Amyotrophic lateral sclerosis
Definition:
Amyotrophic lateral sclerosis (a-mi-oh-TROH-fik LAT-ur-ul skluh-ROH-sis), or ALS, is a serious neurological disease that causes muscle weakness, disability and eventually death. ALS is often called Lou Gehrig's disease, after the famous baseball player who was diagnosed with it in 1939. In the U.S., ALS and motor neuron disease (MND) are sometimes used interchangeably.
Worldwide, ALS occurs in 1 to 3 people per 100,000. In the vast majority of cases — 90 to 95 percent — doctors don't yet know why ALS occurs. About 5 to 10 percent of ALS cases are inherited.
ALS often begins with muscle twitching and weakness in an arm or leg, or with slurring of speech. Eventually, ALS affects your ability to control the muscles needed to move, speak, eat and breathe.
Symptoms:
Early signs and symptoms of ALS include:
Causes:
In ALS, the nerve cells that control the movement of your muscles gradually die, so your muscles progressively weaken and begin to waste away. Up to 1 in 10 cases of ALS is inherited. But the remainder appear to occur randomly.
Researchers are studying several possible causes of ALS, including:
As the disease progresses, people with ALS experience one or more of the following complications:
Breathing problems
ALS eventually paralyzes the muscles needed to breathe. Some devices to assist your breathing are worn only at night and are similar to devices used by people who have sleep apnea (for example, continuous positive airway pressure, or CPAP, masks). In the latter stages of ALS, some people choose to have a tracheostomy — a surgically-created hole at the front of the neck leading to the windpipe (trachea) — to enable the full-time use of a respirator that inflates and deflates their lungs.
The most common cause of death for people with ALS is respiratory failure, usually within three to five years after symptoms begin.
Eating problems
When the muscles that control swallowing are affected, people with ALS can develop malnutrition and dehydration. They are also at higher risk of aspirating food, liquids or secretions into the lungs, which can cause pneumonia. A feeding tube can reduce these risks.
Dementia
Some people with ALS experience problems with memory and making decisions, and some are eventually diagnosed with a form of dementia called frontotemporal dementia.
Treatments and drugs:
Because there's no reversing the course of amyotrophic lateral sclerosis, treatments mainly involve efforts to slow the progression of symptoms and make you more comfortable and independent.
Medications
The drug riluzole (Rilutek) is the first and only medication approved by the Food and Drug Administration for slowing ALS. The drug appears to slow the disease's progression in some people, perhaps by reducing levels of glutamate — a chemical messenger in the brain that's often present in higher levels in people with ALS.
Your doctor may also prescribe medications to provide relief from:
Definition:
Amyotrophic lateral sclerosis (a-mi-oh-TROH-fik LAT-ur-ul skluh-ROH-sis), or ALS, is a serious neurological disease that causes muscle weakness, disability and eventually death. ALS is often called Lou Gehrig's disease, after the famous baseball player who was diagnosed with it in 1939. In the U.S., ALS and motor neuron disease (MND) are sometimes used interchangeably.
Worldwide, ALS occurs in 1 to 3 people per 100,000. In the vast majority of cases — 90 to 95 percent — doctors don't yet know why ALS occurs. About 5 to 10 percent of ALS cases are inherited.
ALS often begins with muscle twitching and weakness in an arm or leg, or with slurring of speech. Eventually, ALS affects your ability to control the muscles needed to move, speak, eat and breathe.
Symptoms:
Early signs and symptoms of ALS include:
- Difficulty lifting the front part of your foot and toes (footdrop)
- 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
Causes:
In ALS, the nerve cells that control the movement of your muscles gradually die, so your muscles progressively weaken and begin to waste away. Up to 1 in 10 cases of ALS is inherited. But the remainder appear to occur randomly.
Researchers are studying several possible causes of ALS, including:
- Gene mutation. Various genetic mutations can lead to inherited forms of ALS, which appear nearly identical to the non-inherited forms.
- Chemical imbalance. People who have ALS typically 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, and scientists have speculated that this may trigger the process that results in ALS.
- Protein mishandling. There's evidence that mishandled proteins within the nerve cells can lead to a gradual accumulation of abnormal forms of these proteins in the cells, eventually causing the nerve cells to die.
As the disease progresses, people with ALS experience one or more of the following complications:
Breathing problems
ALS eventually paralyzes the muscles needed to breathe. Some devices to assist your breathing are worn only at night and are similar to devices used by people who have sleep apnea (for example, continuous positive airway pressure, or CPAP, masks). In the latter stages of ALS, some people choose to have a tracheostomy — a surgically-created hole at the front of the neck leading to the windpipe (trachea) — to enable the full-time use of a respirator that inflates and deflates their lungs.
The most common cause of death for people with ALS is respiratory failure, usually within three to five years after symptoms begin.
Eating problems
When the muscles that control swallowing are affected, people with ALS can develop malnutrition and dehydration. They are also at higher risk of aspirating food, liquids or secretions into the lungs, which can cause pneumonia. A feeding tube can reduce these risks.
Dementia
Some people with ALS experience problems with memory and making decisions, and some are eventually diagnosed with a form of dementia called frontotemporal dementia.
Treatments and drugs:
Because there's no reversing the course of amyotrophic lateral sclerosis, treatments mainly involve efforts to slow the progression of symptoms and make you more comfortable and independent.
Medications
The drug riluzole (Rilutek) is the first and only medication approved by the Food and Drug Administration for slowing ALS. The drug appears to slow the disease's progression in some people, perhaps by reducing levels of glutamate — a chemical messenger in the brain that's often present in higher levels in people with ALS.
Your doctor may also prescribe medications to provide relief from:
- Muscle cramps
- Constipation
- Fatigue
- Excessive salivation
- Excessive phlegm
- Pain
- Depression
- Physical therapy. A physical therapist can
recommend low-impact exercises to maintain your cardiovascular fitness,
muscle strength and range of motion as long as possible, helping you
preserve a sense of independence. Regular exercise can also help improve
your sense of well-being.
- Occupational therapy. An occupational therapist can
help you become accustomed to a brace, walker or wheelchair and may be
able to suggest devices such as ramps that make it easier for you to get
around.
- Speech therapy. Because ALS affects the muscles you use to speak, communication becomes an issue as the disease progresses. A speech therapist can help teach adaptive techniques to make your speech more clearly understood or help you explore alternative methods of communication, such as an alphabet board or simple pen and paper. Later in the disease, a speech therapist can recommend devices such as speech synthesizers and computer-based equipment that may help you communicate. Ask your therapist about the possibility of borrowing or renting these devices
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