have been reported. Here, we report a novel preparation of acute slices from adult mouse spinal cord, allowing visualized whole cell patch-clamp recordings of fluorescent lumbar MN cell bodies from ChAT-eGFP or superoxide dismutase 1-yellow fluorescent protein (SOD1YFP) transgenic animals up to 6 mo of age...
November 25, 2014: Proceedings of the National Academy of Sciences of the United States of America
A R Kherlopian, J P Gerrein, M Yue, K E Kim, J W Kim, M Sukumaran, P Sajda
This paper discusses the creation of a system for computer-aided communication through automated analysis and processing of electrooculogram signals. In situations of disease or trauma, there may be an inability to communicate with others through standard means such as speech or typing. Eye movement tends to be one of the last remaining active muscle capabilities for people with neurodegenerative disorders, such as amyotrophic lateral sclerosis (ALS) also known as Lou Gehrig's disease. Thus, there is a need for eye movement based systems to enable communication...
August 2006: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
Csilla Ari, Angela M Poff, Heather E Held, Carol S Landon, Craig R Goldhagen, Nicholas Mavromates, Dominic P D'Agostino
Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's disease, is a neurodegenerative disorder of motor neurons causing progressive muscle weakness, paralysis, and eventual death from respiratory failure. There is currently no cure or effective treatment for ALS. Besides motor neuron degeneration, ALS is associated with impaired energy metabolism, which is pathophysiologically linked to mitochondrial dysfunction and glutamate excitotoxicity. The Deanna Protocol (DP) is a metabolic therapy that has been reported to alleviate symptoms in patients with ALS...
2014: PloS One
Paul Mehta, Vinicius Antao, Wendy Kaye, Marchelle Sanchez, David Williamson, Leah Bryan, Oleg Muravov, Kevin Horton
PROBLEM/CONDITION: Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig's disease, is a progressive and fatal neuromuscular disease for which no cure has been identified. Although ALS has no known definitive cause, familial ALS (a hereditary form) occurs in 5%-10% of cases. Many hypotheses have been formulated about what causes ALS, including chemical exposures, occupational exposure, military service, infectious agents, nutritional intake, physical activity, and trauma. Worldwide, ALS affects white males aged >60 years more often than any other group...
July 25, 2014: MMWR Supplements
The Als Association Media Relations
No abstract text is available yet for this article.
2013: Rare Diseases
Susana Collado-Vázquez, Jesús María Carrillo
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with a progressive course that affects the corticospinal and spinal cord motor neurons, the main manifestations of which are muscular weakness, amyotrophy and hyperreflexia. It has an incidence of 0.4-2.4 cases/100,000 inhabitants/year, and a prevalence of 4-6 cases/100,000 inhabitants. It is more frequent in adult males over 50 years of age. A number of different neurological diseases have been portrayed in literature, cinema and television, including ALS, which has been presented correctly and realistically...
July 1, 2014: Revista de Neurologia
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