About amyotrophic lateral sclerosis

What is amyotrophic lateral sclerosis
(als or

Amyotrophic lateral sclerosis facts

  • Amyotrophic lateral sclerosis is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells responsible for controlling voluntary muscles.
  • Amyotrophic lateral sclerosis, or ALS, is sometimes called Lou Gehrig's disease.
  • As many as 20,000 to 30,000 people in the United States have ALS, and an estimated 5,000 people in the United States are diagnosed with the disease each year.
  • Only about 5 to 10 percent of all ALS cases are inherited in the family's genes.
  • Early symptoms of ALS are subtle and may include twitching, cramping, or stiffness of muscles; muscle weakness affecting an arm or a leg; slurred and nasal speech; or difficulty chewing or swallowing.
  • No one test can provide a definitive diagnosis of ALS, although the presence of upper and lower motor neuron signs in a single limb is strongly suggestive.
  • The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others. Scientists have discovered that mutations in the gene that produces the SOD1 enzyme were associated with some cases of familial ALS.
  • No cure has yet been found for ALS. The first drug treatment for the disease -- riluzole (Rilutek) is believed to reduce damage to motor neurons by decreasing the release of glutamate. Clinical trials with ALS patients showed that riluzole prolongs survival by several months and extends the time before a patient needs ventilation support. Other treatments for ALS are designed to relieve symptoms and improve the quality of life for patients.

What is amyotrophic lateral sclerosis?

Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig's disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles. The disease belongs to a group of disorders known as motor neuron diseases, which are characterized by the gradual degeneration and death of motor neurons.

Motor neurons are nerve cells located in the brain, brainstem, and spinal cord that serve as controlling units and vital communication links between the nervous system and the voluntary muscles of the body. Messages from motor neurons in the brain (called upper motor neurons) are transmitted to motor neurons in the spinal cord (called lower motor neurons) and from them to particular muscles. In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away (atrophy), and twitch (fasciculations) . Eventually, the ability of the brain to start and control voluntary movement is lost.

ALS causes weakness with a wide range of disabilities (see section titled "What are the symptoms?"). Eventually, all muscles under voluntary control are affected, and patients lose their strength and the ability to move their arms, legs, and body. When muscles in the diaphragm and chest wall fail, patients lose the ability to breathe without ventilatory support. Most people with ALS die from respiratory failure, usually within 3 to 5 years from the onset of symptoms. However, about 10 percent of ALS patients survive for 10 or more years.

Although the disease usually does not impair a person's mind or intelligence, several recent studies suggest that some ALS patients may have alterations in cognitive functions such as depression and problems with decision-making and memory.

ALS does not affect a person's ability to see, smell, taste, hear, or recognize touch. Patients usually maintain control of eye muscles and bladder and bowel functions, although in the late stages of the disease most patients will need help getting to and from the bathroom.



What are the symptoms for amyotrophic lateral sclerosis
(als or

Cramping symptom was found in the amyotrophic lateral sclerosis condition

ALS often starts in the hands, feet or limbs, and then spreads to other parts of your body. As the disease advances and nerve cells are destroyed, your muscles get weaker. This eventually affects chewing, swallowing, speaking and breathing.

There's generally no Pain in the early stages of ALS, and Pain is uncommon in the later stages. ALS doesn't usually affect your bladder control or your senses.



What are the causes for amyotrophic lateral sclerosis
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ALS affects the nerve cells that control voluntary muscle movements such as walking and talking (motor neurons). ALS causes the motor neurons to gradually deteriorate, and then die. Motor neurons extend from the brain to the spinal cord to muscles throughout the body. When motor neurons are damaged, they stop sending messages to the muscles, so the muscles can't function.

ALS is inherited in 5% to 10% of people. For the rest, the cause isn't known.

Researchers continue to study possible causes of ALS. Most theories center on a complex interaction between genetic and environmental factors.



What are the treatments for amyotrophic lateral sclerosis
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Treatments can't reverse the damage of amyotrophic lateral sclerosis, but they can slow the progression of symptoms, prevent complications, and make you more comfortable and independent.

