Symptoms and Complications

The symptoms of ALS are caused by the destruction of motor neurons resulting in gradual deterioration of the muscles. Muscle atrophy often starts in the hands or feet, and moves its way up the arms to the shoulders, or up the legs to the hips. The early symptoms of ALS include muscle weakness, clumsy hand movements, muscle stiffness, and difficulty performing tasks that require delicate movements of the fingers or hands. Muscular weakness in the legs may cause tripping and falling. As the muscles lose strength, some people experience muscle cramps, twitches, or spasms. When ALS progresses, more muscle groups become affected, leading to:

• impaired speech
• difficulty swallowing
• breathing problems caused by a weakened diaphragm

Only motor neurons - and therefore only muscles - are affected with ALS. Bladder and bowel muscles aren't usually involved, and the disease doesn't affect a person's mind or any of the five senses. People remain alert, but often lose the ability to take care of themselves. It can even become impossible to breathe without the help of a respirator.

There are several variants of ALS. Each has different symptoms and a different outcome.

Primary bulbar palsy: The initial or predominant symptoms are impaired speech or swallowing along with wasting of the tongue. The outcome is generally worse in this condition since swallowing and breathing are affected early in the course of the disease.

Progressive muscle atrophy: Muscle weakness and wasting are the principal features. Speech and swallowing are not affected and there is no spasticity. These individuals tend to have a slower course and better outcome.

Primary lateral sclerosis: The main feature is spasticity with no muscle atrophy. The outcome is usually somewhat better.

Making the Diagnosis

When the muscles get progressively weaker - especially if a person's sensations aren't at all impaired - doctors will suspect ALS. A battery of tests may be needed to rule out other potential causes for symptoms. Electromyography (EMG) is used to determine whether the problem is in muscle or the nerve cells. This test works by measuring the electrical activity in the muscle.

An MRI scan of the head or spine may be used to exclude other conditions that can damage or compress nerve cells such as tumors or degenerative disc disease.

Based on where in the body symptoms first appeared, and where or how they progress over time, your doctor can tell the difference between ALS and other conditions.

Treatment and Prevention

Because the exact causes of the disease haven't been pinpointed, ALS can neither be prevented nor cured. Medications are available, however, that may slow the progress of the disease and that deal with some of the symptoms.

Riluzole* was approved by the FDA in 1995. Riluzole appears to prolong the life of people with ALS by at least a few months and more recent studies suggest it slows the progress of ALS, allowing the person more time in the higher functioning states. Riluzole seems to do two things: slow the release of glutamate from neurons and block its effects on glutamate receptors.

Muscle spasms - and sometimes cramps - can be controlled with the medications baclofen or diazepam. Medications can also be used to lower the amount of saliva that's made, to help people who've lost the ability to swallow it.

To avoid choking or inhaling food and saliva into the lungs, a feeding tube might need to be placed into the stomach. People with difficulty breathing will eventually have to go on a respirator. Physical therapy can help people maintain strength in their muscles and control muscle contractions.

Although a person's physical state worsens, his or her mental capacity does not. To cope with the progression of the disease, good emotional support from family and friends is important. Support groups can also be helpful: the ALS Association can be contacted by phone (1-800-782-4747), or by e-mail through their website (www.alsa.org).

*All medications have both common (generic) and brand names. The brand name is what a specific manufacturer calls the product (e.g., Tylenol®). The common name is the medical name for the medication (e.g., acetaminophen). A medication may have many brand names, but only one common name. This article lists medications by their common names. For more information on brand names, speak with your doctor or pharmacist.