Topic Overview
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 counseling, or
joining a support group may help you deal with your feelings. Your family
members may also need support or counseling as your disease gets worse.
ALS is rare. Each year in 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.2 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 usually doesn't affect the ability to think, reason,
or remember things.
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 exam 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.
Physical 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
durable power of attorney. Making these plans ahead of
time will help make sure that your health care choices are followed.
Frequently Asked Questions
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Learning about amyotrophic lateral sclerosis (ALS):
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Being diagnosed:
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Getting treatment:
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Living with ALS:
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End-of-life issues:
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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.
- Difficulty 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.
- Pain during the late stages of the
disease.
Your ability to think, reason, and remember will most
likely not be affected by 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 difficulty 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 more difficult 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 more difficult, 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.
Exams and Tests
A diagnosis of
amyotrophic lateral sclerosis (ALS) is based on a
careful medical history, a physical exam of the nervous system, and tests that
show how the nerves and muscles are working.
If your health
professional thinks you have ALS, you may be referred to a
neurologist for diagnosis and treatment. A medical
history and detailed physical exam 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 exam 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.
Physical therapists
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. These include:
- Glycopyrrolate (Robinul), benztropine, transdermal hyoscine
(Scopolamine), atropine, trihexyphenidyl hydrochloride, or amitriptyline for
extra saliva or drooling. These medicines may be helpful if you have trouble
with the muscles that control swallowing.
- Amitriptyline or fluvoxamine (Luvox) to help
with uncontrollable emotions and mood swings (emotional lability).
- Baclofen (Lioresal), tizanidine (Zanaflex), dantrolene
(Dantrium), or benzodiazepines such as diazepam (Valium), to relieve muscle
stiffness, spasms, and twitching.
- Quinine, phenytoin sodium
(Dilantin), benzodiazepines, or gabapentin to relieve muscle
cramps.
- Antidepressant medicines, to help with depression,
sleeplessness, poor appetite, or fatigue.
- Pain relievers, to reduce
muscle pain, which sometimes develops late in the course of the disease.
- Morphine, to help with breathing problems (dyspnea). Antianxiety
medicines such as diazepam or lorazepam may be helpful in relieving anxiety
caused by breathing problems. The American Academy of Neurology also considers
acupuncture a useful option for breathing problems
when used in addition to medicine or breathing devices.3
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, nonsurgical forms
of ventilation may be used. These include bilevel positive airway pressure
(BiPAP) and noninvasive 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.4 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. Riluzole
is the only medicine approved for the treatment of 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 Web site (www.alsa.org) or at
www.clinicaltrials.gov.
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
physical therapist 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 health professional 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
health care agent 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 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 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 counseling by a psychologist or
psychiatrist.
Your family members may also need support as your
condition deteriorates. Support groups or counseling may be helpful for them as
well. Organizations such as the ALS Association (www.alsa.org) 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
Organizations
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National Institute of Neurological Disorders and
Stroke
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| P.O. Box 5801 |
| Bethesda, MD 20824 |
| Phone: |
1-800-352-9424
(301) 496-5751 |
| TDD: |
(301) 468-5981 |
| Web Address: |
www.ninds.nih.gov |
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The National Institute of Neurological Disorders and
Stroke (NINDS), a part of the National Institutes of Health, is the leading
U.S. federal government agency supporting research on brain and nervous system
disorders. It provides the public with educational materials and information
about these disorders.
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Amyotrophic Lateral Sclerosis Association
(ALSA)
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| 27001 Agoura Road |
| Suite 150 |
| Calabasas Hills, CA 91301-5104 |
| Phone: |
1-800-782-4747 (information and referral hotline)
(818) 880-9007 |
| Fax: |
(818) 880-9006 |
| Web Address: |
http://www.alsa.org |
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This national ALS Association provides up-to-date educational
materials and information about service and support resources to ALS patients
and their families, caregivers, researchers, and health professionals.
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Les Turner Amyotrophic Lateral Sclerosis (ALS)
Foundation
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| 5550 West Touhy Avenue |
| Suite 302 |
| Skokie, IL 60077-3254 |
| Phone: |
1-888-ALS-1107 (1-888-257-1107)
(847) 679-3311 |
| Fax: |
(847) 679-9109 |
| E-mail: |
info@lesturnerals.org |
| Web Address: |
www.lesturnerals.org |
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The Les Turner ALS Foundation is a nonprofit
organization committed to the treatment and elimination of ALS. It funds the
Lois Insolia ALS Center (an ALS treatment center), research programs, support
groups, communication and equipment services, and education programs for people
who have ALS. The Les Turner ALS Foundation is based in the Chicago area and
affiliated with Northwestern University Medical School.
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Muscular Dystrophy Association (MDA)—ALS
Division
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| Muscular Dystrophy Association National Headquarters |
| 3300 East Sunrise Drive |
| Tucson, AZ 85718 |
| Phone: |
1-800-572-1717 |
| E-mail: |
mda@mdausa.org |
| Web Address: |
http://als.mdausa.org |
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This Web site contains ALS information, up-to-date research
announcements, ALS-related publications, ALS-specific chat rooms, and other
materials. It is sponsored by the Muscular Dystrophy Association (MDA).
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References
Citations
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Younger DS, Hanif S (2003). Amyotrophic lateral
sclerosis. In RW Evans, ed., Saunders Manual of Neurologic Practice, pp. 550–554. Philadelphia: Saunders.
-
Ropper AH, Brown RH (2005). 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, 8th ed., pp. 938–949. New York:
McGraw-Hill.
-
Miller RG, et al. (1999). Practice parameter: The care
of the patient with amyotrophic lateral sclerosis (an evidence-based review).
Neurology, 52: 1311–1323.
-
Miller RG, et al. (2007). Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane Database of Systematic Reviews (1).
Credits
|
Author
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Monica Rhodes |
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Editor
|
Kathleen M. Ariss, MS |
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Associate Editor
|
Denele Ivins |
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Associate Editor
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Pat Truman, MATC |
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Primary Medical Reviewer
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E. Gregory Thompson, MD - Internal Medicine |
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Specialist Medical Reviewer
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Colin Chalk, MD, CM, FRCPC - Neurology |
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Last Updated
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August 14, 2008 |
