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What is ALS?


What Is Amyotrophic Lateral Sclerosis?

Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disorder of the nervous system. It is also known as Lou Gehrig’s disease. ALS attacks motor neurons, which are nerve cells in the brain and spinal cord. These cells send messages to muscles throughout the body. In ALS, the motor neurons die. The ability of the brain to start and control muscle movement is lost. As a result, muscles gradually weaken. In time, most muscles are affected, including those that control swallowing and breathing. For most people, weakness tends to progress, causing death in three to five years. About 10 percent of people with ALS live for 10 years or longer with the disease. While ALS can develop at any age, it generally occurs between the ages of 40 and 70. About 5,000 people in the United States are diagnosed with ALS each year.

What Are the Symptoms?

ALS usually comes on slowly. The first signs are often arm or leg weakness, problems with speaking or swallowing, muscle twitches or cramps, loss of muscle bulk, or stiff muscles. Some people laugh or cry more easily. In time, all muscles involved with voluntary movement can be affected. ALS does not affect involuntary muscles, such as those that control the heart and the digestive system.

How Is ALS Diagnosed?

ALS is a difficult disease to diagnose. There is no single test to diagnose ALS. The diagnosis is based on the symptoms and signs of the disease and a series of tests to rule out other diseases. A test of nerves

and muscles called electromyography and nerve conduction studies (EMG/NCS) may help confirm the diagnosis. Many disorders, such as cervical spine disease and conditions that affect the peripheral nerves, can mimic ALS. Seeing a neurologist with experience in diagnosing ALS is important. If there is uncertainty, seeking a second opinion is sometimes helpful.

What Causes ALS?

Two types of ALS occur. Most people have sporadic, or random, ALS, in which the cause is not known. The children of people with sporadic ALS are at no greater risk of developing the disorder than other people. Five percent to 10 percent of those with ALS have familial ALS. They have a family history of the disorder and show a dominant genetic pattern. In this form, offspring have about a 50-percent chance of inheriting the gene mutation, putting them at risk of developing the disease.

What Are the Treatments?

There is no known cure for ALS, but research is promising. The drug riluzole modestly increases survival time. Much can be done to make life easier and longer for people with ALS. The goal of treatment is to maintain function and independence for as long as possible. Therapies are available that can be helpful in treating some symptoms of ALS. Research shows that using an assisted breathing device may increase life expectancy and slow down development of breathing problems. Research also shows that using a feeding tube may help stabilize body weight and prolong survival. Treatments are also available for excess saliva and drooling due to swallowing problems. People with ALS need to take an active role in planning and carrying out their treatment. A neurologist is the doctor best qualified to lead the treatment team. Ideally, physical, occupational, speech, respiratory, and nutrition therapists will also be involved. Research has shown that people who are treated at a specialized, multidisciplinary ALS clinic may live longer and have a better quality of life.

Living with ALS

Medications and assistive devices can help manage the symptoms of ALS. Talk to your doctor about your concerns. Treatments are available for many symptoms. ALS support groups can be a source of help, comfort, and information. You can learn how other people with ALS are managing the physical and emotional changes that are part of the disorder. You can also learn about new ways of managing your symptoms and current research.

Partnering with Your Neurologist

To provide the best care, your neurologist needs to know all about your symptoms and medical history. Likewise, you need to get answers to your questions. Keeping a notebook about your condition and bringing a few well-organized questions to your appointments can be helpful.

For more information about ALS visit http://www.alsa.org/

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