Every 90 minutes, someone is diagnosed with the disease and someone passes away from it.
What is ALS?
ALS, or amyotrophic lateral sclerosis, is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord.
A-myo-trophic comes from the Greek language. "A" means no. "Myo" refers to muscle, and "Trophic" means nourishment – "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.
Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their demise. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, people may lose the ability to speak, eat, move and breathe. The motor nerves that are affected when you have ALS are the motor neurons that provide voluntary movements and muscle control. Examples of voluntary movements are making the effort to reach for a smartphone or step off a curb. These actions are controlled by the muscles in the arms and legs.
There are two different types of ALS, sporadic and familial. Sporadic, which is the most common form of the disease in the U.S., accounts for 90 to 95 percent of all cases. It may affect anyone, anywhere. Familial ALS (FALS) accounts for 5 to 10 percent of all cases in the U.S. Familial ALS means the disease is inherited. In those families, there is a 50% chance each offspring will inherit the gene mutation and may develop the disease. French neurologist Jean-Martin Charcot discovered the disease in 1869.
Recent years have brought a wealth of new scientific understanding regarding the physiology of this disease. There are currently four drugs approved by the U.S. FDA to treat ALS (Riluzole, Nuedexta, Radicava, and Tiglutik). Studies all over the world, many funded by The Association, are ongoing to develop more treatments and a cure for ALS.
Partnership with The ALS Association
Established in 1985, The ALS Association is the only national nonprofit organization fighting ALS on every front. By leading the way in global research, providing assistance for people with ALS through a nationwide network of chapters, coordinating multidisciplinary care through certified clinical care centers, and fostering government partnerships, The Association builds hope and enhances quality of life while aggressively searching for new treatments and a cure.
As the preeminent ALS organization, The Association leads the way in research, care services, public education, and public policy — giving help and hope to those facing the disease. The Association’s nationwide network of chapters provides comprehensive patient services and support to the ALS community. The mission of The ALS Association is to discover treatments and a cure for ALS, and to serve, advocate for, and empower people affected by ALS to live their lives to the fullest.
Who Gets ALS?
Most people who develop ALS are between the ages of 40 and 70, with an average age of 55 at the time of diagnosis. However, cases of the disease do occur in people in their twenties and thirties. ALS is 20 percent more common in men than in women. However, with increasing age, the incidence of ALS is more equal between men and women.
About 90 percent of ALS cases occur without family history, which is known as sporadic ALS. The remaining 10 percent of ALS cases are inherited through a mutated gene, which is known as familial ALS.
For unknown reasons, military veterans are more likely to be diagnosed with the disease than the general public.
Stages of ALS
Once ALS starts, it almost always progresses, eventually taking away the ability to walk, dress, write, speak, swallow, and breathe, and shortening the life span.
The onset of ALS often involves muscle weakness or stiffness as early symptoms. Progression of weakness, wasting, and paralysis of the muscles of the limbs and trunk, as well as those that control vital functions such as speech, swallowing, and breathing, generally follows.
How fast and in what order this occurs is very different from person to person. While the average survival time is three years, about 20 percent of people with ALS live five years, 10 percent will survive 10 years and 5 percent will live 20 years or longer.
Progression is not always a straight line in an individual, either. It is not uncommon to have periods lasting weeks to months where there is very little or no loss of function. There are even very rare examples in which there is significant improvement and recovery of lost function. These ALS "arrests" and "reversals" are, unfortunately, usually transient. Less than 1 percent of people with ALS will have significant improvement in function lasting 12 months or longer.
Symptoms and Diagnosis
ALS is typically a disease that involves a gradual onset. The initial symptoms of ALS can be quite varied in different people. One person may have trouble grasping a pen or lifting a coffee cup, while another person may experience a change in vocal pitch when speaking.
The rate at which ALS progresses can be quite variable from one person to another. Although the mean survival time with ALS is two to five years, some people live five, 10 or more years. Symptoms can begin in the muscles that control speech and swallowing or in the hands, arms, legs or feet. Not all people with ALS experience the same symptoms or the same sequences or patterns of progression. However, progressive muscle weakness and paralysis are universally experienced.
Gradual onset of progressive muscle weakness – which is generally painless -- is the most common initial symptom in ALS. Other early symptoms vary but can include tripping, dropping things, abnormal fatigue of the arms and/or legs, slurred speech, muscle cramps and twitches, and/or uncontrollable periods of laughing or crying.
When the breathing muscles become affected, ultimately, people with the disease will need permanent ventilatory support to assist with breathing.
Since ALS attacks only motor neurons, the senses of sight, touch, hearing, taste and smell are not affected. For many people, muscles of the eyes and bladder are generally not affected.