$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.