- published: 17 Jan 2012
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Amyotrophic lateral sclerosis (ALS), also referred to as motor neuron disease in British English, is the most common form of the motor neuron diseases. The condition is sometimes called Lou Gehrig's disease in North America, after the New York Yankees baseball player who was diagnosed with the disease in 1939. The disorder is characterized by rapidly progressive weakness, muscle atrophy and fasciculations, muscle spasticity, difficulty speaking (dysarthria), difficulty swallowing (dysphagia), and decline in breathing ability.
The disorder causes muscle weakness and atrophy throughout the body caused by degeneration of the upper and lower motor neurons. Unable to function, the muscles weaken and atrophy. Affected individuals may ultimately lose the ability to initiate and control all voluntary movement, although bladder and bowel sphincters and the muscles responsible for eye movement are usually, but not always, spared.
Cognitive function is generally spared for most patients although some (~5%) also have frontotemporal dementia. A higher proportion of patients (~30-50%) also have more subtle cognitive changes which may go unnoticed but are revealed by detailed neuropsychological testing. Sensory nerves and the autonomic nervous system, are generally unaffected meaning the majority of people with ALS will maintain sight, hearing, touch, smell, and taste. Bladder and bowel functions are also rarely affected by ALS.
Henry Louis "Lou" or "Buster"Gehrig (June 19, 1903 – June 2, 1941) was an American baseball first baseman who played 17 seasons in Major League Baseball (MLB) for the New York Yankees (1923–1939). Gehrig set several major league records, including most career grand slams (23) and most consecutive games played (2,130). Gehrig is chiefly remembered for his prowess as a hitter and his durability, a trait which earned him his nickname "The Iron Horse", as well as the pathos of his farewell from baseball at age 36, when he was stricken with amyotrophic lateral sclerosis (ALS). Gehrig was elected to the Baseball Hall of Fame in 1939. In 1969 he was voted the greatest first baseman of all time by the Baseball Writers' Association, and was the leading vote-getter on the Major League Baseball All-Century Team, chosen by fans in 1999.
A native of New York City, he played for the Yankees until his career was cut short by ALS, a disorder now commonly known in the United States and Canada as Lou Gehrig's disease. Over a 15-season span from 1925 through 1939, he played in 2,130 consecutive games. This streak ended only when Gehrig became disabled by the fatal neuromuscular disease that claimed his life two years later. His streak, long considered one of baseball's few unbreakable records, stood for 56 years, until finally broken by Cal Ripken, Jr., of the Baltimore Orioles on September 6, 1995.