Amyotrophic Lateral Sclerosis (ALS)

OVERVIEW AND FACTS

What is ALS?

Amyotrophic lateral sclerosis (ALS), often called “Lou Gehrig’s disease,” is a progressive and invariably fatal neurodegenerative disease. ALS attacks the nerve cells (neurons) that control voluntary muscles causing the neurons to die. When the motor neurons die, they can no longer send impulses to the muscle fibers that normally result in muscle movement and the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively depleted, patients in the later stages of the disease may become totally paralyzed.

Amyotrophic lateral sclerosis/ALS/ Lou Gehrig’s disease belongs to a group of disorders known as motor neuron diseases, which are characterized by the gradual degeneration and death of motor neurons.

Early symptoms of ALS often include increasing muscle weakness, especially involving the arms and legs, speech, and swallowing or breathing. Limbs begin to look “thinner” as muscle tissue atrophies.

Why is ALS called “Lou Gehrig’s Disease”? Lou Gehrig was a hall-of-fame baseball player for the New York Yankees who was diagnosed with ALS in the 1930s. That term is often used by Americans, but ALS has different names in different countries. The French refer to it as Maladie de Charcot, after the man who discovered it. People in England and Australia call it Motor Neurone Disease (MND). Whatever it’s called, the disease and its results are the same.

Facts about ALS:

• ALS is not contagious
• ALS can strike anyone and has no racial, ethnic or socioeconomic boundaries
• Approximately 5,600 people in the U.S. are diagnosed with ALS each year
• As many as 30,000 Americans may have ALS at any given time
• The life expectancy of an ALS patient averages about two to five years
• More than half of all patients live more than three years after diagnosis, but many people live with quality for five years and more

SYMPTOMS AND TYPES

• muscle weakness in the hands, arms, legs or the muscles of speech, swallowing or breathing
• twitching and cramping of muscles, especially those in the hands and feet
• loss of the use of the arms and legs
• difficulty in projecting the voice and “thick speech”
• shortness of breath, difficulty in breathing and swallowing are common in more advanced stages

Although there are many commonalities, symptoms will vary from person to person. Common tell-tale signs may be tripping or stumbling, trouble lifting, uncontrollable periods of laughing or crying or slurred speech. Progressive muscle weakness and paralysis are experienced by all people with the disease and the hands and feet are usually affected first.

Most of the weakening of the muscles starts on the trunk of the body, but will eventually begin to affect speech, swallowing, chewing and breathing. When the breathing muscles become affected, the patient will need a ventilator to breathe.
Unlike some other neuromuscular diseases, the person’s sense of sight, touch, hearing, taste and smell are not affected.

There are three classifications of ALS:

• Sporadic – the most common form of ALS in the United States accounting for 90 to 95% of all cases.
• Familial – occurring more than once in a family lineage. This accounts for a 5 to 10% of all cases in the U.S. but there is a 50% chance each offspring of a parent with ALS will inherit the gene mutation and may develop the disease.
• Guamanian – found in people of Guam and the Trust Territories of the Pacific in the 1950’s.