Neurology

Conditions We Treat

Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig's disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles. In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away, and twitch. Eventually, the ability of the brain to start and control voluntary movement is lost. 

Individuals with ALS lose their strength and the ability to move their arms, legs, and body. When muscles in the diaphragm and chest wall fail, individuals lose the ability to breathe without ventilatory support. The disease does not affect a person's ability to see, smell, taste, hear, or recognize touch, and it does not usually impair a person's thinking or other cognitive abilities. However, several recent studies suggest that a small percentage of patients may experience problems with memory or decision-making, and there is growing evidence that some may even develop a form of dementia. The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others.

Treatment

No cure has yet been found for ALS. However, the FDA has approved the first drug treatment for the disease—riluzole. Riluzole is believed to reduce damage to motor neurons and prolongs survival by several months, mainly in those with difficulty swallowing. Other treatments are designed to relieve symptoms and improve the quality of life for people with ALS. Drugs also are available to help individuals with pain, depression, sleep disturbances, and constipation. Individuals with ALS may eventually consider forms of mechanical ventilation (respirators).

Prognosis

Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses. Individuals have increasing problems with moving, swallowing, and speaking or forming words. Eventually, people with ALS will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms. In later stages of the disease, individuals have difficulty breathing as the muscles of the respiratory system weaken. Although ventilation support can ease problems with breathing and prolong survival, it does not affect the progression of ALS. Most people with ALS die from respiratory failure, usually within 3 to 5 years from the onset of symptoms. However, about 10% of those individuals with ALS survive for 10 or more years.

Helpful Resources

Highland Neurology

NYS Designated Stroke Center

NYS Dept of Health

Use the F.A.S.T. Test

If you think you or someone you know is having a stroke.

Face

Ask the person to smile. Is the face lopsided?

Arm

Ask the person to raise arms. Does one arm drift down?

Speech

Ask the person to repeat a phrase. Does their speech sound strange? Can they do it without slurring words?

Time

Don't waste it. Call 911 now.