Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS), often referred to as Lou Gehrig's disease, is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Approximately 5,600 people in the United States are diagnosed with ALS each year8. It is estimated that as many as 30,000 Americans may have the disease at any given time, with100, 000 throughout the Western world. The total cost of treating ALS patients is approximately $1.25 billion.

In healthy people, motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. In ALS, there is progressive degeneration of the motor neurons, eventually leading to death. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed. However, in most cases, mental faculties are not affected.

It is estimated that in the United States, ALS is responsible for nearly two deaths per 100,000 population annually and occurs two-thirds as frequently as MS. More people die every year of ALS than of MS or Huntington's disease. There appear to be more MS sufferers because MS patients tend to live much longer, some for 30 years or more, while the life expectancy of an ALS patient averages between two to five years from the time of diagnosis.

Current Treatment Options

Today, treating physicians can only offer conventional medications to alleviate ALS symptoms or, in the case of Riluzole, slow the disease’s progression. In the absence of an effective cure, treatment options focus on ways of increasing the patient’s comfort and quality of life. As ALS progresses and muscles weaken, the patient may benefit from rehabilitation services such as physical and occupational therapy, speech therapy and the use of assistive devices to enhance independence and quality of life. As ALS weakens muscles used to breathe, the patient may need a ventilator (breathing machine), initially while sleeping and eventually full time.

Scientists have not yet identified a reliable biological marker for ALS—a biochemical abnormality shared by all patients with the disease. Once such a biomarker is discovered and tests are developed to detect the marker in patients—allowing early detection and diagnosis of ALS—physicians will have a valuable tool to help them follow the effects of new therapies and monitor disease progression.