About the Disease

Amyotrophic lateral sclerosis (ALS), often referred to as "Lou Gehrig's disease," is a progressive motor neuron disease (MND) that attacks nerve cells in the brain and the spinal cord, damaging both upper and lower motor neurons.  Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed. The vast majority of people with ALS maintain total intellectual awareness throughout the disease process. However, some do experience thinking and/or behavior changes due to possible effects of ALS in the frontal temporal lobes of the brain. 

A-myo-trophic comes from the Greek language. "A" means no or negative. "Myo" refers to muscle, and "Trophic" means nourishment. Literally, the word means "no muscle nourishment." When a muscle has no nourishment, it "atrophies" or wastes away. "Lateral" identifies the areas in a person's spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates it leads to scarring or hardening ("sclerosis") in the region.

As motor neurons degenerate, they can no longer send impulses to the muscle fibers that normally result in muscle movement. Early symptoms of ALS often include increasing muscle weakness, especially involving the arms and legs, speech, swallowing or breathing. When muscles no longer receive the messages from the motor neurons that they require to function, the muscles begin to atrophy, or become smaller. Limbs begin to look "thinner" as muscle tissue atrophies.

Although the cause of ALS is not completely understood, the recent years have brought a wealth of new scientific understanding regarding the physiology of this disease.

While there is not a cure or treatment today that halts or reverses ALS, there is one FDA approved drug, Rilutek, that modestly slows the progression of ALS.   Attendance at a multidisciplinary ALS clinic has shown a lifespan increase of almost a year.  Those who attend clinics have also been shown to have a higher quality of life.  A number of new promising drugs are being studied in clinical trials (http://clinicaltrials.gov/). 

Importantly, there are significant devices and therapies that can manage the symptoms of ALS that help people maintain as much independence as possible and prolong survival.  It is important to remember that ALS is a quite variable disease; no two people will have the same journey or experiences.  There are medically documented cases of people in whom ALS “burns out,” stops progressing or progresses at a very slow rate.  No matter what your individual course or situation may be, The ALS Association and your medical team are here to help.