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Amyotrophic Lateral Sclerosis
Definition: 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)

Synonym(s): Lou Gehrig Disease / Motor Neuron Disease, Amyotrophic Lateral Sclerosis / ALS (Amyotrophic Lateral Sclerosis) / Amyotrophic Lateral Sclerosis With Dementia / Amyotrophic Lateral Sclerosis, Guam Form / Dementia With Amyotrophic Lateral Sclerosis / Gehrig's Disease / Guam Form of Amyotrophic Lateral Sclerosis / Lou Gehrig's Disease / Gehrig Disease / Gehrigs Disease / Lateral Scleroses, Amyotrophic / Lou Gehrigs Disease / Sclerosis, Amyotrophic Lateral
MeSH 2010 © U.S. National Library of Medicine®.
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  More information from the Genetics Home Reference Website
What is amyotrophic lateral sclerosis?
Amyotrophic lateral sclerosis is a progressive disease that affects the control of muscle movement by damaging motor neurons, which are specialized nerve cells in the spinal cord and the part of the brain that is connected to the spinal cord (the brainstem). More than 90 percent of amyotrophic lateral sclerosis cases occur in people with no family history of the disorder (sporadic cases). The cause of sporadic cases remains largely unknown. Only a small percentage of amyotrophic lateral sclerosis cases are caused by a known genetic mutation; these case ...

How common is amyotrophic lateral sclerosis?
An estimated 5,000 people in the United States are diagnosed with amyotrophic lateral sclerosis each year. Worldwide, this disorder occurs in 4 to 8 per 100,000 individuals. Only a small percentage of cases arise from a known genetic cause. About 3 percent of sporadic cases and 20 percent of familial cases are considered type 1. Types 2, 4, and 8 are rare disorders, reported in a small number of families.


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  Web resources for "Amyotrophic Lateral Sclerosis"
  English (47)  French (25)  German (100)  Spanish (1)    Italian (5)  Dutch (41)

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view the HON description of the site HONcode - Orphanet Journal of Rare Diseases | Full text | Amyotrophic lateral sclerosiswww.ojrd.com 2
view the HON description of the site HONcode - Amyotrophic Lateral Sclerosis - Symptoms, Causes, Treatments - Better Medicinewww.wrongdiagnosis.com 2
view the HON description of the site HONcode - ASNOM - Association Amicale Santé Navale et d'Outre Merwww.asnom.org  
view the HON description of the site HONcode - Amyotrophic lateral sclerosis - Genetics Home Referenceghr.nlm.nih.gov 15
view the HON description of the site HONcode - Amyotrophic Lateral Sclerosis: Lou Gehrig's Disease - March 15, 1999 - American Family Physicianwww.aafp.org  
view the HON description of the site HONcode - Amyotrophic Lateral Sclerosis: MedlinePluswww.nlm.nih.gov 5
view the HON description of the site HONcode - Amyotrophic lateral sclerosis (ALS) - MayoClinic.comwww.mayoclinic.com  
view the HON description of the site HONcode - Page Not Found: National Institute of Neurological Disorders and Stroke (NINDS)www.ninds.nih.gov 6
view the HON description of the site HONcode - Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig's Disease, Charcot's Disease)www.virtualneurocentre.com 2
view the HON description of the site HONcode - Amyotrophic Lateral Sclerosiswww.fpnotebook.com  
view the HON description of the site HONcode - ALS Overview - Amyotrophic Lateral Sclerosis (ALS)/Lou Gehrig's Disease - HealthCommunities.comwww.neurologychannel.com 5
view the HON description of the site HONcode - ALERT: Potential Marker for Amyotrophic Lateral Sclerosis Progression Identified | MediNEWS.Direct!www.medinewsdirect.com  
view the HON description of the site HONcode - Amyotrophic lateral sclerosiswww.healthcentral.com 3
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Broader term(s): - Spinal Cord Diseases - Central Nervous System Diseases - Nervous System Diseases - Motor Neuron Disease - Neurodegenerative Diseases

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  Medical image(s) for "Amyotrophic Lateral Sclerosis"
click below to enlargeAmyotrophic Lateral Sclerosis  (Chromosome 21)
www.ncbi.nlm.nih.gov
Copyright © NCBI - Genes and Disease Amyotrophic Lateral Sclerosis (Chromosome 21)
click below to enlargeFasciculations may be subtle or severe to the point of 'vermiculation' giving a 'bag of worms' appearance, for example, the infamous 'scrotal tongue' when the motor neurons of the 12th Cranial Nerve in the brainstem are affected.
www.idiom.com
Dr.John's Cybersuite Fasciculations may be subtle or severe to the point of "vermiculation" giving a "bag of worms" appearance, for example, the infamous "scrotal tongue" when the motor neurons of the 12th Cranial Nerve in the brainstem are affected.
Copyright © John M. Friedberg, M.D.
click below to enlargeALS - 3D Medical Animation
www.virtualrespiratorycentre.com
Copyright © Virtual Respiratory Centre ALS - 3D Medical Animation
Broader term(s): - Spinal Cord Diseases
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  Medical News for "Amyotrophic Lateral Sclerosis"
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Broader term(s): - Nervous System Diseases

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  Scientific articles from MEDLINE for "Amyotrophic Lateral Sclerosis"
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Subheadings: complications / diagnosis / embryology / epidemiology / etiology / metabolism / microbiology / surgery / therapy

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  Clinical Trials for "Amyotrophic Lateral Sclerosis"
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Broader term(s): - Spinal Cord Diseases - Central Nervous System Diseases - Nervous System Diseases - Motor Neuron Disease - Neurodegenerative Diseases

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Subheadings: complications / diagnosis / embryology / epidemiology / etiology / metabolism / microbiology / surgery / therapy


  Broader term(s):
      Diseases
          Nervous System Diseases
              Neuromuscular Diseases
                  Motor Neuron Disease 
              Neurodegenerative Diseases
                  Motor Neuron Disease 
              Central Nervous System Diseases
                  Spinal Cord Diseases 

MeSH 2010 © U.S. National Library of Medicine®.

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