You might need an integrated team of doctors trained in many areas and other health care professionals to provide your care. This might prolong your survival and improve your quality of life.

Your team will help you select the right treatments for you. You have the right to choose or refuse any of the treatments suggested.

Medications

The Food and Drug Administration has approved two drugs for treating ALS:

  • Riluzole (Rilutek). Taken orally, this drug has been shown to increase life expectancy by 3 to 6 months. It can cause side effects such as dizziness, gastrointestinal conditions and liver function changes. Your doctor will monitor your blood counts and liver functions while you're on the drug.
  • Edaravone (Radicava). This drug, given by intravenous infusion, has been shown to reduce the decline in daily functioning. Its effect on life span isn't yet known. Side effects can include bruising, headache and shortness of breath. This medication is given daily for two weeks a month.

Your doctor might also prescribe medications to provide relief from other symptoms, including:

  • Muscle cramps and spasms
  • Constipation
  • Fatigue
  • Excessive saliva and phlegm
  • Pain
  • Depression
  • Sleep problems
  • Uncontrolled outbursts of laughing or crying

Therapies

  • Breathing care. You'll eventually have more difficulty breathing as your muscles weaken. Doctors might test your breathing regularly and provide you with devices to assist your breathing at night.

    You can choose mechanical ventilation to help you breathe. Doctors insert a tube in a surgically created hole at the front of your neck leading to your windpipe (tracheostomy) that connects to a respirator.

  • Physical therapy. A physical therapist can address pain, walking, mobility, bracing and equipment needs that help you stay independent. Practicing low-impact exercises can help maintain your cardiovascular fitness, muscle strength and range of motion for as long as possible.

    Regular exercise can also help improve your sense of well-being. Appropriate stretching can help prevent pain and help your muscles function at their best.

    A physical therapist can also help you adjust to a brace, walker or wheelchair and might suggest devices such as ramps that make it easier for you to get around.

  • Occupational therapy. An occupational therapist can help you find ways to remain independent despite hand and arm weakness. Adaptive equipment can help you perform activities such as dressing, grooming, eating and bathing.

    An occupational therapist can also help you modify your home to allow accessibility if you have trouble walking safely.

  • Speech therapy. A speech therapist can teach you adaptive techniques to make your speech more understandable. Speech therapists can also help you explore other methods of communication, such as an alphabet board or pen and paper.

    Ask your therapist about the possibility of borrowing or renting devices such as tablet computers with text-to-speech applications or computer-based equipment with synthesized speech that can help you communicate.

  • Nutritional support. Your team will work with you and your family members to ensure you're eating foods that are easier to swallow and meet your nutritional needs. You might eventually need a feeding tube.
  • Psychological and social support. Your team might include a social worker to help with financial issues, insurance, and getting equipment and paying for devices you need. Psychologists, social workers and others may provide emotional support for you and your family.

Potential future treatments

Clinical studies on promising medications and treatments are occurring for ALS all the time.



What are the risk factors for amyotrophic lateral sclerosis
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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 risk increases with age, and is most common between the ages of 40 and the mid-60s.
  • Sex. Before the age of 65, slightly more men than women develop ALS. This sex difference disappears after age 70.
  • Genetics. Some studies examining the entire human genome found many similarities in the genetic variations of people with familial ALS and some people with noninherited ALS. These genetic variations might make people more susceptible to ALS.

Environmental factors, such as the following, might trigger ALS.

  • Smoking. Smoking is the only likely environmental risk factor for ALS. The risk seems to be greatest for women, particularly after menopause.
  • Environmental toxin exposure. Some evidence suggests that exposure to lead or other substances in the workplace or at home might be linked to ALS. Much study has been done, but no single agent or chemical has been consistently associated with ALS.
  • Military service. Studies indicate that people who have served in the military are at higher risk of ALS. It's unclear what about military service might trigger the development of ALS. It might include exposure to certain metals or chemicals, traumatic injuries, viral infections, and intense exertion.



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