ALSAGLAC: What is ALS?


Date	Event
7/19	Golf Tournament North Ranch Country Club
7/19	Game Day Tournament
8/7	Circle of Care Training
8/21	NinaPalooza
8/24	Walk to D'Feet ALS Kick-Off Party
10/2	Walk to D'Feet ALS Reed Park
10/16	Walk to D'Feet ALS Bakersfield
10/23	2nd Annual Ernie Wallengren Memorial Hoopfest
Click to learn more and to register for events!

Download the latest
Messenger newsletter!
Click here to download!

What is ALS - Lou Gehrig's Disease

What is ALS?

Amyotrophic lateral sclerosis (ALS), often referred to as "Lou Gehrig's disease," is a progressive neurodegenerative disease that attacks nerve cells in the brain and the spinal cord. 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 lead to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With all voluntary muscle action affected, patients in the later stages of the disease become totally paralyzed. Yet, through it all, for the vast majority of people, their minds remain unaffected.

A-myo-trophic comes from the Greek language. "A" means no or negative. "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 nourish 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 and breathing. When muscles no longer receive the messages from the motor neurons that they require to function, the muscles begin to atrophy (waste away). Limbs begin to look "thinner" as muscle tissue atrophies.

What Types of Nerves Make Your Body Work Properly?
(from Living with ALS, Manual 1: What's It All About?)

The body has many kinds of nerves. There are those involved in the process of thinking, memory, and of detecting sensations (such as hot/cold, sharp/dull), and others for vision, hearing, and other bodily functions. The nerves that die when you have ALS are the motor neurons that provide voluntary movements and muscle power. Examples of voluntary movements are your making the effort to reach for the phone or step off a curb; these actions are controlled by the muscles in the arms and legs.

The heart and the digestive system are also made of muscle but a different kind, and their movements are not under voluntary control. When your heart beats or a meal is digested, it all happens automatically. Therefore, the heart and digestive system are not involved in ALS. Breathing also may seem to be involuntary. Remember, though, while you cannot stop your heart, you can hold your breath - so be aware that ALS may eventually have an impact on breathing.

Although the cause of ALS is not completely understood, the 1990's have brought a wealth of new scientific understanding regarding the physiology of this disease.

Perspective from Hiroshi Mitsumoto, M.D., Cleveland Clinic ALSA Center and Chair of ALSA's Medical Advisory Committee: "In a review of ALS published in the Archives of Neurology in 1988, I quote Lewis Thomas. 'The whole field of biomedical science is on the move as never before in the long history of medicine. I don't know what will happen over the next 20 years, but my guess is that we are on the verge of discoveries that will match the best achievement in infectious disease a generation ago.' In ten years - just half of Lewis' prediction - we now know the gene responsible for some familial ALS; we have the first drug we can prescribe for ALS; we have a real animal model for this disease and we have incredibly important knowledge on the cell death mechanisms of motor neurons in ALS. Yes, the progress still appears to be too slow for anyone waiting for a breakthrough, but we are truly on the verge of more exciting discoveries. We have solid reasons for strong hope in ALS." There is no question about whether the cause of ALS will be found; it is only a question of when.

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 as well as other drugs in clinical trials that hold promise.

Initial Symptoms of the Disease

At the onset of ALS the symptoms may be so slight that they are frequently overlooked. With regard to the appearance of symptoms and the progression of the illness, the course of the disease may include the following:

muscle weakness in one or more of the following: hands, arms, legs or the muscles of speech, swallowing or breathing
twitching (fasciculation) and cramping of muscles, especially those in the hands and feet
impairment of the use of the arms and legs
"thick speech" and difficulty in projecting the voice
in more advanced stages, shortness of breath, difficulty in breathing and swallowing

The initial symptoms of ALS can be quite varied in different people. One person may experience tripping over carpet edges, another person may have trouble lifting and a third person's early symptom may be slurred speech. The rate at which ALS progresses can be quite variable from one person to another. Although the mean survival time with ALS is three to five years, many people live five, ten or more years. In a small number of people, ALS is known to remit or halt its progression, though there is no scientific understanding as to how and why this happens. Symptoms can begin in the muscles of speech, 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. But, progressive muscle weakness and paralysis are universally experienced.

Muscle weakness is a hallmark initial sign in ALS, occurring in approximately 60% of patients. Early symptoms vary with each individual, but usually 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.

The hands and feet may be affected first, causing difficulty in lifting, walking or using the hands for the activities of daily living such as dressing, washing and buttoning clothes.

As the weakening and paralysis continue to spread to the muscles of the trunk of the body the disease, eventually affects speech, swallowing, chewing and breathing. When the breathing muscles become affected, ultimately, the patient will need permanent ventilatory support in order to survive.

