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AMYOTROPHIC LATERAL SCLEROSIS (ALS)
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ALS was first found out in 1869 by French neurologist Jean-Martin Charcot, but it wasn't until 1939 that Lou Gehrig brought national and international attention to the disease. Ending the career of one of the most beloved baseball players of all time, the disease is still most closely associated with his name. Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects 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 leads to their death. 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.
ALS, is a nervous system (neurological) disease that causes muscle weakness and impacts physical function.
ALS is often called Lou Gehrig's disease, after the famous baseball player who was diagnosed with it. ALS is a type of motor neuron disease that causes nerve cells to gradually break down and die. In the United States, ALS is sometimes called motor neuron disease.
In most cases, doctors don't know why ALS occurs. A small number of cases are inherited.
ALS often begins with muscle twitching and weakness in an arm or leg, or sometimes with slurring of speech. Eventually, ALS can affect your ability to control the muscles needed to move, speak, eat and breathe. ALS can't be cured and eventually leads to death.
Amyotrophic lateral sclerosis (ALS), often referred to as "Lou Gehrig's Disease," is a progressive neurodegenerative disease that affects 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 leads to their death. 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.
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 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 (become smaller). Limbs begin to look "thinner" as muscle tissue atrophies.
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 are affected 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 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, riluzole, that modestly slows the progression of ALS as well as several other drugs in clinical trials that hold promise.
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.
To learn more about the personal stories of people who are living fully, click here. As one man put it, "I've made ALS part of my life, not my whole life."
Early signs and symptoms of ALS include:
- Difficulty walking, tripping or difficulty doing your normal daily activities
- Weakness in your leg, feet or ankles
- Hand weakness or clumsiness
- Slurring of speech or trouble swallowing
- Muscle cramps and twitching in your arms, shoulders and tongue
- Difficulty holding your head up or keeping a good posture
The disease frequently begins in your hands, feet or limbs, and then spreads to other parts of your body. As the disease advances, your muscles become progressively weaker. This weakness eventually affects chewing, swallowing, speaking and breathing.
However, ALS doesn't usually affect your bowel or bladder control, your senses, or your thinking ability. It's possible to remain actively involved with your family and friends.
In ALS, the nerve cells that control the movement of your muscles gradually die, so your muscles progressively weaken and begin to waste away.
ALS is inherited in 5 to 10 percent of cases. The other cases appear to occur randomly.
Researchers are studying several possible causes of ALS, including:
Gene mutation. Various genetic mutations can lead to inherited ALS, which appears nearly identical to the noninherited form.
Chemical imbalance. People with ALS generally have higher than normal levels of glutamate, a chemical messenger in the brain, around the nerve cells in their spinal fluid. Too much glutamate is known to be toxic to some nerve cells.
Disorganized immune response. Sometimes a person's immune system begins attacking some of his or her body's own normal cells, which may lead to the death of nerve cells.
Protein mishandling. Mishandled proteins within the nerve cells may lead to a gradual accumulation of abnormal forms of these proteins in the cells, eventually causing the nerve cells to die.
Established risk factors for ALS include:
Heredity. Five to 10 percent of the people with ALS inherited it (familial ALS). In most people with familial ALS, their children have a 50-50 chance of developing the disease.
Age. ALS most commonly occurs in people between the ages of 40 and 60.
Sex. Before the age of 65, slightly more men than women develop ALS. This sex difference disappears after age 70.
It may be that in some people ALS, is triggered by certain environmental factors.
Also, some studies examining the entire human genome (genome-wide association studies) found numerous genetic variations that people with familial ALS and some people with noninherited ALS had in common. These genetic variations might make people more susceptible to ALS.
Environmental factors under study that may modify a person's individual risk of ALS include:
Smoking. Smoking cigarettes appears to increase a person's risk of ALS to almost twice that of a nonsmoker. The more years spent smoking, the greater the risk. However, quitting smoking can eventually lower the increased risk.
Lead exposure. Some evidence suggests that exposure to lead in the workplace may be associated with the development of ALS.
Military service. Recent studies indicate that people who have served in the military are at higher risk of ALS. Exactly what about military service may trigger the development of ALS is uncertain, but it may include exposure to certain metals or chemicals, traumatic injuries, viral infections and intense exertion.
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