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What is ALS?

ALS stands for Amyotrophic Lateral Sclerosis, also called Lou Gehrig’s disease in North America and motor neuron disease in the UK. It is a rapidly progressive and fatal neuromuscular disease that is characterized by degeneration of a select group of nerve cells and pathways (motor neurons) in the brain and spinal cord. This loss of motor neurons leads to progressive paralysis of the voluntary muscles. The heart is not a voluntary muscle, and therefore remains unaffected by the disease. However, since breathing is controlled voluntarily by the chest muscles, death usually occurs when the chest muscles are no longer able to help the lungs adequately expel carbon dioxide.
“Amyotrophic” means:

A = absence of
myo = muscle
trophic = nourishment
lateral = side (of spine)
sclerosis = hardening or scarring

ALS is not contagious, but it is fatal. For the most part, the battle is short, with 80 per cent losing their lives within two to five years of diagnosis. Between 10 and 20 per cent live 10 years or more after diagnosis, while others live only a few months. The cause is unknown, but research is being conducted in areas relating to genetic predispositions, viral or infectious agents, environmental toxins and immunological changes.

For some people, the muscles for speaking, swallowing or breathing are the first to be affected. This is known as bulbar ALS. The term “bulbar” refers to the motor neurons located in the brain stem that control the muscles used for chewing, swallowing and speaking. ALS symptoms, and the order in which they occur, vary from one person to another.

In the majority of cases, ALS affects the lower portion of the spinal cord first. This is known as limb onset ALS. In these cases, the first signs of ALS are usually muscle weakness, cramps and weakened reflexes in the arms and legs. The rate of muscle loss can vary significantly from person to person with some patients having long periods with very slow degeneration, and others experiencing rapid degeneration.