Primary Lateral Sclerosis Information Page
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What is Primary Lateral Sclerosis?
Is there any treatment?
What is the prognosis?
What is Primary Lateral Sclerosis?
Primary lateral sclerosis is a rare neuromuscular disease characterized by progressive muscle weakness in the voluntary muscles. Onset of the disorder 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 which produces slowness and stiffness of movement, dragging of the feet (leading to inability to walk), and facial involvement resulting in dysarthria (poorly articulated speech). The disorder usually begins in the legs, however, it may also begin in the tongue or the hands. PLS belongs to a group of disorders known as motor neuron diseases. It occurs when specific nerve cells in the cerebral cortex that control voluntary movement gradually degenerate, causing the muscles under their control to weaken. The disease-which scientists believe is not hereditary-progresses gradually over a number of years, or even decades, leading to stiffness and weakening of the affected muscles. In PLS, there is no evidence of the degeneration of spinal motor neurons or muscle wasting (amyotrophy) that occurs in a similar disorder called amyotrophic lateral sclerosis or ALS (Lou Gehrig's disease). For the first few years (3-5) after symptoms begin there is a possibility that the true diagnosis is ALS, which can have some of the same symptoms as PLS. For this reason, most neurologists follow the affected individual's clinical course for a few years before making a diagnosis of PLS.
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 may help prevent joint immobility, and speech therapy may be useful for patients with dysarthria.
What is the prognosis?
There is no cure for PLS. The disorder is not fatal.
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