NINDS Motor Neuron Diseases Information Page

Skip secondary menu

Motor Neuron Diseases

Information Page

Publications
Organizations
Clinical Trials
News
Research literature

Disclaimer

NINDS is part of the
National Institutes of
Health

Download Adobe Reader

Print-friendly version

Email this page

Condensed from Motor Neuron Diseases Fact Sheet

Table of Contents (click to jump to sections)
What are Motor Neuron Diseases?
Is there any treatment?
What is the prognosis?
What research is being done?

Organizations
Related NINDS Publications and Information
Publicaciones en Español

What are Motor Neuron Diseases?

The motor neuron diseases (MNDs) are a group of progressive neurological disorders that destroy cells that control essential muscle activity such as speaking, walking, breathing, and swallowing. Normally, messages from nerve cells in the brain (called upper motor neurons) are transmitted to nerve cells in the brain stem and spinal cord (called lower motor neurons) and from them to particular muscles. When there are disruptions in these signals, the result can be gradual muscle weakening, wasting away, and uncontrollable twitching (called fasciculations). Eventually, the ability to control voluntary movement can be lost. MNDs may be inherited or acquired, and they occur in all age groups. In adults, symptoms often appear after age 40. In children, particularly in inherited or familial forms of the disease, symptoms can be present at birth or appear before the child learns to walk.

The causes of sporadic (noninherited) MNDs are not known, but environmental, toxic, viral, or genetic factors may be implicated. Common MNDs include amyotrophic lateral sclerosis (ALS), progressive bulbar palsy, primary lateral sclerosis, and progressive muscular atrophy. Other MNDs include the many inherited forms of spinal muscular atrophy and post-polio syndrome, a condition that can strike polio survivors decades after their recovery from poliomyelitis.

Is there any treatment?

There is no cure or standard treatment for the MNDs. Symptomatic and supportive treatment can help patients be more comfortable while maintaining their quality of life. The drug riluzole (Rilutek®), which as of this date is the only drug approved by the U.S. Food and Drug Administration to treat ALS, prolongs life by 2-3 months but does not relieve symptoms. Other medicines that may help reduce symptoms include muscle relaxants such as baclofen, tizanidine, and the benzodiazepines for spasticity; glycopyrrolate and atropine to reduce the flow of saliva; quinine or phenytoin for cramps; anticonvulsants and nonsteroidal anti-inflammatory drugs to relieve pain; tranquilizers to help with sleeping problems; antidepressants; and botulinum toxin, amitriptyline, and other anticholinergic drugs to control drooling. Some patients may require stronger medicines such as morphine to cope with musculoskeletal abnormalities or pain in later stages of the disorders, and opiates are used to provide comfort care in terminal stages of the disease.

Physical and speech therapy, occupational therapy, and rehabilitation may help to improve posture, prevent joint immobility, slow muscle weakness and atrophy, and cope with swallowing difficulties. Applying heat may relieve muscle pain. Assistive devices such as supports or braces, orthotics, speech synthesizers, and wheelchairs help some patients retain independence.

What is the prognosis?

Prognosis varies depending on the type of MND and the age of onset. Some MNDs, such as primary lateral sclerosis, are not fatal and progress slowly. Patients with spinal muscular atrophy may appear to be stable for long periods, but improvement should not be expected. Some MNDs, such as ALS and some forms of spinal muscular atrophy, are fatal.

What research is being done?

The National Institute of Neurological Disorders and Stroke (NINDS) conducts research related to the MNDs in its laboratories at the National Institutes of Health (NIH), and also supports additional research through grants to major medical institutions across the country. Much of this research focuses on finding better ways to prevent, treat, and ultimately cure disorders such as the MNDs.

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

Organizations

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	Muscular Dystrophy Association 3300 East Sunrise Drive Tucson, AZ 85718-3208 mda@mdausa.org http://www.mda.org Tel: 520-529-2000 800-344-4863 Fax: 520-529-5300
ALS Therapy Development Institute 215 First Street Cambridge, MA 02142 info@als.net http://www.als.net Tel: 617-441-7200 Fax: 617-441-7299	Les Turner ALS Foundation 5550 W. Touhy Avenue Suite 302 Skokie, IL 60077-3254 info@lesturnerals.org http://www.lesturnerals.org Tel: 888-ALS-1107 847-679-3311 Fax: 847-679-9109
Project ALS 900 Broadway Suite 901 New York, NY 10003 info@projectals.org http://www.projectals.org Tel: 212-420-7382 800-603-0270 Fax: 212-420-7387	Spastic Paraplegia Foundation 7700 Leesburg Pike 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: 703-495-9261
Synapse: A PLS Newsletter 212 Farm Road Sherborn, MA 01770 synapsePLS@comcast.net http://www.synapsepls.org Tel: 508-653-5246	Families of Spinal Muscular Atrophy P.O. Box 196 Libertyville, IL 60048-0196 info@fsma.org http://www.curesma.org Tel: 847-367-7620 800-886-1762 Fax: 847-367-7623
FightSMA/Andrew's Buddies 1807 Libbie Avenue Suite 104 Richmond, VA 23226 heatherlennon@fightsma.com http://www.fightsma.org Tel: 804-515-0080 Fax: 804-515-0081	Kennedy's Disease Association P.O. Box 1105 Coarsegold, CA 93614-1105 info@kennedysdisease.org http://www.kennedysdisease.org Tel: 559-658-5950
Spinal Muscular Atrophy Foundation 888 Seventh Avenue Suite 400 New York, NY 10019 info@smafoundation.org http://www.smafoundation.org Tel: 877-FUND-SMA (877-386-3762) 646-253-7100 Fax: 212-247-3079	Post-Polio Health International/ 4207 Lindell Blvd. #110 St. Louis, MO 63108-2930 info@post-polio.org http://www.post-polio.org Tel: 314-534-0475 Fax: 314-534-5070

Related NINDS Publications and Information

NINDS Amyotrophic Lateral Sclerosis Information Page
Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's disease) information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
Amyotrophic Lateral Sclerosis Fact Sheet
Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's disease) fact sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
NINDS Spinal Muscular Atrophy Information Page
Spinal muscular atrophy (SMA) information page compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
NINDS Post-Polio Syndrome Information Page
Post-Polio syndrome information compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
Post-Polio Syndrome Fact Sheet
Post-Polio Syndrome fact sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
NINDS Primary Lateral Sclerosis Information Page
Primary lateral sclerosis (PLS) information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
NINDS Monomelic Amyotrophy Information Page
Monomelic amyotrophy (Benign Focal Amyotrophy) information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
Testimony on Amyotrophic Lateral Sclerosis, May 2000
NINDS Director's testimony before the Senate Appropriations Subcommittee on Labor, Health and Human Services, Education and Related Agencies on Amyotrophic Lateral Sclerosis (ALS), May 18, 2000.

Publicaciones en Español

Enfermedades de la Neurona Motora

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.

Return to top

Last updated September 09, 2008