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Amyotropic Lateral Sclerosis
Student Health Information Page compiled by: Jollyn Tyryfter

What is ALS?

Amyotropic Lateral Sclerosis (ALS) is a motor neuron disease also commonly known as Lou Gehrig’s Disease. It was first described in 1869 but did not receive national attention until Lou Gehrig, a famous baseball player, was diagnosed in 1939. ALS is a disease affecting the nerve cells in the brain (upper motor neurons) as well as those in voluntary muscles (lower motor neurons) such as those in the legs, arms, and torso.

These neurons progressively degrade, which causes the muscles to weaken and eventually die, leaving the individual unable to control their movements. Though ALS affects neurons in the brain, it does generally only affects motor neurons. ALS does not affect the nerves for sensory impulses such as nerves responsible for sight, touch, smell, or taste. A small percentage of patients may experience memory loss. There is currently no cure for the disease.

Who is affected by ALS?

Around 5,000 people in the United States are diagnosed each year, and it is one of the most common neuromuscular diseases in the world. An estimated 20,000-30,000 people in the United States are currently living with the disease. Symptoms most frequently begin in those aged 40-60 years, but it does affect people both younger and older. Men are approximately 20% more likely to develop the disease than women. However, these differences between men and women decrease with age and among older adults the rate of people developing ALS is more equally distributed between men and women. Over 90% of those on the National ALS registry are Caucasian.

What are the risk factors for ALS?

There are currently no known risk factors for ALS, though Gulf War military veterans have been shown to be more likely to develop the disease. Studies are ongoing to determine environmental risk factors that may contribute to the disease. Only 5-10% of cases are inherited, and mutations responsible for ALS have been discovered in a dozen genes. The overwhelming majority of cases (90-95% of cases) have no identified risk factors that may explain why the disease occurred.

Early Symptoms

ALS signs can begin in any voluntary muscle in the body but frequently begin in the hands, feet, or limbs. Common early symptoms include small twitches in the muscles, cramps, muscle weakness, slurred speech, and difficulty chewing and swallowing.

Diagnosis

Early diagnosis of ALS can be difficult as the first symptoms that occur are often similar to other neurological diseases. Signs of damage to both upper and lower motor neurons must be present for a diagnosis of ALS. Your family physician will likely refer you to a neurologist who will examine your symptoms and perform necessary tests for diagnosis.

There is currently no test to definitively diagnose ALS. However, there are several tests that physicians use in order to rule out other diseases that have the same symptoms. Since there is no cure for ALS your physician will want to make sure you don’t have another, more treatable disease before making a diagnosis.

Treatment

Rilutek (riluzole) is currently the only medication available to treat ALS. It does not reverse the damage of ALS but may prolong survival time, and can lengthen the time before a patient will need a ventilator for breathing support.

The remaining treatment options are focused on relieving symptoms and improving the quality of life for those who have been diagnosed.

  • Medications can be prescribed to help ease cramps, reduce fatigue, help with constipation, reduce excess saliva and phlegm, and help with depression that may occur after diagnosis
  • Physical Therapy can help fight fatigue and depression as well as strengthen muscles that have not yet been affected.
  • Occupational Therapy can provide help with independence by suggesting devices such as walkers and ramps to help improve mobility.
  • Speech Therapy can assist those with speech difficulties by helping them speak more clearly, or to communicate nonverbally with aids like computer communication systems.
  • Nutritionists can teach patients and caregivers how to prepare appropriate, small meals with easy to swallow foods for those with difficulty chewing or swallowing.
  • Psychiatrist/Psychologist can help with the stress of diagnosis and help patients and caregivers cope.

The speed at which the disease progresses will vary for each individual, but eventually patients will no longer be able to control their muscles and their muscles will begin to waste. Eventually patients may need a feeding tube when they are no longer able to chew and swallow well enough to receive proper nutrition. As control of the chest muscles begin to deteriorate, it will become necessary to use a respirator to support breathing if the patient chooses to explore the option of mechanical ventilation. Respiratory failure remains the most common cause of death for those with ALS.

Average survival rates are between 3-5 years from diagnosis, though 10 percent will live for 10 or more years. It is important to discuss all therapy options with your physician and make sure you understand how the disease is likely to progress.

The National ALS Registry

The Centers for Disease Control and Prevention have established a national ALS registry for those diagnosed. You can help provide scientists and clinicians with information they need to search for a cure. You will be asked questions on your family history, health, and job. Your information will be stored securely and researchers will not have access to your identifying information. By participating you will have access to information on clinical trials, reports, journal articles, and fact sheets.

To participate in the registry go to www.cdc.gov/ALS

Resources and Support

ALS Association
1275 K Street, N.W. Suite 1050
Washington, DC 20005-1717

Muscular Dystrophy Association
3300 East Sunrise Drive
Tucson, AZ 85718-3208
202-407-8580

Les Turner’s ALS Foundation
5550 W. Touhy Avenue Suite 302
Skokie, IL 60077-3254
800-572-888-ALS-1107

ALS Independence:

Christopher and Dana Reeve Paralysis Resource Foundation: