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  Primary Lateral Sclerosis 
  
Reviewed  11-26-2001  

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What is Primary Lateral Sclerosis?
Is there any treatment?
What is the prognosis?
What research is being done?

Organizations

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 generally 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.

What research is being done?
The NINDS conducts a broad range of research on neuromuscular diseases aimed at finding better treatments and, ultimately, preventing and curing these disorders.

Select this link to view a list of studies currently seeking patients.

 Organizations

Spastic Paraplegia Foundation
P.O. Box 1208
Forston, GA 31808
info@sp-foundation.org
http://www.sp-foundation.org
Tel: 978-256-2673

Les Turner ALS Foundation
8142 North Lawndale Avenue
Skokie, IL 60076
info@lesturnerals.org
http://www.lesturnerals.org
Tel: 888-ALS-1107 847-679-3311
Fax: 847-679-9109

Primary Lateral Sclerosis Newsletter
101 Pinta Court
Los Gatos, CA 95032
73112.611@compuserve.com
Tel: 408-356-8227
Fax: same

Synapse: A PLS Newsletter
95 Campion Road
North Andover, MA 01845-1231
SynapsePLS@attbi.com
http://synapsePLS.home.attbi.com
Tel: 978-258-0712


NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.

All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.


Provided by:
The National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892




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