NINDS Primary Lateral Sclerosis Information Page

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What is Primary Lateral Sclerosis?

Primary lateral sclerosis (PLS) is a rare neuromuscular disease with slowly progressive weakness in voluntary muscle movement. PLS belongs to a group of disorders known as motor neuron diseases. In motor neuron diseases, the nerve cells that control voluntary muscle movement degenerate and die. In PLS, the corticospinal motor neurons (often called "upper motor neurons") in the brain are affected. There is no evidence of degeneration of lower motor neurons in the spinal cord or brainstem and there is little muscle wasting (what doctors call "amyotrophy"). Symptoms include weakness, muscle stiffness and spasms (spasticity), clumsiness, slowing of movement, and problems with balance. Onset of PLS usually occurs after age 40. The symptoms often begin with problems in the legs, but may also start with hand clumsiness or changes in speech. PLS progresses gradually over a number of years, or even decades. Scientists do not believe PLS has a simple hereditary cause. There are similar, but rare, hereditary childhood disorders termed "juvenile PLS." The diagnosis of PLS requires extensive testing to exclude other diseases. When symptoms begin, PLS may be mistaken for amyotrophic lateral sclerosis or ALS (Lou Gehrig's disease) and the diagnosis of PLS can be delayed for several years.

Is there any treatment?

What is the prognosis?

What research is being done?

NIH Patient Recruitment for Primary Lateral Sclerosis Clinical Trials

• At NIH Clinical Center
• Throughout the U.S. and Worldwide

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.

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Last updated March 12, 2009