Amyotrophic Lateral Sclerosis

Alternative Names: Lou Gehrig's disease.

Amyotrophic lateral sclerosis (ALS) is a progressive, fatal neurological disease affecting as many as 20,000 Americans with 5,000 new cases occurring in the United States each year.  The disorder belongs to a class of disorders known as motor neuron diseases.

Incidence; Causes and Development

ALS strikes in mid-life.  Men are about one-and-a-half times more likely to have the disease than are women.

ALS occurs when specific nerve cells in the brain and spinal cord that control voluntary movement gradually degenerate.  The loss of these motor neurons causes the muscles under their control to weaken and waste away, leading eventually to paralysis.

Signs and Symptoms

ALS manifests itself in different ways, depending on which muscles weaken first.  Symptoms may include tripping and falling, loss of motor control in hands and arms, difficulty speaking, swallowing and/or breathing, persistent fatigue, and twitching and cramping, sometimes quite severely.

Treatment and Prevention

There is no cure for ALS, nor is there a proven therapy that will prevent or reverse the course of the disorder.  The FDA has approved riluzole, the first drug that has been shown to prolong the survival of ALS patients.  Patients may also receive supportive treatments that address some of their symptoms.

Prognosis

ALS is usually fatal within five years of the initial diagnosis.