WHAT IS ALS/MND?

Amyotrophic Lateral Sclerosis or Motor Neurone Disease is a progressive, usually fatal neuromuscular disease. It attacks motor neurones in the spinal cord and lower brain which transmit signals from the brain to the voluntary muscles throughout the body. When motor neurones die as a result of ALS/MND, the ability of the brain to control muscle movement is lost. When muscles fail to receive messages, they weaken and waste away (atrophy). ALS/MND is also known as Lou Gehrig's Disease and Maladie de Charcot, after Jean-Martin Charcot, the French neurologist who first described it in 1869.

WHAT ARE THE SYMPTOMS OF ALS/MND?

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 (called fasciculations) and mood swings including 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 onset, which means that the throat, 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/MND. Muscle wasting gradually spreads to the muscles of the trunk of the body, and the disease eventually affects swallowing, chewing and breathing. Complete paralysis ultimately results, usually occurring within two to five years of diagnosis. When the diaphragm is attacked, the patient is unable to breathe unaided and faces permanent ventilatory support in order to survive.

ALS/MND attacks only 'motor' neurones so 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: "With keen mind and open eyes we watch ourselves die."

WHAT IS THE AVERAGE LIFE EXPECTANCY?

Between two and five years for the newly diagnosed person, although improved medical care is resulting in longer and more productive lives for people with ALS/MND. Twenty percent will live more than five years and up to ten percent will survive more than ten years.

HOW COMMON IS ALS/MND?

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/MND is considered 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 USA are newly diagnosed with ALS each year. That's about 13 new cases every day. Many scientists believe the incidence of ALS/MND is increasing, though some feel this may be due to improved understanding of the illness and better diagnostic techniques. ALS/MND affects about seven or 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/MND. 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/MND. There are around 1300 people in Australia with ALS/MND and on average one person dies each day from ALS/MND.

HOW IS ALS/MND DIAGNOSED?

At present there is no definitive means of diagnosis of ALS/MND. Most diagnoses are made by eliminating all other possibilities - ailments with symptoms resembling those of ALS/MND. Neurologists use a number of clinical tests to establish a profile, including blood testing, MRI (Magnetic Resonance Imaging) scans and EMGs (Electro Myographs).

WHO IS LIKELY TO GET ALS/MND?

Anyone can get ALS/MND. Most who develop ALS/MND 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/MND occurs in greater percentages as men and women grow older. ALS/MND strikes men slightly more frequently than women. About one in 800 adult men, and one in 1,200 adult women die of ALS/MND. ALS/MND occurs throughout the world with no racial, ethnic or socioeconomic boundaries. 90+% of ALS/MND cases are people with no family history of the disease. The remaining cases are classified as familial or inherited ALS/MND.

WHAT DOES "AMYOTROPHIC LATERAL SCLEROSIS" MEAN?

"A-myo-trophic" comes from the Greek. "A" means no or negative, "Myo" refers to muscle and "Trophic" means nourishment: thus "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.

WHY IS IT ALSO KNOWN AS "LOU GEHRIG'S DISEASE"?

Lou 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/MND.

ALS/MND claimed the lives of actor David Niven, US Senator Jacob Javits and Jon Stone, co-creator of Sesame Street, died in 1997 of ALS/MND. Physicist Stephen Hawking is still alive 34 years after diagnosis and communicates using his thumb to operate a voice synthesizer.

ARE THERE DIFFERENT TYPES OF ALS/MND?

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

WHEN WAS ALS/MND FIRST DISCOVERED?

ALS/MND was first described in 1869 by Jean-Martin Charcot, an innovative French neurologist. The disease was first known as "Maladie de Charcot".

WHAT CAUSES ALS/MND?

The cause of ALS/MND 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 (the SOD or Superoxide Dimutase gene). While only 10% of ALS/MND 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/MND occurs in those with a genetic predisposition to motor neuron degeneration, and that the illness is then triggered by environmental factors. In Nov 1996, Dr. Jeffrey Rothstein of Johns Hopkins University found that 42% of sporadic ALS/MND patients have a defect in the gene that controls the protein EAAT2, a protein that helps to regulate the brain's glutamate levels, well known to be a factor in motor neuron degeneration.

CAN YOU "CATCH" ALS/MND?

It cannot be "caught" and is not contagious. Though some scientists believe it is possible that ALS/MND is caused by a slow-acting or latent "virus", there is absolutely no evidence that it is contagious: there is no increased incidence among medical personnel who deal with ALS/MND patients.

WHAT ABOUT ENVIRONMENTAL CAUSES?

The very high incidence of ALS/MND 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 aluminum, which can poison the body and cause ALS/MND symptoms.

IS THERE HOPE FOR PEOPLE WITH ALS/MND?

ALS/MND can have a devastating effect on patients and their families. As they struggle to cope with the prospect of advancing disability and death, it consumes their emotional and financial reserves. It is a costly disease in its later stages, demanding both extensive nursing care and expensive equipment. But there is always hope. Recent medical discoveries have created new drugs that seem to slow the progression of the disease. Others have shed light on factors that will encourage the regrowth of nerve cells. Advances in knowledge of other neurological diseases also continue to shed light on causes and treatment of ALS/MND.

WHAT CAN I DO TO HELP?

ALS/MND takes a tremendous toll on the physical, financial, and emotional resources of its victims and their families. ALS/MND societies and support groups around the world do much to help, but there's always need for more. If you know someone with ALS/MND, offer your time to relieve the family members of the constant strain. Or just talk, listen, 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, specialised equipment, therapy, and nursing care, for an open-ended time frame. 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/MND. Support groups, universities, medical and scientific associations, drug companies, and many individuals are represented, most with links to other sites.

Click on the ZazAngels logo for links to a variety of  important and informative ALS-related websites:

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Links -- ALS Association Websites and Other Sites of Interest 

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Copyright © 1999 by Michael Zaslow's ZazAngels. All rights reserved.
01/04/06 05:14:21 PM