ALS is a progressive and fatal neurodegenerative disease that attacks the nerve cells responsible for muscle movement. There is no cure or treatment.
Click to enlarge (credit Detroit Free Press)
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is a progressive and fatal neurodegenerative disease that attacks the nerve cells in the brain and spinal cord responsible for voluntary muscle movement, known as motor neurons.
In ALS, the motor neurons deteriorate leading to muscle weakness, paralysis, atrophy and eventually respiratory failure. As this happens, the muscle tissues become hardened and scarred because no movement is being stimulated. The disease is characterized by individuals being unable to move any part of their body, unable to speak or communicate verbally, and dependent on a feeding tube for nutrition.
Other nerve cells, such as sensory neurons that bring information from sense organs to the brain, remain healthy. As a result, none of the senses—hearing, eyesight, smell, taste, or touch—are impeded by ALS. In most cases, cognitive functions are also unaffected, leaving people with ALS prisoners in their own bodies.
The average life span post-diagnosis is two to five years. There is no cure or treatment.
Common questions
▾ What does "ALS" mean?
ALS is an abbreviation for amyotrophic lateral sclerosis. This means:
Amyotrophic: absence of muscle nourishment
Lateral: the sides of the spinal cord where motor neurons are located
Sclerosis: the part of the spinal cord that is affected by the disease develops hardened, scarred tissue
▾ Are there other names for ALS?
In the United States, the disease is usually called Lou Gehrig's Disease or ALS. Canada, South America, Belgium, Netherlands, and Israel also use the name "ALS". In France, ALS is known as Maladie de Charcot (Charcot’s Disease). In the United Kingdom, Australia, and parts of Europe, ALS is referenced as Motor Neurone Disease, or MND.
▾ Who gets ALS?
Like cancer, anyone can get ALS. The disease is most common in people between the ages of 40 to 70. It affects people of all genders, ages, races, ethnicities, and socioeconomic statuses.
▾ Can ALS run in families?
Yes. About 10 percent of ALS cases are familial, while the remaining 90 percent are sporadic. C9ORF72 and SOD1 are the most common genes associated with familial ALS. Learn more here
▾ How common is ALS?
In the United States, someone is diagnosed with and someone dies of ALS every 90 minutes. That's about 5,600 people dying and being diagnosed each year. There are about 30,000 people living ALS in the U.S. and about 500,000 worldwide. ALS is considered an orphan disease because it affects a small percentage of the general population.
The worldwide incidence rate (new cases) of ALS is 2 per 100,000 people and the prevalence rate (total cases) is 6 per 100,000 people.
▾ How is the disease diagnosed?
There is no definitive test for ALS. It is often diagnosed through a combination of general diagnostic tests and a process of elimination. General diagnostics can include genetic testing (for familial ALS), electromyography tests, MRIs, and muscle biopsies, among other tests.
▾ How long do people live with ALS?
On average, people live with ALS for 2 to 5 years after diagnosis. Approximately 50 percent of all patients live with ALS for 3 or more years. 20 percent of all patients live more than 5 years, but only half of those live longer than 10 years. Half of those diagnosed with ALS live for 3 years or less.
▾ How quickly does the disease progress?
There is no set progression curve. ALS develops differently in each person.
▾ Is there a treatment or cure?
No effective treatment has been identified to stop the disease's progression or prevent onset. Both Rilutek and Radiclava are drugs that have been approved for use in the treatment of ALS in the United States, however both only offer a very small effect, which, in the case of Rilutek, has usually been described as a three-month life extension.
▾ Are certain populations more likely to develop ALS?
Yes. Veterans are twice as likely as the general population to develop the disease, though it is not known why (ALS is considered a service-related condition).
While there is not an increased risk known for athletes, many professional football players have been diagnosed with the disease. It is believed that CTE, toxic plaque on the brain that can develop from multiple head injuries, may have a role.
Recent statistical studies suggest that the disease is more prevalent in white populations than it is in African American, Asian or Hispanic populations. Additionally, for individuals under 65, ALS affects men at a higher rate than it does women.
▾ Are there related diseases to ALS?
Yes. Other neurodegenerative diseases include Alzheimer's, Parkinson's, Multiple Sclerosis (MS), and Frontotemporal Dementia. Scientific discoveries for one disease can help lead to further breakthroughs for the others.
It's estimated that by 2025, 1 in every 25 Americans will be diagnosed with a neurodegenerative disease, and that half of the current population will be diagnosed with one these disease by the time they're 80 years old.
Additionally, aside from ALS, the category of motor neurone diseases also includes conditions such as Primary Lateral Sclerosis (PLS), Progressive Bulbar Palsy (PBP), and and Progressive Muscular Atrophy (PMA).
The content contained on this page is for informational purposes only and is not intended as verified medical information or advice.