Primary lateral sclerosis (PLS) is a rare neuromuscular disease characterized by progressive muscle weakness in the voluntary muscles. PLS belongs to a group of disorders known as motor neuron diseases. Motor neuron diseases develop when the nerve cells that control voluntary muscle movement degenerate and die, causing weakness in the muscles they control. Onset of PLS usually occurs after age 50. Symptoms may include difficulty with balance, weakness and stiffness in the legs, and clumsiness. Other symptoms may include spasticity (sudden, involuntary muscle spasms) in the hands, feet, or legs; foot dragging, and speech problems due to involvement of the facial muscles. The disorder usually begins in the legs, but it may also start in the tongue or the hands. The disease-which scientists believe is not hereditary-progresses gradually over a number of years, or even decades. In PLS, there is no evidence of the degeneration of spinal motor neurons or muscle wasting (amyotrophy) that occurs in amyotrophic lateral sclerosis or ALS (Lou Gehrig's disease), which it resembles. Diagnosis of PLS is often delayed because it is mistaken for ALS.
Treatment
for individuals with PLS is symptomatic. Baclofen and tizanidine may
reduce spasticity. Quinine or phenytoin may decrease cramps. Physical
therapy often helps prevent joint immobility. Speech therapy may be
useful for those with involvement of the facial muscles.
PLS
is not fatal. There is no cure, and the progression of symptoms
varies. Some people may retain the ability to walk without
assistance, but others eventually require wheelchairs, canes, or other
assistive devices.
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My work place was close to where I lived.
When I first noticed something was
wrong when I begin to fall. Not really fall but I would fold up, my
knees would give way and down I would tumble. I never got hurt bad but,
I sure did not understand what was happening to me. My voice started to
change and I was not able to project my voice and I noticed it was
becoming more difficult to compete with the noise of a passing car, tv,
radio, etc..