Amyotrophic Lateral Sclerosis (ALS)

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Amyotrophic Lateral Sclerosis (ALS)

Topic Overview

Picture of the brain

What is amyotrophic lateral sclerosis?

Amyotrophic lateral sclerosis, or ALS, is a disease in which certain nerve cells in the brain and spinal cord slowly die. These nerve cells are called motor neurons, and they control the muscles that allow you to move the parts of your body. ALS is also called Lou Gehrig’s disease.

People with ALS gradually become more disabled. How quickly the disease gets worse is different for everyone. Some people live with ALS for several years. But over time, ALS makes it hard to walk, speak, eat, swallow, and breathe. These problems can lead to injury, illness, and eventually death.

It can be very scary to learn that you have ALS. Talking with your doctor, getting counselling, or joining a support group may help you deal with your feelings. Your family members may also need support or counselling as your disease gets worse.

ALS is rare. Each year in Canada, the United States, and most of the world, only 1 to 2 people out of 100,000 get ALS. Men get ALS slightly more often than women do. ALS can occur at any age, but it most often starts in middle-aged and older adults.1

What causes ALS?

Doctors don't know what causes ALS. In about 1 case out of 10, it runs in families.1 This means that 9 times out of 10, a person with ALS doesn't have a family member with the disease.

ALS is not contagious. So it can't be spread from one person to another.

What are the symptoms?

The first sign of ALS is often weakness in one leg, one hand, the face, or the tongue. The weakness slowly spreads to both arms and both legs. This happens because as the motor neurons slowly die, they stop sending signals to the muscles. So the muscles don't have anything telling them to move. Over time, with no signals from the motor neurons telling the muscles to move, the muscles get weaker and smaller.

Over time, ALS also causes:

  • Muscle twitching.
  • Trouble using your hands and fingers to do tasks.
  • Problems with speaking, swallowing, eating, walking, and breathing.

ALS does not cause numbness, tingling, or loss of feeling.

Just because you have muscle weakness, fatigue, stiffness, and twitching doesn't mean that you have ALS. Those symptoms can also be caused by other conditions. So talk to your doctor if you have those symptoms.

How is ALS diagnosed?

It can be hard for your doctor to tell if you have ALS. It may not be clear that you have the disease until symptoms get worse or until your doctor has done more testing. To find out if you have ALS, your doctor will do a physical examination and will ask you about your symptoms and past health. You will also have tests that show how your muscles and nerves are working.

Tests needed to confirm ALS may include:

If your doctor thinks that you have ALS, he or she may also refer you to a neurologist to make sure.

How is it treated?

There is no cure for ALS, but treatment can help you stay strong and independent for as long as possible. It can also help you avoid other problems from ALS.

Physiotherapy and occupational therapy can help you stay strong and make the most of the abilities you still have. Speech therapy can help you keep your ability to talk after problems with speech begin. And there are several medicines that doctors can use to help relieve your symptoms and keep you comfortable.

What decisions will you face as ALS progresses?

If you or a family member has ALS, learn as much as you can about the disease and how to take care of it. How much treatment you want for the problems caused by ALS is a personal choice that only you and your loved ones can make. Your values, wants, and needs are important things to think about as you make choices about your care.

As ALS symptoms get worse, you may have to choose which treatments you want for things like problems with breathing and eating. As you make these choices, keep in mind that what's right for one person with ALS may not feel right for another person. It’s important to talk about your treatment options and share your concerns with your doctor.

You may want to put your health care choices in writing. This is called an advance directive or a living will. It gives you control over your own medical care when you can't make decisions or speak for yourself. You may also want to choose a friend or family member to speak for you. This is called a substitute decision-maker. Making these plans ahead of time will help make sure that your health care choices are followed.

