Lou Gehrig's Disease (Amyotrophic Lateral Sclerosis)

Lou Gehrig's disease, or amyotrophic lateral sclerosis (a-my-o-TROF-ik LATer-al skle-RO-sis) (ALS), is a rare disease. It is hereditary in some cases, but in most cases, its cause is unknown. ALS is not an infectious disease, meaning people cannot catch it from someone who has it. ALS affects the body's neurons, or nerve cells, that control movement of the voluntary (skeletal) muscles.

What Is Lou Gehrig's Disease?

Lou Gehrig's disease or amyotrophic lateral sclerosis (ALS) is a progressive and fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles (such as those in the arms, legs, and face). It belongs to a group of disorders known as motor neuron diseases, which are characterized by the gradual degeneration and death of motor neurons.

There are many different types of ALS, differentiated by their signs, symptoms, and genetic association. Sporadic ALS is the most common form of ALS, and occurs in people with no apparent history of the disorder in their family. Family members of a person with sporadic ALS are not considered to be at an increased risk for developing the disease. Symptoms of sporadic ALS usually first develop in a person's late 50s or early 60s. Less common is familial ALS, meaning there is a family history of ALS or a related condition called frontotemporal dementia (FTD), which is a progressive brain disorder that affects personality, behavior, and language. The familial form of ALS is usually inherited; it only requires one parent to carry the gene responsible for the disease. The signs and symptoms of familial ALS typically first appear in a person's late 40s or early 50s. Rarely, people with familial ALS develop symptoms in childhood or their teenage years. It a rare form of the disorder known as juvenile ALS.

Lou Gehrig's disease does not affect a person's ability to see, smell, taste, hear, or recognize touch. Patients usually maintain control of eye muscles and bladder and bowel functions. In late stages of the disease most people with ALS need help getting to and from the bathroom.




British scientist Stephen Hawking has amyotrophic lateral sclerosis. ALS causes the muscles to waste away but does not affect the mind. Hawking, whose specialty is theoretical physics, is known as a visionary thinker;





British scientist Stephen Hawking has amyotrophic lateral sclerosis. ALS causes the muscles to waste away but does not affect the mind. Hawking, whose specialty is theoretical physics, is known as a visionary thinker; he has given many lectures using a computerized speech synthesizer.

How Common Is Lou Gehrig's Disease?

Lou Gehrig's disease is one of the most common neuromuscular diseases worldwide. As of 2016, more than 12,000 people in the United States had a diagnosis of ALS. It affects people of all races and ethnic backgrounds. The disease is more common among white males, non-Hispanics, and persons aged 60 to 69 years, but younger and older people can also develop the disease. Men are affected more often than women. Sporadic ALS accounts for 90 to 95 percent of all ALS cases. About 5 to 10 percent of all ALS cases are inherited.

A variant of ALS, known as Western Pacific ALS, had a particularly elevated incidence among people on the Pacific island of Guam in the 1950s and 1960s. The rate later declined, and researchers believe that an undetermined environmental factor may have been the cause.

What Happens to People with Lou Gehrig's Disease?

ALS affects the upper and lower motor neurons * in the body. The upper motor neurons are located in the cerebral cortex * of the brain and send signals for muscle movement to the spinal cord, where the lower motor neurons then carry the signals to the muscles. Damage to the upper motor neurons results in such symptoms as heightened gag reflex or other reflexes, and stiff muscles. Damage to the lower motor neurons includes muscle weakness, cramps, and twitches. In ALS, both upper and lower motor neurons degenerate or die. In either case, they no longer function. For this reason, symptoms of both upper and lower motor neuron damage are part of the diagnosis of ALS.

Typically, the disease produces weakness and paralysis * in the muscles it affects, and it causes these muscles to waste away. About 40 percent of people with ALS first notice very minor symptoms, such as clumsiness in their hands when they try to perform such routine tasks as buttoning a shirt. Others may feel weakness in their legs or notice that their speech has become slower.

LOU GEHRIG'S DISEASE

Lou Gehrig (1903–1941), who played first base for the New York Yankees from 1923 to 1939, was one of the greatest players in the history of baseball. His lifetime batting average was .340, and he hit .361 in six World Series. He twice led the American League in home runs, and he once tied for home runs with Babe Ruth. In 1934 he not only led the American League in home runs with 49, but also in batting average (363) and in runs batted in (165).

