Description

Amyotrophic lateral sclerosis (a-my-o-TROE-fik LAT-ur-ul skluh-ROE-sis), also known as amyotrophic lateral sclerosis, is a disease of the nervous system that affects nerve cells in the brain and the spinal cord. ALS causes of the loss of muscle control. The disease gets worse over time.

ALS is often called Lou Gehrig's disease after the baseball player who was diagnosed with it. The exact cause of the disease is not yet known. A small number of cases are hereditary.

ALS often begins with muscle twitching and weakness in an arm or leg, difficulty swallowing or slurred speech. Eventually, als affects control of the muscles needed to move, speak, eat and breathe. There is No cure for this deadly disease.

Symptoms

The symptoms of ALS vary from person to person. The symptoms depend on which nerve cells are the most affected. ALS often begins with muscle weakness that spreads and gets worse with time. Symptoms may include:

  • Difficulty walking or doing normal daily activities.
  • Stumble and fall.
  • Weakness in the legs, feet or ankles.
  • Hand weakness or clumsiness.
  • Difficulty speaking or difficulty swallowing.
  • The weakness associated with muscle cramps and spasms in the arms, shoulders and tongue.
  • Untimely, crying, laughing or yawning.
  • Thought or behavior changes.

ALS often begins in the hands, feet, arms, or legs. Then it spreads to other parts of the body. The muscles weaken as more nerve cells die. This eventually affects chewing, swallowing, speaking and breathing.

There is usually no pain in the early stages of amyotrophic lateral sclerosis . The pain also is not common in the later stages. The COMMONWEALTH does not usually affect control of the bladder. Also, in general, does not affect the senses, including the ability to taste, smell, touch and hear.

Causes

ALS affects the nerve cells that control voluntary muscle movements such as walking and talking. These nerve cells called motor neurons. There are two groups of motor neurons. The first group extends from the brain to the spinal cord to the muscles throughout the body. Are called upper motor neurons. The second group extends from the spinal cord to the muscles throughout the body. They are referred to as the lower motor neurons.

ALS causes of both groups of motor neurons to slowly deteriorate and die. When motor neurons are damaged, they stop sending messages to the muscles. As a result, the muscles can't function.

Approximately 10% of people with amyotrophic lateral sclerosis , a genetic cause can be identified. For the rest, the cause is not known.

Researchers are studying the possible causes of ALS . Most theories center of a complex interaction between genes and factors in the environment.

Risk factors

Established risk factors for ALS include:

  • Genetics. Approximately 10% of people with ALS , a risk gene is passed from one member of the family. This is called hereditary ALS . In most people with inherited ALS , their children have a 50% chance of inheriting the gene.
  • Age. The risk increases with age until the age of 75. ALS is more common between the ages of 60 and the middle 80's.
  • Sex. Before the age of 65 years, slightly more men than women develop ALS . This sex difference disappears after 70 years of age.

Environmental factors, such as the following, have been associated with an increased risk of ALS .

  • The habit of smoking. The evidence supports that smoking is an environmental risk factor for ALS . Women who smoke appear to be at higher risk, particularly after the menopause.
  • Environmental toxin exposure. There is some evidence that suggests that exposure to lead and other substances in the workplace or in the home could be linked to ALS . Much study has been done, but not an agent or other chemical has been consistently associated with amyotrophic lateral sclerosis .
  • Military service. Studies indicate that people who have served in the armed forces are at greater risk of ALS . It is not clear what about the military service might trigger the COMMONWEALTH . May include exposure to certain metals or chemicals, traumatic injuries, infections, viral, or an intense workout.

Complications

As the disease progresses, amyotrophic lateral sclerosis, causes complications, such as:

Breathing problems

Over time, ALS leads to weakness of the muscles used for breathing. People with ALS may be that you need a device such as a mask ventilator to help breathing at night. The device is similar to what someone with sleep apnea might use. This type of device is compatible with the person's breathing through a mask over the nose, the mouth, or both.

Some people with advanced ALS choose a tracheostomy. This is a surgically created hole in the front of the neck that leads to the trachea. A fan may work better in a tracheostomy mask.

The most common cause of death in people with ALS is respiratory failure. Half of people with ALS die within 14 to 18 months of diagnosis. However, some people with ALS to live 10 years or more.

Speaking of problems

Most people with ALS develop the weakness of the muscles used to form speech. This usually begins with slow speech and occasional slurring of the words. Then it becomes difficult to speak clearly. This can progress to the point that others can't understand the speech. Other forms of communication and technology it uses to communicate.

Power problems

People with ALS may develop weakness of the muscles involved in swallowing. This can lead to malnutrition and dehydration. They are also at higher risk of getting food, liquid or saliva into the lungs, which can cause pneumonia. A feeding tube can reduce these risks and to ensure adequate hydration and nutrition.

Dementia

Some people with this condition have problems with the language and decision making. Some are eventually diagnosed with a form of dementia called frontotemporal dementia.

