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$Unique_ID{BRK04218}
$Pretitle{}
$Title{Shy-Drager Syndrome}
$Subject{Shy-Drager Syndrome Progressive Autonomic Failure PAF Orthostatic
Hypotension Orthostatic Hypotension in Neurological Disease Postural
Hypotension Parkinson's Disease }
$Volume{}
$Log{}
Copyright (C) 1986, 1987, 1988, 1990, 1991 , 1992 National Organization
for Rare Disorders, Inc.
242:
Shy-Drager Syndrome
** IMPORTANT **
It is possible the main title of the article (Shy-Drager Syndrome) is not
the name you expected. Please check the SYNONYMS listing on the next page to
find alternate names, disorder subdivisions, and related disorders covered by
this article.
Synonyms
Progressive Autonomic Failure
PAF
Orthostatic Hypotension
Orthostatic Hypotension in Neurological Disease
Postural Hypotension
Information on the following disease can be found in the Related
Disorders section of this report:
Parkinson's Disease
General Discussion
** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or
treatment purposes. If you wish to obtain more information about this
disorder, please contact your personal physician and/or the agencies listed
in the "Resources" section of this report.
Shy-Drager Syndrome is due to an impairment of the autonomic nervous
system. This disorder is primarily characterized by low blood pressure
associated with dizziness or momentary blackouts upon standing. Initial
symptoms usually include bladder problems, which can become more severe.
Sexual impotence may occur in males. Other symptoms resembling those of
Parkinson's disease may develop with time. The course of Shy-Drager Syndrome
is variable, and some patients may have mild symptoms for up to twenty years.
Symptoms
The initial symptoms of Shy-Drager Syndrome may be bladder problems and/or
sexual impotence in males. The urinary difficulties may become progressively
more severe. Dizziness or momentary blackouts often occur as a consequence
of low blood pressure that occurs when the patient stands up (orthostatic
hypotension). Patients may also experience constipation, and urinary or
rectal incontinence. Additionally, dry skin due to loss of the ability to
sweat, vision disturbances, and loss of pigment in the iris of the eyes can
occur.
Symptoms not related to the autonomic nervous system abnormalities may
include slowness of movement, unsteady gait, slurred speech, mild tremors,
and loss of balance or other symptoms resembling those of Parkinson's
disease. (For more information on Parkinson's Disease, choose "Parkinson" as
your search term in the Rare Disease Database).
In later stages, patients may develop breathing problems such as loud
respirations (stridor), or episodes of momentary breathing stoppages during
sleep (apnea). Heart beat irregularities may also occur. Chewing,
swallowing, speaking and breathing may become increasingly difficult as this
disorder progresses.
Causes
The exact cause of Shy-Drager Syndrome is not known, although scientists
believe it may have either environmental or genetic predisposition causes.
Symptoms are caused by an impairment of the autonomic nervous system. The
autonomic nervous system is involved with autonomous functions essential for
our survival such as heart rate, breathing, sweating, intestinal, urinary and
sexual functions. It also controls skin and body temperature and how our
body responds to stress.
Environmental causes are often related to occupational exposure to such
toxins as: metal dusts and fumes, plastic monomers and additives, organic
solvents, and pesticides.
Affected Population
Shy-Drager Syndrome affects males twice as often as females. However, the
incidence of the syndrome in the general population is not known. According
to one study, Shy-Drager Syndrome was incorrectly diagnosed as Parkinson's
disease in approximately 1.8 percent of cases of Parkinson's disease
patients. Age of onset of this disorder ranges from thirty-seven to seventy-
five years, with an average onset at fifty-five years of age.
Related Disorders
Symptoms of the following disorder can be similar to those of Shy-Drager
Syndrome. Comparisons may be useful for a differential diagnosis:
Parkinson's disease is a slowly progressive neurological disorder
characterized by tremor, muscular rigidity, slowness of movement, balance
problems and difficulty in initiating movements. In Parkinson's disease,
there are degenerative changes in certain areas of the brain, and a decrease
in dopamine levels in neurons associated with these areas. Parkinsonian
symptoms, however, may very rarely be secondary to strokes or tumors in the
brain. Exposure to certain drugs and toxins may also be associated with
symptoms of Parkinson's disease.
Parkinson's disease may affect the autonomic nervous system although
symptoms are usually milder than those among Shy-Drager Syndrome patients.
In some cases, the distinction between these two disorders is hard to
determine. The nervous system dysfunction is usually confined to one system
in Parkinson's, whereas many systems may be affected by Shy-Drager Syndrome.
(For more information on this disorder, choose "Parkinson" as your search
term in the Rare Disease Database).
Therapies: Standard
Treatment of Shy-Drager Syndrome is aimed at controlling symptoms.
Antiparkinson medication must be used with caution because it can lower blood
pressure, thus causing blackouts. To relieve the low blood pressure, dietary
increases of salt and fluid may be beneficial. Elastic stockings may be worn
on the legs. Drugs to elevate blood pressure such as corticosteroid
derivatives must be carefully monitored by a physician to avoid side-effects.
Surgical insertion of an artificial feeding tube (gastrostomy), or an
artificial breathing tube (tracheostomy) may be necessary for treatment of
breathing and swallowing difficulties. On rare occasions, a pacemaker may be
implanted to correct heart irregularities.
Therapies: Investigational
Researchers are investigating the orphan drug Midodrine as a treatment for
Shy-Drager Syndrome. Those interested in participating in clinical research
trials can ask their doctor to contact:
Roberts Pharmaceuticals
Meridian Center III
6 Industrial Way West
Eatontown, NJ 07724
(201) 389-1182
The National Institute of Neurological Disorders and Stroke (NINDS) is
seeking certain individuals affected by Shy-Drager Syndrome for participation
in a clinical research project. For complete information, those interested
should have their physicians contact:
Ms. Linda Nee, M.S.W. or Dr. Ronald Polinsky
NINCDS Medical Neurology Branch
Bldg. 10, Rm. 5N236
Bethesda, MD 20892
(301) 496-8850
Clinical trials are underway to study taxonomy and therapy od Orthostatic
Hypotension. Interested persons may wish to contact:
Dr. Italo Biaggioni
AA 3228 MCN
Vanderbilt University GCRC
Nashville, TN 37232
(615) 343-6499
to see if further patients are needed for this research.
This disease entry is based upon medical information available through
January 1992. Since NORD's resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.
Resources
For more information on Shy-Drager Syndrome, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
Shy-Drager Syndrome Support Group
1607 Silver Ave., S.E.
Albuquerque, NM 87106
(505) 243-5118
David Robertson, MD
Autonomic Dysfunction Center
Vanderbilt University
Nashville, TN 37232-2195
(615) 343-6499
International Tremor Foundation
360 West Superior Street
Chicago, IL 60610
(312) 664-2344
NIH/National Institute of Neurological Disorders & Stroke (NINDS)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5751
(800) 352-9424
References
CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.: W. B. Saunders Co., 1988. Pp. 1466, 2106-7, 2153.
THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme
Research Laboratories, 1987. Pp. 407, 1427.