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NINDS Motor Neuron Diseases Information Page

Condensed from Motor Neuron Diseases Fact Sheet

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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.  MNDs occur more commonly in men than in women, and symptoms may 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 treat excessive saliva; and anticonvulsants and nonsteroidal anti-inflammatory drugs to relieve pain.  Panic attacks can be treated with benzodiazepines.  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.  Proper nutrition and a balanced diet are essential to maintaining weight and strength.

What is the prognosis?

Prognosis varies depending on the type of MND and the age of onset.  Some MNDs, such as primary lateral sclerosis and Kennedy disease, 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. 

NIH Patient Recruitment for Motor Neuron Diseases Clinical Trials

Organizations

Column1 Column2
ALS Association
1275 K Street, N.W.
Suite 1050
Washington, DC   20005
advocacy@alsa-national.org
http://www.alsa.org
Tel: 202-407-8580
Fax: 202-289-6801

Muscular Dystrophy Association
3300 East Sunrise Drive
Tucson, AZ   85718-3208
mda@mdausa.org
http://www.mda.org
Tel: 520-529-2000 800-572-1717
Fax: 520-529-5300

ALS Therapy Development Institute
300 Technology Square
Suite 400
Cambridge, MA   02139
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
3960 Broadway
Suite 420
New York, NY   10032
info@projectals.org
http://www.projectals.org
Tel: 212-420-7382 800-603-0270
Fax: 212-420-7387

Spastic Paraplegia Foundation
4 Sherwood Hill Rd
Sherman, CT   06784-2001
information@sp-foundation.org
http://www.sp-foundation.org
Tel: 1-877-SPF-GIVE (1-877-773-4483)
Fax: 877-SPF-GIVE

Families of Spinal Muscular Atrophy
925 Busse Road
Elk Grove Village, IL   60007
info@fsma.org
http://www.curesma.org
Tel: 800-886-1762
Fax: 847-367-7623

Fight SMA
1321 Duke Street
Suite 304
Alexandria, VA   22134
web@fightsma.org
http://www.fightsma.org
Tel: 703-299-1144

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

 
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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 April 16, 2014