NINDS Amyotrophic Lateral Sclerosis (ALS) Information Page

What is Amyotrophic Lateral Sclerosis (ALS)?

Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig's disease or classical motor neuron 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.  Symptoms are usually first noticed in the arms and hands, legs, or swallowing muscles.  Muscle weakness and atrophy occur on both sides of the body.  Individuals with ALS lose their strength and the ability to move their arms and legs, and to hold the body upright. When muscles in the diaphragm and chest wall fail to function properly, individuals lose the ability to breathe without ventilatory support.  The disease does not affect a person's ability to see, smell, taste, hear, or recognize touch.  Although the disease does not usually impair a person's mind or personality, several recent studies suggest that some people with ALS may develop cognitive problems involving word fluency, decision-making, and memory.  The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others.

Is there any treatment?

What is the prognosis?

What research is being done?

• New gene mutations linked to ALS and nerve cell growth dysfunction
  Researchers have linked newly discovered gene mutations to some cases of the progressive fatal neurological disease amyotrophic lateral sclerosis – ALS, also known as Lou Gehrig’s disease. Shedding light on how ALS destroys the cells and leads to paralysis, the researchers found that mutations in this gene affect the structure and growth of nerve cells.
• Genetic mutation linked to inherited forms of ALS, dementia
  Scientists have identified the most common genetic cause known to date for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).
• New model of ALS is based on human cells from autopsied tissue
  By isolating cells from patients' spinal tissue within a few days after death, researchers funded by the National Institute of Neurological Disorders and Stroke have developed a new model of amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease.  They found that during the disease, cells called astrocytes become toxic to nerve cells – a result previously found in mouse models of familial ALS. The new study shows that astrocytes derived from people with sporadic ALS are also toxic to neurons.
• Induced Pluripotent Stem Cells Give Investigators a New Window into Neurological Disease
  Update on the three consortia funded by NINDS through ARRA to develop iPS cell lines from individuals with Parkinson’s disease, amyotrophic lateral sclerosis (ALS), and Huntington’s disease.
• New Links Found between Familial and Sporadic ALS
  In people with sporadic ALS, it is possible to detect SOD1 protein that is genetically normal but has properties similar to mutant SOD1 protein associated with about 20% of familial ALS cases.
• Spinocerebellar Ataxia 2 Gene is a Risk Gene for ALS
  Repeat expansions in the ataxin-2 gene are known to cause spinocerebellar ataxia type 2, but it turns out that smaller expansions in the gene increase the risk of ALS.
• Advances in Disease Modeling for ALS and FTD Workshop: Executive Summary
   
• MicroRNA Triggers Protective Response in Mice with ALS
  New study shows that a molecule called microRNA-209 mounts a protective response to repair nerve-muscle connections in mice with ALS.
• Therapy Investigated for ALS May Find New Role in Kennedy’s Disease
  Mouse model of SBMA is rescued by transgenic mIGF-1.
• Genetic Factor Extends Survival in People with ALS
  In a genome-wide association study, researchers identified a genetic factor that extends the survival of people with ALS.
• Support Cells Trigger Neuron Death in ALS
  2007 news article on research showing that astrocytes with SOD1 mutations produce a toxin that kills motor neurons.
• Large-Scale Gene Study Identifies Clues about Sporadic ALS
  2007 news article about a whole-genome association study of sporadic ALS, from the National Institute of Neurological Disorders and Stroke (NINDS).
• Neurons Grown From Embryonic Stem Cells Restore Function In Paralyzed Rats
  Press Release: Neurons Grown From Embryonic Stem Cells Restore Function In Paralyzed Rats
• What's Old is New Again - Antibiotic Protects Nerves By Removing Excess Glutamate
  February 2005 news article on research showing that the antibiotic ceftriaxone delays nerve damage in a mouse model for amyotrophic lateral sclerosis (ALS).
• Senataxin Gene Linked to Juvenile-Onset ALS
  June 2004 news summary on discovery of a gene for a rare, juvenile-onset form of amyotrophic lateral sclerosis (ALS).
• Misbehaving Molecules: 3-Dimensional Pictures of ALS Mutant Proteins Support Two Major Theories About How the Disease is Caused
  May 2003 press release on research showing how mutant proteins may lead to familial amyotrophic lateral sclerosis (ALS).
• Amyotrophic Lateral Sclerosis (ALS) Fact Sheet
  
  Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's disease) fact sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
• Doubling Up: Researchers Combine a Common Dietary Supplement with an Antibiotic to Treat Lou Gehrig's Disease
  January 2003 news summary on a drug combination that slows the progression of amyotrophic lateral sclerosis (ALS) in an animal model.
• Minocycline Delays Onset and Slows Progression of ALS in Mice
  May 2002 news summary on a possible new treatment for amyotrophic lateral sclerosis (ALS)
• Testimony on Amyotrophic Lateral Sclerosis, May 2000
  NINDS Director's testimony before the Senate Appropriations Subcommittee on Labor, Health and Human Services, Education and Related Agencies on Amyotrophic Lateral Sclerosis (ALS), May 18, 2000.