What is Amyotrophic Lateral Sclerosis (ALS)?

Amyotrophic Lateral Sclerosis is also known as Lou Gehrig’s disease or maladie de Charcot. It is a neuromuscular disorder that affects the nerve cells and spinal cord progressively leading to the paralysis of the muscles. ALS progresses rapidly and is usually fatal within five years following the initial diagnosis.

Jean-Martin Charcot (known as the founder of modern neurology) was a French neurologist and was the first to describe the symptoms that define amyotrophic lateral sclerosis, or ALS. Known in France as Maladie de Charcot, the disease is more commonly known in the U.S. as Lou Gehrig’s Disease, after the famous baseball player who died of Amyotrophic Lateral Sclerosis. It is a neuromuscular disease characterized by the degeneration of precise nerve cells in the brain and spinal cord. Little by little, this deterioration affects the brain’s capacity to move and control voluntary muscles. With a lack of stimulation to these muscles by the brain, they begin to atrophy, leading to progressive paralysis.

ALS is the most commonly known and common type of motor neuron disease, which also includes primary lateral sclerosis, as well as various forms of paralysis and partial paralysis.

Currently, about 2,500 to 3,000 people in Canada suffer from this disease and two new cases are reported per 100,000 people every year. This disease is not contagious and most of the reported cases are sporadic: meaning that no one in the affected person’s immediate or extended family has had ALS. The disease is hereditary in only 5 to 10% of cases, and mostly affects people between the ages of 40 and 75; cases in children are extremely rare.

To date, there is no treatment to significantly prolong the life of patients with ALS. Hope lies in the important actions by the associations, volunteers, ambassadors and researchers who all share the same mission of eradicating this devastating disease. Fundraising events and the generous contributions of donors support patient services and research in finding the most effective treatments for Amyotrophic Lateral Sclerosis, and eventually a cure.

Some facts about ALS

  • ALS is not contagious.
  • Origin of the name:
    • A = absence of
    • myo = muscle
    • trophic = nourishment
    • Lateral = side (of spine)
    • Sclerosis = hardening
  • ALS may also be called:
    • Lou Gehrig’s Disease
    • Motor Neuron Disease (MND)
    • Maladie de Charcot
  • ALS usually strikes between the ages of 40 and 75.
  • Approximately 2,500 to 3,000 Canadians over 18 years old currently live with ALS.
  • Juvenile ALS is extremely rare.
  • ALS affects all ethnic and socio-economic groups.
  • The mortality rate, meaning the number of new diagnosis per year is approximately 2 per 100,000people.
  • ALS is a fatal disease with no effective treatment or cure.
  • Life expectancy is usually between two and five years, but a small percentage of people live for 10 years or more.
  • More people die every year of ALS than of muscular dystrophy, multiple sclerosis and cystic fibrosis combined.
  • ALS is the most common cause of neurological death in Canada.
  • ALS affects slightly more men than women.