Amyotrophic Lateral Sclerosis (ALS)

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According to the ALS Association, approximately 5,600 people in the U.S. are diagnosed with ALS each year. It is further estimated that as many as 30,000 Americans may have the disease at any given time.

ALS, also known as Lou Gehrig’s disease, can strike anyone. It is a progressive and fatal neurodegenerative disease that attacks nerve cells in the brain and spinal cord, resulting in muscle weakness and atrophy.

The only nerve cells affected are the lower motor neurons, which control movement of the limbs, swallowing, and some aspects of breathing. However, the senses and cognitive functions remain normal. Pain does not accompany the disease at any stage. For the vast majority of people, their faculties remain intact while their body progressively degenerates.

Amyotrophic Lateral Sclerosis (ALS): Symptoms

Not all people with ALS experience the same symptoms or the same sequences or patterns of progression. But, progressive muscle weakness and paralysis are hallmarks of this relatively rare disease.

Approximately 60% of ALS patients experience muscle weakness and stiffness as the initial symptom. In the early stages of the disease, an individual may also experience:

  • Increasing weakness in one limb, especially in a hand
  • Clumsiness with the hands
  • Difficulty walking
  • Impaired speech

As the disease progresses, patients experience:

  • Weakening of other limbs, perhaps accompanied by twitching, muscle cramping, and exaggerated, faster reflexes
  • Problems with chewing, swallowing, and breathing
  • Drooling in some cases
  • Subtle, light twitches under the skin called fasciculations
  • Eventual paralysis.

Amyotrophic Lateral Sclerosis (ALS): Diagnosis

ALS is often difficult to diagnose because the symptoms are similar to those of other neuromuscular disorders. In addition to performing a thorough physical and neurological exam, your neurologist will request nerve conduction velocity (NCV) and electromyogram (EMG) studies to test for nerve damage. He or she may also employ the following tests to rule out muscular dystrophy, multiple sclerosis, spinal cord tumors, or other neuromuscular diseases:

  • Magnetic resonance imaging (MRI scan) may be used to rule out spinal cord disease.
  • Blood may be analyzed to detect the presence of heavy metals such as lead in the blood.
  • Laboratory tests may detect abnormal proteins or hormone levels associated with other neurological diseases.

A lumbar puncture may be performed to analyze the cerebrospinal fluid for genetic abnormalities.

Amyotrophic Lateral Sclerosis (ALS): Treatment

Although no treatment slows or halts the progression of ALS, there are a number drugs and therapies available to help manage symptoms and make living with ALS easier.

The medication Riluzole (Rilutek®) is approved for the treatment of ALS. This drug, which decreases the release of glutamate, provides symptomatic relief, helps prevent complications, and helps patients achieve optimal function and quality of life.

In the early stages of ALS, physical therapy can improve circulation and help prolong muscle use. Various medications may be prescribed as the disease progresses to help control worsening symptoms:

  • Baclofen relieves stiffness in the limbs and throat.
  • Nutritional supplements called branched-chain amino acids (BCAAs) can help slow muscle decline and weight loss.
  • Phenytoin may ease cramps.
  • Tricyclic antidepressants can help control excess saliva production.

Other therapies, such as occupational therapy and speech therapy, are also used to treat ALS. Because ALS patients require a diet of high-energy foods that are easy to swallow, a nutritionist is usually assigned to their patient care team.

Amyotrophic Lateral Sclerosis (ALS): Resources

ALS Association (ALSA)
27001 Agoura Road
Suite 150
Calabasas Hills, CA 91301-5104
Tel: 818-880-9007
Fax: 818-880-9006

Les Turner ALS Foundation
5550 W. Touhy Avenue, Suite 302
Skokie, IL 60077-3254
Tel: 888-ALS-1107
Fax: 847-679-9109

Muscular Dystrophy Association
3300 East Sunrise Drive
Tucson, AZ 85718-3208
Tel: 520-529-2000
Fax: 520-529-5300

Project ALS
3960 Broadway
Suite 420
New York, NY 10032
Tel: 212-420-7382 or 800-603-0270
Fax: 212-781-3241

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