Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and fatal neurological disease that attacks the nerve cells responsible for controlling muscles. The disease was identified and named in 1874 by a French physician, Jean Martin Charcot. In the United States, the disease is often called Lou Gehrig’s disease after the baseball legend Lou Gehrig, who died from ALS in 1941.
When motor neurons die, the ability of the brain to initiate and control muscle movement dies with them. The disease appears to strike randomly, although there is a clear genetic link in a small percentage of cases. The earliest symptoms often include muscle twitches called fasciculations. However, doctors are unable to predict how ALS will progress in any individual because the disease affects different people in different ways. For instance, some patients have more limb involvement early on, while others have a bulbar version of the disease, and experience difficulty speaking, chewing, and swallowing. Patients in the later stages of ALS are totally paralyzed—yet, through it all, their minds remain unaffected. Denervation of the respiratory muscles and diaphragm is typically the cause of death.
Currently, only one drug is specifically approved by the FDA for treatment of ALS. Therapy generally is limited to supportive care (relieving symptoms, when possible, and addressing quality-of-life issues). Patients with ALS live two to five years, on average, after diagnosis, with about 10 percent surviving for 10 years or longer. A little more than 5,600 people in the United States are diagnosed with ALS each year (that’s 15 new cases a day). It is estimated that as many as 30,000 Americans have the disease at any given time. (Find more information from the National Institute of Neurological Disorders and Stroke here. )