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Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)

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A degenerative disorder affecting upper MOTOR NEURONS in the brain and lower motor neurons in the brain stem and SPINAL CORD. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, FASCICULATION, hyperreflexia, DYSARTHRIA, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous ASTROCYTES and atrophy of anterior SPINAL NERVE ROOTS and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94)

Also Known As:

Lou Gehrig's Disease; Motor Neuron Disease, Amyotrophic Lateral Sclerosis; Lou Gehrig Disease; ALS (Amyotrophic Lateral Sclerosis); Amyotrophic Lateral Sclerosis With Dementia; Amyotrophic Lateral Sclerosis, Guam Form; Amyotrophic Lateral Sclerosis-Parkinsonism/dementia Complex 1; Amyotrophic lateral sclerosis, Parkinsonism/Dementia complex of Guam; Dementia With Amyotrophic Lateral Sclerosis; Gehrig's Disease; Guam Form of Amyotrophic Lateral Sclerosis; Lou-Gehrigs Disease; Disease, Lou-Gehrigs; Gehrig Disease; Gehrigs Disease; Lateral Scleroses, Amyotrophic; Sclerosis, Amyotrophic Lateral

Networked: 5913 relevant articles (140 outcomes, 584 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neurodegenerative Diseases (Neurodegenerative Disease)
2.	Parkinson Disease (Parkinson's Disease)
3.	Alzheimer Disease (Alzheimer's Disease)
4.	Stroke (Strokes)
5.	Huntington Disease (Huntington's Disease)

Experts

1.	Trojanowski, John Q: 53 articles (11/2015 - 03/2004)
2.	Andersen, Peter M: 48 articles (04/2015 - 12/2002)
3.	Brown, Robert H: 46 articles (08/2015 - 05/2002)
4.	Ludolph, Albert C: 42 articles (12/2015 - 10/2002)
5.	Lee, Virginia M-Y: 39 articles (11/2015 - 03/2004)
6.	Shaw, Pamela J: 37 articles (10/2014 - 06/2002)
7.	Meininger, Vincent: 36 articles (11/2015 - 01/2002)
8.	Shaw, Christopher E: 36 articles (03/2015 - 12/2002)
9.	Sobue, Gen: 35 articles (11/2015 - 01/2002)
10.	Beal, M Flint: 34 articles (04/2014 - 04/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Amyotrophic Lateral Sclerosis:

