What Is Lou Gherig's Disease?

Lou Gehrig is a baseball legend, but he is also known for the disease that brought the legend down and caused him to leave the game. Lou Gehrig's disease is formally known as amyotrophic lateral sclerosis (ALS). It's a neuromuscular disease that weakens and eventually destroys the body's motor neurons. In essence, it cuts off communication between the brain and the skeletal muscles.

What's in a Name?

• In the United States, ALS is called Lou Gehrig's Disease because New York Yankee Gehrig was diagnosed with it in the 1930s. The disease is called other names in other countries. The Australians and English call ALS motor neuron disease. The French call ALS Maladie de Charcot, named after Dr. Jean-Martin Charcot who first wrote about the disease in 1869.

How Common Is It?

• Only 30,000 people in the United States currently have ALS out of roughly 308 million people. Said another way, one person in every 10,266 has ALS. About 5,600 new diagnoses are made each year. More men than women are diagnosed with it, and nearly all of the patients (93 percent) are Caucasian. Life expectancy after diagnosis is generally three years (50 percent of patients) to 5 years (20 percent of patients) with most diagnoses being made in middle age.

Effects

• ALS causes the motor neurons to atrophy, which, in turn, causes the patient to gradually lose control of his muscles. The disease begins as a weakening in the extremities. The patient will eventually not be able to do anything that requires voluntary muscle control, such as moving, breathing, eating or speaking.

Types of ALS

• Familial ALS is hereditary and is caused by gene mutation. It represents about 5 percent to 10 percent of the cases. The more common type of ALS is sporadic ALS, which attacks people at random with no known cause or risk factors.

Diagnosis

• It may take some time to make a diagnosis of ALS because the symptoms can be attributed to other diseases as well. A doctor may use an electromyogram to see if the muscles aren't working because of damaged nerves. Other tests such as magnetic resonance imaging, spinal taps, blood tests, and nerve and muscle biopsies help the doctor narrow the possible causes to one.

Treatment

• There is no known cure for ALS. Treatments can slow the progression and help make a patient comfortable, but that is all. Rilutek is an FDA-approved drug for slowing the progression of ALS. Some doctors will prescribe drugs to treat muscle cramping. Physical therapy can help strengthen muscles that have begun to atrophy. A ventilator may eventually be required to help the patient breathe.