What is ALS?

ALS (Amyotrophic lateral sclerosis) is also referred to as Lou Gehrig’s disease, Maladie de Charcot or Motor Neuron Disease.

ALS is a very serious disease. Average age at diagnosis is around 55 years, with the disease progressing more rapidly the older the person is at diagnosis. Most people with ALS die from respiratory failure within 3 to 5 years after the disease’s onset, though about 10% of ALS patients survive 10 years or more. Currently, there is no cure for the disease. The only approved drug (Rilutek) modestly improves patients’ survival by an average of 3 months.

The process is rapidly degenerative and attacks the nerve cells responsible for voluntary movements, called motor neurons. Once the motor neurons die, the muscles that they command do not receive orders, and they weaken, twitch (fasciculation) and finally waste away (atrophy). Little by little patients lose the ability to carry things, to walk, to speak, to eat and finally to breathe without ventilatory support.

It differs from Alzheimer’s Disease in that it usually does not attack parts of the brain responsible for the “mind” of a person (cognition) and memory or only at the latter stages of the disease. ALS also differs in that it does not impair people’s ability to see, smell, taste or recognize touch.

Despite enjoying a considerably smaller profile and level of understanding, more people die of ALS on a yearly basis than Multiple Sclerosis.

Famous People with Amyotrophic lateral sclerosis (ALS) or Lou Gehrig's Disease

Lou Gehrig - (June 19, 1903 – June 2, 1941), was an American baseball player in the 1920s and 1930s who played for the New York Yankees until his career was cut short by amyotrophic lateral sclerosis (ALS), now commonly known in the United States and Canada as Lou Gehrig's disease. Throughout his career, Gehrig was considered an excellent base runner, and set several major league records. He still holds the record for most career grand slams. However, a year into the diagnosis, his coordination and speed had deteriorated significantly. "Don't think I am depressed or pessimistic about my condition at present," Lou Gehrig wrote following his retirement from baseball. Struggling against his ever-worsening physical condition, he added, "I intend to hold on as long as possible and then if the inevitable comes, I will accept it philosophically and hope for the best. That's all we can do." Gehrig finally lost his battle in 1941.

Stephen Hawking - An internationally renowned Physicist, Hawking is a well-known example of a person with MND. After being told that he would not live for more than two years, having been diagnosed at 21 with amyotrophic lateral sclerosis (ALS), Hawkins has defied doctor’s pronouncements by surviving with the disease for more than four decades. He describes himself as lucky despite his disease. Its slow progression has allowed him time to make influential discoveries and has not hindered him from having, in his own words, "a very attractive family." The symptoms of ALS are very similar to those of Cerebral Palsy, Hawking cannot walk, talk, breathe easy, swallow and has difficulty in holding up his head.

Mao Zedong - (1893-1976) Chinese military and political leader, who led the Communist Party of China (CPC) to victory against the Kuomintang (KMT) in the Chinese Civil War, and was the leader of the People’s Republic of China (PRC) from its establishment in 1949 until his death in 1976. Regarded as one of the most important figures in modern world history, Mao is still a controversial figure today, over thirty years after his death. He died of amyotrophic lateral sclerosis, commonly known in the U.S as Lou Gehrig's Disease and elsewhere as Motor Neurone Disease. Mao had been in poor health for several years and had declined visibly for some months prior to his death.

Lead Belly - Huddie William Ledbetter, (January, 1888 – December 6, 1949) was an American folk and blues musician, notable for his clear and forceful singing, his virtuosity on the twelve-string guitar, and the rich songbook of folk standards he introduced. Although he most commonly played the twelve-string, he could also play the piano, mandolin, harmonica, violin, concertina, and accordion. In 1949 he began his first European tour with a trip to France, but fell ill before its completion, and was diagnosed with amyotrophic lateral sclerosis, or Lou Gehrig's disease. Lead Belly died later that year in New York City.

David Niven - James David Graham Niven (March 1, 1910 – July 29, 1983) was an Academy Award-winning English actor and novelist, best known for his roles as Phileas Fogg in Around the World in 80 Days and Sir Charles Litton, a.k.a. "the Phantom," in The Pink Panther. In 1980, Niven began experiencing fatigue, muscle weakness, and a warble in his voice. A 1981 interview on Michael Parkinson's talk show alarmed family and friends; viewers wondered if Niven had either been drinking or suffered a stroke. He blamed his slightly slurred voice on the shooting schedule on the film he'd been making. Later that same year he was diagnosed with amyotrophic lateral sclerosis. Niven died in Switzerland on July 29, 1983 of motor neurone disease (Lou Gehrig's Disease) at age 73.