Primary Lateral Sclerosis
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What is Primary Lateral Sclerosis?Is there any treatment?What is the prognosis?What research is being done?OrganizationsWhat is Primary Lateral Sclerosis?
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.
Is there any treatment?
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.
What is the prognosis?
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.
What research is being done?
The NINDS conducts a broad range of research on neuromuscular disorders such as PLS. This research is aimed at developing
techniques to diagnose, treat, prevent, and ultimately cure these devastating diseases.
Select this link
to view a list of studies currently seeking patients.
Organizations
Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892
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on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined
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Last updated February 14, 2007
