What is ALS/MND?
What are the symptoms of ALS?
What is the average life expectancy?
How common is ALS?
How is ALS diagnosed?
Who is liable to get ALS?
What does "Amyotrophic Lateral Sclerosis" mean?
Why is it also known as "Lou Gehrig's Disease"?
Are there different types of ALS?
When was ALS first discovered?
What causes ALS?
Can you "catch" ALS?
What about environmental causes?
Is there any treatment available?
What can I do to help?
Where can I learn more?
What is ALS/MND?
ALS/MND---ALS stands for Amyotrophic Lateral Sclerosis. Although the name Motor Neurone Disease is used synonymously in some countries, ALS is actually just one of several diseases categorized as Motor Neuron Diseases. In the USA, ALS is also known as Lou Gehrig's Disease, and in French as Maladie de Charcot. ALS is a rapidly progressive neuromuscular disease. It attacks motor neurons in the spinal cord and lower brain which transmit signals from the brain to the voluntary muscles throughout the body. When motor neurons die as a result of ALS, the ability of the brain to control muscle movement is lost. When muscles fail to receive messages, they weaken and atrophy. Weakness progresses to paralysis and death occurs from respiratory failure. Respiratory failure can be prevented only if the patient opts to be placed on a ventilator. If ventilated, the patient may live for many more years. The paralysis however will continue to progress until all movement is lost. There is no known cure.

What are the symptoms of ALS?
The groups of muscles affected and the order in which they are affected varies from one person to another. Early symptoms usually include tripping, dropping things, abnormal fatigue of the arms and/or legs, slurred speech, muscle cramps and twitches and uncontrollable periods of laughing or crying. The hands and feet may be affected first, causing difficulty in walking or using the hands for the activities of daily living such as dressing, washing and buttoning clothes.

About 25% of patients have bulbar (throat) onset, which means that voice and swallowing are first affected. About 50% have arm onset, and 25% leg onset.

The disease frequently takes its toll before being positively diagnosed: many patients are significantly debilitated before learning that they have ALS.

Muscle wasting gradually spreads to the muscles of the trunk of the body, and the disease eventually affects swallowing, chewing and breathing. Complete paralysis eventually results, usually occurring within two to five years of diagnosis.

When the diaphragm is attacked, the patient is unable to breathe for him/herself, and faces permanent ventilatory support in order to survive.

ALS attacks only 'motor' neurons. Sight, touch, hearing, taste, smell and muscles of the eyes and bladder are generally not affected. Sexual function and drive are not affected. The mind is not affected, and remains sharp despite the progressive degenerating condition of the body.

Dennis Kaye, a Canadian ALS victim, wrote an inspirational book on his struggle: "Laugh? I Thought I'd Die". He said of his like: "With keen mind and open eyes We watch ourselves die."

What is the average life expectancy?
This is between two and five years for the newly diagnosed person. Improved medical care is resulting in longer and more productive lives for people with ALS. Twenty percent will live more than five years and up to 10% will survive more than ten years.

Back to top

How common is ALS?
Once thought rare, ALS is now fairly common.

In terms of INCIDENCE (how often does it strike), a normal population produces about 2 new cases per 100,000 every year. (A 1995 Dalhousie University Study found in that there was a 2.03 per 100,000 population incidence in Nova Scotia.)

(As ALS is terminal and incurable, death rates are very close to incidence rates.)

According to the United States National Institute of Health, some 5,000 people in the U.S. are newly diagnosed with ALS each year. (That's about 13 new cases every day!)

Many scientists believe the incidence of ALS is increasing, though some feel this may be due to improved understanding of the illness and better diagnostic techniques.

In terms of PREVALENCE (how many are affected at any one time), ALS affects about seven to eight people out of every 100,000.

It is estimated that as many as 30,000 Americans have the disease at any given time, and that some 300,000 Americans who are alive and apparently well today will die with ALS. In Canada, from a population of about 30 million, two to three Canadians die every day of ALS. Over 2,500 Canadians currently have ALS. Some 20,000 to 25,000 Canadians who are alive and apparently in good health will die of ALS.