Frequently Asked Questions

Learning about amyotrophic lateral sclerosis (ALS):

Being diagnosed:

Getting treatment:

Living with ALS:

End-of-life issues:

Symptoms

The symptoms of amyotrophic lateral sclerosis (ALS) include:

  • Weakness or clumsiness in the hands and feet.
  • Gradual loss of strength of the arms and legs.
  • Inability to control the use of the arms and legs.
  • Muscle twitches (fasciculations).
  • A stiff, clumsy walking style.
  • Problems swallowing, speaking, and breathing. You may notice a problem with drooling.
  • Fatigue.
  • Trouble controlling your emotions. You may burst into tears or start laughing for no reason.
  • Muscle cramps, especially at night, which may occur late in the course of the disease.

It is important to remember that having muscle weakness, fatigue, stiffness, and twitching doesn't necessarily mean that you have ALS.

The first sign of ALS is usually slight weakness in one leg, one hand, the face, or the tongue. Other problems may include increasing clumsiness and trouble performing tasks that require precise movements of the fingers and hands. Muscle twitching may also occur. The weakness slowly spreads to the arms and legs over a period of months or years. As the nerves continue to waste away and decrease in number, the muscle cells that would normally be stimulated by those nerves also start to waste away, and the muscles weaken.

ALS is a progressive, disabling disease. Walking, speaking, eating, swallowing, breathing, and other basic functions become harder with time. These problems can lead to injury, illness, and other complications.

Respiratory problems are the most common serious complication of ALS. As the muscles in the throat and chest area become weak, swallowing and coughing become harder, which may sometimes cause food and saliva to be inhaled into the windpipe (aspiration). This may lead to pneumonia. Breathing problems tend to get worse as the disease progresses, increasing the risk of infection and respiratory failure.

Pneumonia, pulmonary embolism, lung failure, and heart failure (probably due to lack of adequate breathing as the chest muscles weaken) are the most common causes of death among people with ALS. In most cases, death will occur within 3 to 5 years after symptoms begin, although some people live for many years, even decades.

Examinations and Tests

A diagnosis of amyotrophic lateral sclerosis (ALS) is based on a careful medical history, a physical examination of the nervous system, and tests that show how the nerves and muscles are working.

If your doctor thinks you have ALS, you will be referred to a neurologist for diagnosis and treatment. A medical history and detailed physical examination of the nervous system can usually help the neurologist diagnose your condition. Tests needed to confirm the diagnosis include electromyogram (EMG) and nerve conduction studies. EMG helps measure how well and how quickly the nerves and muscles are functioning. Nerve conduction studies test nerve function.

Depending on your doctor's findings during the nervous system examination and the results of EMG or nerve conduction studies, other tests may be needed to rule out other possible causes of your symptoms. These tests may include a nerve or muscle biopsy (tissue sample), blood tests, or imaging tests, such as a computed tomography (CT) scan or magnetic resonance imaging (MRI).

ALS may be difficult to diagnose, and the diagnosis may not become clear until symptoms have progressed or until additional testing and observation have taken place. If ALS is suspected, evaluation by a specialist who has experience treating and diagnosing ALS may lead to an earlier diagnosis. Although it does not affect the course or outcome of the disease, an early diagnosis may:

  • Give you more time to make decisions about the future and take advantage of the time before symptoms become severe.
  • Help you avoid inappropriate treatment resulting from an incorrect diagnosis.
  • Give you the opportunity to participate in clinical trials of new treatments for ALS.

Treatment Overview

Although there is no cure for amyotrophic lateral sclerosis (ALS), treatment can help you maintain strength and independence, manage symptoms, and avoid complications for as long as possible. Treatment also focuses on providing emotional support as your disability increases.

Physiotherapists and occupational therapists can help you maintain strength and function and make the most of your remaining abilities. Speech therapists can help you maintain your ability to communicate after speaking problems begin.

Medicines may be used to relieve symptoms and make you more comfortable.