Gehrig, whose nickname was the Iron Horse, also set a major league record in 1939 for playing 2,130 consecutive games, a record that held for more than five decades. His career ended in that year, however, when he developed ALS. His fame drew attention to this condition, and in the United States, it had the common name of Lou Gehrig's disease after that.

Lou Gehrig was elected to the Baseball Hall of Fame in 1939. He showed his well-known winning spirit during the recognition day held in his honor at Yankee Stadium on July 4, 1939, shortly after he received the ALS diagnosis. He said: “Fans, for the past two weeks you have been reading about the bad break I got. Yet today I consider myself the luckiest man on the face of this earth … I may have had a tough break, but I have an awful lot to live for.”

Gehrig died of ALS just two years later at the age of 37. Several books as well as the 1942 film Pride of the Yankees chronicled his story.

People usually die within a few years after they develop ALS. In a very few cases, symptoms may stabilize at some point, remaining the same for years without getting any worse.

How Is Lou Gehrig's Disease Diagnosed?

Physicians can distinguish ALS from other diseases of the nervous system by its usually late onset in life and by the progressive nature (worsening of symptoms) of the illness. The fact that the sense of touch is not affected also helps in diagnosis. Like ALS, multiple sclerosis (MS), a more common disease, affects the nervous system. Unlike ALS, however, MS attacks the nervous system in a number of different ways rather than involving only the motor nerves.

Physicians perform different kinds of medical tests to diagnose ALS. Tests may include the following:




Amyotrophic lateral sclerosis is caused by the degeneration and death of motor neurons in the spinal cord and brain, which normally convey electrical messages from the brain to the muscles to stimulate movement in the arms, legs, trunk, neck,





Amyotrophic lateral sclerosis is caused by the degeneration and death of motor neurons in the spinal cord and brain, which normally convey electrical messages from the brain to the muscles to stimulate movement in the arms, legs, trunk, neck, and head.
Illustration by Electronic Illustrators Group. © 2016 Cengage Learning®.

How Is Lou Gehrig's Disease Treated?

As of 2016, Lou Gehrig's disease had no known cure, but a few medications can ease the muscle spasms and possibly even slow the rate at which symptoms get worse. People who have ALS maintain an independent lifestyle as much as possible by taking different measures. Physical therapy, such as massage and regulated exercises, can reduce disability somewhat by helping the muscles to work better. Such aids as canes, walkers, and wheelchairs can help people with ALS remain independent. Mechanical ventilation (machine-assisted breathing support) is often needed in the later stages of the disease.

How ALS Got Its Medical Name

French neurologist Jean-Martin Charcot (1825–1893) was the first to describe ALS and gave the disease its name.

Charcot noted in detail the wasting of muscles (amyotrophy) and the hardening (sclerosis) of motor nerves along the sides (laterally) of the spinal cord. In France, ALS is known as la maladie de Charcot (meaning “Charcot's disease”) and in Great Britain it is called motor neurone disease.

See also Multiple Sclerosis (MS) • Paralysis

Resources

Books and Articles

Hawking, Stephen. My Brief History. New York: Bantam, 2013.

Websites

National Institute of Neurological Disorders and Stroke. “Amyotrophic Lateral Sclerosis (ALS) Fact Sheet.” http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_ALS.htm (accessed May 12, 2016).

ALS Association. “Who Gets ALS.” http://www.alsa.org/about-als/facts-you-should-know.html?referrer=https://www.google.com/ (accessed July 26, 2015).

Organizations

ALS Association. 1275 K St. NW, Suite 250, Washington, DC 20005. Website: http://www.alsa.org (accessed November 17, 2015).

National Institute of Neurological Disorders and Stroke. PO Box 5801, Bethesda, MD 20824. Telephone: 301-496-5751; Toll-free: (800) 352-9424. Website: http://www.ninds.nih.gov (accessed November 17, 2015).

* neurons are nerve cells. Most neurons have extensions called axons and dendrites through which they send and receive signals from other neurons.

* cerebral cortex (suh-REE-brul KOR-teks) is the part of the brain that controls functions such as conscious thought, listening, and speaking.

* paralysis (pah-RAH-luh-sis) is the loss or impairment of the ability to move some part of the body.

Disclaimer:   This information is not a tool for self-diagnosis or a substitute for professional care.

(MLA 8th Edition)