How is ALS diagnosed?
At present there is no definitive means of diagnosis of ALS. Most diagnoses are made by eliminating all other possibilities--ailments whose symptoms resemble those of ALS. Neurologists use a number of clinical tests to establish a profile, including blood testing, EMG, MRI, etc.

Who is liable to get ALS?
Anyone can get ALS. Most who develop ALS are between the ages of 40 and 70. There are, however, cases of the disease attacking persons in their twenties and thirties. Generally though, ALS occurs in greater percentages as men and women grow older. ALS strikes men slightly more frequently than women. About one in 800 adult men, and one in 1,200 adult women die of ALS.

ALS occurs throughout the world with no racial, ethnic or socioeconomic boundaries.

90% of ALS cases are people with no family history of the disease. Ten percent of the cases are classified as familial or inherited ALS.

What does "Amyotrophic Lateral Sclerosis" mean?
"A-myo-trophic" comes from the Greek language. "A" means no or negative. "Myo" refers to muscle, and "Trophic" means nourishment---"No muscle nourishment." When a muscle has no nourishment, it "atrophies" or wastes away. "Lateral" identifies the areas in a person's spinal cord where portions of the nerve cells that nourish the muscles are located. As this area degenerates it leads to scarring or hardening--"sclerosis"--in the region.

Back to top

Why is it also known as "Lou Gehrig's Disease"?
In other countries, ALS is sometimes called Motor Neuron Disease. This is actually a broader category which includes diseases besides ALS. In the USA, it is called Lou Gehrig Disease after Lou Gehrig. Gehrig, "The Iron Horse", was a baseball superstar in the late 20's and 30's, one of the "Golden Ages" of baseball. After 14 glory-filled seasons, Lou Gehrig's career, and his life, were cut short by ALS. Though today LouGehrig's name may bring "the illness" and "the streak" to mind, he was in his day a true superstar. His accomplishments will forever keep him among the legends of the game. Gehrig hit for power, hit for average, and was a consistent run producer. He was twice named the American League's Most Valuable Player. In his 14 full seasons the Yankees won seven World Series championships. The Wall Street Journal recently ran an article which stated that statistically, as measured by "contribution to team success" Lou Gehrig was the greatest player of all time, and that his compensation in today's market would exceed $10 million per year.

Are there different types of ALS?
There are three common classifications, and numerous sub-categories:
Sporadic: the most common form of ALS
Familial: less than 10% of ALS cases suggest genetic inheritance
Guamanian: a high number of cases of ALS occur in Guam and the Trust Territories of the Pacific

When was ALS first discovered?
ALS was first described in 1869 by Jean-Martin Charcot, a trail-blazing French neurologist.

What causes ALS?
The cause of ALS is not yet known. While many theories are being researched, at present neither a cure nor a means of prevention is known.

In 1993, scientists announced in a paper published in the British journal "Nature" that they had isolated the gene associated with about 20% of the cases of the inherited form of the disease. While only 10% of ALS patients have this genetic predisposition, there is no evidence of a clinical difference between the familial and the sporadic forms of the illness.

A currently favoured theory combines genetics and toxicity (poisons): that sporadic ALS occurs in those with a genetic predisposition to motor neuron degeneration, and that the illness is then triggered by environmental insult. In Nov 1996, Dr. Jeffrey Rothstein of Johns Hopkins University found that 42% of sporadic ALS patients have a defect in the gene that controls the protein EAAT2, a protein which helps to regulate the brain's glutamate levels, well known to be a factor in motor neuron degeneration.

Back to top

Can you "catch" ALS?
ALS cannot be "caught" -- it is not contagious. Though some scientists believe it is possible that ALS is caused by a slow-acting or latent "virus", there is absolutely no fear that it is contagious: there is no increased incidence among medical personnel who deal with ALS patients.

What about environmental causes?
The very high incidence of ALS on the island of Guam, in Western New Guinea and on Kii peninsula of Japan may provide some clues about environmental influences. Heavy metals such as lead and mercury are suspected causes, as is aluminium, which can poison the body and cause ALS symptoms.