1.	Riluzole FDA LinkGeneric 11/07/2003 - "These results suggest that riluzole, a drug used in amyotrophic lateral sclerosis (ALS), might be beneficial for the treatment of MS." 11/01/2013 - "Riluzole is a drug approved for the treatment of amyotrophic lateral sclerosis (ALS) and may be effective for the treatment of other neurodegenerative and neuropsychiatric disorders. " 01/01/2006 - "Indeed, initial evidence from our group suggests that the antiglutamatergic agent riluzole (Rilutek), which was developed for the treatment of amyotrophic lateral sclerosis, is effective in treatment-resistant OCD. " 10/15/2013 - "Although riluzole is FDA approved and has been shown to be safe and effective in the context of amyotrophic lateral sclerosis, its efficacy and safety in the context of CSM remain unknown. " 05/01/2013 - "In an attempt to determine the mechanisms by which riluzole exerts neuroprotective effects, in particular to dissect the relative contributions of inhibition of glutamatergic transmission and Na+ channel modulation, the present study utilized a combination of cortical and peripheral axonal excitability approaches to monitor changes in excitability and function in patients with amyotrophic lateral sclerosis. "
2.	LithiumIBA 08/01/2009 - "In this article we provide an overview of the intersection between amyotrophic lateral sclerosis (ALS) and the autophagy pathway and discuss the potential protective effects of lithium through mechanisms that recruit autophagy and other effects. " 05/01/2012 - "To determine the safety and efficacy of lithium for the treatment of amyotrophic lateral sclerosis (ALS) in a randomised, placebo controlled, double blind, sequential trial. " 12/01/2013 - "The study by Al-Chalabi et al. state that the therapeutic activity of lithium in amyotrophic lateral sclerosis (ALS) is worth investigating. " 04/01/2013 - "Lithium in patients with amyotrophic lateral sclerosis (LiCALS): a phase 3 multicentre, randomised, double-blind, placebo-controlled trial." 05/01/2012 - "Lithium lacks effect on survival in amyotrophic lateral sclerosis: a phase IIb randomised sequential trial."
3.	AntioxidantsIBA 08/01/2008 - "In the opinion of the reviewers, there is insufficient evidence of efficacy of individual antioxidants, or antioxidants in general, in the treatment of people with amyotrophic lateral sclerosis. " 01/01/2007 - "There is insufficient evidence of efficacy of individual antioxidants, or antioxidants in general, in the treatment of people with amyotrophic lateral sclerosis. " 01/01/2005 - "There is insufficient evidence of efficacy of individual antioxidants, or antioxidants in general, in the treatment of people with amyotrophic lateral sclerosis. " 01/01/2004 - "There is insufficient evidence of efficacy of individual antioxidants, or antioxidants in general, in the treatment of people with amyotrophic lateral sclerosis. " 08/01/2006 - "Cell-permeable peptide antioxidants as a novel therapeutic approach in a mouse model of amyotrophic lateral sclerosis."
4.	Glutamic Acid (Glutamate)FDA Link 01/01/1993 - "Since recent studies provided evidence for abnormal glutamate metabolism in amyotrophic lateral sclerosis, we measured amino acid levels in the fasting plasma of 52 ALS patients and an equal number of controls of a similar age. " 01/01/2015 - "Altered synaptic transmission with excess glutamate release has been implicated in the loss of motoneurons occurring in amyotrophic lateral sclerosis (ALS). " 11/01/2014 - "Glutamate excitotoxicity might contribute to the pathophysiology of amyotrophic lateral sclerosis. " 07/01/2014 - "The neurotoxic effects of cerebrospinal fluid (CSF) from patients with amyotrophic lateral sclerosis (ALS) have been reported by various authors who have attributed this neurotoxicity to the glutamate in CSF-ALS. " 09/01/2012 - "Non-cell autonomous pathology is widely accepted to determine the demise of motoneurons (MNs) in amyotrophic lateral sclerosis (ALS) with astrocytes, GFAP and glutamate transport suggested to play roles in reactive astrogliosis. "
5.	Insulin-Like Growth Factor I (IGF-1)IBA 01/01/2002 - "The main objective of this review was to examine the efficacy of recombinant human insulin-like growth factor I in amyotrophic lateral sclerosis. " 09/01/2012 - "Functional improvement in mouse models of familial amyotrophic lateral sclerosis by PEGylated insulin-like growth factor I treatment depends on disease severity." 01/01/2007 - "To examine the efficacy of recombinant human insulin-like growth factor I in amyotrophic lateral sclerosis. " 07/01/2004 - "Insulin-like growth factor I (IGF-I) is currently in clinical trials for treatment of amyotrophic lateral sclerosis (ALS), but little is known about how it promotes the survival of motor neurons. " 01/01/2002 - "Types of studies: all randomised controlled clinical trials involving recombinant human insulin-like growth factor I treatment of amyotrophic lateral sclerosis. "