  • For extra saliva or drooling, your doctor may prescribe glycopyrrolate, benztropine, scopolamine, atropine, trihexyphenidyl, or amitriptyline. These medicines may be helpful if you have trouble with the muscles that control swallowing.
  • For uncontrollable emotions and mood swings (emotional lability), your doctor may prescribe amitriptyline or fluvoxamine (Luvox).
  • For muscle stiffness, spasms, and twitching, your doctor may prescribe baclofen (Lioresal), tizanidine (Zanaflex), dantrolene (Dantrium), or diazepam ( for example, Valium).
  • For muscle cramps, your doctor may prescribe phenytoin (for example, Dilantin), benzodiazepines, or gabapentin (Neurontin).
  • For depression, your doctor may prescribe antidepressant medicines. These may also help with sleeplessness, poor appetite, or fatigue.
  • For pain late in the course of the disease, your doctor may prescribe pain relievers.
  • For breathing problems (dyspnea), your doctor may use morphine.
  • For anxiety, your doctor may prescribe antianxiety medicines such as diazepam (for example, Valium) or lorazepam (for example, Ativan).

If you are having problems getting enough food or taking medicine, a tube can be inserted through the skin into the stomach (percutaneous endoscopic gastrostomy, or PEG). The tube provides an easier way to get adequate nutrition and take medicines.

Breathing devices may be needed as the muscles of the chest become affected. Initially, extra oxygen can be given through a nasal cannula, a flexible plastic tube that is placed in the nostrils. For people who have mild breathing problems, non-surgical forms of ventilation may be used. These include bilevel positive airway pressure (BiPAP) and non-invasive positive pressure ventilation (NPPV), which deliver air or extra oxygen through a face mask. But in some cases, an opening in the neck leading to the windpipe (tracheostomy) may have to be created and oxygen delivered through a tube in the opening.

At first, breathing devices may be needed only part of the time, such as when you are sleeping. As the disease and breathing problems get worse, you may need to be on a breathing machine all the time.

Riluzole

A medicine called riluzole (Rilutek) may prolong survival by about 2 months.2 It is not known exactly how the medicine works, but it may slow the release of certain brain chemicals (neurotransmitters) that are believed to play a role in ALS.

There are some disadvantages to taking riluzole. Although it has been shown to prolong survival for about 2 months, it does not improve symptoms or quality of life in ways that people with ALS, their caregivers, or their doctors have been able to detect. Most people tolerate riluzole very well, but it can cause side effects, including nausea, vomiting, weakness, dizziness, and coughing. Because riluzole can cause liver problems, people taking the drug need to have their liver checked on a regular basis. Treatment with riluzole is also expensive, and it may not help some people.

Scientists are testing many other medicines to see if they work for people with ALS. An updated list of clinical trials to test these drugs in people with ALS can be found on the ALS Association website (www.alsa.org) or at www.clinicaltrials.gov.

Scientists are testing many other medicines to see if they work for people with ALS. More information on clinical trials can be found on the ALS Society of Canada website (www.als.ca).

Home Treatment

If you have amyotrophic lateral sclerosis (ALS), it is important for you and your family to learn to cope with the effects of the disease. Much of your care will take place at home, and good home treatment can sometimes help manage symptoms and prevent complications.

Early in the course of the disease, light aerobic exercise and slow, easy stretching may help you stay strong, reduce fatigue and depression, and prevent muscle cramps and spasms. A physiotherapist can help plan safe physical activities and evaluate your changing needs throughout the course of the disease.

People who have ALS often have problems chewing and swallowing. Making changes in your diet can help you avoid choking and ensure adequate nutrition. These might include eating smaller, more frequent meals and choosing foods that are soft and easy to swallow.

As ALS progresses, it will become more and more difficult to move around and perform basic daily activities. A variety of supportive devices and equipment can help you avoid injury and remain independent. These may include:

  • A neck (cervical) collar to support the head if the neck muscles become weak.
  • Foot and ankle braces, a cane, a walker, or a wheelchair to help you stay mobile.
  • Ramps, handrails, a raised toilet seat, or a shower seat.
  • Erasable writing tablets, voice amplifiers, or other devices to help you communicate if speaking becomes difficult.
  • Cough assist devices. These help you cough if you are unable to cough strongly enough to clear your airway and prevent pneumonia.

