Question: What are the known causes and treatments for ALS or Lou Gehrig's disease?

Dr. Hibberd's Answer:

Amyotrophic lateral sclerosis (ALS) is a slow-onset, debilitating degenerative neurological disorder that affects 20,000 to 30,000 people in the United States with 5,000 new cases diagnosed annually. Approximately 10 percent of all ALS cases are inherited, while the remaining 90 percent appear to be random and appear to have no known common origin or cause. Unfortunately, we have no cure.

Though it does not target memory or intelligence, it does cause a progressive loss of nerve function. It attacks nerve cells that are essential to initiate and control voluntary muscle movement (motor neurons both upper and lower motor neurons) in the brain and spinal cord. Damage to these motor neurons results in neuronal death, resulting in the inability of the brain to send messages to the muscles. The muscles gradually weaken and atrophy (shrink in size) and the victim eventually loses control of voluntary movement.

Patients lose the control of their arms, legs, and body, and most victims die of respiratory failure within three to five years. At least 10 percent of victims may live 10 or more years after symptoms begin.

Treatment is supportive, and much research is underway into its cause and treatment. We have medications to help reduce spacticity, fatigue, muscle cramps, and excessive salivation and secretions. Often mood modifiers, antidepressants, as well as medications for pain, sleep, and anxiety are useful supplements.

An FDA-approved drug treatment (Riluzole) that seems to delay damage to the motor neurons, and helps delay the need for ventilator support is available, but this effect is limited and seems to last only for several months in many. Physical and occupational therapy will help victims accommodate their disabilities.

Nighttime breathing assistance with masks supplying positive pressure airway support are useful adjuncts, and can be used part-time or full-time by patients as needed without insertion of invasive breathing tubes. Mechanical ventilation is always an option, but, of course, it has its limitations.