6.	CreatineIBA 05/01/2011 - "We demonstrated that creatine produced an extension of survival, improved motor performance, and a reduction in loss of motor neurons in a transgenic mouse model of amyotrophic lateral sclerosis (ALS). " 12/01/2010 - "A phase I, pharmacokinetic, dosage escalation study of creatine monohydrate in subjects with amyotrophic lateral sclerosis." 04/01/2003 - "A randomized sequential trial of creatine in amyotrophic lateral sclerosis." 01/01/2010 - "In patients already diagnosed with clinically probable or definite amyotrophic lateral sclerosis (ALS), creatine at doses ranging from 5 to 10 g per day did not have a statistically significant effect on survival, ALS functional rating revised scores (ALSFRS-R) progression or percent predicted forced vital capacity (FVC) progression." 07/01/2007 - "In addition, creatine has direct antioxidant effects, which may be of importance in amyotrophic lateral sclerosis. "
7.	superoxide dismutase 1IBA 10/01/2010 - "Vaccinations targeting extracellular superoxide dismutase 1 (SOD1) mutants are beneficial in mouse models of amyotrophic lateral sclerosis (ALS). " 01/01/2011 - "RNA interference (RNAi) is a potential cure for amyotrophic lateral sclerosis (ALS) caused by dominant, gain-of-function superoxide dismutase 1 (SOD1) mutations. " 10/01/2009 - "In this issue of Genes & Development, Hetz and colleagues (pp. 2294-2306) surprisingly show that inhibition of the UPR by knockout of XBP-1 causes a massive increase in autophagy, enhances clearance of superoxide dismutase 1 (SOD1) aggregates, and delays the development of amyotrophic lateral sclerosis. " 10/01/2015 - "Drug discovery for amyotrophic lateral sclerosis (ALS) has experienced a surge in clinical studies and remarkable preclinical milestones utilizing a variety of mutant superoxide dismutase 1 model systems. " 06/25/2015 - "In this study, we analyzed the spatiotemporal alterations of phospholipid composition in the spinal cord of an amyotrophic lateral sclerosis (ALS) mouse model (G93A-mutated human superoxide dismutase 1 transgenic mice [SOD1(G93A) mice]) using imaging mass spectrometry (IMS), a powerful method to visualize spatial distributions of various types of molecules in situ. "
8.	Biological Markers (Surrogate Marker)IBA 01/01/2011 - "Our results indicate that PBMC multiprotein biomarkers could contribute to determine amyotrophic lateral sclerosis diagnosis, differential diagnosis, disease severity and progression, and may help to elucidate pathogenic mechanisms." 09/01/2009 - "Improved biomarkers would facilitate the diagnosis and treatment of amyotrophic lateral sclerosis (ALS). " 01/01/2016 - "The identification of circulating biomarkers is needed to facilitate diagnosis and prognosis of amyotrophic lateral sclerosis (ALS) and to offer indicators of therapeutic response in clinical trials. " 09/01/2014 - "There is an urgent need to identify reliable biomarkers of amyotrophic lateral sclerosis (ALS) progression for clinical practice and pharmacological trials. " 06/01/2014 - "This approach can be used not only for individual clinical work-up purposes, but enlarges the spectrum of potential non-invasive surrogate markers as a neuroimaging-based read-out for amyotrophic lateral sclerosis studies within a clinical context. "
9.	Proteins (Proteins, Gene)IBA 02/01/2002 - "This study concerns the immunohistochemical investigation of synaptic proteins in the anterior horn of amyotrophic lateral sclerosis (ALS). " 10/21/2015 - "Using a candidate gene screen, we report that loss of RAD-23 protects against the toxicity of proteins known to aggregate in amyotrophic lateral sclerosis. " 03/01/2015 - "Autophagy-linked FYVE protein (Alfy) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS)." 01/01/2015 - "Interest in the proteins has been heightened by the discovery that gene mutations in UBQLN2 cause dominant inheritance of amyotrophic lateral sclerosis (ALS). " 12/01/2014 - "The proteins associated with amyotrophic lateral sclerosis also showed distinct differences between cell lines and primary motoneurons, providing a molecular basis for understanding fundamental alterations between cell lines and neurons with respect to neuronal pathways with relevance for disease mechanisms. "
10.	Nerve Growth Factors (Neurotrophins)IBA 08/01/2000 - "This model thus represents an attractive model to evaluate potential protective effects of neurotrophic factors for adult-onset motoneuron diseases, such as amyotrophic lateral sclerosis. " 09/01/1997 - "Finally, the current status of clinical trials for amyotrophic lateral sclerosis using neurotrophic factors will be discussed, as well as recent reports that neurotrophic factors can exert adverse effects on neuronal survival." 07/25/1997 - "Immunocytochemical studies of neurotrophins in cerebral motor cortex in Amyotrophic Lateral Sclerosis." 01/01/2014 - "Analysis of neurotrophic factors in limb and extraocular muscles of mouse model of amyotrophic lateral sclerosis." 01/01/2004 - "Neurotrophic factors and amyotrophic lateral sclerosis."