Diagnosis

Amyotrophic lateral sclerosis, also known as amyotrophic lateral sclerosis , it can be difficult to diagnose early because it can have symptoms similar to those of other diseases. Tests to rule out other diseases or help diagnose ALS may include:

  • Electromyogram (EMG). A needle is inserted through the skin into various muscles. The test records the electrical activity of your muscles when they contract and when they are at rest. This can determine if there is a problem with the muscles or nerves.
  • The nerve conduction study. This study measured the nerves ' ability to send impulses to the muscles in different areas of the body. This test can determine if you have nerve damage. EMG and nerve conduction studies are almost always done together.
  • The magnetic resonance imaging. The use of radio waves and a powerful magnetic field, an mri produces detailed images of the brain and the spinal cord. An mri can reveal tumors of the spinal cord, disc herniations in the neck, or other conditions that could be causing your symptoms. The highest resolution of the camera can sometimes see ALS changes themselves.
  • Blood and urine tests. The analysis of blood and urine samples in the laboratory can help to eliminate other possible causes of your symptoms. Serum neurofilament light levels, which are measured from blood samples, are usually high in people with ALS . The test can help to make an early diagnosis of the disease.
  • Spinal tap, also known as a lumbar puncture. This involves extracting a sample of cerebrospinal fluid for laboratory testing. The cerebrospinal fluid is removed through a small needle is inserted between two bones in the lower part of the back. The fluid of the spinal cord appears typical in people with ALS, but, you can discover another cause of the symptoms.
  • The muscle biopsy. If your doctor believes you may have a disease of the muscle in place of the COMMONWEALTH , you could undergo a muscle biopsy. While you are under local anesthesia, a small piece of muscle is removed and sent to a laboratory for analysis.
  • In the nerve biopsy. If your doctor believes you may have a nerve disease in place of ALS , you may be subjected to a biopsy of the nerve. While you are under local anesthesia, a small piece of nerve is removed and sent to a laboratory for analysis.

Treatment

The treatments cannot reverse the damage of the COMMONWEALTH , but may delay the progression of symptoms. You can also help prevent complications and make you more comfortable and independent.

You might need a team of health care providers and doctors trained in many areas to provide your care. The team works together to prolong their survival and improve their quality of life.

His team works to select the right treatment for you. You have the right to choose or reject any of the treatments suggested.

Drugs

The Food and Drug Administration has approved two drugs for the treatment of ALS :

  • Riluzole (Rilutek, Exservan, Tiglutik). It is taken by mouth, this drug may increase the life expectancy by about 25%. Can cause side effects such as dizziness, gastrointestinal disorders, and liver problems. Your health care provider usually oversees the function of your liver with the periodic drawing of blood while you are taking the medication.
  • Edaravone (Took Root). This drug may also reduce the rate of decline in daily functioning. It is given through a vein in your arm or by mouth as a liquid. The effect on the duration of life is not yet known. Side effects may include bruising, headache, and difficulty walking. This drug is administered daily for two weeks each month.

Your doctor may also prescribe treatments for the relief of other symptoms, such as:

  • Muscle cramps and spasms.
  • The constipation.
  • Fatigue.
  • Excessive saliva and phlegm.
  • Pain.
  • Depression.
  • Sleep problems.
  • Uncontrollable fits of laughter or crying.
  • An urgent need to urinate.
  • The swelling of the legs.

Therapies

When the ALS affects your ability to breathe, speak, and move, therapies and other forms of support can help.

  • The breathing of attention.Most people withALSeventually have more trouble breathing as the muscles weaken. Your health care provider might test your regular breathing and provide devices known as mechanical ventilation to assist their breathing in the night. You may choose the use of a respirator with a mask that can be easily applied and removed. This is known as non-invasive ventilation. Some people eventually undergo a surgery that creates a hole in the front of the neck, which leads to his windpipe. This is called a tracheostomy. A tube that is inserted in the hole is connected to a respirator to help them breathe. Sometimes people withALSwho have a trach also have a type of surgery called a laryngectomy. This surgery prevents food from entering the lungs.
  • The physical therapy.A physical therapist can treat the pain, walking, mobility, strengthening and equipment needs that help you to stay independent. The practice of low-impact exercises can help maintain your cardiovascular health, muscle strength and range of motion for as long as possible. Regular exercise can also help improve your sense of well-being. Proper stretching can help prevent the pain and help the muscles to function at its best. A physical therapist can also help to overcome the weakness by the use of a brace, walker, or wheelchair. The therapist can 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 the 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 house, to allow access if you have problems to walk safely.
  • Speech therapy.A speech therapist can teach adaptive techniques to make your speech more understandable. Speech therapists can also help you find other ways to communicate. These may include the use of a smart phone app, the alphabet of the board, or pen and paper. Talk with your therapist about the possibility of recording your own voice to be used by a text-to-speech application.
  • Nutritional support. Your computer normally works with you and members of your family to ensure that you are eating foods that are easier to swallow and meet their nutritional needs. You can choose to have a feeding tube is placed to when it becomes too difficult to swallow.
  • Psychological and social support. Your computer might include a social worker to help with the financial issues, insurance, and get all the equipment and the payment for the devices that you need. Psychologists, social workers, and others who can provide emotional support for you and your family.

