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Studies with patients

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Is there any treatment?
Orthostatic hypotension in Shy-Drager syndrome is treatable, but there is not known effective treatment for the progression central nervous system degeneration. The general treatment course is aimed at controlling symptoms. Antiparkinsonian medication, such as L-dopa, may be helpful. To relieve low blood pressure while standing, dietary increases of salt and fluid may be beneficial. Medications to elevate blood pressure, such as salt-retaining steroids, are often necessary, but they can cause side effects and should be carefully monitored by a physician. Alpha-adrenergic medications, non-steroidal anti-inflammatory drugs, and sympathomimetic amines are sometimes used. Sleeping in a head-up position at night reduces morning orthostatic hypotension. An artificial feeding tube or breathing tube may be surgically inserted for management of swallowing and breathing difficulties.

What is the prognosis?
Shy-Drager syndrome usually ends in the patient’s death by 7 to 10 years after diagnosis. Breathing problems such as aspiration, stridor (high-pitched breathing sounds due to airway obstruction), or cardiopulmonary arrest are common causes of death.

What research is being done?
The NINDS carries out and funds research about disorders of the autonomic nervous system, including Shy-Drager syndrome. This research is aimed at discovering ways to diagnose and treat disorders of the autonomic nervous system and ultimately to cure or prevent them.