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.

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.

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.

Research

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. 

Organizations

ALS Association
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.

1275 K Street, N.W.
Suite 250
Washington, DC 20005
advocacy@alsa-national.org
http://www.alsa.org
Tel: Washington
Fax: 202-289-6801

Muscular Dystrophy Association
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.

National Office - 222 S. Riverside Plaza
Suite 1500
Chicago, IL 60606
mda@mdausa.org
http://www.mda.org
Tel: Chicago
Fax: 520-529-5300

ALS Therapy Development Institute
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.

300 Technology Square
Suite 400
Cambridge, MA 02139
info@als.net
http://www.als.net
Tel: Cambridge
Fax: 617-441-7299

Les Turner ALS Foundation
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.

5550 W. Touhy Avenue
Suite 302
Skokie, IL 60077-3254
info@lesturnerals.org
http://www.lesturnerals.org
Tel: Skokie
Fax: 847-679-9109

Content Provided By

NINDS Disorders is an index of neurological conditions provided by the National Institute of Neurological Disorders and Stroke. This valuable tool offers detailed descriptions, facts on treatment and prognosis, and patient organization contact information for over 500 identified neurological disorders.

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