AMYOTROPHIC LATERAL SCLEROSIS

Amyotrophic lateral sclerosis (ALS) is also referred to as Lou Gehrig's Disease, after the famous New York Yankees first base player who died from this neuromuscular disease. The cause of ALS is not known. The disease affects the nerve cells that run from the brain to the spinal cord to the muscles of the body. ALS leads to the degeneration of these nerve cells and, ultimately, to paralysis of the body.

ALS occurs in about 3 people per 100,000 population. Approximately 5000 new cases are diagnosed in the United States each year. Men and women are equally affected. ALS can be sporadic, meaning there is no family history of the disease, or it can be inherited (10% of the cases are inherited).

The disease is progressive, but the rate of progression varies from patient to patient. Unfortunately, there is no cure for ALS at this time.

Muscle weakness is the first symptom noted by patients. The weakness is usually noted in the hands and described as clumsiness. The weakness can involve both sides but typically to a different degree. Twitching and cramping of the hands and feet is commonly associated with the weakness. The weakness progresses with time and can involve the speech, swallowing, and breathing. ALS usually does not involve sensation, bowel, bladder, or thinking (cognition).

As the disease progresses, immobility and paralysis of the upper and lower extremities increases and swallowing and breathing become even more labored.

There is no specific blood test to detect ALS. The diagnosis is usually made by careful physical examination, special radiograph (x-ray) tests called computed tomography (CT) scans, and magnetic resonance imaging (MRI) scans along with specific nerve studies called EMG (electromyogram). A biopsy (obtaining tissue with a needle and looking at it under the microscope) of the muscle and the nerve can also help make the diagnosis.

At the present time there is no cure for ALS. There are many ongoing trials looking for specific drug therapy. A drug called riluzole has recently been approved for use in patients with ALS. Other drugs are also being investigated for uses in ALS (see below for the address and telephone number of the ALS Association).

Other therapies are directed at symptomatic relief. Rehabilitative devices include splints for the hand, braces for foot drop and limb support, and wheelchairs for mobilization.

Antibiotics are available in cases of pneumonia that may develop because of difficulty swallowing, leading to aspiration of mouth contents into the lungs. In more advanced stages, feeding tubes can be used to maintain nutrition and respiratory support can be offered.

• Report any symptoms of muscle weakness, specifically paralysis, to your physician.
• Seek a specialty opinion by a neurologist and a facility experienced in dealing with patients with ALS.
• Discuss your wishes with family members. For example, do you want to be placed on a ventilator (breathing machine) if your breathing stops because of the disease? Do you want life-sustaining efforts or CPR (cardiopulmonary resuscitation) if your breathing or heart stops?
• Appoint a next of kin to make legal and medical decisions for you in case you are no longer able to do this.

• Don't push yourself with exercise. Set your limits, and stop and rest when you feel you need to.
• Don't be afraid to seek opinions from centers experienced in ALS. Ongoing trials are available and these centers can give you the most up-to-date research trials available.

• If you have any weakness or paralysis.
• If you are short of breath.
• If you have a fever.
• If you can no longer get around by yourself and need social service support or nursing support.
• If you have made important decisions about life-sustaining measures.

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