NINDS Primary Lateral Sclerosis Information Page

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What 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.

NIH Patient Recruitment for Primary Lateral Sclerosis Clinical Trials

Organizations

Spastic Paraplegia Foundation
Spastic Paraplegia Foundation PO Box 1208 Fortson, GA 31808 Phone: 877-773-4483 Email: information@sp-foundation.org www.sp-foundation.org
Suite 123
Falls Church, VA   22043
information@sp-foundation.org
http://www.sp-foundation.org
Tel: 1-877-SPF-GIVE (1-877-773-4483)
Fax: 877-SPF-GIVE

ALS Association
27001 Agoura Road
Suite 150
Calabasas Hills, CA   91301-5104
advocacy@alsa-national.org
http://www.alsa.org
Tel: 818-880-9007 800-782-4747
Fax: 818-880-9006

Synapse: A PLS Newsletter
212 Farm Road
Sherborn, MA   01770
synapsePLS@comcast.net
http://synapsepls.org
Tel: 508-653-5246

 


Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892



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.

Last updated February 14, 2007