Muscle Diseases - Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis, ALS, is a degenerative neurological disease in which the nerve cells that control muscles die, resulting in loss of muscle movement and the wasting away of muscles from lack of use.
The first signs of ALS, commonly known as Lou Gehrig's Disease (named for the New York Yankees player who died of it at 38), are the inability to do simple tasks, such as turn a door knob or button a shirt. The condition starts on one side of the body, then moves to the other side. As muscles begin to degenerate, the person loses weight. Eventually, ALS sufferers lose control of their ability to speak, to move, to swallow, or sometimes even to breathe. Mentally, they remain alert, trapped in a withering body.
Since there is no cure for ALS, treatment focuses on controlling symptoms. One drug, riluzole, has been shown conclusively to prolong survival of ALS patients. Scientists have also discovered a defective gene believed to be the cause of the inherited form of the disease (about ten percent of the cases). Antispasmodic drugs, such as diazepam, are available as are drugs to help patients with impaired ability to swallow.