Primary lateral sclerosis (PLS) is a progressive degenerative
motor neuron disease, meaning that nerve cells in the body gradually die off.
PLS affects only some of the nerve cells in the body--those that control
voluntary movement of muscles. It is not known why it occurs, or exactly how the
nerve cells are destroyed. Primary lateral sclerosis seems to begin between 35
and 65 years of age. It is not known how many people in the world are affected
by primary lateral sclerosis, although it does seem to be rare.
Symptoms
The loss of the nerve cells causes muscles
to become stiff and difficult to move. Typically, the muscle problems start in
the legs, and move up the body to the trunk, arms, and muscles in the face and
neck. The disease progresses slowly, usually over a few years.
Diagnosis
There is no specific test for primary lateral sclerosis, so most of the time the
diagnosis occurs as the result of eliminating other possible causes of the
symptoms. Because amyotrophic lateral sclerosis (ALS, or Lou Gehrig's disease)
is more common, primary lateral sclerosis is often confused with it. However,
primary lateral sclerosis does not produce muscle wasting throughout the body as
ALS does, and therefore does not affect the respiratory muscles.
Treatment
There is no specific treatment or cure for primary lateral
sclerosis, so treatment focusing on relieving its symptoms. Medications can
reduce pain and muscle spasms. Physical therapy can also help reduce spasms and
provide devices to help with walking, talking, and eating. Support groups such
as the Spastic Paraplegia Foundation can help individuals and their families
adapt to the physical changes the disease brings. Primary lateral sclerosis
brings progressive disability and loss of function but does not affect the mind
or shorten the life span directly.
MGM
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