Since ALS attacks only motor neurons, the sense of sight, touch, hearing, taste and smell are not affected. For many people, muscles of the eyes and bladder are generally not affected.

For the vast majority of people, their mind and thoughts are not impaired and remain sharp despite the progressive degenerating condition of the body.

Incidence of ALS

ALS is one of the most devastating disorders that affects the function of nerves and muscles. Based on U.S. population studies, a little over 5,000 people in the U.S. are diagnosed with ALS each year. (That's 14 new cases a day.) It is estimated that as many as 30,000 Americans have the disease at any given time.

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 persons in their twenties and thirties. Generally though, ALS occurs in greater percentages as men and women grow older. ALS is 20% more common in men than in women. However with increasing age, the incidence of ALS is more equal between men and women.

Half of all people affected with ALS live at least three or more years after diagnosis. Twenty percent live five years or more; up to ten percent will survive more than ten years.

Forms of ALS
Three classifications of ALS have been described:

Sporadic - the most common form of ALS in the United States - 90 to 95% of all cases.
Familial - occurring more than once in a family lineage (genetic dominant inheritance) accounts for a very small number of cases in the United States - 5 to 10% of all cases.
Guamanian - an extremely high incidence of ALS was observed in Guam and the Trust Territories of the Pacific in the 1950's.

The most common form of ALS in the United States is "sporadic" ALS. It may affect anyone, anywhere.

"Familial" ALS (FALS) means the disease is inherited. Only about 5 to 10% of all ALS patients appear to have genetic or inherited form of ALS. In those families, there is a 50% chance each offspring will inherit the gene mutation and may develop the disease.

Facts About ALS

The onset of ALS is insidious with muscle weakness or stiffness as early symptoms. Inevitable progression of 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 follows.
In most cases, mental faculties are not affected. Also, ALS is not contagious.
It is estimated that ALS is responsible for nearly two deaths per hundred thousand population annually. More people die every year of ALS than of Huntington's disease or multiple sclerosis.
A little over 5,000 people in the U.S. are diagnosed with ALS each year. The incidence of ALS (two per 100,000 people) is five times higher than Huntington's disease and about equal to multiple sclerosis. It is estimated that as many as 30,000 Americans have the disease at any given time.
The life expectancy of an ALS patient averages about two to five years from the time of diagnosis. Half of all affected live more than three years after diagnosis.
About twenty percent of people with ALS live five years or more and up to ten percent will survive more than ten years and five percent will live 20 years. There are people in whom ALS has stopped progressing and a small number of people in whom the symptoms of ALS reversed.
ALS occurs throughout the world with no racial, ethnic or socioeconomic boundaries.
ALS can strike anyone. Someone you know or love will die from ALS unless a cure or prevention is found.
Present treatment of ALS is aimed at symptomatic relief, prevention of complications and maintenance of maximum optimal function and optimal quality of life. Most of this, in the later stages, requires nursing management of a patient who is alert but functionally quadriplegic with intact sensory function, bedridden and aware he or she is going to die.
In 1991 a team of ALSA-funded researchers linked familial ALS to chromosome 21. In 1993 the research team identified a defective SOD1 gene on chromosome 21 as responsible for many cases of familial ALS. Further study indicated over 60 mutations (structural defects) in the SOD (superoxide dismutase) enzyme which alters the enzyme's ability to protect against free radical damage to motor neurons. These studies open possibilities for future therapies or strategies to effectively mediate both familial and sporadic ALS. But much more research on the SOD enzyme is needed. Also, researchers have not ruled out other gene involvement (on other chromosomes) in ALS.
The financial cost to families of persons with ALS is exceedingly high. It is estimated that in the advanced stages, care can cost an average of $200,000 a year. Patients' and relatives' entire savings are quickly depleted because of the extraordinary cost involved in the care of ALS patients.
Rilutek®, the first treatment to alter the course of ALS, was approved by the FDA in late 1995. This antiglutamate drug appears to prolong the life of persons with ALS by at least a few months and more recent studies suggest Rilutek® slows the progress of ALS, allowing the patient more time in the higher functioning states. Rilutek is manufactured by Aventis Pharmaceuticals.

The information contained on this web site may not be published, broadcast or otherwise distributed without the prior written authorization of The ALS Association.

The ALS Association Greater Los Angeles Chapter • P.O. Box 565, Agoura Hills, CA 91376-0565, Tel: (818) 865-8067
The information contained on this web site may not be published, broadcast or otherwise distributed without the prior written authorization of The ALS Association.

ALSGLAC Webmaster | Privacy Policy | Link Policy and Disclaimer
Site designed by Henderson Multimedia. Hosting Donated by