Amyotrophic lateral sclerosis (also known as Lou Gehrig's disease) is a devastating neurodegenerative disease. Those living with the disease become progressively paralyzed due to degeneration of the upper and lower motor neurons in the brain and spinal cord.
For reasons that are not completely understood, motor neurons in the brain and spinal cord begin to disintegrate. Because these damaged nerves to the body do not carry signals from the brain, the muscles begin to weaken and deteriorate from the lack of stimulation and use.
The first signs of progressive paralysis are usually seen in the hands and feet. They include weakness in the legs, difficulty walking, and clumsiness of the hands when washing and dressing. Eventually, almost all muscles under voluntary control, including those of the respiratory system, are affected. Despite the paralysis, however, the mind and the senses remain intact. Death is usually caused by respiratory failure or pneumonia.
Diagnosis is often difficult because the causes of ALS remain unknown. In more than 90 percent of cases, ALS is sporadic, arising in individuals with no known family history of the disorder. In the other 5 to 10 percent of cases, ALS is familial — transmitted to family members because of a gene defect.
Approximately 2,500 - 3,000 Canadians currently live with ALS. Once diagnosed, physical therapy and rehabilitation methods can help strengthen unused muscles. Various drugs can ease specific problems, such as twitching and muscle weakness, but there is no cure.
Eighty percent of people with ALS die within two to five years of diagnosis.