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. 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. 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. 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.  http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?CMD=search&term=motor+neuron+disease+AND+human[mh]+AND+english[la]+AND+(review[ptyp]+OR+review+literature[mh]+OR+practice+guideline[ptyp]+OR+guideline[ptyp]+OR+clinical+trials[mh]+OR+clinical+trial[ptyp]+OR+consumerj[sb])&db=PubMed&orig_db=PubMed&filters=on&pmfilter_EDatLimit=2+Years 2 http://clinicaltrials.gov/ct2/results?term= /disorders/motor_neuron_diseases/pubs_motor_neuron_diseases.htm /disorders/motor_neuron_diseases/xml_motor_neuron_diseases.xml xml_motor_neuron_diseases.xml V6 ALS Association

1275 K Street, N.W.

Suite 1050 Washington DC 20005 advocacy@alsa-national.org http://www.alsa.org 202-407-8580 202-289-6801 Nonprofit voluntary health organization dedicated to the fight against amyotrophic lateral sclerosis. Funds global research and sponsors advocacy programs, a network of chapters, and certified centers and clinics located nationwide. V160 Muscular Dystrophy Association

3300 East Sunrise Drive

Tucson AZ 85718-3208 mda@mdausa.org http://www.mda.org 520-529-2000 800-572-1717 520-529-5300 Voluntary health agency that fosters neuromuscular disease research and provides patient care funded almost entirely by individual private contributors. MDA addresses the muscular dystrophies, spinal muscular atrophy, ALS, Charcot-Marie-Tooth disease, myasthenia gravis, Friedreich's ataxia, metabolic diseases of muscle, and inflammatory diseases of muscle, for a total of more than 40 neuromuscular diseases. V733 ALS Therapy Development Institute

300 Technology Square

Suite 400 Cambridge MA 02139 info@als.net http://www.als.net 617-441-7200 617-441-7299 Founded in 1999, the ALS Therapy Development Foundation is dedicated to discovering and developing treatments for ALS. The organization, which focuses on a concentrated drug discovery program for ALS, has collaborated with 28 for-profit companies and academic researchers to perform advanced investigations. V8 Les Turner ALS Foundation

5550 W. Touhy Avenue

Suite 302 Skokie IL 60077-3254 info@lesturnerals.org http://www.lesturnerals.org 888-ALS-1107 847-679-3311 847-679-9109 Supports medical research, patient services, and promotes awareness and education to find the cause(s), effective treatments, and ultimately, a cure for amyotrophic lateral sclerosis. V613 Project ALS

3960 Broadway

Suite 420 New York NY 10032 info@projectals.org http://www.projectals.org 212-420-7382 800-603-0270 212-420-7387 Not-for-profit organization that raises awareness and significant funds toward effective treatments and a cure for ALS, also known as Lou Gehrig’s disease. V806 Spastic Paraplegia Foundation

4 Sherwood Hill Rd

Sherman CT 06784-2001 information@sp-foundation.org http://www.sp-foundation.org 1-877-SPF-GIVE (1-877-773-4483) 877-SPF-GIVE Volunteer driven, non-profit organization dedicated to finding the cures for Hereditary Spastic Paraparesis (HSP) and Primary Lateral Sclerosis (PLS). V212 Families of Spinal Muscular Atrophy

925 Busse Road

Elk Grove Village IL 60007 info@fsma.org http://www.curesma.org 800-886-1762 847-367-7623 International non-profit foundation dedicated to accelerating the development of a treatment or cure for SMA through the funding of research, drug discovery programs, and clinical trials. Assists families through informational programs and support services; sponsors an equipment pool; and educates the public and the medical community about SMA. V599 Fight SMA

1321 Duke Street

Suite 304 Alexandria VA 22134 web@fightsma.org http://www.fightsma.org 703-299-1144 National nonprofit organization committed to accelerating a cure for spinal muscular atrophy. V783 Kennedy's Disease Association

P.O. Box 1105

Coarsegold CA 93614-1105 info@kennedysdisease.org http://www.kennedysdisease.org 559-658-5950 Non-profit resource that offers support and information about spinal bulbar muscular atrophy or Kennedy's Disease. Works to increase awareness and to raise funds in support of research. Sponsors support groups. V823 Spinal Muscular Atrophy Foundation

888 Seventh Avenue

Suite 400 New York NY 10019 info@smafoundation.org http://www.smafoundation.org 877-FUND-SMA (877-386-3762) 646-253-7100 212-247-3079 Non-profit foundation dedicated to accelerating the development of a treatment or cure for SMA. V108 Post-Polio Health International

4207 Lindell Blvd.

#110 St. Louis MO 63108-2930 info@post-polio.org http://www.post-polio.org 314-534-0475 314-534-5070 Works to enhance the lives and independence of polio survivors and ventilator users through education, advocacy, research, and networking programs.