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Dystonias

(Also Called 'Focal Dystonia', 'Generalized Dystonia', 'Hemidystonia', 'Multifocal Dystonia', 'Segmental Dystonia')
 
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What is Dysautonomia?

Dysautonomia refers to a disorder of autonomic nervous system (ANS) function. Most physicians view dysautonomia in terms of failure of the sympathetic or parasympathetic components of the ANS, but dysautonomia involving excessive ANS activities also can occur. Dysautonomia can be local, as in reflex sympathetic dystrophy, or generalized, as in pure autonomic failure. It can be acute and reversible, as in Guillain-Barre syndrome, or chronic and progressive. Several common conditions such as diabetes and alcoholism can include dysautonomia. Dysautonomia also can occur as a primary condition or in association with degenerative neurological diseases such as Parkinson's disease. Other diseases with generalized, primary dysautonomia include multiple system atrophy and familial dysautonomia. Hallmarks of generalized dysautonomia due to sympathetic failure are impotence (in men) and a fall in blood pressure during standing (orthostatic hypotension). Excessive sympathetic activity can present as hypertension or a rapid pulse rate.

Is there any treatment?

There is no cure for dysautonomia. Secondary forms may improve with treatment of the underlying disease. In many cases treatment of primary dysautonomia is symptomatic and supportive. Measures to combat orthostatic hypotension include elevation of the head of the bed, frequent small meals, a high-salt diet, and drugs such as fludrocortisone, midodrine, and ephedrine.

What is the prognosis?

The outlook for patients with dysautonomia depends on the particular diagnostic category. Patients with chronic, progressive, generalized dysautonomia in the setting of central nervous system degeneration have a generally poor long-term prognosis. Death can occur from pneumonia, acute respiratory failure, or sudden cardiopulmonary arrest in such patients.

What research is being done?

The NINDS supports and conducts research on dysautonomia. This research aims to discover ways to diagnose, treat, and, ultimately, prevent these disorders.

Organizations

National Dysautonomia Research Foundation
P.O. Box 301
Red Wing, MN 55066-0301
ndrf@ndrf.org
www.ndrf.org
Tel: 651.267.0525
Fax: 651.267.0524

National Organization for Rare Disorders (NORD)
P.O. Box 1968
(55 Kenosia Avenue)
Danbury, CT 06813-1968
orphan@rarediseases.org
www.rarediseases.org
Tel: 203.744.0100 Voice Mail 800.999.NORD (6673)
Fax: 203.798.2291

Dysautonomia Foundation
315 W. 39th Street, Suite 701
New York, NY 10018
info@familialdysautonomia.org
www.familialdysautonomia.org
Tel: 212.279.1066
Fax: 212.279.2066

Familial Dysautonomia Hope Foundation, Inc. (FD Hope)
121 South Estes Drive, Suite 205-D
Chapel Hill, NC 27514-2868
info@fdhope.org
www.fdhope.org
Tel: 919.969.6636

Shy-Drager/Multiple System Atrophy Support Group, Inc.
P.O. Box 279
Coupland, TX 78615
www.shy-drager.org
Tel: 866.DS.4999 (737.4999)
Fax: 512.251.3315

Dysautonomia Youth Network of America, Inc.
1301 Greengate Court
Waldorf, MD 20601
info@dynakids.org
www.dynakids.org
Tel: 301.705.6995
Fax: 301.638.DYNA

Source: National Institutes of Health; National Institute of Neurological Disorders and Stroke

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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 4/24/2009...#6006