Is there any treatment available?
While no drug has been found which has been proven to prevent, reverse, or stop the progression of ALS, a drug which may slow the progression is now available. Rilutek has been shown to extend life expectancy by approximately three months. Earlier use of Rilutek or use of Rilutek in combination with other drugs may prove even more effective. Information on Rilutek is available from your doctor or from several websites including this one from its manufacturer:

http://www.alsinfo.com

Note -- This info is tailored for US customers. Information for other countries is available but limited to contact phone numbers. To access them, go to: http://www.rp-rorer.com/

Other drugs being developed include the Sanofi drug (SR57746A) and various nerve growth factors. These drugs remain in the research stage.

Other areas of on-going research focus on finding the cause rather specific treatment. This includes investigation into a possible viral link, the role of calcium binding proteins, coenzyme Q10, enzyme SOD1, and glutamate toxicity.

Efforts also continue to identify and develop tests for the presence of additional autoantibodies in patients with Motor Neuron Diseases. The presence of antibodies suggest the patient may benefit from immunosuppressive treatments. The discovery of some of these antibodies and successful treatment of patients has resulted in a revision of the diagnostic criteria for ALS. Similarly, there is new emphasis on the possible misdiagnosis of some patients with Lyme disease as having ALS.

Drugs such as Neurontin, Baclofen, snake venom, etc. were developed for other diseases and continue to mentioned as possibly helpful in ALS. At this time none of these drugs have been proven effective in ALS although some continue to be tested. Because these drugs are already approved for other uses by regulating agencies they can be prescribed by physicians and are therefore sometimes used by individual doctors outside of a formal research program. This may be in response to a specific request by a patient, as an attempt to control a specific symptom in a particular patient, or simply the individual doctor's belief that he has seen some positive effect from use.

While conventional medicine cannot offer a cure or treatment that slows progression substantially, it does offer supportive care such as medication to ease muscle spasms, techniques to reduce the risk of choking, varying levels of respiratory support, therapy to maximize mobility and flexibility, and equipment to assist with activities of daily living.

Alternative medicine also offers a variety of treatments. The value of these treatments varies considerably. Some, such as use of dietary supplements containing anti-oxidants, creatine, Vit. E, are possible solutions suggested by medical research. Many of these products are now being evaluated in controlled tests, but as "natural" products they are readily available over the counter and so are being widely used by individual ALS patients. Some of these may have benefit, others may turn out to be just the latest fad in ALS treatment.

Other treatments such as removal of dental mercury filling have been tested and rejected by the medical community but continue to have strong supporters and create controversy.

Still others continue to be promoted despite a lack of any scientific basis. In general, ALS patients should be highly suspicious of treatments supported only by testimonial evidence (recommendations from supposed users), those purported to be effective in a large number of unrelated diseases, those which have been available for many years but never subjected to controlled research, and those offered by only a single doctor or clinic.

Back to top

What can I do to help?
ALS takes a tremendous toll on the physical, financial, and emotional resources of its victims and their families. ALS societies and support groups around the world do much to help, but there's always need for more.

If you know someone with ALS, offer your time to relieve the family members of the constant strain. Or just to talk, to listen, to be a friend.

Spread the word. We must continue to raise public awareness of this devastating killer. Get involved, find out, write, phone, talk, shout about it.

Give money. Patients often need expensive home renovations, specialized equipment, therapy, and nursing care, for an open-ended time frame. And science needs funding to intensify the search for cause and cure, so we can eliminate this nightmare.

Where can I learn more?
The Internet and World Wide Web contain a tremendous amount of information on ALS. Support groups, universities, medical and scientific associations, drug companies, and many individuals are represented, most with links to other sites. A separate FAQ on ALS Internet Resources will be posted regularly along with this FAQ.

This "ALS Frequently Asked Questions" is posted at the http://www.alslinks.com newsgroup "sci.med.diseases.als"

For updated information on the latest research and developments, please visit the ALS Association's website: www.alsa.org/patient/news.cfm?CFID=2245065&CFtoken=4715066

MDA's ALS Division website: als.mdausa.org

Back to top

© 2000-2005 Charlie Wedemeyer Family Outreach. All rights reserverd.
Designed and hosted by Quixotic Design
Email questions or comments to
info@cwfo.org