Amyotrophic lateral sclerosis (ALS, sometimes called Lou Gehrig’s disease, Maladie de Charcot or motor neurone disease) is a chronic, progressive,invariably fatal neurological disease. ALS is marked by gradual degeneration of the nerve cells in the central nervous system that control voluntary muscle movement. The disorder causes muscle weakness and atrophy throughout the body.
In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away (atrophy), and have fasciculations because of denervation. Eventually, the ability of the brain to start and control voluntary movement is lost. However, even patients in advanced stages of the disease may retain the same intelligence, memory, and personality they had before its onset.
ALS is caused by the death of motor neurons in the spinal cord and brain. Normally, these neurons convey electrical messages from the brain to the muscles to stimulate movement in the arms, legs, trunk, neck, and head. As motor neurons die, the muscles they enervate cannot be moved as effectively, and weakness results. In addition, lack of stimulation leads to muscle wasting, or loss of bulk.
Involvement of the upper motor neurons causes spasms and increased tone in the limbs, and abnormal reflexes. Involvement of the lower motor neurons causes muscle wasting and twitching (fasciculations).
One of the symptoms is the patient will feel weakness in their feet and ankles, causing their feet to drag when they walk (called foot drop). This can be corrected with the help of a drop foot brace.
