Amyotrophic Lateral Sclerosis (ALS)
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Amyotrophic lateral sclerosis (ALS), often referred to as "Lou
Gehrig's Disease," is a progressive neurodegenerative disease that
affects nerve cells in the brain and the spinal cord. Motor neurons reach
from the brain to the spinal cord and from the spinal cord to the muscles
throughout the body. The progressive degeneration of the motor neurons
in ALS eventually leads to their death. When the motor neurons die, the
ability of the brain to initiate and control muscle movement is lost.
With voluntary muscle action progressively affected, patients in the
later stages of the disease may become totally paralyzed.
comes from the Greek language. "A" means no or
negative. "Myo" refers to muscle, and "Trophic" means
nourishment–"No muscle nourishment." When a muscle has no nourishment,
it "atrophies" or wastes away. "Lateral" identifies
the areas in a person's spinal cord where portions of the nerve cells
that signal and control the muscles are located. As this area degenerates
it leads to scarring or hardening ("sclerosis") in the region.
motor neurons degenerate, they can no longer send impulses to the muscle
fibers that normally result in muscle movement. Early symptoms of ALS
often include increasing muscle weakness, especially involving the arms
and legs, speech, swallowing or breathing. When muscles no longer receive
the messages from the motor neurons that they require to function, the
muscles begin to atrophy (become smaller). Limbs begin to look "thinner" as
muscle tissue atrophies.
The diagnosis of Amyotrophic Lateral Sclerosis
or Lou Gerhig's Disease is a devastating moment. The disease is universally
fatal and at present there is no known cure. However many patients receive
the diagnosis from physicians who also feel that they have nothing to
offer patients. At Phoenix Neurological Associates we recognize a few
important facts about ALS. First of all, it is vital to be sure of the
diagnosis and to be sure that all other possible diseases have been excluded.
This requires physicians with years of experience in neuromusculaar diseases.
Secondly, once the diagnosis is reached we believe that this is the beginning
of our care for the patient, not the end. We use a multidisciplinary
approach to help patients with ALS remain as independent and active for
as long as possible. We are proud to be one of only 33 certified ALS
clinics in the country.
This condition is often abbreviated as ALS, but is also known
as Lou Gehrig's disease, named after the famous baseball player who died
of the disease. ALS is a motor neuron disease that destroys the nerve
cells responsible for carrying signals from the brain to the muscles.
When these cells die, one loses the ability to control the muscles that
enable us to move, speak, breathe, and swallow, and the muscles gradually
weaken and waste. The majority of the symptoms of ALS are directly related
to muscle weakness. This weakness can begin in any part of the body and
progresses very slowly. There are often muscle twitches, called fasciculations,
and muscle cramps. Numbness, tingling, and pain are not common. The disease
does not usually affect the nerves in the brain, so that one’s thinking
is generally not affected.
The diagnosis of ALS is one of exclusion because at the onset
ALS may mimic other disorders like strokes, carpal tunnel syndrome, or
other neurological conditions. There is no specific, definitive test
to diagnose ALS. Thus a physician will typically perform many different
tests to rule out other diseases, including magnetic resonance imaging
(MRI) of the brain and spinal cord, electrodiagnostic studies, including
needle electromyography (EMG) and nerve conduction studies (NCS), along
with a variety of blood and urine tests. A diagnosis of ALS is reached
after evaluating the results of all the tests, along with the patient's
medical history, and physician's physical examination.
The EMG is the
most important test in this work-up because it gives the physician accurate
information about what is happening within the nerves. The needle EMG
results can also be used to rule out others diagnoses. Once the diagnosis
of ALS is made, it is appropriate to get a confirmatory second opinion
given the gravity of this disease.
There is no known cause for ALS. Researchers have looked into
theories including environmental exposure and a variety of infections
and have not yet discovered the source for the disease. In 10% of patients
with ALS there is a family history of the disease. Approximately 20%
of these familial cases of ALS have a specific gene, called Superoxide
Dismutase 1 which is abnormal. This gene can be tested for through
a blood test. Otherwise there is no other specific genetic relationship
for the majority of the cases of ALS.
There is one FDA approved drug to treat ALS, Rilutek. This
drug was shown to increase survival of patients by approximately three
months. Other than Rilutek there are no known drugs or supplements that
have been proven to increase survival.
The majority of the treatment for
patients with ALS is focused on managing a patient’s symptoms. We believe
this is best accomplished through a multidisciplinary clinic (Link to
clinic) which brings together a variety of specialized therapists, such
as physical therapy, occupational therapy, speech therapy, and respiratory
therapy. We have established this type of a clinic at Good Samaritan
Hospital in Phoenix, Arizona. Here we can manage symptoms that arise
by providing specific equipment to compensate for the weakness that develops.
Trials have shown that patients followed in these multidisciplinary clinics
live 6-12 months longer.
As there is no known cause for the disease we do not have a cure
yet. Once the disease begins the average life expectancy is 3-5 years.
Patients pass away from an inability to swallow effectively or breathe
effectively. At present there are numerous research trials looking to
explore avenues to find cures. Much of this research focuses on a concept
known as excitotoxicity. This theory implies that certain levels of chemicals
that excite muscles and nerves are necessary to be able to move. However
too much of these chemicals causes nerve death. Unfortunately there
is still a great deal of work that needs to be done before we will have
a cure for this disease.
ALS Clinical Trials
by our physicians: