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What is Shy-Drager Syndrome?

Multiple system atrophy with autonomic failure, also called Shy-Drager syndrome, is a progressive disorder of the central and autonomic nervous systems. The disorder is characterized by postural hypotension--an excessive drop in blood pressure which causes dizziness or momentary blackouts upon standing or sitting up. There are 3 types of Shy-Drager syndrome: Parkinsonian-type which may include symptoms of Parkinson's disease such as slow movement, stiff muscles, and mild tremors; cerebellar-type which may include problems such as loss of balance and the tendency to fall; and combination-type which may include symptoms of both types 1 and 2. Parkinsonian symptoms and symptoms of autonomic dysfunction such as constipation and sexual impotence in males predominate early in the course of the disease. Constipation may be unrelenting and hard to manage in some patients. Shy-Drager may be difficult to diagnose or underdiagnosed in the early stages because it may take years for some key symptoms to reveal themselves. For the majority of patients, blood pressure is unstable--often fluctuating up and down--and causes severe headaches. Other symptoms may also develop, such as generalized weakness, double vision and/or other vision disturbances, impairment of speech, sensory changes, difficulties with breathing and swallowing, irregularities in heart beat, inability to sweat, and diarrhea.

Is there any treatment? Shy-Drager is often difficult to treat because of the fluctuations in blood pressure. The general treatment course is aimed at controlling symptoms. Anti-Parkinson medication, such as L-dopa, may be helpful but should be used with caution because it can lower blood pressure, causing blackouts. To relieve low blood pressure, dietary increases of salt and fluid may be beneficial. Medications to elevate blood pressure such as corticosteroids may cause side effects and should be carefully monitored by a physician. Alpha-adrenergic medications, metoclopramide, ergotamine derivatives, and indomethacin are useful in many cases. Sleeping in a head-up position at night may reduce headaches and morning dizziness. An artificial feeding tube or breathing tube may be surgically inserted for management of swallowing and breathing difficulties. In rare cases, a pacemaker may be implanted to correct heart irregularities.

What is the prognosis?

Shy-Drager usually ends in death 7 to 10 years after the onset of symptoms. Pneumonia is the most common cause of death, although irregularities in heart beat or choking may be responsible for death in some patients.

What research is being done?

The NINDS supports research on disorders of the autonomic nervous system including Shy-Drager syndrome. This research is aimed at discovering ways to prevent, diagnose, and treat disorders of the autonomic nervous system and, ultimately, to find cures for them.


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