Shy-Drager Syndrome

Shy-Drager syndrome, like Parkinson's disease, causes tremors when muscles are at rest (resting tremor). It also causes internal body processes, such as blood pressure control, to malfunction.

Shy-Drager syndrome usually develops between the ages of 37 and 75. It is more common among men. The cause is unknown. This syndrome progresses, eventually resulting in death.

Shy-Drager syndrome results from deterioration of the parts of the brain that control internal body functions. These parts include nerve cells of the brain stem and spinal cord. They control such functions as heart rate, blood pressure, breathing rate, the amount of stomach acid secreted, and the speed at which food passes through the digestive tract.

Symptoms

Like Parkinson's disease, Shy-Drager syndrome causes tremors, rigid muscles, and problems with movements. Walking and speaking become slow. Like people with Parkinson's disease, people with Shy-Drager syndrome may shuffle, have difficulty taking a first step, freeze in the middle of a movement, and have to take many small, quick steps to prevent a fall. Speech may become slurred.

In addition, Shy-Drager syndrome interferes with the regulation of blood pressure, heart rate, bladder and bowel function, body temperature, and focusing of the eyes. When people with this disorder stand up, blood pressure falls dramatically. They then feel dizzy, light-headed, or faint. This disorder is called orthostatic hypotension. Blood pressure may increase when they lie down.

Less sweat, tears, and saliva are produced. So people do not tolerate heat well, and their eyes and mouth are dry. People may have difficulty urinating or become constipated, sometimes severely. Uncontrollable loss of urine (urinary incontinence) or loss of control of bowels with leakage and soiling (fecal incontinence) may develop. In men, erectile dysfunction (impotence) may develop. Walking may become unsteady, and movements may become uncoordinated.

Late in the disorder, people may become confused and have difficulty controlling their emotions. Mental function may decline slightly. Breathing and swallowing become difficult, and the heart beats irregularly.

Diagnosis and Treatment

Doctors suspect Shy-Drager syndrome on the basis of symptoms. No tests can directly confirm the diagnosis. However, a tilt table test may be done to determine whether the body's control of blood pressure is abnormal. For the test, a person is strapped to a motorized table, which tilts the person who is lying flat to an almost standing position. Blood pressure and heart rate are continuously monitored during the test.

No treatment can cure the disorder, but some symptoms can be relieved. Usually, the drugs used to relieve the symptoms of Parkinson's disease are less effective in treating Shy-Drager syndrome. However, different people respond differently.

Several measures can help relieve the symptoms resulting from a fall in blood pressure when a person stands up.

• Consuming more salt and drinking a lot of water (which may increase the volume of blood and thus help increase blood pressure)
• Drinking caffeinated beverages early in the day (which may increase blood pressure slightly)
• Wearing fitted elastic stockings up to the waist
• Raising the head of the bed (which can help prevent blood pressure from increasing too much when a person lies down)
• Avoiding extreme heat
• Abstaining from alcoholic beverages
• Eating small meals
• Getting up slowly
• Not straining during a bowel movement

The drug fludrocortisone can help prevent blood pressure from becoming too low. This drug increases blood pressure because it causes the body to retain salt and water. Midodrine may help prevent blood pressure from decreasing too much when a person stands. Taking propranolol at night can help prevent blood pressure from increasing too much when a person lies down.

Outlook

Shy-Drager syndrome results in death, usually due to pneumonia, 7 to 10 years after symptoms begin. People with Shy-Drager syndrome should prepare advance directives, indicating what kind of medical care they want at the end of life.