Therapies and Procedures

1.	Tracheostomy 05/01/2010 - "Prolonging survival in amyotrophic lateral sclerosis: efficacy of noninvasive ventilation and uncuffed tracheostomy tubes." 01/01/2013 - "Randomised and quasi-randomised controlled trials involving non-invasive or tracheostomy assisted ventilation in participants with a clinical diagnosis of amyotrophic lateral sclerosis, independent of the reported outcomes. " 10/01/2010 - "Tracheostomy in amyotrophic lateral sclerosis: a 10-year population-based study in Italy." 01/01/2009 - "Randomised and quasi-randomised controlled trials involving non-invasive or tracheostomy assisted ventilation in participants with a clinical diagnosis of amyotrophic lateral sclerosis. " 09/01/1995 - "The purpose of this study was to determine which pulmonary function variables best predicted the potential for prolonging survival of individuals with amyotrophic lateral sclerosis (ALS) by the use of physical medicine respiratory muscle aid alternatives to tracheostomy for ventilatory support and airway suctioning. "
2.	Ketogenic Diet 08/01/2013 - "Ultimately, determining whether a high-fat or ketogenic diet could be beneficial in amyotrophic lateral sclerosis will require large randomized, placebo-controlled clinical trials. " 08/01/2013 - "High-fat and ketogenic diets in amyotrophic lateral sclerosis." 01/01/2006 - "A ketogenic diet as a potential novel therapeutic intervention in amyotrophic lateral sclerosis." 11/01/2008 - "A growing body of literature suggests the ketogenic diet may be beneficial in certain neurodegenerative diseases, including Alzheimer disease, Parkinson's disease, and amyotrophic lateral sclerosis. " 01/01/2014 - "In regard to neurological disorders, ketogenic diet is recognized as an effective treatment for pharmacoresistant epilepsy but emerging data suggests that ketogenic diet could be also useful in amyotrophic lateral sclerosis, Alzheimer, Parkinson's disease, and some mitochondriopathies. "
3.	Transplantation (Transplant Recipients) 11/01/2007 - "We conclude that transplantation of adult OB-NPCs is therapeutic for mouse amyotrophic lateral sclerosis." 03/01/2014 - "The US Food and Drug Administration-approved trial, "A Phase 1, Open-Label, First-in-Human, Feasibility and Safety Study of Human Spinal Cord-Derived Neural Stem Cell Transplantation for the Treatment of Amyotrophic Lateral Sclerosis, Protocol Number: NS2008-1," is complete. " 03/01/2014 - "Intraspinal neural stem cell transplantation in amyotrophic lateral sclerosis: phase 1 trial outcomes." 01/01/2012 - "Our previous series of studies have proven that olfactory ensheathing cell (OEC) transplantation appears to be able to slow the rate of clinical progression after OEC transplantation in the first 4 months and cell intracranial (key points for neural network restoration, KPNNR) and/or intraspinal (impaired segments) implants provide benefit for patients (including both the bulbar onset and limb onset subtypes) with amyotrophic lateral sclerosis (ALS). " 01/01/2012 - "Mesenchymal stromal cell transplantation in amyotrophic lateral sclerosis: a long-term safety study."
4.	Positive-Pressure Respiration (PEEP) 03/01/2004 - "Noninvasive positive pressure ventilation (NIPPV) is associated with improved survival in amyotrophic lateral sclerosis (ALS) and has been widely recommended. " 03/01/2001 - "Objective measures of the efficacy of noninvasive positive-pressure ventilation in amyotrophic lateral sclerosis." 03/01/2001 - "The impact of noninvasive positive-pressure ventilation (NIPPV) on pulmonary function studies, quality of life, and survival was assessed in patients with amyotrophic lateral sclerosis. " 05/01/2013 - "Non-invasive positive-pressure ventilation in patients with amyotrophic lateral sclerosis: spinal versus bulbar form." 12/01/2012 - "Various breathing abnormalities (Neurology 2009; 73: 1218) have been proposed as indicators for the introduction of non-invasive positive-pressure ventilation (NIV) in patients with amyotrophic lateral sclerosis (ALS). "
5.	Electrodes (Electrode) 04/01/2015 - "We were also able to custom design, rapidly manufacture, safely implant, and confirm the efficacy of personalized electrodes, including the capability to attain meaningful high-gamma-band information in an amyotrophic lateral sclerosis patient. " 01/01/2014 - "We were also able to custom design, rapidly manufacture, safely implant and confirm the efficacy of personalized electrodes, including the capability to attain meaningful high gamma-band information in an amyotrophic lateral sclerosis patient. " 01/01/2015 - "This study examines the innervation zone (IZ) in the biceps brachii muscle in healthy subjects and those with amyotrophic lateral sclerosis (ALS) using a 20-channel linear electromyogram (EMG) electrode array. " 02/01/2014 - "This study presents a simple high-density surface EMG method for automatic detection of spontaneous action potentials from surface electrode array recordings of patients with amyotrophic lateral sclerosis. " 02/01/2014 - "Both analyses were applied to computer simulations of surface EMG baseline with the presence (representing activity data) or absence (representing reference data) of hidden muscle activity, as well as surface electrode array EMG baseline recordings of healthy control and amyotrophic lateral sclerosis (ALS) subjects. "