Lou Gehrig s Disease: An Introduction

Susan McQuillan

Reviewed by QualityHealth's Medical Advisory Board

What Is Lou Gehrig’s Disease?

Amyotrophic Lateral Sclerosis (ALS), a degenerative neurological condition more commonly known as Lou Gehrig’s disease, strikes between 5,000 and 6,000 Americans each year. The disease damages the brain and spinal cord cells that control muscle movement. It most often starts locally, so patients may initially experience weakness in an arm, a leg, or the muscles of the face. Over time, the disease spreads to involve muscles throughout the body, including those that allow us to breathe.

Who Is At Risk?

Both men and women develop ALS, but the condition is slightly more common in men. The average age of patients diagnosed with ALS is 57, but people as young as 20 have developed the condition. Younger patients have a somewhat better prognosis.

In up to 10 percent of all cases, the disease is passed from generation to generation; this is called familial ALS. Although researchers have discovered genetic factors that cause familial ALS, no knows what causes the disease in most patients. Environmental factors, inflammation, and complex genetic interactions are all being investigated as causes.

How ALS Affects Patients

As ALS progresses and the disease spreads through the body, patients experience exaggerated reflexes, lack of coordination, fatigue, and weight loss. Assistance with walking, eating, and even breathing become necessary to extend the lifespan for many ALS patients. "Loss of strength is the hallmark symptom of this condition," says Lucie Brujin, Phd, Chief Scientist at the ALS Association. "Some patients also experience thinking difficulties, and a small number develop dementia."

The average life expectancy upon diagnosis is generally two to five years, but the ALS Association reports that up to 10 percent of patients will live ten years or more. The actual cause of death for most patients is related to weakness of respiratory muscles (those associated with breathing).

What Can Be Done

Although ALS currently has no cure, it can be treated. The FDA-approved ALS medication Riluzole slows the progress of the disease; other medications can help with stiffness, cramps, excessive saliva, depression and many other symptoms. Care at clinics, including many sponsored by the ALS Association, can help preserve quality of life. At these clinics, patients are seen by their neurologists, as well as by specialists in respiratory therapy, physical and occupational therapy, and nutritional support.

Lucie Brujin, PhD, MBA, reviewed this article.