Shy-Drager Syndrome
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What is Multiple System Atrophy with Orthostatic Hypotension?Is there any treatment?What is the prognosis?What research is being done?OrganizationsWhat is Multiple System Atrophy with Orthostatic Hypotension?
Multiple system atrophy with orthostatic hypotension is the current classification for a neurological disorder that was once
called Shy-Drager syndrome. A progressive disorder of the central and autonomic nervous systems, it is characterized by orthostatic
hypotension (an excessive drop in blood pressure when standing up) which causes dizziness or fainting. Multiple system atrophy
can occur without orthostatic hypotension, but it is rare. Doctors classify the disorder into 3 types: the Parkinsonian-type includes symptoms of Parkinson's disease such as slow movement, stiff muscles, and tremor; the cerebellar-type, which causes problems with coordination and speech; and the combined-type, which includes symptoms of both parkinsonism and cerebellar failure. Problems with urinary incontinence, constipation, and
sexual impotence in men happen early in the course of the disease. Other symptoms include generalized weakness, double vision
or other vision disturbances, difficulty breathing and swallowing, sleep disturbances, and decreased sweating. Because the
disease resembles others, a correct diagnosis may take years.
Is there any treatment?
There is no cure for multiple system atrophy with orthostatic hypotension. Treatment is aimed at controlling symptoms. Anti-Parkinson
medication such as Sinemet may improve the general sense of well-being. Medications to elevate blood pressure while standing
are often used, but may cause high blood pressure when lying down. Individuals should sleep with the head of the bed elevated.
An artificial feeding tube or breathing tube may be required for problems with swallowing and breathing.
What is the prognosis?
Most individuals with multiple system atrophy with orthostatic hypotension die within 7 to 10 years after the onset of symptoms.
A problem with the respiratory system is the most common cause of death.
What research is being done?
The NINDS supports research on disorders of the autonomic nervous system, including multiple system atrophy with orthostatic
hypotension. This research is aimed at developing techniques to diagnose, treat, and prevent these disorders.
Select this link
to view a list of studies currently seeking patients.
Organizations
Shy-Drager/Multiple System Atrophy Support Group, Inc.
P.O. Box 279
Coupland,
TX
78615
http://www.shy-drager.org
Tel: 866-SDS-4999 (737-4999)
Fax: 512-251-3315
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WE MOVE (Worldwide Education & Awareness for Movement Disorders)
204 West 84th Street
New York,
NY
10024
wemove@wemove.org
http://www.wemove.org
Tel: 212-875-8312 866-546-3136
Fax: 212-875-8389
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National Dysautonomia Research Foundation
P.O. Box 301
Suite 160
Red Wing,
MN
55066
ndrf@ndrf.org
http://www.ndrf.org
Tel: 651-267-0525
Fax: 651-267-0524
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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.
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Last updated February 13, 2007