The breathing of attention. The majority of people with ALS, finally, they have more difficulty breathing as the muscles weaken. Your health care provider might test your regular breathing and provide devices known as mechanical ventilation to assist their breathing in the night.

You may choose the use of a respirator with a mask that can be easily applied and removed. This is known as non-invasive ventilation. Some people eventually undergo a surgery that creates a hole in the front of the neck, which leads to his windpipe. This is called a tracheostomy. A tube that is inserted in the hole is connected to a respirator to help them breathe. Sometimes, people with ALS who have a trach also have a type of surgery called a laryngectomy. This surgery prevents food from entering the lungs.

The physical therapy. A physical therapist can treat the pain, walking, mobility, strengthening and equipment needs that help you to stay independent. The practice of low-impact exercises can help maintain your cardiovascular health, muscle strength and range of motion for as long as possible.

Regular exercise can also help improve your sense of well-being. Proper stretching can help prevent the pain and help the muscles to function at its best.

A physical therapist can also help to overcome the weakness by the use of a brace, walker, or wheelchair. The therapist can 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 the 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 house, to allow access if you have problems to walk safely.

Speech therapy. A speech therapist can teach adaptive techniques to make your speech more understandable. Speech therapists can also help you find other ways to communicate. These may include the use of a smart phone app, the alphabet of the board, or pen and paper.

Talk with your therapist about the possibility of recording your own voice to be used by a text-to-speech application.

Potential future treatments

Based on the current understanding of ALS , the researchers are conducting clinical studies on the promise of drugs and treatments.

Coping and support

The learning takes the ALS can be devastating. The following tips can help you and your family deal:

  • Take the time to grieve. The news that you have a very serious condition that reduced his mobility and independence is hard to listen to. You and your family can go through a period of mourning and the pain after the diagnosis.
  • Be optimistic. Your computer can help you focus on your skills and healthy lifestyle. Some people with ALS live longer than the 3 to 5 years usually associated with this condition. Some of live 10 years or more. Maintain an optimistic outlook can help to improve the quality of life of people with ALS .
  • Think beyond the physical changes. Many people with ALS lead rewarding lives despite physical limitations. Try to think of the COMMONWEALTH, as only a part of your life, not your entire identity.
  • Join a support group. You can find consolation in a support group with other people who have amyotrophic lateral sclerosis . Loved ones to help you with your care may benefit from a support group of other COMMONWEALTH caregivers. Find support groups in your area by talking with your doctor or by contacting the ALS Association.
  • Make decisions about your medical care in the future.Planning for the future, allows you to be in control of the decisions about your life and your care. It also decreases the burden on your loved ones. With the help of your health care provider, nurses, or social workers, you can decide if you want to extend the life of the procedures. You can decide where you want to spend your last days. You might want to consider hospice care options. Planning for the future can help you and your loved ones to calm anxieties.
  • Considering getting involved inALSresearch. ALS research is working to find a cure for amyotrophic lateral sclerosis . Consider joining a clinical trial, providing samples for the research and to join the National ALS Registry. The registry is open to all people with ALS . Many institutions collect samples for research to better understand the disease.

Make decisions about your medical care in the future. Planning for the future, allows you to be in control of the decisions about your life and your care. It also decreases the burden on your loved ones. With the help of your health care provider, nurses, or social workers, you can decide if you want to extend the life of the procedures.

You can decide where you want to spend your last days. You might want to consider hospice care options. Planning for the future can help you and your loved ones to calm anxieties.

Preparing for your appointment

Your primary care provider may be the first to recognize the symptoms of ALS . Your provider will likely refer you to a medical doctor trained in nervous system conditions, known as a neurologist, to establish a diagnosis.

What you can do

You may need many tests to diagnose your condition. The diagnostic process can be stressful and frustrating. These strategies can give you a greater sense of control.

  • Keep a diary of symptoms. Before you see a neurologist, start using a calendar or a notebook to write down when and how you notice symptoms. Record information about your problems with walking, coordination of hands, speech, swallowing, and / or involuntary muscle movements. Your notes may show a pattern that is useful for its diagnosis.
  • Find a neurologist and the care team. An integrated care team led by neurologist is usually the most appropriate for the COMMONWEALTH of attention. Your team is usually communicates with each other and are familiar with their needs.

What to expect from your doctor

Your primary care provider is likely that the review of your family's medical history and your symptoms. Your neurologist and your primary care doctor can perform a physical and neurological examination. This can include evidence of your:

  • Reflections.
  • The muscle strength.
  • The muscle tone.
  • The senses of touch and sight.
  • Coordination.
  • Balance.
Symptoms and treatment of Amyotrophic lateral sclerosis (ALS)