There are lots of other devices that can help with bathing, eating, dressing, and communicating. Talk to an occupational therapist for suggestions on what devices might be best and what changes might make it safer and easier for you to live in your home. Ask your doctor about having a home health nurse come to your home to find out about your needs.

Planning for the future

If you or someone in your family has ALS, learn as much as you can about the disease and the options for care. As the disease progresses, you may have choices about care and treatment. As you face these decisions, remember that what's right for one person with ALS may not feel right for another person. What you value and want are important factors in making these choices.

Some people want every possible medical treatment to sustain life, while others prefer measures to maintain their comfort without prolonging life. It may be helpful to think about what kind of medical treatment you want. Discuss and decide how aggressively you want to treat complications of the disease, such as breathing problems, difficulty eating, or pneumonia. These decisions may need to be revisited throughout the course of the disease because you may change your mind over time. Some questions for you to consider include the following:

  • Do you want a tracheostomy when breathing problems become severe? Tracheostomy provides long-term breathing assistance by making an opening in your neck that leads to your windpipe: oxygen can then be delivered through a tube in the opening. There are less invasive forms of breathing assistance that can be used prior to tracheostomy, but most people with the disease eventually face the decision of whether they want long-term mechanical assistance.
  • What type of treatment, if any, do you want if pneumonia or another serious lung infection develops?
  • Do you want a feeding tube placed in your stomach when your ability to swallow is reduced or lost?

You may find it helpful and comforting to state your health care choices in writing, in the form of an advance directive or living will, while you are still able to make and communicate these decisions. Also think about who you would choose as your substitute decision-maker to make and carry out decisions about your care if you were unable to speak for yourself. For more information, see the topics:

A time may come when your goals or the goals of your loved one may change from treating ALS to maintaining comfort and dignity. Hospice palliative care health professionals focus on relieving pain and other symptoms and providing comforting surroundings at the end of life. For more information, see the topic Hospice Palliative Care.

Finding support

The progressive, disabling nature of ALS and the fact that it has no cure make it a very difficult disease to cope with. In addition to medical care, you will need emotional support from family, friends, doctors, and other caregivers. Support groups made up of other people who have ALS may be very helpful, as may counselling by a psychologist or psychiatrist.

Your family members may also need support as your condition deteriorates. Support groups or counselling may be helpful for them as well. Organizations such as the ALS Society of Canada (www.als.ca) provide information on support services for people with ALS and for their families. For more information, see the Other Places to Get Help section of this topic.

Other Places To Get Help

Organization

ALS Society of Canada
265 Yorkland Boulevard
Suite 300
Toronto, ON  M2J 1S5
Phone: 1-800-267-4ALS (1-800-267-4257)
Web Address: www.als.ca
 

The ALS Society of Canada works with regional partners across the country to provide support and information, and promote awareness and understanding of ALS. The society Web site provides links to provincial chapters throughout Canada.


References

Citations

  1. Ropper AH, Samuels MA (2009). Syndrome of muscular weakness and wasting without sensory changes section of Degenerative diseases of the nervous system. In Adams and Victor's Principles of Neurology, 9th ed., pp. 1058–1070. New York: McGraw-Hill.
  2. Miller RG, et al. (2007). Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane Database of Systematic Reviews (1).

Other Works Consulted

  • Miller RG, et al. (2009). Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review). Neurology, 73(15): 1218–1226.
  • Miller RG, et al. (2009). Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: Multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review). Neurology, 73(15): 1227–1233.

Credits

By Healthwise Staff
Primary Medical Reviewer Anne C. Poinier, MD - Internal Medicine
Primary Medical Reviewer Brian D. O'Brien, MD - Internal Medicine
Specialist Medical Reviewer Barrie J. Hurwitz, MD - Neurology
Last Revised September 21, 2010

This information does not replace the advice of a doctor. Healthwise, Incorporated disclaims any warranty or liability for your use of this information.