ALS (Lou Gehrig’s disease): what you need to know

Celebrities, sports figures and media personalities have been doused with frigid water in the Ice Bucket Challenge to raise awareness and money for Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease.

But how many people actually know what the disease is? Below is an overview of ALS and some frequently asked questions.

What is ALS?
It is a “rapidly progressive” and fatal neurological disease that attacks nerve cells called motor neurons that control voluntary muscles in the body, such as in the arms, legs and face.

How does the disease work?
Located in the brain and spinal cord, the neurons are responsible for transmitting electrical signals from the brain to the muscles that control the voluntary movements.

Over time, the neurons degenerate and cannot transmit the electrical impulses to the muscles, which cause the latter to weaken and become smaller (atrophy).

Since the neurons are unable to regenerate, the disease is not reversible.

Why is it also called Lou Gehrig’s Disease?
Baseball player Lou Gehrig, who played for the New York Yankees from 1923 to 1939, was diagnosed with ALS in 1939 and died in 1941 at the age of 37. At the peak of his career, he was known as “Ironman” due to his strength and agility.

What are some of the symptoms?

• Unsteady gait
• Extreme fatigue
• Muscle twitching and cramping
• Weak muscles
• Difficulty in swallowing and chewing
• Slurred speech
• Tripping or falling

While some ALS sufferers who are at the later stages of the disease are confined to a wheelchair, other people whose breathing muscles are affected require ventilation support.

How is the disease diagnosed?
Since some of the symptoms of ALS are associated with other diseases, it can be difficult for doctors to diagnose it.

A series of diagnostic tests are done, such as electrodiagnostic tests including electomyography and nerve conduction study, magnetic resonance imaging, muscle and/or nerve biopsy, and other examinations.

Is the disease genetic?
ALS strikes people without family history of the disease in 90 per cent of cases, according to ALS Canada. It can occur at any age but is most often diagnosed in middle or late adulthood.

What are the mortality rates?
Based on ALS Canada’s data, 80 per cent of people with ALS die within two to five years of being diagnosed, while others die within a few months. Ten per cent of those affected can live 10 years or longer.

How many people are affected?
The Stem Cell Network says around 3,000 Canadians and 30,000 Americans live with ALS. It affects people of all ethnic backgrounds, and men have a higher risk (1 in 400) than women (1 in 500) in developing ALS.

Can ALS be treated?
There is no known cure or treatment to reverse the disease. However, there are some drugs that can help to control the symptoms.

Various associations, such as ALS Canada, provide help and information for people living with ALS on how to cope with the disease. Below is its Manual for People Living with ALS.

ALS Canada: 2012 Manual for People Living with ALS

Click here to read the manual on your mobile or tablet device.