Information on Amyotrophic Lateral Sclerosis from the National Institute of Neurological Disorders and Stroke
Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig's disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles. In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away, and twitch. Eventually the ability of the brain to start and control voluntary movement is lost. Individuals with ALS lose their strength and the ability to move their arms, legs, and body. Read more at the National Institute of Neurological Disorders and Stroke.
Information on ALS from the Robert Packard Center for ALS Research at Johns Hopkins
ALS is often referred to as a syndrome because the disease becomes apparent in various patterns. ALS occurs rarely and spontaneously. Currently, there is no cure for amyotrophic lateral sclerosis. Read extensive information at the Robert Packard Center for ALS Research at Johns Hopkins website.
Information on Amoytrophic Lateral Sclerosis from Mayoclinic.com
Amyotrophic lateral sclerosis at Mayoclinic.com.
Information on ALS from the ALS Association
Amyotrophic lateral sclerosis (ALS), often referred to as "Lou Gehrig's Disease," is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed. Read more at the ALS Association.
Living and dying with ALS
Slowly being robbed of the use of his body, Toronto Sun writer Jerry Gladman and his family come to grips with a frightening diagnosis. Read his story at focusonals.com.
Meals for Easy Swallowing
Swallowing can become a signficant problem for patients with ALS. The following collection of recipes is derived from our patients and their creative spouses who translated their caring into foods that look good, taste good, are easy to chew and swallow, and minimize discomfort. Learn more at the MDA-ALS division.
Milestones in ALS Research
Highlighting ALS Research Efforts. Supported By The Muscular Dystrophy Association Read the highlights at the Muscular Dystrophy Association.
Podcast: Life with Lou Gehrig's Disease
About two years ago, commentator Darcy Wakefield was diagnosed with ALS, or Lou Gehrig's disease, at age 33. A few months later, she wrote a commentary about running -- and losing her ability to do it. Since then, her disease has progressed, sometimes in ways she didn't anticipate. Listen to the podcast from the National Public Radio.
Video: Living with Lou Gehrig's Disease
Leo Greene, a reporter and videographer for the Inland Valley Daily Bulletin, was diagnosed with amyotrophic lateral sclerosis. Leo will explain his thoughts and feelings as he tackles the disease, and hopes to educate the public and bring more attention to an overlooked illness that afflicts more than 5,000 Americans each year. View the 4 min 17 sec video from www.dailybulletin.com
Podcast: Dietary Issues for Patients with Lou Gehrig’s Disease
In this nutrition podcast, registered dietician Nina Crowley goes over eating guidelines for patients with ALS, also referred to as Lou Gehrig’s disease. Listen to the podcast at the Medical University of South Caroline Podcast Library.
Practice Parameter Update: Caring for the Patient with Amytrophic Lateral Sclerosis
Drug, respiratory, and evidence based therapies. Read more at the American Academy of Neurology.
Abstract: Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis (ALS) is a fatal degenerative motor neuron disorder. Ten percent of cases are inherited; most involve unidentified genes. We report here 13 mutations in the fused in sarcoma/translated in liposarcoma (FUS/TLS) gene on chromosome 16 that were specific for familial ALS.Abstract available at Pubmed.
Full Text: Reduced expression of the Kinesin-Associated Protein 3 (KIFAP3) gene increases survival in sporadic amyotrophic lateral sclerosis
To identify genetic variants associated with susceptibility and phenotypes in sporadic ALS, we performed a genome-wide SNP analysis in sporadic ALS cases and controls. No SNPs were associated with risk of sporadic ALS, site of onset, or age of onset. We have identified a variant within the KIFAP3 gene that is associated with decreased KIFAP3 expression and increased survival in sporadic ALS. These findings support the view that genetic factors modify phenotypes in this disease and that cellular motor proteins are determinants of motor neuron viability. Full text available at Pubmed.
Full Text: Activated protein C therapy slows ALS-like disease in mice by transcriptionally inhibiting SOD1 in motor neurons and microglia cells
Activated protein C (APC) is a signaling protease with anticoagulant activity. Here, we have used mice expressing a mutation in superoxide dismutase-1 (SOD1) that is linked to amyotrophic lateral sclerosis (ALS) to show that administration of APC or APC analogs with reduced anticoagulant activity after disease onset slows disease progression and extends survival. Full text available at Pubmed.
Abstract: Smoking may be considered an established risk factor for sporadic ALS.
A 2003 evidence-based review of exogenous risk factors for sporadic amyotrophic lateral sclerosis (ALS) identified smoking as the only risk factor that attained "probable" (more likely than not) status, based on 2 class II studies. The purpose of the current, evidence-based, update was to see if the conclusion of the previous review needed to be modified, based on studies published since. Abstract available at Pubmed.
Full Text: Workplace Exposures and the Risk of Amyotrophic Lateral Sclerosis
Background
Occupation has been suggested to play a role in amyotrophic lateral sclerosis (ALS) etiology, but detailed information on the importance of specific workplace exposures is lacking.
Objectives
Our aim was to assess the relationship between workplace exposures and the risk of ALS and to evaluate potential interactions between these exposures and smoking. Free full text available at Pubmed.
Full Text: A conditioning lesion provides selective protection in a rat model of Amyotrophic Lateral Sclerosis.
Amyotrophic Lateral Sclerosis (ALS) is neurodegenerative disease characterized by muscle weakness and atrophy due to progressive motoneuron loss. The death of motoneuron is preceded by the failure of neuromuscular junctions (NMJs) and axonal retraction. Thus, to develop an effective ALS therapy you must simultaneously preserve motoneuron somas, motor axons and NMJs. A conditioning lesion has the potential to accomplish this since it has been shown to enhance neuronal survival and recovery from trauma in a variety of contexts. Free full text available at Pubmed.
Full Text: Latent cluster analysis of ALS phenotypes identifies prognostically differing groups.
Amyotrophic lateral sclerosis (ALS) is a degenerative disease predominantly affecting motor neurons and manifesting as several different phenotypes. Whether these phenotypes correspond to different underlying disease processes is unknown. We used latent cluster analysis to identify groupings of clinical variables in an objective and unbiased way to improve phenotyping for clinical and research purposes. Free full text available at Pubmed.
Full Text: Depression and Quality of Life in Patients With Amyotrophic Lateral Sclerosis
There is increasing debate on the issue of whether to facilitate the end-of-life decisions of severely disabled patients with diseases such as amyotrophic lateral sclerosis (ALS). The rationale for not providing life-sustaining treatment to severely disabled patients is that a poor quality of life is expected after such treatment. Our studies have shown, however, that ALS patients can experience a satisfactory quality of life without depressive manifestations even if they are severely physically impaired, including in the terminal phase. Full text available at Pubmed.
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