Amyotrophic Lateral Sclerosis (ALS)
[
en Español]
What is amyotrophic lateral sclerosis (ALS)?
Amyotrophic lateral sclerosis
(ALS), sometimes called Lou Gehrig's Disease, is a
progressive brain disease that attacks the nerve cells (neurons)
that control voluntary muscles. This disease belongs to a group
of motor neuron disorders (e.g., muscular dystrophy, multiple
sclerosis, Parkinson's disease) that lead to the gradual
degeneration and death of motor neurons, the nerve cells located
in the brain, brainstem, and spinal cord. These motor neurons
serve as connections from the nervous system to the muscles in
the body.
Imagine wanting to brush your teeth. The motor neurons in the
brain (
upper motor neurons
) have to send a message to the motor neurons in the spinal cord
(
lower motor neurons
). The lower motor neurons then send the message to the muscles
that are needed to brush your teeth (arms, mouth, etc.). ALS
causes both the upper and lower motor neurons to degenerate or
die. Both the brain and spinal cord lose the ability to initiate
and send messages to the muscles in the body. The muscles, which
are unable to function, gradually
atrophy
(waste away) and
fasciculate
(twitch).
ALS does not impair a person's mind. It affects only the
motor neurons. Personality, intelligence, memory, and
self-awareness remain the same. The senses of sight, smell,
touch, hearing, and taste remain intact as well.
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What are some signs or symptoms of ALS?
ALS first affects an individual's ability to speak loudly
and clearly. Eventually, it completely prevents speaking and
vocalizing. Some of the earliest speech-related symptoms of ALS
may include:
- nasal speech quality (talking out of your nose)
- difficulty pronouncing words due to weak, tight, or stiff
speech muscles (
dysarthria)
- difficulty with lengthy sentences or conversation
As muscles for breathing weaken, it becomes more difficult for
patients to speak loud enough to be understood. Eventually
extensive muscle atrophy eliminates one's ability to vocalize
and speak altogether.
Patients may also experience difficulty chewing and
swallowing. Initially only some food consistencies are affected,
but over time it becomes difficult for the patient to swallow
even pureed foods and saliva. People with ALS get tired easily
and they may not have the energy to eat an entire meal. All of
these factors make it difficult to maintain adequate nutrition
and weight. Eventually doctors may decide that it is best to
insert a feeding tube into the person's stomach to make sure
they receive enough nutrition.
In some cases, initial symptoms affect only one leg or arm.
Patients may have awkwardness and stumbling when walking or
running. They may have difficulty lifting objects or with tasks
that require manual dexterity (e.g., buttoning a shirt, tying a
shoe, turning a key). Eventually the individual will not be able
to stand or walk, get in and out of bed without help, or use
hands and arms to perform activities of daily living, such as
washing and dressing.
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How is ALS diagnosed?
There is no specific laboratory test for ALS, making it hard
to diagnose. The diagnosis is made using clinical findings in
conjunction with results of electrodiagnostic studies and the
absence of evidence of other disorders. According to the
diagnostic guidelines of the World Federation of Neurology, there
must be lower motor neuron degeneration detected by clinical
electrophysiological or neuropathologic examination, signs of
upper motor neuron degeneration by clinical examination, and
progressive spread of signs within a region of the body or to
other regions.
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What treatments are available for people with ALS?
Presently, there is no cure for ALS. However, as more and more
research is done on brain diseases, doctors are learning more
about what causes ALS and how to best treat it. This has led to
the development of medications to treat ALS. Riluzole, an
antiglutamate agent, is the first FDA-approved drug for the
treatment of patients with ALS. Although Riluzole is not a cure,
results of clinical trials indicate some promise in prolonging
life, especially with
bulbar
patients. The current trend is to evaluate potential additive or
synergistic effects of drug combinations (how well two or more
drugs may work better than one alone).
As with any incurable disease, the state of the art in
treatment for ALS is symptom management (also referred to as
palliative care
). According to the World Health Organization, palliative care is
active care of the total patient. Individuals with ALS receive
the best treatment from multidisciplinary clinical teams that
specialize in neuromuscular disorders. Intervention deals with
symptoms that occur over the course of the disease process. The
ALS Association and the Muscular Dystrophy Association sponsor
clinics in many U.S.cities (information can be obtained from
their Web sites-see list below).
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What other organizations have information about ALS?
This list is not exhaustive, and inclusion does not imply
endorsement of the organization or the content of the Web site by
ASHA.
The following Web sites can provide information and support
for people with ALS and their families:
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See Also: