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Amyotrophic lateral sclerosis




from a US site in the US, the address is :(looking for it)
What is ALS?

Amyotrophic lateral sclerosis (ALS) is a progressive disease of
the nervous system. The cause is not known and there is no
cure, although progress is being made on both fronts. ALS is
also known as Lou Gehrig's disease after the famous baseball
player who died from it.
 
ALS attacks motor neurons, which are among the largest of all
nerve cells in the brain and spinal cord. These cells send
messages to muscles throughout the body. In ALS, motor neurons
die and the muscles do not receive these messages. As a result,
muscles weaken as they lose their ability to move. Eventually,
most muscle action is affected, including those which control
swallowing and breathing, as well as major muscles in the arms,
legs, back and neck. There is, however, no loss of sensory
nerves, so people with ALS retain their sense of feeling,
sight, hearing, smell and taste. The mind is not affected by
this disease and people with ALS remain fully alert and aware
of events. The course of ALS is extremely variable and it is
difficult to predict the rate of progression in any single
patient. For the majority of people with ALS, weakness tends to
progress over a three-to-five year period.
ALS can strike anyone, at any age, but generally ALS occurs
between the ages of 40 and 70. According to the National
Institutes of Health, some 4,600 people in the United States
are newly diagnosed with ALS each year. About 4 to 6 people per
100,000 worldwide get ALS. In a small percentage of patients,
ALS is genetic.

What are the Symptoms?
The first signs of ALS are often arm and leg weakness, muscle
wasting and faint muscle rippling. These symptoms occur because
muscles are no longer receiving the nutrient signals they need
for growth and maintenance -- a result of motor neurons dying.
ALS nerve degeneration may also cause muscle cramps and vague
pains, or problems with speech and swallowing. Some people with
the disease may lose some control over their emotional
responses. They may laugh or cry much more easily than in the
past. Eventually, all voluntary muscle action is affected.
 

How is ALS Diagnosed?

There is no specific test for diagnosing ALS. However, several
tests -- including nerve conduction studies and electromyogram
(EMG) -- are used to measure how well and quickly the nerves
are working. Ruling out other causes of muscular weakness is
important because ALS often mimics other treatable diseases.
Diagnosis requires special skills and neurologic tests. People
with ALS symptoms usually are referred to neurologists, who
specialize in the nervous system. Diagnosis may take several
months since an important part of the diagnostic process is to
confirm disease progression.
 

What causes ALS?
The cause of ALS is unknown. It attacks its victims at random.
However, it was recently discovered that five to ten percent of
those with ALS show a definite genetic pattern. In this rare
form, about one-half of the offspring may develop ALS. These
people show a gene defect that affects an enzyme called
superoxide dismutase. This enzyme eliminates toxic substances
called free radicals. Free radicals can cause nerve cells to
die and are associated with a number of diseases and even
implicated in aging itself. For most people with ALS, the vast
majority of their children are not at any greater risk to
develop this disease than the general population. This type of
ALS is often called "sporadic ALS" due to its unpredictable
nature.
ALS researchers have found no difference between the symptoms
and disease progression in the sporadic and genetic forms of
ALS. Therefore, since the genetic and acquired forms of ALS
appear to be similar, an understanding of the cause of the
genetic form could lead to treatment for all forms of the
disease.
 

Treatment

While there is no cure for ALS, research to solve the ALS
puzzle is ongoing. Scientific advances have led to approval of
the first treatment for the disease -- a medication that may
increase survival time. Other treatments under investigation
include several nerve growth factors which may help maintain
quality of life by maintaining nerve function. While each of
these therapies represent a step forward for people with ALS, a
cure remains to be discovered.
For the majority of people with ALS, the primary treatment
remains the management of ALS symptoms. Patients need to take
an active role in the design of their treatment regimen.
Ideally, ALS management involves physical, occupational,
speech, respiratory and nutrition therapy. For instance,
certain drugs and the application of heat or whirlpool therapy
may help to relieve muscle cramping. Exercise can help maintain
muscle strength and function. Exercise, however, is recommended
in moderation. Drugs also may be used to help combat fatigue,
but in some patients may worsen muscle cramps.
As the disease progresses, various assistive devices will help
a person with ALS maintain their independence and ensure
personal safety. For example, an ankle/foot brace can improve
function and conserve energy, as well as help avoid injury.
When neck, trunk and shoulder weakness makes walking or sitting
difficult, cervical collars, perhaps with an additional chest
and head strap, provide helpful support. A reclining chair is
preferable to a headrest to relieve fatigue of neck muscles.
There are also numerous devices to assist in feeding, dressing
and maintaining personal hygiene. Eventually, more substantial
equipment, such as wheelchairs, scooters, lifts and hospital
beds may be required.
It is important to know that speech therapists can help with
speech and swallowing difficulties as they develop. Also, drug
treatments can help patients who develop excessive saliva and
drooling. Family members of people with ALS should be
instructed in the Heimlich maneuver to provide assistance in a
life-threatening choking episode. Feeding tubes may be
necessary to maintain nutrition, as may breathing devices when
the disease affects the muscles of the chest. However, with
these supportive devices, there are physical, emotional and
financial implications, and their use should be discussed with
a physician well in advance of when the need arises. Managing
the symptoms is a process that is challenging for people with
ALS, their caregivers, and their medical team.
Of all the disabilities that affect a person with ALS, one of
the most devastating and most common is the progressive loss of
the ability to communicate. However, advances in computer
technology mean that persons with ALS today have vital new
electronic communications options that can be adapted to their
individual capabilities.
 

Progress through Research

Significant progress is being made in the study of ALS.
Although there is still no cure, recent clinical trials have
shown that some drugs affect nerve cell activity and may
increase the survival time for people with ALS. Newly developed
animal models of the genetic form of the disease, so-called
transgenic ALS mice, offer neurologic researchers the ability
to test therapies in mice. There is great hope that this and
other neuroscientific advances will lead to a cure in humans.
Talk with your doctor about being involved in future clinical
trials or about the drugs currently available for the treatment of this disease.
8/01/05
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